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Endocardial fibroelastosis: an unusual cause of pulmonary hypertension in pregnancy
1Medical Corps, United States Army, Department of Obstetrics and Gynecology, Tripler Army Medical Center, Honolulu, Hawaii, USA.
Insights
Pregnancy in women with severe pulmonary hypertension from endocardial fibroelastosis is rare but achievable. Modern obstetric care and hemodynamic monitoring can lead to successful outcomes for both mother and child.
Area of Science:
- Cardiology
- Obstetrics
- Genetics
Background:
- Endocardial fibroelastosis (EFE) with severe pulmonary hypertension (PH) is a rare, often fatal pediatric condition.
- Women with EFE rarely reach reproductive age, making pregnancy cases undocumented.
- Advances in medical care allow more EFE patients to reach reproductive age.
Observation:
- A 23-year-old woman with congenital EFE and severe PH presented at 20 weeks gestation.
- Pregnancy management included cardiac catheterization, bed rest, medications (theophylline, digoxin), and oxygen therapy.
- Labor at 35 weeks involved invasive hemodynamic monitoring and epidural anesthesia, with postpartum pulmonary artery pressure management using nitroglycerin.
Findings:
- This case demonstrates the first reported successful pregnancy in a woman with severe pulmonary hypertension due to endocardial fibroelastosis.
- Maternal pulmonary artery pressures were managed effectively throughout pregnancy and postpartum.
- The fetus was delivered at 35 weeks gestation.
Implications:
- Successful pregnancy is possible for women with EFE and severe PH.
- Modern obstetric and critical care techniques are crucial for managing these high-risk pregnancies.
- This case highlights the importance of multidisciplinary care for reproductive-aged women with complex congenital heart disease.
Abstract:
Pulmonary hypertension due to endocardial fibroelastosis is usually diagnosed during infancy and childhood and is almost uniformly lethal when severe. Since females with this disorder rarely reach reproductive age, no cases of successful pregnancy in the presence of this severe cardiopulmonary disease have been reported. A 23-year-old Caucasian primigravida with a history of congenital endocardial fibroelastosis and severe pulmonary hypertension presented at 20 weeks' gestation. Following cardiac catheterization, the pregnancy was managed with bed rest, oral theophylline and digoxin, and low-flow oxygen therapy. After spontaneous onset of labor at 35 weeks, invasive hemodynamic monitoring and epidural anesthesia were initiated. Worsening of maternal pulmonary artery pressures postpartum was relieved by intravenous nitroglycerin infusion. Recent advances in medical care have resulted in more women with endocardial fibroelastosis reaching reproductive age. Successful pregnancy outcome is possible using established techniques of modern obstetric care.