The impact of prenatal counselling on mothers of surviving children with hypoplastic left heart syndrome: A qualitative interview study

Abstract Objective To explore the role of antenatal counselling in how parents make treatment decisions following an antenatal diagnosis of Hypoplastic Left Heart Syndrome (HLHS). Background Antenatal counselling is a critical part of patient management following a diagnosis of fetal congenital heart disease; however, there is a very limited evidence base examining how parents actually experience antenatal counselling and make decisions in this context. Methods Semi‐structured interviews were conducted with women who had received an antenatal diagnosis of HLHS. Interviews were digitally recorded, anonymised and transcribed verbatim. A thematic content analysis was performed using a constant comparative approach. Results Eight mothers of surviving children with HLHS were interviewed. Eight key themes emerged including new perspectives on how women receive antenatal counselling and how it affects their decision making. Three themes in particular are new to the literature: (a) Mothers of children with HLHS reported feelings of intense guilt that arose in the antenatal period around potentially causing the condition in their child. (b) For this group of women, recollections of perceived pessimism during antenatal counselling had a lasting impact. (c) Despite support from partners or extended family, women nevertheless experienced a strong sense that antenatal decision making was largely a ‘maternal’ responsibility. Conclusions When recounting their experiences of antenatal counselling, mothers of surviving children with HLHS offer new perspectives that can guide fetal cardiologists in how best to support their individual patients. Further research is needed to comprehensively understand the experience of prospective parents counselled for severe forms of fetal congenital heart disease.


| INTRODUC TI ON
An antenatal diagnosis of a severe congenital anomaly such as hypoplastic left heart syndrome (HLHS) often requires parents to assimilate a large amount of complex information at a time of intense emotional and psychological distress. However, there is little empirical evidence available from parents who have faced these experiences. A better understanding of the challenges faced by parents in these circumstances may help to inform a more relevant, supportive and intuitive model for antenatal counselling.
Hypoplastic left heart syndrome is a rare form of congenital heart disease in which the left side of the heart fails to develop normally in utero. As a result, there is obstruction to blood flow through the left side of the heart. The syndrome varies in its presentation and encompasses underdevelopment of the mitral valve, left ventricle, aorta and aortic arch. HLHS has been reported to occur in approximately 0.016% to 0.036% of all live births. 1 There are several options available in the management of this condition: a three-staged reconstructive surgical technique, termination of the pregnancy and compassionate supportive therapy only. All surgical options are non-curative or 'palliative' procedures, with the ultimate goal of producing a 'single ventricle' or 'Fontan' circulation, whereby the right ventricle ejects to the aorta and pulmonary blood flow is driven by central venous pressure alone. This strategy carries significant peri-operative and interstage mortality risks and is associated with long-term morbidity, including exercise intolerance, ventricular dysfunction, arrhythmias, protein-losing enteropathy, plastic bronchitis and neurodevelopmental impairment. 3,4 Stage one of this approach, generally termed the 'Norwood Procedure', involves enlargement of the atrial septal defect, connection of the aorta to the pulmonary artery, aortic arch reconstruction and the insertion of a systemic-to-pulmonary shunt. 2 Despite some recent debate about the role of compassionate care in HLHS, 5 the consensus remains that this is a potentially life-limiting condition with no cure and that treatment decisions should be guided wherever possible by the wishes of the family.
Several studies [6][7][8] have been conducted looking at the parental experience of caring for a child with HLHS but little work exists on the experience of diagnosis and prenatal counselling. Previous studies have looked at parental decision making in the neonatal period 9 and yet increasingly, due to improvements in prenatal screening, the diagnosis of HLHS is made antenatally. 10 This is potentially a critical moment for a pregnant couple, and one which may have a significant impact on how they will continue to interact with health-care professionals thereafter. It has previously been emphasised that the information given at diagnosis, the manner in which it is presented, and the parents' understanding and interpretation of that information are critical factors in shaping parents' perceptions and management decisions. 11 Antenatal counselling in HLHS therefore represents a unique window in which doctors have a responsibility to communicate effectively and sensitively with parents in order to support their decision making.
The 2014 review of Children's Heart Surgery Services in Leeds 12 sought feedback from families and highlighted antenatal diagnosis and care as a key area for improvement. Families felt that the counselling and support provided following the diagnosis of the congenital heart condition was inadequate, and there was a perception of little compassion or understanding. Similarly in the United States, parents receiving a diagnosis of congenital heart disease report sometimes perceiving their doctors to be lacking in compassion and empathy. 11,13,14 Parents' perception of the information communicated to them about an antenatal diagnosis, as well as the manner in which that information is communicated to them by health-care professionals, has been shown to impact on their ability to cope with the diagnosis. 15 Similarly, how parents conceptualise the decision-making process, and their role within it, will also impact on how they cope in the longer term. 16 In this study, we sought to interview parents with an antenatal diagnosis of HLHS, using their first-hand accounts of the decision-making process to explore the impact of prenatal counselling on how these difficult decisions are navigated in the antenatal period.

