Lipoprotein(a) (Lp(a)) contributes to the formation to atherosclerosis, promotes inflammation and stimulates prothrombotic processes.

One hundred seventeen adult congenital heart disease (ACHD) patients and 152 controls were studied to compare serum Lp(a) concentrations in different subgroups of congenital heart abnormalities. Analytically, Lp(a), total cholesterol, low-density lipoprotein (LDL) cholesterol, high-density lipoprotein cholesterol, triglycerides, and C-reactive protein (CRP) were studied. In congenital heart disease patients, N-terminal-pro-B-type natriuretic peptide levels were also determined.

Thirty-nine (25.6%) patients in the control group and 33 (28.2) ACHD patients (2 hypoxemic and 31 nonhypoxemic) had a Lp(a) concentration higher than 30 mg/dL. No significant differences were seen between patients with Lp(a) concentration ≤30 or >30 mg/dL after performing a binary logistic regression multivariate analysis including as covariates all the variables that showed significance (P < .001) between the case and control groups (age, gender, CRP, and total, LDL, and high-density lipoprotein cholesterol) besides being congenital or not. Similarly, no significant differences were found between ACHD patients with Lp(a) concentration lower and higher than 30 mg/dL after performing a multivariate analysis in which age, sex, body mass index, LDL-cholesterol levels, and being or not hypoxemic were included as covariates. Pearson's correlation showed a significant positive correlation (0.27) between LDL-cholesterol and Lp(a) concentrations (P = .004) and between CRP and N-terminal-pro-B-type natriuretic peptide levels (0.19) in ACHD patients (P = .035).

Adult congenital heart disease patients showed lower serum total cholesterol and LDL-cholesterol levels than no-congenital patients although no significant differences were seen in Lp(a) concentrations between both groups.

Structural heart interventions require the use of relatively large-diameter delivery sheaths or latex nylon net balloon catheters, which results in a relatively large-diameter venous puncture point. At present, femoral venous hemostasis is achieved by manual compression. A temporary figure-of-eight subcutaneous suture has been introduced to achieve immediate postprocedural femoral venous hemostasis after using a ≥24Fr sheath in an adult. This method is not well evaluated in children.

We report our experience using this technique in children treated with 7–14Fr sheaths. From May 2009 to March 2012, 104 children (ages ranging from 3 to 10 years, mean 8.1 ± 1.6 years) with atrial septal defects (n = 86) and pulmonary valve stenosis (n = 18) underwent percutaneous transcatheter interventions. Sheath sizes used were ≥7Fr (7Fr, n = 5; 8Fr, n = 29; 9Fr, n = 14; 10Fr, n = 15; 12Fr, n = 19; 14Fr, n = 22).

A total of 102 patients had immediate femoral vein hemostasis, and 2 developed a femoral vein hematoma requiring manual compression. One patient was diagnosed with a femoral artery pseudoaneurysm during hospitalization. On follow-up, there was no evidence of hematoma or thrombosis.

The “figure-of-eight” suture technique is effective and safe, achieving immediate hemostasis after the use of large femoral vein sheaths in children.

Left-sided cardiac lesions have a birth prevalence of approximately 1 in 1000 and have been shown to be heritable in pedigree studies. A large microdeletion at 16p12.1 is associated with childhood developmental delay, and initial studies describing this deletion identified left-sided lesions as an enriched phenotype compared with a control population.

The aim of this study is to determine whether patients with left-sided cardiac lesions have an increased frequency of 16p12.1 microdeletions as compared with control populations.

A cohort of 262 probands with left-sided lesions, including 53 with isolated aortic stenosis/bicuspid aortic valve, 83 with coarctation of the aorta with or without aortic stenosis/bicuspid aortic valve, and 126 with hypoplastic left heart syndrome were assessed for copy number variation at 16p12.1. The control cohort included 595 patients with conotruncal defects as a cardiac control and 971 healthy children.

We detected one patient in the left-sided lesion cohort with a large duplication partially overlapping the reported 16p12.1 microdeletion, along with one patient each in the conotruncal and control cohorts with a deletion in the same region. None of these patients had dysmorphic features, extracardiac malformations, or developmental delay.

In our cohort, structural variation at 16p12.1 was not identified with increased frequency in patients with left-sided lesions as compared with controls.

To describe the incidence, characteristics, and outcomes of hospitalized children and young adults with aortic dissection (AD).

Retrospective review.

The Pediatric Health Information System database, a multiinstitutional administrative database.

All index cases of patients <30 years old hospitalized with AD between January 2004 and June 2011.

The primary outcome measure was inpatient mortality.

Of 3 800 964 hospitalizations, AD was identified in 124 (<1%), accounting for 110 patients (69% male, P = .003) at a median age of 12.9 (interquartile range 3.9–16.8) years with a bimodal distribution in infancy and late adolescence. Associated diagnoses included congenital heart disease (CHD) (38%), trauma (24%), connective tissue disease (CTD) (16%), and isolated hypertension (HTN) (8%). Common CHD diagnoses included aortic arch (24%) and valve (21%) disease, hypoplastic left heart syndrome (10%), and transposition of the great arteries (10%). CHD patients were younger and more likely to undergo inpatient non–AD-related cardiovascular procedures compared with other diagnostic groups (P < .001 for both). Marfan and Ehlers–Danlos syndrome were present in 72% and 11% of CTD patients, respectively. Overall in-hospital mortality in patients with AD was 13% compared with 1% in the database population (odds ratio 12.0, 95% confidence interval 6.9–21.1). By diagnostic category, mortality was 22% in HTN, 22% in CTD, 12% in CHD, and 4% in trauma.

AD is rare in children and young adults but most commonly occurs in CHD and CTD and in males. AD is associated with high inpatient mortality.

The study aims to describe substance use, dental hygiene, and physical activity in adult survivors with single ventricle physiology (SVP) and to compare the behaviors with matched controls, while the patients are particularly at risk for general health problems.

The present study is part of a larger research project on long-term outcomes in adult patients with SVP. A cross-sectional, case-control study including 59 patients out of 83 eligible patients participated in the study (response rate 71%). The patients were matched to 172 healthy controls. A questionnaire was mailed to the patients. Nonresponders did not differ significantly from the included participants on age, gender, or physical function.

The study was conducted at the Heart Center of Copenhagen University Hospital or the Department of Cardiology, Aarhus University Hospital.

In these patients, 85% report alcohol consumption (92% in controls; odds ratio [OR] = 0.91; P = 0.575); 26% admit “binge drinking” (41% in controls; OR = 0.56; P = 0.041); 20% are cigarette smokers (36% in controls; OR = 0.59; P = 0.100); 12% have used cannabis over the past year (15% in controls; OR = 0.80; P = 0.596); 20% have had no dental visits during the last year (25% in controls; OR = 1.07; P = 0.684); 46% are not flossing their teeth (32% in controls; OR = 1.32; P = 0.239); and 39% are not physically active (24% in controls; OR = 1.63; P = 0.069).

While in general there was no significant differences in overall health behaviors between SVP patients and controls, SVP patients are less physically active and are less likely to binge drink.

The purpose of this study is to report a single institution's experience with abdominal coarctation in children and report associated comorbidities.

Abdominal coarctation is a rare condition, accounting for less than 2% of aortic coarctations. Single patients with abdominal coarctation have been reported with additional vascular disease in pediatric patients.

Our echocardiography database between January 2001 and January 2012 was searched to identify all patients with abdominal coarctation. Relevant clinical data were reviewed.

Nine patients were identified with abdominal coarctation. Median age at diagnosis was 4.7 years (IQR 1.1–14.3 years). Additional cardiac diagnoses were found in three patients: one had moderate aortic regurgitation and aortic root dilatation; one had mild aortic regurgitation, severe mitral regurgitation, and atrial flutter; and one had a thoracic coarctation previously repaired. Eight patients (89%) had an associated noncardiac comorbidity. Comorbidities included: Takayasu arteritis (n = 3), systemic lupus erythematosus (n = 1), epidermal nevus syndrome (n = 1), abdominal hemagioma (n = 1), Williams syndrome (n = 1), and renal artery stenosis (n = 2). Intervention was performed in four patients (57%): two underwent surgical grafting and two had angioplasty with stent placement. Patients with surgical grafting required no further intervention, whereas both patients who underwent angioplasty and stenting required further stent placement.

Abdominal coarctation is a rare anomaly. It is frequently associated with other vascular abnormalities. Vasculitis should be suspected in children with abdominal coarctation. All patients, even if treated, require continued close observation.

To examine the knowledge and attitudes of anesthesia providers in relation to the care of adult congenital heart disease (ACHD) patients presenting for noncardiac surgery.

A novel survey was designed and administered to 168 anesthesiologists across a single academic department in a range of practice environments.

None.

Survey responses, including true/false, multiple choice, and Likert scale questions.

A total of 118 anesthesiologists (response rate = 70%) completed the survey. Knowledge scores ranged from 0 to 19 (median [interquartile range] = 7 [5–13]) out of a possible maximum of 20. Total knowledge scores differed significantly by fellowship background (P = .004), with higher scores in those with cardiac (11 [7–15], P = .005) and pediatric (12 [6–15], P = .001) fellowship training, but not in those with critical care, obstetric, regional, or pain management training. Scores also differed by frequency of providing care for cardiopulmonary bypass cases and frequency of providing care for patients under 2 years of age (P < .001 for both), but not by gender or years removed from residency. Respondents reported only moderate levels of comfort with a range of questions about providing perioperative or obstetric care to ACHD patients, with decreasing levels of comfort reported in patients with more complex lesions.

Within the context of the limitations of a single-institution survey design, the low levels of knowledge and comfort we observed suggest that providers may benefit from improved training and protocols for ensuring adequate preparedness for the care of ACHD patients.

Congenital heart disease (CHD) has an incidence of ∼0.8–1%. Outcome of previously diagnosed CHD patients awaiting surgery (either correction or palliation) in a developing country setting is unknown. We strive to determine the outcome of patients with CHD awaiting surgery who present to pediatric intensive care unit (PICU) setting with an acute illness.

Retrospective cross-sectional chart review.

Pediatric intensive care unit of The Aga Khan University Hospital, Karachi, Pakistan.

Medical records of infants (1–12 months) with CHD awaiting surgery presenting to the PICU with an acute illness between January 2009 and June 2012 were included. Newly diagnosed CHD patients, those not requiring PICU admission, and those transferred to another hospital were excluded.

A total of 34 infants met the inclusion criteria. Median age at presentation was 5 months. Seventy-four percent of the infants had CHD lesion characterized by increased pulmonary blood flow (shunt lesions). Though none of the patients met the strict criteria for sepsis or pneumonia, 74% were admitted with a diagnosis of pneumonia or sepsis. Only 15% of patient had congestive heart failure as an admitting diagnosis. Oxygen therapy was given to 94% of these patients. Fifty-nine percent of these patients expired during the admission, 95% of those expired had multiorgan dysfunction.

