Recent advances in the clinical management of end-stage pediatric pulmonary hypertension.

PURPOSE OF REVIEW: This review aims to give an overview of recent advances in the clinical management of children <18 years of age with end-stage pulmonary hypertension refractory to conventional medical therapy.

RECENT FINDINGS/SUMMARY: The increased awareness and characterization of pediatric pulmonary vascular disease has amounted more data toward creating an accurate, and comprehensive, risk prediction tool. This underscores the importance of serial outpatient re-assessment, as early consideration of advanced pharmaceutical and interventional therapies, as well as lung transplantation, improves outcomes in children. Despite the emergence and approval of new therapies against pulmonary hypertension in adults, pediatric-specific complications have not yet been adequately explored. It is, however, encouraging that several trials are ongoing to address this issue. Interventional strategies to unload the right ventricle are increasingly being utilized, although evidence remains scarce in this area. Extra-corporeal membrane oxygenation may, in addition to being a bridge to lung transplantation in well-selected patients, be considered as a bridge to catheter-based or surgical interventions. Management of pediatric right ventricular failure in the setting of advanced pulmonary hypertension is a truly complex clinical challenge at the intersection of cardiac, vascular and respiratory physiology and requires careful integration of invasive hemodynamic assessment, pulmonary vasodilator therapy, ventilatory strategy, and timely consideration of mechanical circulatory support.