Uneventful pregnancy and delivery in a patient with idiopathic pulmonary hypertension: a case report

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.

Primary pulmonary hypertension and pregnancy

Primary pulmonary hypertension (PPH) is a disorder intrinsic to the pulmonary vascular bed characterized by sustained elevation in pulmonary artery pressure and pulmonary vascular resistance with normal pulmonary artery wedge pressure, in the absence of a known cause. Cardiovascular disorders are the most common cause of morbidity and mortality in pregnant women. Risk is so great with some cardiovascular abnor-malities that recommendation of avoidance or interruption of pregnancy is supportable, one of these conditions is pulmonary hypertension. In this article two patients with primary pulmonary hypertension who sustained two pregnancies each are described. Both patients had uneventful pregnancies and deliveries, with all neonates surviving. When evaluating primary pulmonary hypertension and pregnancy, early studies reported a maternal mortality rate as high as 50%. More recent studies report a maternal mortality of 30%. In this article, cases of primary pulmonary hypertension undergoing pregnancy from 1978 to 2005 were reviewed, revealing a 22% maternal mortality for the total number of pregnancies. Despite advances in treatment, primary pulmonary hypertension continues to carry considerable maternal morbidity and mortality, and prevention of pregnancy is still the main recommendation. Early diagnosis and treatment is critical and a multi-disciplinary approach is required when dealing with a patient with PPH who desires to continue pregnancy.

Bullous pemphigoid: course of the disease. A study of 25 patients

Different studies on the course of bullous pemphigoid have suggested that there may be significant variations in the survival of these patients based on their ethnicity or region of origin. Because of the lack of studies on patients with bullous pemphigoid in the Caribbean, specifically in Puerto Rico, a retrospective analysis about the outcome of patients with this disease in the region was performed. Twenty-five (25) patients were included in this study. The patients were diagnosed as bullous pemphigoid by clinical, histopathologic and immunopathologic assessment. Medical records were reviewed and those patients that were alive were contacted and examined on a follow up visit. Nine were men and sixteen were women; the age at the time of the diagnosis ranged from 54 to 90 year-old (mean 72); 13 were alive after at least 5 years of follow-up and 12 had died. The age at the moment of death ranged from 67 to 95 year-old (mean 83). Of those patients living, 85 were in remission, while 15 had active disease. In general, patients had a disease of mild severity, but, still, 68 of them received systemic corticosteroid therapy. Five of patients had to be admitted due to disease; two of them died while at the hospital. This data suggests that bullous pemphigoid in Puerto Ricans is a disease with a relatively benign course, but which can be fatal in the elderly, especially, if it requires admission to the hospital and the patient receives high doses of systemic corticosteroids