Patients with CHD awaiting surgery and who admitted to the PICU with acute illness are at high risk for mortality. Stringent criteria to diagnose pneumonia or sepsis should be used in these patients.

To investigate impact of prophylactic peritoneal dialysis (PD) on clinical outcomes and inflammatory cytokines in children following cardiac surgery with cardiopulmonary bypass.

Prospective before-and-after nonrandomized cohort study.

Pediatric cardiovascular intensive care unit in tertiary hospital.

Fifty-two consecutive neonates and infants at high risk for postoperative fluid overload following cardiopulmonary bypass. All had PD catheters placed during primary cardiac surgery.

Initial 27 patients were managed with passive peritoneal drainage and diuretics (controls). Following 25 patients were started on prophylactic PD in immediate postoperative period and managed per PD protocol (+PD).

Cumulative fluid balance, indices of disease severity, and clinical outcomes were prospectively collected. Plasma interleukin-6 and interleukin-8 were measured immediately before-and-after cardiopulmonary bypass and at 24 and 48 hours post–cardiopulmonary bypass.

Demographics, diagnoses, and intraoperative variables were similar. Median net fluid balance was more negative in +PD at 24 hours, −24 mL/kg (interquartile range: −62, 11) vs. +18 mL/kg (interquartile range: −26, 11), P = .003, and 48 hours, −88 mL/kg (interquartile range: −132, −54) vs. −46 mL/kg (interquartile range: −84, −12), P = .004. +PD had median 55 mL/kg less fluid intake at 24 hours, P = .058. Peritoneal drain, urine, and chest tube output were comparable over first 24 hours. Mean inotrope score was lower in +PD at 24 hours. +PD had earlier sternal closure—24 hours (interquartile range: 20, 40) vs. 63 hours (interquartile range: 44, 72), P < .001—and a trend toward shorter duration of mechanical ventilation—71 hours (interquartile range: 49, 135) vs. 125 hours (interquartile range: 70, 195), P = .10. +PD experienced lower serum concentrations of interleukin-6 and interleukin-8 at 24 hours.

Prophylactic PD is associated with greater net negative fluid balance, decreased inotrope requirements, and lower serum concentrations of inflammatory cytokines in the early postoperative period.

To determine whether caregivers of children with single ventricle heart defects identified as having feeding disorder will report more frequent feeding dysfunction, or maladaptive mealtime behavior and/or interactions, when compared with reference populations.

As part of routine evaluation, parents of children evaluated at the Feeding, Swallowing, and Nutrition Center at the Children's Hospital of Wisconsin completed previously validated questionnaires to assess feeding dysfunction and parental stress. Parental responses for single ventricle patients were compared with all other children evaluated with a feeding disorder.

Questionnaires were completed in eight patients with single ventricle heart defects. The mean age was 36 ± 23 months, with five females (63%). Mean weight-for-age z-score was −1.4 ± 0.9. Compared with noncardiac feeding clinic children, there was more reported child resistance to eating (83 ± 15% vs. 44 ± 2%; P = .05). Single ventricle parents were more likely to report distress (50 ± 18% vs. 21 ± 2%; P = .04) and a difficult child (63 ± 17% vs. 31 ± 2%; P = .05). There was also more defensive responding among parents of single ventricle children (63 ± 17% vs. 29 ± 2%; P = .04).

Single ventricle patients evaluated for disordered feeding more frequently showed resistance to eating and parental distress than noncardiac feeding clinic patients. Parents of these children underestimated the degree of feeding difficulty by defensive responding and had more parental distress. These findings suggest that feeding dysfunction can contribute to longer-term feeding and growth problems in single ventricle patients with feeding disorder.

To examine the willingness of grown-ups with congenital heart disease (GUCH) to participate in the GUCH Training Program-Individualised (GTI), an exercise program specifically designed for GUCH, and to identify factors affecting their willingness to participate.

In this cross-sectional study, all outpatient GUCH of the University Medical Center Groningen in The Netherlands, living within a 30-km radius of Groningen (n = 311), were asked to participate.

In total, 116 (37%) of the 311 GUCH who are invited to participate in our study returned completed questionnaires. The median age of the respondents was 40 (interquartile range 31–50) years and 55% were women.

Respondents (n = 116) completed a questionnaire that queried physical activity, perceived physical fitness, psychosocial determinants (motivation, self-efficacy, and social support) related to physical activity, and willingness to participate in GTI.

Of the 116 respondents, 68 (59%) were willing to participate in GTI. They were less physically active, had worse perceived physical fitness, were less satisfied with their fitness, were generally more motivated to engage in physical activity, and had more social support than patients unwilling to participate. The best logistic regression model predicting willingness to participate in GTI included the variables perceived physical fitness and motivation for physical activity in general.

Asking GUCH to participate in an exercise program supervised by physical therapists is a good strategy. Taken into account nonresponse, a participation rate in the exercise program of over 20% is to be expected. Perceived physical fitness and motivation for physical activity in general are important predictors of patients' willingness to participate.

Determine incidence and risk factors for possible paradoxical embolic events in patients who have Ebstein anomaly with severe tricuspid regurgitation.

Retrospective study of clinical and imaging data.

Tertiary care center.

Patients undergoing clinical evaluation and echocardiography prior to cardiac surgery for Ebstein anomaly (1975–2010) performed at age ≥ 40 years.

Mean age of 128 patients (81 female) was 53 ± 9 years. All had severe tricuspid regurgitation. Twenty-four (19%) had previous cardiac surgery (at <40 years), including 17 for interatrial shunt closure. Most (112 [88%]) had New York Heart Association functional class III/IV heart failure; 84 (66%) had interatrial shunting (58 had an atrial septal defect and 29 had a patent foramen ovale [3 had both]). During their lifetime, 29 patients (23%) had a history of ≥1 possible paradoxical embolic events (stroke or transient ischemic attack, brain abscess, or myocardial infarction). The best predictors of preoperative possible paradoxical embolic events were an atrial septal defect (P = .002) and older age at surgery (P = .007). There was no association of possible paradoxical embolic events with cardiovascular risk factors (hypertension, dyslipidemia, smoking, or family history of coronary artery disease) (all P ≥ .3) or atrial fibrillation (P = .69). Median age at occurrence of paradoxical embolism was 49 (range, 1.5–74 years).

Possible paradoxical embolic events are common in adults with Ebstein anomaly and severe tricuspid regurgitation and are strongly associated with atrial septal defect. In patients with atrial septal defect or patent foramen ovale, shunt closure should be considered to reduce risk of possible paradoxical embolic events.

To assess the effect of nutritional status and cardiovascular risk on hospital outcomes after congenital heart surgery in infants and children.

Retrospective study.

Cardiac intensive care unit in a tertiary-care children's hospital.

One hundred twenty-one patients <24 months of age admitted to the cardiovascular intensive care unit (CVICU) for >48 hours following cardiac surgery.

Demographics, Risk Adjustment for Congenital Heart Surgery-1 (RACHS-1), Paediatric Index of Mortality 2, and Pediatric Risk of Mortality III scores were obtained on admission. CVICU nutritional intake was calculated for 7 days. Energy and protein needs were estimated using recommended guidelines. Risk Adjustment for Congenital Heart Surgery-1 was categorized as (1–3) or (4–6). Malnutrition was categorized by Waterlow criteria and correlated with mortality risk, days of mechanical ventilation, and hospital and CVICU length of stay.

Ninety-one patients who underwent cardiac surgery were categorized as RACHS-1 (1–3) and RACHS-1 scores of (4–6) (n = 30). Patients with RACHS-1 (4–6) had higher mortality risk by Pediatric Risk of Mortality III (4.9% vs. 2.6%, P < .01), longer CVICU (10.4 days vs. 4.8 days) and hospital stays (28 days vs.14 days), and more days of mechanical ventilation (4 days vs. 2 days) (all P < .005) than RACHS-1 (1–3). The prevalences of acute protein-energy malnutrition and chronic protein-energy malnutrition were 51.2% and 40.5%. The median hospital stay for mild, moderate, and severe chronic protein-energy malnutrition was 31, 10, and 22.5 days, respectively, vs. normal, 15 days (Kruskal–Wallis, P < .005). The average energy and protein requirements met on day 7 were 68 ± 27(SD)% and 68 ± 40%, respectively.

Although nearly half of the patients were malnourished at surgery, only two-thirds of their recommended caloric and protein requirements were provided by week 1. To improve hospital outcomes, care should be taken to optimize the nutritional condition of infants and children prior to and following surgical correction of congenital heart disease to improve hospital outcomes.

We report a single institute experience of transcatheter pulmonary valvotomy using the soft end of a guidewire followed or not by a systemic-pulmonary shunt in patients with pulmonary atresia with intact ventricular septum (PAIVS) or critical pulmonary stenosis (CPS). In addition, we compare patients with or without an additional source of flow to support the pulmonary circulation after successful pulmonary valvotomy.

All neonates with PAIVS or CPS who underwent primary transcatheter pulmonary valvotomy between January 2004 and December 2010 were reviewed retrospectively. Some of them needed an additional source of flow to support the pulmonary circulation. We performed a comparison between those who required an additional source of pulmonary flow and those who did not.

The initial procedure was successful in 20 out of 22 patients (seven of nine with PAIVS; all of 13 with CPS), but 10 of them needed an additional source of flow to support the pulmonary circulation: nine had arterial duct stenting and one had surgical Blalock-Taussig shunt. There were no deaths or major acute complications, except for femoral artery occlusion in three patients. The bipartite right ventricular morphology, the tricuspid z-score of ≤−0.74, the tricuspid to mitral valve ratio of ≤0.9, and the z-score of the diastolic interventricular septal thickness ≥2.37 in preprocedural examination showed more tendency of needing shunt placement.

Transcatheter pulmonary valvotomy using the soft end of a guidewire followed or not by the arterial duct stent implantation was an effective approach in those patients. The angiographic distinction between CPS and PAIVS did not affect anything in this study including the procedural method, success, and odds for reintervention. The degree of right ventricle cavity hypoplasia provided the main restriction to forward flow after pulmonary valvotomy.

We evaluated immediate and midterm results of transcatheter closure of atrial septal defects (ASDs) and patent foramen ovale (PFO) using various closure devices.

The study included four hundred fourteen patients (one hundred eighty-two men, two hundred thirty-two women; mean age 39 ± 12.3 years; range 17–67 years) who underwent transcatheter closure of secundum ASD (n = 193) or PFO (n = 221). All the patients were evaluated by transthoracic echocardiography and transesophageal echocardiography before the procedure. Transcatheter closure was performed by using Amplatzer (n = 184), Occlutech Figulla (n = 209), or BioSTAR (n = 21) devices. Closure of ASDs was performed under general anesthesia with transesophageal echocardiography guidance, and closure of PFOs was performed under local anesthesia with transthoracic echocardiography guidance. Follow-up controls were at 1, 6, and 12 months and annually thereafter. The median follow-up periods of ASD and PFO patients were 43 and 30 months.