| DE S I G N & S E T TING
This was a single-centre interview study with parents. Potential participants (parents of patients with a diagnosis of HLHS) were identified using the fetal cardiology database and eligible parents who received a diagnosis of HLHS during their pregnancy from 1995 to 2014 were invited to participate. A purposive sampling strategy was used. We chose this timeframe owing to the fact that 1995 was the year that Guy's and St. Thomas' Hospital started offering the Norwood procedure, as part of a three-staged surgical intervention for HLHS.
Both parents who had a surviving child with HLHS and mothers who had opted for a termination of pregnancy were approached.
Participants were invited to participate by letter and provided with detailed patient information sheets about the study and a copy of the interview topic guide. Written consent was obtained prior to the interviews. Semi-structured interviews were digitally recorded, anonymised and transcribed verbatim. Topics covered included parental understanding of HLHS, parental memories of being given the diagnosis and discussions that took place at the time, factors which influenced the parental decision-making process, the doctor's role in the decision-making process and the ongoing impact of the diagnosis on the parents' and child's life.
Interviews lasted on average 50 mins, ranging between 26 minutes and 1 hour 26 minutes. All interviews were undertaken by SB.
Data were analysed using a thematic content analysis employing a constant comparative approach. Open coding of early data was performed to generate categories. This enabled the range of concepts used by participants to be identified and to extend the analysis so the research question could better be understood in terms of ideas from the data itself. An inductive approach was thus applied such that coding of extracts and a constant comparative approach enabled the team to develop concepts and connections and bring these together into the themes presented. A reflective diary was completed immediately after each interview to provide additional context. Three randomly selected interviews were coded separately by MB-L and compared across the team.
Consensus on emergent themes was reached through regular discussions. Using an iterative process and in-depth conversation amongst the coders and co-authors, it was determined that saturation was achieved well before the study ended. Illustrative quotes are presented in the findings.

Ethical approval was granted by the NRES Committee South East
Coast-Surrey (REC Reference 14/LO/1557).

| Participants
Given concerns from the clinical team that some families would find contact from the research team upsetting, the clinicians were invited to compile a list of patients they felt were eligible and able to participate. Families who the clinical team felt were too vulnerable to be approached were excluded from the list of potential par-  Table 1.

| RE SULTS
Thematic analysis of the interview data revealed eight key themes as detailed below.

| Emotional distress and feelings of guilt
Mothers talked about the profound emotional distress they expe-

| Determination to understand the condition
Having been given the diagnosis of HLHS mothers discussed how overwhelming they found the information given to them and how difficult it was to understand all of the details about the condition and the treatment options presented to them. This sensation of being overwhelmed was met with a sense of determination to better understand the condition in order to better inform their decision making. Mothers responded by seeking out information: from leaflets, from the internet or from other families affected by HLHS. They frequently emphasised the importance of other people's stories in helping them come to an understanding of their own situation.

