Case report: Peripartum cardiomyopathy in a woman with hypopituitarism
Article first published online: 5 DEC 2005
BJOG: An International Journal of Obstetrics & Gynaecology
Volume 113, Issue 1, pages 123–124, January 2006
How to Cite
Morton, A. and Kannan, S. (2006), Case report: Peripartum cardiomyopathy in a woman with hypopituitarism. BJOG: An International Journal of Obstetrics & Gynaecology, 113: 123–124. doi: 10.1111/j.1471-0528.2005.00807.x
- Issue published online: 5 DEC 2005
- Article first published online: 5 DEC 2005
- Accepted 5 October 2005.
A 12-year-old girl underwent transphenoidal partial resection and subsequent X-ray treatment to a 10-mm diameter craniopharyngioma in 1988. She developed panhypopituitarism within one year of treatment and was commenced on replacement treatment with thyroxine, cortisone acetate, oestrogen and progesterone. Growth hormone (GH) was not used as she had achieved satisfactory height. At age 25, she was documented to have GH deficiency with her serum insulin-like growth factor-I (IGF-1) being 10 nmol/L (normal 20–55 nmol/L). She conceived with in vitro fertilisation at age 28. At 22 weeks of gestation, cardiac investigations were performed because of a two-year history of intermittent palpitations. Echocardiography revealed normal left ventricular size and function with an ejection fraction of 60%, Holter monitoring disclosed frequent ventricular ectopics. At 35 weeks of gestation, she developed mild gestational hypertension that did not require any antihypertensive treatment. There were no features to suggest preeclampsia. She proceeded to have an elective caesarean section with spinal anaesthesia and intravenous hydrocortisone cover at 38 weeks of gestation. The caesarean section was performed because of maternal request. Her son weighed 3330 g. Twelve hours postpartum, she described progressive dyspnea. Her oxygen saturation was 83% with 8 L/minute oxygen via a Hudson mask, she was afebrile, pulse was 120 min and regular, blood pressure was 160/105 mm Hg and respiratory examination revealed bilateral crackles. Peripartum fluid balance was positive 1 L, no non-steroidal anti-inflammatory drugs were given. ECG was normal, chest X-ray was consistent with pulmonary oedema and ventilation—perfusion scanning was reported as a low probability for pulmonary embolism. Echocardiography revealed a severely dilated left ventricle (end-diastolic diameter 68 mm, end-diastolic volume 187 mL) with moderate global systolic dysfunction with ejection fraction of 37% consistent with peripartum cardiomyopathy (PPCM). No left ventricular thrombus was seen. Right ventricular systolic function appeared mildly reduced. There was moderate eccentric mitral regurgitation with the valve appearing structurally normal. The estimated right ventricular systolic pressure was 26 mmHg. She was treated with frusemide, perindopril and clexane and subsequently was commenced on warfarin and carvedilol. She did not lactate. GH replacement was considered but was deferred in view of progressive improvement in her left ventricular function on serial echocardiography and also because of the uncertain relationship between her longstanding GH deficiency and PPCM. At 12 months postpartum her ejection fraction had returned to normal. She is not contemplating further pregnancies. Long term GH replacement has not been considered because of cost issues as this treatment is not subsidised in Australia.
We were able to identify outcome data for 83 pregnancies to mothers with GH deficiency and/or hypopituitarism from the published literature. On the basis of 18 pregnancies to 9 hypopituitary women, Overton et al.1 concluded these were high risk cases with half of the live births on or below the tenth centile for weight, caesarean section rate of 100% (six emergency, four elective), two (11%) second trimester intrauterine fetal deaths and five (28%) first trimester miscarriages. In one live birth the mode of delivery was unknown. They proposed the high adverse pregnancy outcome rate may relate to uterine defects secondary to endocrine deficiency. In contrast, Curran et al.2 found no adverse effects of GH deficiency in terms of fetal outcome and low maternal morbidity in 25 pregnancies to 16 mothers with GH deficiency due to a variety of causes. They concluded that GH replacement therapy is probably not essential for GH-deficient females during pregnancy. From approximately 10 weeks of gestation to term, placental GH gradually replaces pituitary GH, which becomes undetectable.3 The description of progressive elevation of serum IGF1 in a pregnant woman with Pit-1 deficiency supports the hypothesis that placental GH is the prime regulator of maternal serum IGF1 during pregnancy.4 GH deficiency is associated with decreased left ventricular ejection fraction at rest and on effort, decreased diastolic filling at rest, decreased myocardial wall thickness and decreased exercise duration and capacity. The severity of cardiac impairment correlates with the severity of GH deficiency. GH treatment of adults with GH deficiency is associated with a significant positive effect on left ventricular mass, left ventricular end-diastolic diameter and stroke volume.5 Patients with deficiency of pituitary hormones other than GH who are adequately replaced have systolic performance, diastolic filling and haemodynamics similar to controls. GH has an established role in cardiac development and modulates myocardial structure and function. GH deficiency is also associated with vascular abnormalities including decreased aortic compliance, increased intimamedia thickness and endothelial dysfunction.6
This is the first case of which we are aware of PPCM occurring in the setting of hypopituitarism. The incidence of PPCM has varied in reported series but is probably between 1 in 3000 and 15,000 pregnancies.7 Risk factors include maternal age greater than 30 years, multiparity, women of African descent, pregnancy with multiple fetuses, pre-eclampsia, maternal cocaine abuse, selenium deficiency and long term oral tocolytic therapy. Women with a history of PPCM who regain normal resting left ventricular size and performance on resting echocardiography have an approximate 20% risk of recurrence of PPCM in subsequent pregnancies, and decreased contractile reserve has been demonstrated in these women by the use of a dobutamine challenge test.8
This case of PPCM in a mother with GH deficiency may simply represent the co-occurrence of two uncommon disorders. Another explanation is that the patient's longstanding GH deficiency led to subclinical left ventricular dysfunction not detected by resting echocardiography, which was then unmasked by the cardiovascular stresses of gestational hypertension, delivery and the postpartum state. Thus, it would seem prudent to perform a rigorous test of left ventricular contractile reserve such as stress echocardiography before embarking on attempting pregnancy in any woman with GH deficiency, and for these mothers to be closely observed for cardiac decompensation in the peripartum period.