The mean device size was 19.3 ± 6.2 mm for ASD patients and 24.6 ± 2.6 mm for PFO patients. The mean procedural and fluoroscopy times were 22.3 ± 4.7 and 4.1 ± 1.9 minutes for ASD closure and 12.4 ± 3.2 and 3.1 ± 1.2 minutes for PFO closure, respectively. Procedural device embolization occurred in only two patients (0.48%). During follow-up, recurrent embolic events occurred in four patients (1.8%) after PFO closure, and no residual shunts were seen after ASD closure. Device thrombosis developed in two ASD patients during the procedure and in one PFO patient at 12th month of the follow-up (0.72%).

Transcatheter closure of PFOs and secundum-type ASDs using the Amplatzer, Occlutech Figulla, and BioSTAR devices is an efficacious and safe therapeutic option.

Cardiomyopathy (CM) is an inevitable consequence of Duchenne muscular dystrophy, and electrocardiographic changes, right ventricular hypertrophy in particular, have been proposed to serve as an early marker for CM. To evaluate this concept, we assessed the correlation between R wave height in lead V₁ and echocardiographic findings in boys with Duchenne muscular dystrophy.

Serial echocardiograms and electrocardiograms (n = 800) were performed during each clinic visit in a cohort of 155 boys with Duchenne muscular dystrophy. Precordial R wave height in lead V₁ was measured. Echocardiographic parameters included ejection fraction (EF), shortening fraction, and left ventricular end-diastolic dimension. Data were analyzed using Pearson correlation and linear regression.

Ages ranged from 1.8 to 37.2 years (mean 14.7 ± 5.9 years). Seventy-one patients had CM and 318/800 echocardiograms had an EF < 55%. Older patients tended to have a lower EF, but there was no correlation between age and left ventricular end-diastolic dimension. R wave height in lead V₁ correlated poorly with both left ventricular end-diastolic dimension (r = 0.096, P =.0078) and EF (r = 0.096, P =.0088) for the whole cohort as well as those studies demonstrating an EF <55% (left ventricular end-diastolic dimension r = 0.089, P =.12 and EF r = −0.044, P =.94). No individual patient demonstrated significant correlation between R wave height in lead V₁ and left ventricular end-diastolic dimension or EF. Left ventricular end-diastolic dimension showed a moderate negative correlation with EF for the whole cohort (r = −0.394, P <.001) as well as those with an EF < 55% (r = −0.376, P <.001).

The precordial R wave height in V₁ correlates poorly with the presence of depressed left ventricular function and is not prognostic for the development of CM. While not predictive for CM, the electrocardiogram remains vital to cardiac screening for boys with Duchenne muscular dystrophy due to risk for other cardiac manifestations such as arrhythmias.

A subset of children with repaired congenital heart disease (CHD) may require tracheostomy for ongoing ventilatory support. Data on outcomes of children with CHD and tracheostomy are scarce. Our objectives were to describe indications for tracheostomy and outcomes, including readmission data in this population.

This is a retrospective chart review of children (<18 years old) with CHD who underwent tracheostomy at a single center over a 12-year period. Exclusion criteria were prematurity with isolated patent ductus arteriosus ligation. Outcomes until discharge and data on all readmissions after the initial discharge were reviewed.

A total of 21 subjects with CHD underwent tracheostomy at a median (range) age of 4 (1–84) months and mean (standard deviation) weight of 7.2 (5.9) kg. The most common indication for tracheostomy was tracheomalacia with ventilator-dependent respiratory failure (14/21 subjects), followed by subglottic stenosis (5) and vocal cord palsy (2). Genetic syndromes were present in 13 (62%) subjects. The mean (standard deviation) post-tracheostomy length of stay was 55 (35) days. All subjects survived to discharge; 17 (81%) required home ventilation. A total of 11 (52%) subjects died during follow-up, all of whom were mechanically ventilated while three (14%) children underwent successful decannulation. The mean number of nonelective readmissions decreased from 2.4/patient-year in the first year to 1.4/patient-year in the second year, respectively. The commonest reasons for readmission were respiratory deterioration, infections, and mechanical tracheostomy-related problems.

The majority of children with CHD who underwent tracheostomy did so for ventilator dependence and tracheomalacia and had coexisting genetic syndromes. About half the cohort died; among survivors, readmissions were common but decreased after the first year. These results underscore the ongoing mortality and morbidity risks faced by this vulnerable population.

Robust risk-adjustment algorithms are often necessary if data from clinical registries is to be used to compare rates of important clinical outcomes between participating centers. Although such algorithms have been successfully developed for surgical and catheter-based cardiac interventions in children, outcomes of pediatric and congenital catheter ablation have not been modeled with respect to case mix.

A working group was appointed by the Pediatric and Congenital Electrophysiology Society to develop a risk-adjustment algorithm for use in conjunction with a modernized, multicenter registry database. Expert consensus was used to develop relevant outcome measures, an inclusive list of possible predictors, and estimates of associated incremental risk. Historical data from the Pediatric Radiofrequency Ablation Registry was reanalyzed using multivariate regression to create statistical models of ablation outcomes.

Acute ablation failure and serious adverse event rates were modeled as outcomes. Statistical modeling was performed on 4486 cases performed in 19 centers. For ablation failure rate, a simple model including general category of arrhythmia mechanism and presence of structural congenital heart disease accounted for ∼71% of outcome variance. The model was useful for identification of between-center variability in the historical data set. Although expert consensus predicted the need for a more complex model, predicted univariate effects were similar to those generated by statistical modeling. Serious adverse events were too infrequent to permit statistical association with any predictive variable, but could be compared with the mean rate observed among all centers.

A substantial component of the intercenter variability of acute ablation outcomes in a historical database of pediatric and congenital ablation patients may be accounted for by a simple statistical model, exposing variations in outcome specific to centers. This will be a useful initial model for use a modern registry for pediatric catheter ablation outcomes.

Percutaneous pulmonary valve implantation is frequently used as a less invasive method in patients with conduit dysfunction. The common valve type cannot be used in conduits with a diameter larger than 22 mm. There has been limited experience concerning the used of the SAPIEN Transcatheter Heart Valve, produced for use in conduits with a large diameter. This study presents hemodynamic and early follow-up results from a single center in Turkey concerning the use of the SAPIEN Transcatheter Heart Valve in different types of conduits and different lesions.

Between October 2010 and July 2012, seven SAPIEN Transcatheter Heart Valve implantations were performed. There was mixed type 2 pure insufficiency with stenosis and insufficiency in five patients. Three different conduits were used, and one native pulmonary artery process was performed. Patients were followed for hemodynamic findings, functional capacities, valve competence, reshrinking, and breakage in the stent, and the results were evaluated.

Implantations were successfully performed in all patients. Right ventricular pressures and gradients were significantly reduced, and there was no pulmonary regurgitation in any patient. Functional capacities evidently improved in all patients except for one with pulmonary hypertension. No major complication was observed. During the mean time of follow-up (7.2 ± 4.7 months), no valve insufficiency or stent breakage was observed.

Procedural results and short-term outcomes of the SAPIEN Transcatheter Heart Valve were very promising in the patients included in the study. The SAPIEN Transcatheter Heart Valve can be a good alternative to surgical conduit replacement, particularly in patients with larger and different types of conduits.

The HELEX Septal Occluder (HSO) was approved by the Food and Drug Administration for closure of secundum atrial septal defects (ASD) in 2006. However, little mid-term follow-up information is available. The object of this study was to examine mid-term outcomes after HSO implantation

A retrospective chart review was performed on the first 74 patients who underwent ASD closure with the HSO between 4/00–10/04. Only patients who left the catheterization laboratory after successful implantation and had a minimum follow-up of 5 years were selected for analysis.

Thirty-three patients met entry criteria. At implantation the mean age was 10.9 years and the median ASD size was 15.1 mm. At a median follow-up of 88 months, 26 patients had complete ASD closure, 6 had trivial left-right shunts, and 1 had a significant residual shunt having a reintervention. Fluoroscopy, performed in 27 patients revealed a frame fracture in 3 patients (9%), all of whom were asymptomatic and had effective ASD closure. All 3 devices were 30 mm or 35 mm HSO and were left in place. One patient developed first-degree heart block and remained asymptomatic with no progression. There were no instances of late device embolization, cardiac perforation, erosion, or death.

Following successful implantation, HSO results in effective closure of secundum ASD in mid-term follow-up. Late complications appear to be rare with the exception of frame fracture, which in this series did not result in clinical sequelae.

We evaluated left ventricular dimensions and aortic arch z-scores in infants who underwent balloon angioplasty (BAP) or surgery for coarctation of aorta (CoA). We searched for risk factors predicting recoarctation.

Between 2007–2011, 27 male and 17 female infants (mean age 2.93 ± 4.78 months, range 2 days–24 months) with CoA were evaluated. Left ventricular dimensions, systolic functions, mitral and aortic annuli, transverse aortic arch, isthmus, coarctation site, and diaphragmatic aorta measurements were done and z-scores were determined before intervention.

Six patients underwent primary operation, 38 patients had BAP (86.4%). Associated cardiac pathologies in operated patients were double outlet right ventricle (n = 2), atrioventricular septal defect (n = 1), Ebstein's anomaly (n = 1), arch hypoplasia (n = 2). Twelve patients (27.2%) had simple coarctation. Ventricular septal defect was the most frequent associated cardiac pathology (n = 20, 45.4%). The patients were followed for 10.22 ± 8.21 months. Among 33 primary successful BAP's, 14 had recoarctation (42%). Eleven patients were primarily operated (including 5 with unsuccessful BAP), two had recoarctation (18%). Abdominal and transverse aorta values and z-scores were significantly lower in the recoarctation group (7.15 ± 2.12 mm and 6.07 ± 1.86 mm respectively in the “no-recoarctation group”; vs. 5.53 ± 0.75 mm and 4.94 ± 1.53 mm in the “recoarctation group” P <.05). Abdominal aorta z-score of 0.42 was 88.9% sensitive and 53.8% specific to predict recoarctation (area under ROC curve: 0.618–0.902, P <.05).

Although BAP for native coarctation is still a controversial treatment option due to frequent restenosis rates, abdominal aorta z-score of 0.42 could correctly eliminate recoarctation in 89% of these cases. This cutoff value might help us choose patients for primary BAP and decrease the recoarctation rate after BAP.

Infants with Congenital Heart Disease (CHD) often exhibit growth failure. This can affect anthropometric and neurodevelopmental outcomes well into childhood. To determine the resting energy expenditure (REE), body composition, and growth in infants with CHD at 3 months of age, with the secondary aim to identify predictors of REE as compared with healthy infants.

