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The BDR team chose to include all babies with all types of VSDs. Using these criteria Delaware's prevalence of 78.7 was higher than that reported by other states (whose prevalence ranges from 1.6 to 70.0 per 10,000 live births) (National Birth Defects Prevention Network, ). Delaware's prevalence is similar to other states when small muscular VSDs are excluded. Birth Defects Research (Part A) 106:888-893, 2016. © 2016 Wiley Periodicals, Inc.

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Purpose of review Although pregnancy in pulmonary arterial hypertension (PAH) is considered high risk and contraindicated, the incidence is rising. It is paramount to understand the pathophysiology and effective management strategies to ensure optimal outcomes for maternal and fetal survival. Recent findings In this review, we highlight the outcomes of recent case series of PAH patients in pregnancy, with a focus on proper risk assessment and target goals of PAH therapy. These findings support the notion that the pillars of PAH management, including pulmonary vascular resistance reduction resulting in right heart functional improvement, and widening of the cardiopulmonary reserve, should serve as a blueprint for PAH management in pregnancy. Summary Multidisciplinary and tailored management of PAH in pregnancy, with emphasis on optimizing right heart function prior to delivery, can result in excellent clinical outcomes in a referral pulmonary hypertension center.