| Value of clear explanations
Many mothers spoke of the positive experience they felt they had at the tertiary referral centre and much of this was attributed to feeling that health professionals gave clear and detailed explanations.  given the wrong way. I believe that they were given the wrong way because the first option, she, the first thing she offered me was an abortion (…) it shouldn't be the first option.

| A sense of responsibility for decision making
The mothers interviewed expressed how the weight of a decision to pursue a termination of pregnancy would have been difficult for them to bear. It was clear, however, that they felt a sense of responsibility as parents, to be the ones making the decision for their unborn child, and that this was not a decision they saw as appropriate for health professionals or other families to make on their behalf. Many mothers spoke of how much they valued not being pressurised by the doctors who counselled them and feeling that the decision was entirely up to them as parents.
Yeah, it was my decision, it wasn't their decision and it was never their, like they never made me feel like they was saying "she needs it now and we're making the decision over you" so it was always left to me to decide what I wanted to do rather than being pushed into anything.

| Maternal responsibility
Whilst mothers reported discussing the decision to continue with the pregnancy with their partner and others, their sense of nevertheless needing to make the decision alone was surprisingly strong.
The choice to pursue a medical termination is ultimately, from a legal standpoint, that of the mother alone. The mothers interviewed reflected this profound sense of a 'maternal' responsibility in the decision-making process, as opposed to a shared 'parental' responsibility. This was often tied to the realisation that they would be acting as the principal carer for the child after birth.

I mean ultimately it's my body I can do what I want.
But obviously as parents it's me and (the father).

(Participant 2)
Because I knew that it was going to be me, being the main, being the carer, because obviously my husband is out at work.  The role of the clinician, in this case the fetal cardiologist, is to impart a significant amount of important information to the parents in order to best prepare them to make the right decision for their family as to how to progress with the pregnancy. In the case of a severe congenital heart disease such as HLHS, this includes preparing them for the reality of a lifetime of medical care, multiple surgeries and the possibilities of disability or even death. Yet they are also forging a pivotal relationship with that family, and the quality of that relationship and the memories of these early consultations will have a lasting impact.

| Family ethics
The emotional distress at receiving a severe antenatal diagnosis such as HLHS cannot be overestimated and the mothers in this study spoke openly about their shock, fear and sadness at the time of diagnosis. However, what is particularly poignant in these narratives is the feelings of intense guilt shouldered by mothers, who often blame themselves for causing the condition. This emphasises a crucial role for clinicians when counselling, to seek to reassure and assuage any feelings of guilt at an early stage. The women in this study described their impulse to thoroughly understand and research the diagnosis.
This echoes previous work which found 'information seeking' to be a prominent mechanism used by some parents to overcome uncertainty when faced with a prenatal diagnosis of a severe congenital anomaly. 17 The strong recollections of perceived pessimism during early antenatal counselling is another important finding. Whilst it is always the clinicians' responsibility to offer all treatment options available to the patient, the manner and even the order in which these options are presented may have an important influence on later perceptions of counselling. In particular, initial counselling at screening (ie that undertaken before tertiary cardiology review) should be extremely circumspect with regard to the diagnosis, prognosis and management options.
The mothers in this group felt a strong sense of being personally responsible for decision making and valued the fact that their clinicians did not make them feel pressured into any particular treatment path. When faced with making decisions about their pregnancy, the women in this group favoured neutrality and a non-directive approach from their clinician. This further supports previous findings that whilst some health-care professionals may perceive these decisions as being too burdensome for parents to make, parents themselves often conceptualise such decisions as part of their parental responsibility, and may feel offended when professionals provide information in what the parents perceive as a biased manner. 18,19 Interestingly the women in our group emphasised how beyond them identifying a 'parental' responsibility for decision making, they felt a strong sense of it being a 'maternal' decision. This was linked to their sense of ownership over treatment decisions about their own body but also to their anticipated role as main carers for their future child. We do not know how generalisable this finding is across all women.
In the cohort interviewed, all with continuing pregnancies, the reasons given for this decision were largely unanimous in their formulation of the desire 'to give their child a chance'. Here, we see that the mothers in this group felt an early sense of responsibility to advocate for their future child by keeping all options open.
Research has shown that parents of children with life-threatening illnesses often feel driven by a need to leave no stone unturned, | 1229 BERTAUD ET Al. and hence, they will pursue treatment options relentlessly, even those that may have low odds of success. 20 Another study that considered how parents make the choice to pursue surgery for HLHS also found that a sense of hope and seeking a way to 'fix' the problem were key motivators for the decision. 21  Finally, this work reinforces the notion that the relationship between parents and clinicians is critical to decision making. 23 With this in mind, clinicians may be able to better support the antenatal decision-making process by considering the nature of this relationship before, during and after counselling; seeking to understand at an early stage of the process how parents may view their potential options. By making space for a pregnant couple to lay out their views first, the clinician may be better able to assess their practical, psychological and emotional needs, and tailor their counselling accordingly.