This descriptive study is a subanalysis of a prospective study investigating predictors of growth in postoperative infants with CHD compared with healthy infants. Growth measurements, REE, and body composition were obtained in all infants. Analysis included chi-square for association between categorical variables, t-tests, ANOVA and ANCOVA. Outcome measures included the REE as determined by indirect calorimetry, anthropometric z-scores and body composition at 3 months of age.

Participants were recruited from the Cardiac Intensive Care Unit of a large, urban, pediatric cardiac center and pediatric primary care practices.

The analysis included 93 infants, 44 (47%) with CHD. Of the infants with CHD, 39% had single ventricle (SV) physiology. There was no difference in REE related to cardiac physiology between infants with CHD and healthy infants or between infants with SV and biventricular (BV) physiology. Anthropometric z-scores for weight (−1.1 ± 1.1, P < 0.001), length (−0.7 ± 1.1, P < 0.05), and head circumference (−0.6 ± 1.2, P < 0.001) were lower in infants with CHD at 3 months of age. The percentage of body fat (%FAT) in postoperative infants with SV (24% ± 6, P = 0.02) and BV (23% ± 5, P < 0.001) physiology were lower than in healthy infants (27% ± 5), with no difference in REE.

At 3 months of age, there was no difference in REE between postsurgical infants with CHD and healthy infants. Infants with CHD had lower growth z-scores and %FAT. These data demonstrate decreased %FAT contributed to growth failure in the infants with CHD.

The study aims to describe the long-term cardiological and psychological results of our first surgical cohort of arterial switch operation (ASO) patients and compare the results with our earlier series of Mustard patients.

Twenty-four survivors of ASO operated in our center (1985–1990) were evaluated by electrocardiography, echocardiography, magnetic resonance imaging, exercise testing, 24-hour Holter-monitoring, and health-related quality of life questionnaire. The results were compared with 58 adult Mustard patients who were evaluated in 2001 using the same study protocol.

Arterial switch operation was performed at a median age of 13 days and Mustard operation at 2 years. Median follow-up was 22 years (range 20–25) and 25 years (22–29), respectively. After ASO, survival was better (P =.04). The event-free survival after 22 years was 77% after ASO vs. 44% after Mustard (P =.03). Good systemic ventricular function was present in 93% after ASO vs. 6% after Mustard (P <.01). Exercise capacity in ASO was 85% of predicted, compared with 72% in Mustard patients (P =.01). Aortic regurgitation was found in 21% of ASO patients vs. 16% in Mustard patients. Arterial switch patients vs. Mustard patients reported significantly better quality of life and less somatic complaints.

The progression made in surgical treatment for transposition of the great arteries from Mustard to ASO has had a positive impact on survival, cardiac function, exercise capacity, and also self-reported quality of life and somatic complaints. Longer follow-up is warranted to monitor aortic regurgitation.

Many adults with transposition of the great arteries have an anatomic right ventricle functioning as the systemic ventricle and are known to develop congestive heart failure, premature cardiac death, and need for cardiac transplantation. Predictors of poor clinical outcome and functional status in patients with left ventricular failure do not always apply to these patients. We aimed to identify predictors of poor functional status in those patients with a systemic right ventricle.

We performed a prospective study of 51 adults with transposition of the great arteries and systemic right ventricles. Demographic, clinical, laboratory, and imaging data were collected, and patients completed a Minnesota Living with Heart Failure Questionnaire (MLHFQ). Comparisons were made between those patients with d-type transposition of the great arteries (dTGA) who have undergone prior atrial switch and those with congenitally corrected transposition (ccTGA). A correlation analysis was performed to identify predictors of poor functional status, as determined by a 6-minute walk distance test.

Median age was 30 years (range 19–65). Median B-type natriuretic peptide was 48 pg/mL (range 16–406). There were 27 patients (53%) with moderate-severe right ventricular dysfunction and 10 (20%) with moderate-severe tricuspid valve regurgitation. The median MLHFQ score was 9 (range 0–78) and 6-minute walk test was 510 m (range 231–703). Forty-one patients had a diagnosis of dTGA atrial switch and 11 patients had ccTGA. Patients with ccTGA were significantly older (40 vs. 28 years, P =.004) and had more tricuspid valve regurgitation (P =.02). Despite this, their MLHFQ scores were significantly lower (2.5 vs. 17, P =.04) and they walked further (635 vs. 504 m, P =.02). Predictors of a short 6-minute walk distance included short stature (P =.009) and dTGA (P =.002). The patient's self-assessment of poor health, as measured by an increased New York Heart Association class (P =.003) and a decreased MLHFQ score (P >.0001) also correlated. B-type natriuretic peptide levels, right ventricular dysfunction, severity of tricuspid valve regurgitation, need for pacemaker, and clinical signs of heart failure did not correlate with exercise tolerance.

Traditional parameters used to predict outcomes in patients with left ventricular failure are not predictive in patients with a systemic right ventricle. Instead, patient's self-assessment of functional status did correlate with objective functional status.

Multiple muscular ventricular septal defects (VSDs) in children can be difficult to treat and a range of techniques has been advocated. These include pulmonary artery banding, interventional catheter closure, and a variety of surgical approaches. When there are apical muscular defects and associated coarse trabeculations in the right ventricle (RV) producing a “Swiss cheese” pattern, a large patch extending on to the RV free wall and excluding part of the apex has been used.

We assessed four adult patients who had surgery 22 to 45 years ago to treat muscular VSD by patches which excluded the RV apex.

Ages ranged from 22 to 50 years. Re-presentations were for polycythemia, cyanosis, syncope, and atrial flutter. Echocardiography showed bidirectional flow from left ventricle to apex of RV, no pulmonary hypertension, small-sized RV with diastolic dysfunction, enlarged right atria, reopening of patent foramen ovale (PFO) in three, and positive bubble studies with right to left shunting in two. Catheterization confirmed elevated right atrial and RV end diastolic pressures. Two patients had evidence of hepatic cirrhosis. One woman had device closure of PFO, but has right heart failure. One man had redo surgical closure of VSD and PFO. Another patient is being considered for a Glenn shunt to take some load off RV.

Surgical closure of muscular VSD by large patch with RV apical exclusion gives good early results. However, long term in adult life, the reduced size of RV, and diastolic dysfunction cause problems. These include reopening of PFO with cyanosis, right heart failure, cirrhosis, and arrhythmias.

With the success of early repair, continued functional assessment of repaired congenital heart disease is critical for improved long-term outcome. Pulmonary regurgitation, which is one of the main postoperative sequelae of congenital heart disease involved with the right ventricle (RV) such as tetralogy of Fallot and transposition of the great arteries, results in progressive RV dilatation coupled with pulmonary artery (PA) obstruction causing elevated RV pressures. The appropriate timing of intervention to correct these postoperative lesions remains largely subjective. In the present study, we evaluated an energy-based end point, namely energy transfer ratio (e_MPA), to assess the degree of RV and PA inefficiency in a group of congenital heart disease patients with abnormal RV-PA physiology.

Eight patients with abnormal RV-PA physiology and six controls with normal RV-PA physiology were investigated using a previously validated technique that couples cardiac magnetic resonance imaging and invasive pressure measurements.

The mean e_MPA of the patient group (0.56 ± 0.33) was significantly lower (P <.04) than that of the control group (1.56 ± 0.85), despite the fact that the patient group had a significantly higher RV stroke work indexed to body surface area (RV SW_I) than the control group (0.205 ± 0.095 J/m² vs. 0.090 ± 0.038 J/m²; P <.02).

We determined that the patients had inefficient RV-PA physiology due to a combination of RV dilatation with pulmonary regurgitation and RV outflow obstruction leading to an elevated end-systolic pressure. Using coupled magnetic resonance imaging and invasive pressure measurements, e_MPA is determined to be a sensitive energy-based end point for measuring RV-PA efficiency. It may serve as a diagnostic end point to optimize timing of intervention.

The study aims to assess whether the increasing use of cardiovascular magnetic resonance imaging in place of diagnostic cardiac catheterization in the management of pediatric patients with congenital heart disease has had an impact on pediatric cardiac care.

Retrospective analysis of data was used.

The study was performed at the Evelina Children's Hospital Cardiology Department.

Elective diagnostic cardiac catheterization or magnetic resonance imaging (MRI) from 2005–2010 are included (n = 896).

Indication, length of stay, and incidence of complications were recorded. In cases used to plan surgery, 30-day survival following the procedure was recorded. Surgical outcomes were compared between the two groups. Surgical outcomes planned using MRI were compared with national outcomes from Congenital Cardiac Audit Database.

For catheterizations (50 patients, [31 male, median age 3 years, interquartile range 1 to 12]), median hospital stay was 1 day (interquartile range 0 to 3), and complications occurred in 11 (22%). Median hospital stay for MRI (846 patients [517 male, median age 3 years, interquartile range 0 to 9]) was significantly shorter: 0 days (interquartile range 0 to 1, P <.001), with fewer complications (16 [1.9%], P <.0001). Twenty-four catheter and 283 MRI patients underwent surgery within 18 months. One catheter patient (2.0%) and four MRI patients (1.4%) died within 30 days (P =.48).

Replacing catheterization with cardiovascular magnetic resonance imaging has resulted in reduced rates of complication and shorter hospital stays without a significant impact on surgical outcome.

Atrial tachyarrhythmias, particularly atrial flutter and fibrillation, are commonly associated with congenital heart disease and are a major cause of morbidity and mortality. The Cox-maze procedure, introduced by Dr. James Cox in 1987, is effective at controlling atrial fibrillation in structurally normal hearts. Though the Cox-maze procedure has been used for atrial tachyarrhythmias in patients with congenital heart disease, few studies have looked at its effectiveness.

A retrospective chart review was performed on 24 patients with congenital heart disease who underwent the Cox-maze procedure at the Medical College of Wisconsin from 2004 through 2010.

Mean age at time of Cox-maze procedure for the cohort was 40.9 years (range, 14 to 66 years). The most common congenital heart diseases among the patients included tetralogy of Fallot (n = 8) and atrioventricular septal defect (n = 4). All patients had concomitant cardiac procedures with the most common being right ventricular outflow tract reconstruction (n = 10), tricuspid valve repair (n = 8), and atrial septal defect repair (n = 7). Prior to the Cox-maze procedure, arrhythmias consisted of atrial flutter or intratrial reentrant tachycardia (n = 19) and atrial fibrillation (n = 5). There were three early postoperative deaths and one late postoperative death. Follow-up was available for 19 of 21 (90%) survivors with a mean length to follow-up from Cox-maze procedure of 2.8 years (range, 0.14–5.7 years). At last follow-up, 14 (74%) of the survivors remained arrhythmia-free.

