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Pulmonary Arterial Hypertension in the Setting of Pregnancy – A Case Series and Standard Treatment Approach.

J. Smith

J. Mueller

Curt Daniels


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Conference: 2012 International PHA Conference and Scientific Sessions

Release Date: 06.22.2012

Presentation Type: Abstracts

Smith JS1, Mueller J1, Daniels CJ2

1. University of Louisville Medical Center. Division of Pulmonary, Allergy, Critical Care and Sleep Medicine, Louisville, Kentucky.

2. The Ohio State University Medical Center Division of Cardiovascular Medicine, Columbus, Ohio.

BACKGROUND: Pregnancy in women with PAH is associated with a maternal mortality of 30-50% despite modern treatment modalities. The majority of maternal deaths in PAH patients occur either during labor and delivery or within one month post-partum, attributed to a mismatch of physiologic limitations of PAH and the changes that occur with pregnancy and delivery. In the Unites States, there is no consensus on PAH management in pregnancy.We present five cases of gravid PAH patients successfully managed at our institution with a description of our standardized multidisciplinary treatment approach.

METHODS: We present five cases of gravid PAH patients successfully managed at our institution with a description of our standardized multidisciplinary treatment approach.

RESULTS: Five consecutive cases of pregnancy with significant pulmonary hypertension were managed at our center with a mean age of 30 years old (21-37). Three cases had PAH with congenital heart disease, one with idiopathic PAH, and one with SLE associated PAH. Four of the patients tolerated pregnancy well. One patient (Case 3) developed progressive right heart failure to NYHA-FC IV. Anticoagulation was maintained in all cases with enoxaparin. Intravenous prostacyclin was initiated on average at 32 weeks gestation with one patient on therapy prior to conception(Case 3). All delivered at term without maternal or fetal mortality. Four patients delivered vaginally while one underwent Cesarean section. Peri-partum complications include two patients with anemia and volume depletion responding to blood transfusions (Case 3, 5).

CONCLUSIONS: We report our institutions management of five pregnant patients with severe PAH with no fetal or maternal mortality. We attribute a methodical and multidisciplinary approach to our successful outcomes. We advocate development of a standardized approach to the treatment of PAH in the setting of pregnancy in an effort to further improve maternal-fetal survival.