Uneventful Pregnancy and Delivery in a Patient with Idiopathic Pulmonary Hypertension: a Case Report

Abstract

Idiopathic pulmonary hypertension is a rare disease characterized by sustained elevation of the pulmonary artery pressure and pulmonary vascular resistance, normal pulmonary artery wedge pressure, in the absence of a known cause. Prior reports suggest a very high maternal mortality in patients with idiopathic pulmonary hypertension undergoing pregnancy, and for that the recommendation has been avoidance of pregnancy (or termination if the patient is already pregnant). On the other hand, there have been multiple reports of patients with idiopathic pulmonary hypertension sustaining pregnancy and labor without major complications. This case report illustrates the course of pregnancy and labor in a patient diagnosed with idiopathic pulmonary hypertension. At age 24, the patient started with symptoms of shortness of breath and chest pain, and upon evaluation she was found with moderately severe idiopathic pulmonary hypertension. One year and 8 months later the patient becomes pregnant, and begins follow up with gynecology and cardiology. During this time the patient was asymptomatic, and did not have any clinical evidence of pulmonary hypertension. The risks of pregnancy were discussed with the patient, and she decided to continue pregnancy. She had an uneventful pregnancy, complicated only by preterm labor at 34 weeks and 5 days of gestation. She had spontaneous labor and delivered vaginally a healthy baby boy, weighting 4 pounds and 12 ounces. No invasive monitoring was used. The mother and the baby were discharged home 48 hours postpartum. Seven months later the patient returned for evaluation, presenting evidence of severe pulmonary hypertension. She has been followed up ever since by a cardiologist and currently is stable but symptomatic. This report adds to the amount of evidence that suggests that pregnancy and labor in a patient with idiopathic pulmonary hypertension may have a better outcome than previously reported. The decision of undertaking and/or continuing pregnancy in a patient with idiopathic pulmonary hypertension relies ultimately on the patient’s choice, but should be done on an individual basis after careful evaluation of the risks. Finally, the need of close follow up with a multidisciplinary team is mandatory in the patient with idiopathic pulmonary hypertension that wishes to undergo pregnancy.