In patients with congenital heart disease and atrial tachyarrhythmias, the majority were rendered arrhythmia-free by the Cox-maze procedure.

The aim of this study is to determine the relationship between the cardiac catheterization findings and pulsed-wave (PW) Doppler and Doppler tissue imaging (DTI) in pulmonary arterial hypertension patients with congenital heart disease with intracardiac shunts.

The present study aims to determine the relationship between the cardiac catheterization findings and PW Doppler and Doppler tissue imaging (DTI) in patients who have pulmonary arterial hypertension patients due to congenital heart disease with intracardiac shunts. Echocardiographic measurements were performed at the catheter angiography laboratory with concurrent catheterization. Left and right ventricle inflow velocities were recorded with PW Doppler and DTI studies. Maximum tricuspid regurgitation velocity (TS) was recorded in cases with measurable levels by continuous-wave Doppler. Moreover, the correlations among the echocardiographic values and invasive hemodynamic measures such as systolic pulmonary arterial pressure (PAPsystolic), mean pulmonary arterial pressure (PAPmean), diastolic pulmonary arterial pressure (PAPdiastolic) and pulmonary vascular resistance index (PVRI) were evaluated.

A negative correlation was found between TE′/TA′ and PAPsystolic, PAPdiastolic and PAPmean (P = 0.008, r = −0.480; P = 0.001, r = −0.584; P = 0.001, r = −0.567, respectively). ME/ME′ was also found to be negatively correlated with PAPdiastolic, PAPmean and PVRI (P = 0.002, r = −0.556; P = 0.005, r = −0.502; P = 0.027, r = −0.411, respectively). The concurrent use of TE′/TA′ (cut-off value <2.6) and TS had a sensitivity of 79% and a specificity of 93% for distinguishing between patients with healthy controls.

When used in conjunction with conventional methods, TE′/TA′ has the highest sensitivity and specificity in distinguishing between patients and healthy controls.

The use of intraoperative transesophageal echocardiography (iTEE) in neonates ≤4 kg has not been systematically described. We sought to describe the use of and determine risk factors for iTEE probe insertion failure in small infants. We also sought to develop an algorithm for predicting the likelihood of iTEE probe insertion failure.

A retrospective chart review of all neonates ≤4 kg who underwent cardiac surgery at our institution from 12/2001 to 12/2006 was performed. Patients who underwent operations that did not typically require TEE were excluded. Risk factors for TEE probe insertion failure were assessed.

Of 310 neonates who met the inclusion criteria, 219 (70%) underwent successful iTEE. Lower weight (P <.001), abnormal craniofacial anatomy (P =.03), prematurity (P =.015), and 22q11 deletion (P =.04) were independently associated with iTEE probe insertion failure. Stratified by weight, there was an 80% predicted probability of iTEE probe insertion failure for infants weighing: 2 kg with any two of the above risk factors and 3 kg with any three of the above risk factors. There was less than an 80% predicted likelihood of iTEE probe insertion failure for infants weighing 4 kg regardless of other risk factor status.

iTEE can be successfully performed in the majority of neonates ≤4 kg undergoing cardiac surgery. However, there are identifiable risk factors for iTEE probe insertion failure. A weight-based algorithm may help determine neonates at risk for iTEE probe insertion failure. Smaller TEE probes may benefit this patient population.

Most adults with congenital heart disease (CHD) are interested in discussing matters related to advance care planning (ACP) early in the disease course, yet few such conversations actually occur. We aimed to evaluate factors that impact these discussions between patients and adult CHD providers.

Two hundred adult CHD outpatients completed a survey that included factors that might impact ACP discussions with their doctors. In parallel, forty-eight providers within the Canadian Adult Congenital Heart Network completed a similar online survey. Responses were compared between the groups.

Most providers (85%) worried that they were unable to reliably estimate life expectancy and believed that patients were not ready for end-of-life discussions if their estimated life expectancies were beyond 5 years (63%) or beyond 10 years (79%). In contrast, only 24% of patients, independent of disease complexity, thought they were not ready to talk about ACP. Most providers (83%) reported that greater certainty about patients' prognoses would help them discuss ACP. Patients thought that such discussions were best facilitated when they had trust in their doctors (85%) and believed their doctors are good at taking care of patients with CHD (78%).

Despite the fact that challenges to prognostication exist, discussions about ACP should not be reserved for patients with a severely reduced life expectancy. Most patients want these discussions regardless of the complexity of their disease. The trusting and close patient–doctor relationship in adult CHD, often evolving over many years, may provide an excellent platform from which to initiate such discussions.

There is a fivefold increase in the frequency of intracranial aneurysm (IA) in adults with coarctation of the aorta (CoA). Current guidelines for management of adults with CoA recommend computed tomography angiography (CTA) or magnetic resonance imaging of the intracranial vessels. However, this recommendation has not been universally accepted. The purpose of our study was to prospectively perform CTA of the intracranial vessels in adults with CoA to evaluate the prevalence and identify high-risk features of this complication.

From January 2008 to February 2011, adults ≥18 years of age with CoA were prospectively enrolled in a screening program with CTA of the intracranial vessels. Analyses of prognostic variables were performed with both Fisher's exact and two sample t-test. Forty-three patients (58% female, 33.55 ± 10.21 years) with CoA completed CTA of the intracranial vessels. Five patients (11%) were found to have IA. Patients with IA were older than those without (45.6 ± 8.17 vs. 30.89 ± 7.89, P = 0.0003). There were no statistically significant differences detected between measurements of fasting lipid profiles, C-reactive protein, brain natriuretic peptide, and homocysteine levels among CoA patients with and without IA (P = not significant).

Prospective screening of adults with CoA confirmed the increased prevalence of IA but also identified increased age as the sole risk factor. These data suggested that screening is justified particularly in the fourth and fifth decades of life. Further studies are required that focus on the development, natural history, and treatment of IA.

The relationship between resource utilization and postoperative length of stay (PLOS) following congenital heart disease surgery is unknown.

We performed a retrospective cohort study using data from the Pediatric Health Information Systems database. We included subjects 1 month to 1 year of age with a PLOS of ≤1 month following elective, complete repair of tetralogy of Fallot (TOF) during 2004–2008 at children's hospitals that performed ≥10 such surgeries during the study period. We constructed three generalized linear models to assess the relationships of total costs, laboratory costs, and imaging costs during the first three postoperative days with overall PLOS. Race/ethnicity, insurance type, sex, and presence of a genetic syndrome (by ICD-9 codes) were included in the models as fixed effects; hospital of surgery was included as a random effect.

For 1161 eligible surgical encounters at 36 children's hospitals, mean PLOS was 7.1 days (median = 6 days). Mean total, laboratory, and imaging costs for the first three postoperative days were $26 455, $2941, and $813, respectively. Most subjects were male (58.9%), did not have a genetic syndrome (88.3%), were non-Hispanic white (58.3%), and had either public or private insurance (41.0% and 39.1%, respectively). An estimated increase in total costs of $4600 or laboratory costs of $700 in the first three postoperative days was associated with a 1-day increase in PLOS. Imaging costs were not associated with PLOS.

Increased resource utilization is not associated with a shorter PLOS following elective TOF repair, and it may be associated with longer PLOS.

Pulmonary arterial hypertension due to congenital heart disease (CHD-PAH) has a poor prognosis. We sought to determine whether the biomarker high-sensitivity troponin T (hsTnT) measured on routine visit at the outpatient clinic is associated with prognosis.

Consecutive adult CHD-PAH (86% Eisenmenger syndrome) patients referred for advanced medical therapy between January 2005 and March 2007 in the Academic Medical Center in Amsterdam. Patients with severe renal impairment were excluded.

The primary outcome was mortality.

Of all 31 patients (mean age 45 ± 12 years) with CHD-PAH, eight patients died during a median follow-up of 5.6 (range 1.6 to 6.8) years. A hsTnT level >0.014 μg/L was the 99th percentile cutoff of the normal distribution and therefore defined as elevated. At baseline, elevated levels of hsTnT were found in eight patients (26%). In univariate Cox regression, hsTnT elevated at baseline, NT-pro-BNP and right ventricular function were determinants of death (P < .05 for all). Patients with elevated levels of hsTnT showed a significantly higher mortality rate as compared to patients with normal hsTnT levels (62% vs. 13%, P = .005).

Levels of hsTnT were abnormal in a substantial proportion of CHD-PAH patients. A significant inverse relationship was found between hsTnT and survival.

Infants with single ventricle physiology have a high mortality and poor somatic growth during the interstage period. We retrospectively assessed the impact of pharmacotherapy in this population using a multicenter database.

Records for 395 patients (63.5% boys) with single ventricle were obtained from the National Pediatric Cardiology Quality Improvement Collaborative registry. Median of five medications were prescribed per patient at discharge after stage 1 palliation (interquartile range 3 to 6); the most common medications being aspirin (95.7%), diuretics (90.4%), angiotensin convertase enzyme inhibitors (37.7%), proton pump inhibitors (33.4%), H2 receptor blockers (30.6%), and digoxin (27.6%). Interstage mortality was 9.4%. Digoxin use was associated with lower risk of death (P =.03) on univariable analysis, however no single medication was an independent predictor on regression analysis. Change in weight-for-age z-score was studied as outcome of somatic growth with 36.3% patients showing a decrease during the interstage period. Total number of medications prescribed to a patient showed a negative correlation with the interstage change in z-score (r = −0.19, P =.002). On univariable comparisons, use of metoclopramide and lansoprazole were associated with decreased z-score (P =.004 and.041, respectively) although linear regression failed to identify any agent as independent predictor.

Children with single ventricle have high mortality and a profound medication burden. No individual medication is independently associated with better survival or weight gain during interstage period. Despite widespread use, proton pump inhibitors and prokinetic agents are not associated with better outcomes and may be associated with poor growth.

Heart failure (HF) accounts for >3 million hospital admissions annually in adults with acquired cardiovascular disease, but there are limited data on HF admissions in adults with congenital heart disease (ACHD). The purpose of this study was to test the hypotheses that HF admissions are common in ACHD and associated with significant morbidity and mortality.

The 2007 Nationwide Inpatient Sample was used to assess national prevalence, morbidities, and risk factors for mortality during hospitalizations among ACHD with HF.

Of the 84 308 (95% CI 71 345–97 272) ACHD admissions in the United States in 2007, 17 193 (95% CI 14 157–20 229) had a diagnosis of HF (20%). ACHD with HF was associated with an increased risk of death compared to ACHD without HF (OR 3.3, 95% CI 2.6–4.1). On multivariable analysis independent risk factors for mortality included nonoperative intubation (OR 6.1, 95% CI 3.3–11.4), sepsis (OR 4.3, 95% CI 2.4–7.4), and acute myocardial infarction (OR 3.2, 95% CI 1.8–5.7). Cardiac defects associated with an increased risk of mortality included ventricular septal defects (VSDs) (OR 1.8, 95% CI 1.0–3.4).

