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The impact of pulmonary hypertension (PH) on survival in cystic fibrosis (CF) remains unclear.To determine the influence of PH on survival in the CF population.The United Network for Organ Sharing database was queried from 1987 to 2013 to identify first-time lung transplant candidates who were tracked from wait list entry date until death or censoring to determine influence of PH. Using right heart catheterization measurements, mild PH was defined as mean pulmonary artery pressure greater than or equal to 25 mm Hg and severe greater than or equal to 35 mm Hg.Of 2,781 CF patients, 2,100 were used for univariate analysis, 764 for Kaplan-Meier survival function, 687 for multivariate Cox models, and 576 and 132 for matching on the propensity of mild PH and severe PH, respectively. Univariate Cox analysis found significant differences in survival for mild PH (hazard ratio [HR], 1.747; 95% confidence interval [CI], 1.387-2.201; P0.001) and severe PH (HR, 2.299; 95% CI, 1.639-3.225; P0.001). Further assessment by multivariate Cox models identified significant risk for death associated with mild PH (HR, 1.757; 95% CI, 1.367-2.258; P0.001) and severe PH (HR, 2.284; 95% CI, 1.596-3.268; P0.001). Cox regression stratified on matched pairs of PH cases and control subjects confirmed the risk for death for mild PH (HR, 1.919; 95% CI, 1.290-2.85; P = 0.001) and severe PH (HR, 4.167; 95% CI, 1.709-10.157; P = 0.002).The manifestation of PH is associated with significantly increased risk for death in CF patients with advanced lung disease.

Pregnancy in patients with pulmonary arterial hypertension (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 1 month postpartum. Cardiovascular collapse is attributed to a mismatch between the physiologic limitations of PAH and the changes that occur with pregnancy and delivery. In the Unites States, there is no consensus on the management of PAH in pregnancy. Several case reports have been published describing improved maternal-fetal outcomes, likely due to new advanced PH therapies, earlier diagnosis of PAH, and an adoption of a multidisciplinary treatment approach. We present five cases of gravid PAH patients successfully managed at our institution with a description of our standardized multidisciplinary treatment approach.