| Study limitations
Whilst both parents were invited to participate in the study, ultimately our interviews were solely with mothers, leaving the paternal voice out of the picture. Very limited research exists as to the father's experience of an antenatal diagnosis of a severe congenital anomaly. Existing evidence suggests that fathers set their own needs aside to attend to the supportive needs of their pregnant partner whilst focusing on coming to a joint decision regarding whether to continue or terminate the pregnancy. 24 We were only able to recruit families who had chosen to continue with the pregnancy following the diagnosis of HLHS and all had a surviving child at the time of interview. This data therefore only represents a very specific cohort of parents and can only offer perspectives on how some women experience antenatal counselling and decision making. Notably absent from this study are women who opted for termination of pregnancy, representing a significant limitation to some of the findings in this work. 8 out of the 13 families with surviving children with HLHS elected to participate versus 0 out of the 6 families who had chosen a termination of pregnancy. Further research is needed into how parents who choose termination or compassionate supportive care following a diagnosis of HLHS experience the decision-making process.
Interviews were conducted retrospectively with a mean time from the child's birth of 65 months. The mothers' account of their antenatal decision-making process is hence subject to recall bias and their narrative will undoubtedly have been shaped by their subsequent experience of caring for a child with HLHS.
Furthermore, the field of fetal medicine and treatment of congenital heart disease is a rapidly changing area and we cannot assume that for any pregnant women there will be unity in their views or experiences. Nonetheless, there is value in trying to elucidate the underpinnings of how these women made decisions during their pregnancy.

| Future work
Despite considerable advances in antenatal diagnostic technologies in recent years, there is a paucity of research examining parental experiences in-depth. 25  By understanding the decision-making process for families, we can better enrich the relationship between parents and clinicians and work towards more tailored and intuitive counselling. These questions would be best answered by prospective research into antenatal decision making which could capture the experience of all parents faced with a similar diagnosis.

| CON CLUS IONS
When recounting their experiences of antenatal diagnosis of a severe congenital heart defect, mothers of surviving children with HLHS offer unique insights into the process of antenatal decision making. Training and guidance for those undertaking prenatal counselling in congenital heart disease should take into account these findings. There is a need for further prospective work into how parents with an antenatal diagnosis of severe congenital heart disease navigate treatment decisions with their clinicians in order to improve and individualise antenatal counselling. not necessarily those of the NIHR, the Wellcome Trust, the Health Foundation, the NHS, or the Department of Health. This research received no specific grant from any funding agency in the public, commercial or not-for-profit sectors. We would like to acknowledge the parents who gave up their time to be interviewed for this study.

CO N FLI C T O F I NTE R E S T
We declare no competing interests.

E TH I C A L A PPROVA L
Ethical approval was granted by the NRES Committee South East Coast-Surrey (REC Reference 14/LO/1557).

DATA AVA I L A B I L I T Y S TAT E M E N T
The data that support the findings of this study are available on request from the corresponding author. The data are not publicly available due to privacy or ethical restrictions.