In this large population-based study, HF-related hospitalizations were common in ACHD and associated with an increased risk of death compared to non-HF admissions. The risk of mortality is increased with the diagnoses of VSDs and the presence of specific comorbidities such as respiratory failure and sepsis.

Necrotizing enterocolitis (NEC) is a significant cause of morbidity and mortality in neonates with complex single-ventricle anatomy undergoing stage I palliation. Hybrid approach is another option for initial single-ventricle palliation. The goal of this study was to determine if there were differences in echocardiographic indices between patients undergoing the hybrid procedure who developed NEC vs. those that did not develop NEC.

Retrospective chart review was performed on patients who underwent the hybrid procedure. Patients were included if an echocardiogram with adequate Doppler tracings through the patent ductus arteriosus stent was available. Echocardiographic indices measured included antegrade velocity-time integral (VTI), retrograde VTI, effective VTI, VTI regurgitant fraction, VTI retrograde/VTI antegrade ratio, calculated cardiac output, peak antegrade velocity through the ductal stent, retrograde/antegrade time ratio, and percent regurgitant time. Indices were compared in patients who developed NEC (NEC Group) and those who did not develop NEC (No NEC Group). NEC was defined as a Bell Stage ≥2.

Sixty-nine patients met inclusion criteria. Eight of the 69 patients developed NEC. There was no significant difference between the NEC and No NEC Group for antegrade VTI (10.4 ± 3.2 cm vs. 12.7 ± 4.4 cm), retrograde VTI (5.3 ± 1.5 cm vs. 6.1 ± 2.2 cm), effective VTI (5.1 ± 2.9 cm vs. 6.6 ± 3.3 cm), VTI regurgitant fraction (53.6 ± 14.7% vs. 49.7 ± 13.6%), and VTI retrograde/VTI antegrade ratio (0.54 ± 0.15 vs.0.50 ± 0.14). Cardiac output (4.2 ± 1.4 L/min/m² vs. 4.8 ± 1.8 L/min/m²) and peak velocity (117.5 ± 28.9 cm/s and 142.4 ± 42.6 cm/s) were also not different between the NEC and No NEC Groups. Furthermore, retrograde/antegrade time ratios (1.6 ± 0.2 vs. 1.7 ± 0.3) and percent retrograde time (60.6 ± 3.0% vs. 62.0 ± 4.0%) were not different between the NEC and No NEC Groups.

Echocardiographic indices were not sensitive in determining the development of NEC in patients undergoing the hybrid procedure. Larger studies with more sensitive imaging techniques are required to help risk stratify NEC in this complex patient population.

Long QT syndrome (LQTS) is a cardiac channelopathy predisposing to syncope and sudden death secondary to LQT-triggered ventricular arrhythmias. Long QT syndrome has been regarded as a purely electrical disease. Recent reports have shown by echocardiography that LQTS patients have contraction abnormalities that are associated with cardiac arrhythmias. The purpose of this study was to determine the spectrum and prevalence of echocardiographic anomalies in a large cohort of patients diagnosed genetically and/or clinically with LQTS.

Two-dimensional and Doppler echocardiographic studies performed during medical evaluation in Mayo's LQTS Clinic were reviewed for 216 LQTS patients. Echocardiograms were evaluated for morphologic abnormalities and atrial and ventricular size and function. Left atrial volume was indexed by body mass. Arrhythmic events were defined as a history of aborted cardiac arrest, documented ventricular tachycardia or fibrillation, and syncope.

While 75% of patients had normal standard echocardiograms, 54 patients (25%) had at least one abnormal echocardiographic finding. Most common were subclinical cardiomyopathic changes, including increased left atrial volume index (n = 25), left or right ventricular enlargement (n = 7), and grade I–II diastolic dysfunction (n = 7). Left atrial volume index was higher in LQTS patients with arrhythmic events compared with those without (24.4 ± 5.5 mL/m² vs. 22.3 ± 6.1 mL/m², P =.02). Corrected QT intervals and left atrial volume index correlated significantly albeit weakly (r² = 0.04, P <.01). Concomitant congenital heart disease was found in two patients.

Subclinical cardiomyopathic changes were found in nearly 20% of LQTS patients. Left atrial enlargement was the most common finding and was associated with prolonged corrected QT and arrhythmic events. These changes may stem from underlying contraction abnormalities caused by ion channel dysfunction.

Guidewires used in pediatric catheterization are typically floppy, soft, or J-tipped, and are generally assumed to be atraumatic. A recent sentinel case suggested that such wires may cause clinically significant pulmonary artery (PA) injury. We sought to determine the incidence of wire-related PA injury as a cause of “idiopathic” airway bleeding (endobronchial blood) during interventional cardiac catheterization in patients with congenital heart disease.

The Children's Hospital Boston database of cardiac catheterizations was reviewed for adverse events (AEs) indicating possible PA injury occurring between September 2006 and August 2011. Procedure notes were reviewed, and when the clinical scenario was suggestive of wire injury or was not clear, relevant angiograms were reviewed.

One thousand forty-seven cases involving PA dilation were performed in the period of interest. Five cases of probable wire injury were identified, suggesting an incidence of approximately 0.5 per 100 cases. Of these five cases, trauma was judged due to floppy-tipped wires in two, soft-tipped wires in two, and a J-tipped wire in one. In three cases, the distal wire was looped such that the leading segment was stiffer than the wire tip. Clinical manifestations of wire injury comprised contrast within the airway, vessel aneurysm/tear, obstructive intimal flap, blood from the endotracheal tube, hemothorax, and wedge defect on lung scan. These injuries were relatively benign and did not result in instability or prolonged bleeding.

Wire injury to the PAs is relatively uncommon, although possible with even floppy-tipped wires. The mechanism and implications of such injuries are markedly different than balloon-mediated vascular tears.

Following repair of congenital heart disease (CHD), adult patients are at risk for reduced exercise capacity. Restrictive lung disease (RLD) may contribute to reduced exercise capacity in this population. The aim of this study was to determine the prevalence of RLD and its impact on exercise tolerance in the adult with CHD.

One hundred consecutive adult patients with CHD, who underwent routine cardiopulmonary exercise testing with spirometry, were evaluated. Clinical data were obtained by retrospective chart review.

Patients from 10 major diagnostic groups were identified. The median age for the cohort was 31 years (range 18–63) and included 43 males and 57 females. Most patients, 79%, had at least one previous surgical procedure. Based on spirometry and flow/volume loops, 50 patients were classified as normal pulmonary function, 44 patients had patterns suggestive of RLD, 4 suggestive of mixed (obstructive and restrictive), and 2 indeterminate. Risk factors associated with RLD include history of multiple thoracotomies (odds ratio = 9.01, P =.05) and history of atrial arrhythmias (odd ratio = 4.25, P =.05). Overall, 56% of the patients had abnormal exercise capacity. Spirometry suggestive of RLD was a significant risk factor for decreased exercise capacity (odds ratio = 3.65, P =.03). Patients with spirometry suggesting RLD also had lower exercise duration (P =.004) and a higher New York Heart Association Functional Class (P =.02). History of previous surgery and decreased heart rate reserve were also significant risk factors for decreased exercise capacity.

Abnormal spirometry suggestive of RLD is common in the adult with CHD and is a significant risk factor for decreased exercise tolerance in this population. Further studies are needed to evaluate the relationship between RLD and exercise intolerance and its relationship to mortality in the adult with CHD.

Postoperative left ventricular dysfunction is associated with poor prognosis in adults with severe chronic aortic regurgitation and published practice guidelines aim to minimize this risk. However, only limited information exists in pediatrics. The goal of this study was to define preoperative risk factors for postoperative left ventricular dysfunction in children with chronic aortic regurgitation.

Patients fulfilling the following criteria were included in this study: (1) age at preoperative echocardiogram ≤18 years; (2) ≥moderate aortic regurgitation; (3) ≤mild aortic valve stenosis; (4) no additional valve disease/shunt; (5) underwent aortic valve surgery for aortic regurgitation; and (6) available preoperative and ≥6-month postoperative echocardiograms with adequate information. Primary outcome was postoperative left ventricular dysfunction defined as ejection fraction z-score < −2.

Median ages at diagnosis and surgery of the 53 eligible patients were 6.9 (0.04–17.2) and 13 years (1.2–22.4), respectively. Compared with patients whose postoperative left ventricular ejection fraction was normal, those with left ventricular ejection fraction z-score < −2 (n = 10) had significantly higher preoperative left ventricular end-diastolic and systolic volumes and dimensions and lower indices of systolic function. Preoperative left ventricular ejection fraction z-score < −1 was the most sensitive (89%; confidence interval [CI] 52, 100) but least specific (58%; CI 41, 73), whereas left ventricular end-systolic diameter z-score ≥ 5 was the most specific (95%; CI 84, 99) but least sensitive (60%; CI 26, 88) outcome identifier. A combination of shortening fraction z-score < −1 or end-systolic diameter z-score ≥ 5 best identified postoperative left ventricular dysfunction with an area of 0.819 under the receiver-operator characteristic curve.

Lower indices of left ventricular systolic function and severity of dilation identify children at risk for postoperative left ventricular dysfunction after aortic valve surgery. These identifiers are similar to predictors defined in adult patients albeit with different threshold values.

With advances in cardiac care, patients with congenital heart disease, including single ventricle (SV) physiology, now survive into adulthood. These patients often suffer from congestive heart failure (CHF) with overexpression of serum biomarkers. Strain and strain rate (SR) may better describe the myocardial mechanics of a failing SV. Our objective was to determine the correlation between strain/SR and biomarkers in adult patients with SV and CHF.

Adult patients (age ≥8 years) with a SV were enrolled. Strain/SR in a 16-segment model of a SV was measured using 2D speckle echocardiography. Serum levels of interleukin 6, interleukin 8, matrix metalloproteinase 9, procollagen I C-terminal peptide (PCIP), cross-linked carboxy-terminal telopeptide of type I collagen (ICTP), pro-B-type natriuretic peptide, nitrotryrosine, tissue growth factor beta (TGF-β), tumor necrosis factor alpha, vascular endothelial growth factor, and creatinine (Cr) were measured. Patients underwent a complete 6 Minute Walk Test (MWT). Pearson correlation coefficient was used. P <.05 was considered significant.

Ten patients with SV (LV = 7, RV = 3) were enrolled. Mean age was 35.5 years (25–42 years). Mean single ventricular ejection fraction (SVEF) was 47%. ICTP correlated with the basal, mid, and apical anterolateral SR, as well as apical anterior and inferior SR. PCIP correlated with mid anterolateral, basal anteroseptal, and mid inferolateral SR. TGF-β correlated with apical inferior SR. Cr correlated with mid inferior-septal and apical lateral SR. 6 MWT negatively correlated with the apical anterior septum SR.

ICTP, Cr, and PCIP correlated best with segmental SR values. Our results provide a preliminary platform for future studies to follow the results of treatment modalities using strain/SR and biomarkers for CHF in this population.

Weight gain during the interstage (IS) period for hypoplastic left heart (HLHS) patients has been associated with improved outcomes. IS home monitoring has been shown to improve mortality. No data exist on IS weight gain and home monitoring effects on weight gain for HLHS patients undergoing the hybrid procedure.

Goal of this study was to describe the weight gain of patients with HLHS undergoing the hybrid procedure during the IS period, to determine if weight parameters were associated with mortality, and to determine if home monitoring improved weight gain.

Retrospective review was performed. Patients were included if they had the diagnosis of HLHS and underwent hybrid procedure. Baseline demographics, surgical dates, and all IS weights were recorded.

Forty-four patients met inclusion criteria, 24 patients had IS monitoring. Time period evaluated was from April 2006 to June 2011. Mean birth weight of the total population was 3.13 ± 0.61 kg, age at hybrid was 5.84 ± 4.10 days, weight z-score at hybrid discharge was −1.66 ± 1.01, age at pre-Stage II was 6.12 ± 1.37 months, IS weight gain was 16.85 ± 5.94 g/day, and weight z-score pre-Stage II was −2.25 ± 1.28. Monitored patients had significantly higher weight z-score pre-Stage II (−1.67 ± 0.98 vs. −2.82 ± 1.28) and lower change in weight z-score (−0.26 ± 0.97 vs. −1.24 ± 1.06). Eight patients died IS. There was a significant difference in weight gain per day in those that survived the IS period (17.87 ± 4.75 g/day vs. 12.28 ± 8.65 g/day). There were no significant differences in weight characteristics in patients that survived the Stage II procedure (n = 28) vs. those that did not (n = 7).

Home monitoring improved IS weight gain in patients undergoing the hybrid procedure. Decreased weight gain per day was associated with IS mortality.

Children with congenital heart disease may experience significant psychosocial morbidity related to impaired quality of life (QOL). The aim of this study was to evaluate the clinical utility of health-related QOL assessment in a pediatric cardiology outpatient clinic.

The Pediatric Quality of Life Inventory (PedsQL) 4.0 Generic Core Scales were completed by a convenience sample of 176 patients, aged 8–18 years, being seen in a pediatric cardiology clinic. Three cardiologists enrolled in this study reviewed the completed PedsQL during the clinic visit and recorded their responses to items reported to be a problem “Often” or “Almost Always.” This utilization of the instrument was compared to standardized scoring and the practicality and perceived usefulness of the practice was evaluated by physician interview.

PedsQL responses showed 38% of patients reporting significant (Often or Almost Always) problems on at least one domain (19% Physical Functioning, 18.2% Emotional Functioning, 11.4% Social Functioning, and 22.3% School Functioning problems). Using standardized scoring, the prevalence of scores below the cutoff score for clinically significant impaired QOL in each domain ranged from 10% to 20%, with agreement between scoring methods ranging from 89% to 93%, sensitivity 68% to 86%, and specificity 89% to 97%. Cardiologists reported interventions in 30.1% of patients. They found that the PedsQL was easy to use, did not interfere with clinic operations, required minimal time (1–5 minutes), and provided information that had an important impact on their practice in some patients.

This study demonstrates the clinical utility of health-related QOL assessment using the PedsQL in a pediatric cardiology outpatient setting. Identification of significant impairments in QOL can impact clinical decision making and may change psychosocial outcomes in children with congenital heart disease.

The right ventricle (RV) supports the systemic circulation in patients who have had an intraatrial repair of transposition of the great arteries or have congenitally corrected transposition. There is concern about the ability of a systemic RV to support the additional volume load of pregnancy, and previous studies have reported deterioration in RV function following pregnancy. However, conditions with a systemic RV are also associated with progressive RV dysfunction over time. To date, no study has examined whether the deterioration associated with pregnancy is due to the physiological changes of pregnancy itself, or is part of the known deterioration that occurs with time in these patients.

Women who had undergone pregnancy under the care of the Adult Congenital Heart Disease Unit at the Queen Elizabeth Hospital were retrospectively identified and matched to separate male and nulliparous female controls. Functional status (New York Health Association [NYHA]), RV function, and systemic atrioventricular valve regurgitation were recorded for each group at baseline, postpregnancy (or at 1 year for control groups) and at latest follow-up.

Eighteen women had 31 pregnancies (range 1–4) resulting in 32 live births. There were no maternal but one neonatal death. At baseline, there was no significant difference in NYHA class or RV function between pregnancy and control groups. In postpregnancy, there was a significant deterioration in the pregnant group alone for both NYHA class (P = 0.004) and RV function (P = 0.02). At latest follow-up, there was a significant deterioration in RV function in all three groups. There was still a reduction from baseline in NYHA of women who had undergone pregnancy (P = 0.014), which again was not seen in the controls groups.

This study suggests that pregnancy is associated with a premature deterioration in RV function in women with a systemic RV. These women are also more symptomatic, with a greater reduction in functional class compared with patients with a systemic RV who do not undergo pregnancy. This study will allow this cohort of women to be more accurately counseled as to the potential long-term risks of pregnancy.

The surgical management of adults with congenital heart disease (ACHD) offers a great challenge, with a large number of anomalies with complex pathophysiology necessitating specific treatments. Pre- and postoperative morbidity has been relatively high, and the influencing factors are not completely identified. We sought to evaluate the incidence and predictors of postoperative complications following surgery for ACHD centralized to a Swedish cardiothoracic center.

Between April 2003 and May 2012, 191 consecutive patients with ACHD underwent 192 surgical procedures at our department. Pre-, intra-, and postoperative data were prospectively entered in a clinical database and retrospectively reviewed. Multivariate analysis was used to identify determinants of postoperative complications as a composite end point.

The 30-day mortality was 0.5%. Overall survival was 98.3% ± 1.0 at 1 year and 98.3% ± 1.0 at 5 years postoperatively. Repeat sternotomy had to be performed in 94 patients (49%). New onset atrial fibrillation or flutter was the most prevalent (13%, n = 17/135) postoperative complication. Independent risk factors for major postoperative complications were age (odds ratio [OR] 1.81/10 year increment, P = 0.001; 95% confidence interval [CI] 1.29–2.53), reduced (<50%) systemic left ventricle ejection fraction (OR 3.61, P = 0.031; 95% CI 1.13–11.6), and the duration of cardiopulmonary bypass (OR 3.34/60 minute increase, P < 0.001; 95% CI 2.03–5.49).

Our present data suggest that surgery in ACHD can be performed in centralized units with an excellent early and midterm survival. The incidence of postoperative complications was relatively low consisting mainly of supraventricular arrhythmias. In our opinion, ACHD surgery should be performed in centralized units with experienced surgeons in a dedicated multidisciplinary team for optimized postoperative management.

Cardiorenal interactions adversely impact the prognosis in heart failure patients an effect which crucially involves increased central venous pressure (CVP). However, it is unclear whether the same pathophysiology operates in adults with congenital heart disease (CHD).

The present study was designed to assess cardiorenal interactions in adults with CHD after biventricular repair.

We measured the kidney length (KL, cm/m) and renal resistive index (RI) in 77 consecutive patients and 30 controls. We also measured hemodynamics, plasma B-type natriuretic peptide level, 24-hour creatinine clearance, and peak oxygen uptake in each patient. The CVP correlated with the KL (r = 0.44, P <.001) and the RI was greater in the patients (P <.0001). The high RI was independently determined by the CVP, aortic pressure, and cardiac index (P <.05–.001), and correlated with the 24-hour creatinine clearance (r = −0.30, P <.05). The RI correlated closely with the neurohumoral activations and peak oxygen uptake (|r| = 0.45–0.50, P <.0001), and the patients with a traditional criteria of high RI (≥0.70) had a higher incidence of cardiovascular events that required unscheduled hospitalization (hazard ratio = 2.78, 95% confidence interval 1.26–6.10, P <.05). Multivariate Cox model with the cutoff values of KL ≥68 cm/m and RI ≥0.74 revealed that a greater KL (hazard ratio = 4.03, 95% confidence interval 1.46–11.1, P <.01) as well as B-type natriuretic peptide (P <.001) independently predicted the events.

Hemodynamics, especially a high CVP, independently predicted the enlarged kidney and abnormal intrarenal flow dynamics that are closely associated with heart failure severity and cardiovascular events in adults with CHD after biventricular repair.

The purpose of this study is to describe the outcomes of cardiac catheterizations performed by pediatric interventional cardiologists in an adult catheterization laboratory on adult patients with congenital heart disease (CHD).

With improved survival rates, the number of adults with CHD increases by ∼5%/year; this population often requires cardiac catheterization.

From January 2005 to December 2009, two groups of patients were identified, an adult group (>21 years) and an adolescent group (13–21 years), who had catheterizations performed by pediatric interventional staff.

Fifty-seven catheterizations were performed in 53 adults, while 59 were performed in 47 adolescents. The male to female ratio differed significantly between groups; only 15/53 (28%) of adults were male vs. 26/47 (55%) of adolescents (P =.006). Among adults, 27 had previously corrected CHD, 16 with atrial septal defect (ASD), and six with patent foramen ovale (PFO). This differed significantly from the adolescents, where only 30 had previously corrected CHD, seven with ASD, and one with PFO (P =.012). Among adults who were catheterized, interventions were performed on 28/53 (53%). All interventions were successful and included ASD/PFO closure, patent ductus arteriosus occlusion, coarctation dilation, pulmonary artery dilations, and one saphenous vein graft aneurysm closure. Nineteen adults had coronary angiography performed by adult interventionalists in consult with pediatric interventionalists. Two complications occurred among adults (3.8%) vs. one complication (2%; P = 1) among adolescents. No femoral vessel complications or catheterization-associated mortality occurred.

Cardiac catheterizations can be performed effectively and safely in adults with CHD by pediatric interventional cardiologists in an adult catheterization laboratory.

Iron deficiency anemia is the most common single cause of anemia worldwide. The purpose of our study was to estimate the prevalence of anemia in adult congenital heart disease (ACHD) patients, compare different hematology parameters between hypoxemic and nonhypoxemic ACHD patients, and determine which parameters detect iron deficiency anemia in hypoxemic ACHD patients.

ACHD patients were studied and blood samples collected for determination of hemoglobin, derived red cell indices, serum iron, apoferritin, total iron-binding capacity, transferrin saturation index, C-reactive protein (CRP), and N-terminal proB-type natriuretic peptide (NT-proBNP) levels.

Two hundred seventy-eight ACHD patients, mean age 31.6 ± 14.3 years old, were studied. One hundred sixty-seven (60%) patients were male. Two hundred forty-five patients were nonhypoxemic and 33 patients were hypoxemic. Hypoxemic ACHD patients had significant higher hemoglobin concentration (g/dL) (17.5 ± 3.5 vs. 14.6 ± 1.7, P <.001), red cell distribution width (RDW) (%) (17.0 ± 3.3 vs. 14.1 ± 7.6, P <.034), apoferritin (ng/mL) (19.8 [4.1–147.2] vs. 38.0 [6.7–191.2], P =.019), CRP (mg/dL) (0.50 [0.0–3.8] vs. 0.12 [0.0–1.4], P <.001), and NT-proBNP (pg/mL) (409.3 [33.3–9830.8] vs. 5.2 [0.0–1068.4], P <.001) levels than nonhypoxemic ACHD patients. Serum iron, total iron-binding capacity, and transferrin saturation index were not statistically significant between hypoxemic and nonhypoxemic ACHD patients. In the hypoxemic group, 15 (45%) patients had apoferritin levels <20 ng/mL and eight (24%) patients developed microcytosis and hypochromia. A RDW above the normal range (>14.5%) in hypoxemic ACHD patients allowed the detection of an apoferritin level <20 ng/mL with a sensitivity of 93%.

RDW seems to be a useful and economic tool to detect low serum apoferritin levels in hypoxemic ACHD patients.

The aim of the current study is to describe the long-term clinical and hemodynamic characteristics of adult patients with single-ventricle physiology who have not undergone the Fontan operation and consequently have remained cyanotic.

Adult patients at the Ahmanson/UCLA Adult Congenital Heart Disease Center with non-Fontan single-ventricle physiology who had undergone cardiac catheterization between 2005 and 2011 were included. Echocardiographic and cardiac catheterization data were reviewed.

Mean estimated single ejection fraction was 56 ± 8%. Eight of 13 subjects had documented E/E′ data with a mean of 6.44. Seven subjects had both A′ and E′ data documented, of which two subjects exhibited A′ > E′. Mean ventricular end-diastolic pressure (MVEDP) was 15.77 ± 4.91 mm Hg, and was >12 mm Hg in eight of the 13 patients (62%). MVEDP was also analyzed by age, and in the single-ventricle patients was 13.55 ± 4.12 mm Hg in those <50 years of age, compared with 20.75 ± 1.89 mm Hg in those >50 years of age (P = .003). MVEDP prior to inhaled pulmonary vasodilator administration was 14.75 ± 5.5 mm Hg, compared to 15.00 ± 6.78 mm Hg in the postvasodilator group (P = .48). Subjects with end-diastolic pressure (EDP) <12 had a mean brain natriuretic peptide (BNP) of 108 ± 197 pg/mL, while subjects with EDP >12 had a mean BNP of 234.5 ± 127.36 pg/mL (P = .11)

Cyanotic adult single-ventricle patients not palliated with Fontan completion have preserved single-ventricle systolic function but develop elevated ventricular filling pressure with increasing age. Only invasive hemodynamic measurements demonstrated elevated ventricular filling pressures, while traditional echo/Doppler criteria for diastolic dysfunction were not met. Aging with cyanotic single-ventricle physiology is associated with a greater degree of filling pressure elevations than in the general population. Single-ventricle patients with EDP >12 exhibited markedly elevated BNP compared to those with normal EDP.

The purpose of this study was to evaluate the association between fetal development of congenital heart defects (CHD) and maternal prepregnancy body mass index (BMI, kg/m²) in the largest population-based case-control study to date.

Mounting evidence implicates maternal obesity as a risk factor for birth defects. However, the association between maternal obesity and CHD in offspring has been less completely described.

We conducted a study of CHD using linked birth-hospital discharge records for Washington State between 1992–2007. All infants with CHD (n = 14 142) were identified based on ICD9-CM discharge diagnosis codes. 141 420 controls, frequency matched on year of delivery, were selected at random from among infants without CHD. Maternal BMI was calculated from maternal prepregnancy data. Odds ratios (OR) and 95% confidence intervals (CI) for the association of CHD relative to maternal BMI were calculated, adjusted for gestational diabetes.

Infants with CHD were more likely to have an obese mother (OR 1.22, 95% CI 1.15–1.30). The strength of association increased with increasing BMI (BMI 30–34.9: OR 1.16, 95% CI 1.07–1.25; BMI 35–39.9: OR 1.25, 95% CI 1.13–1.39; BMI > = 40: OR 1.49, 95% CI 1.32–1.69). The association was greatest for left and right ventricular outflow tract defects (OR 1.27, 95% CI 1.02–1.59 and OR 1.43, 95% CI 1.20–1.69, respectively). Hypoplastic left heart syndrome was markedly associated (OR 1.86, 95% CI 1.13–3.05). There was no association with conotruncal defects (OR 1.04, 95% CI: 0.82–1.33).

We confirmed the association between CHD and maternal obesity and observed increasing risk with increasing obesity. Outflow tract defects appear uniquely associated. A greater risk of CHD among offspring is an important outcome of maternal obesity, and suggests a need for targeted medical management strategies.

Right ventricular outflow tract (RVOT) reconstruction necessitates frequent reoperation. To understand the early outcomes, we analyzed our results to provide the intra- and postoperative morbidity and mortality. We hypothesized that multiple previous sternotomies do not influence the morbidity, mortality, or survival.

We performed a retrospective review of patients who underwent reoperative RVOT reconstruction at the University of Rochester Medical Center and SUNY Upstate Medical Center from January 1, 2000 to December 31, 2009. Patients were divided into three groups based upon the number of previous sternotomies: Group 1 with one, Group 2 with two, and Group 3 with three or more previous sternotomies.

220 patients had reoperative RVOT reconstruction, 103 in Group 1, 71 in Group 2, and 46 in Group 3. There was no difference in the percentage of inadvertent cardiotomy between groups (Group 1: 2%, Group 2: 1%, Group 3: 2%; P =.9) The number of previous sternotomies had no effect upon infection, arrhythmia, or the percentage of patients who received a red blood cell transfusion (Group 1: 56%, Group 2: 49% Group 3: 43%; P =.3). Perioperative mortality for the entire group was 3/220 (1.4%), with no difference between groups. At a mean follow-up of 39 months, there was a survival of 98% for Groups 1 and 3 and 97% for Group 2 (P =.7).

Reoperative RVOT reconstruction can safely be performed with limited morbidity and mortality. The number of previous sternotomies does not influence the rate of cardiotomy, red blood cell transfusion, or early outcome.

The hybrid stage I procedure is an alternative palliative strategy for patients with hypoplastic left heart syndrome who traditionally have undergone the Norwood operation. At our institution, the hybrid stage I procedure is employed only for patients with high operative risk. Our objective was to describe our use of a pressure guidewire during the hybrid stage I procedure to assess quantitatively pulmonary artery band adequacy.

After reviewing the charts on all high-risk patients who underwent a hybrid stage I procedure at our institution, we compared two groups of patients: those who underwent the standard hybrid stage I palliation (standard cohort) and those with pressure wire-facilitated assessment of distal branch pulmonary artery pressure (pressure wire cohort) to evaluate the impact of pressure guidewire use on procedural risk, radiation time, patient outcomes, and need for reoperation for pulmonary artery band adjustment.

The pressure guidewire was used in 8 of 14 patients at the time of hybrid stage I procedure and was successful and without complication in all attempts. In the standard cohort, 67% of patients needed reoperation for pulmonary artery band adjustment, compared to 12.5% of patients in the pressure wire cohort (P =.09). Procedure time, radiation exposure, and survival to hospital discharge were not different between groups.

This novel use of a pressure guidewire to assess quantitatively pulmonary artery band adequacy at the time of placement is feasible, safe and may decrease the need for reoperation for pulmonary artery band adjustment.

This study presents the long-term follow-up of patients who developed left lung perfusion (LLP) abnormalities following patent ductus arteriosus (PDA) closure with various device types.

The study includes 23 adult and pediatric patients who had undergone transcatheter PDA closure and were shown to have decreased LLP (<40%) by the first scintigraphy performed within the average follow-up period of 14.0 ± 8.12 months (2.0–30 months). For PDA closure, the Amplatzer duct occluder was used in 12 patients, and coils were used in 11. Within the average period of 58.91 ± 12.93 months (37–85 months) after transcatheter PDA closure, a second lung perfusion scintigraphy was performed.

In 13 out of 23 patients (56.5%), LLP improved by the time of the second scintigraphy. Improved and unimproved patients did not differ with regard to age, weight, body surface area, PDA diameter, ampulla diameter, and PDA length at the time of PDA closure and the second scintigraphy. There was no significant difference with regard to the percent of improved patients between the different device types (P =.88). The left pulmonary artery indexes were also insignificantly different (P =.446). Patients with persistent LLP abnormality had significantly higher average Doppler velocity index [(LPA blood flow velocity—RPA blood flow velocity) / MPA blood flow velocity] × 100 (P =.007) and PDA diameter/length. If Doppler velocity index ≥50% is taken as the cutoff value, it is possible to predict persisting LLP abnormality with 80% sensitivity and 76% specificity. Left lung perfusion abnormality was found to persist in patients with PDA diameter/length ≥0.5 with 80% sensitivity and 92.3% specificity.

The LLP abnormalities seen after PDA closure with various devices eventually improve to normal in the majority of patients during long-term follow-up. Patients whose PDA length is shorter than its diameter are at risk of developing LLP abnormalities that persist long-term.

To identify the risk factors that could predict postoperative outcome after aortic valve replacement in pediatric patients with isolated aortic regurgitation (AR).

There is controversy regarding the appropriate timing of surgery in asymptomatic or minimally symptomatic patients with isolated AR. In the pediatric age group, there are limited studies in this regard and most of them are on combined aortic valve stenosis and regurgitation.

All patients with biventricular physiology and morphologic left ventricle (LV) who underwent aortic valve surgery for AR from January 1988 to July 2010 were included in the study. Demographic, clinical, and echocardiographic data were collected at presurgical visit, early postoperative, 1 year, and most recent follow-up.

Among 53 patients (36 males), 18 had LV end-diastolic diameter (LVEDD) z-score >4 standard deviation (SD) (group I) and 35 had LVEDD <4 SD (group II). Forty-one had long-term follow-up. Mean age at surgery was 11.6 ± 5.9 years; mean follow-up was 6.9 ± 5.6 years. Preoperative LVEDD >4 SD predicted persistent LV dilation (>2 SD) at early post-op (P < .05) and 1 year follow-up (P = .09). Preoperative decreased LV function (fractional shortening <28%) was the only significant predictor of persistent LV dysfunction at most recent follow-up and requirement for repeat interventions (P < .01). Most have reduction of LV dimensions in the immediate postoperative period to normal limits.

In children with AR, preoperative LV dysfunction and extreme LV dilation (>4 SD) are significant predictors of incomplete LV remodeling or persistent LV dysfunction.