Full Recovery of Right Ventricular Systolic Function in Children Undergoing Bilateral Lung Transplantation for Severe PAH

ABSTRACT Background . We investigated whether RV function recovers in children with pulmonary arterial hypertension (PAH) and RV failure undergoing lung transplantation (LuTx). Methods . Prospective observational study of 15 consecutive children, 1.9-17.6 years old, with PAH receiving bilateral LuTx. We performed advanced echocardiography (Echo) and cardiac magnetic resonance imaging (MRI), followed by conventional and strain analysis, pre- and ∼6 weeks post-LuTx. Results . After LuTx, RV/LV end-systolic diameter ratio (Echo), RV volumes and systolic RV function (RVEF 63 vs. 30 %; p Conclusion . We demonstrate full recovery of RV systolic function in children within two months after LuTx for severe PAH, independently of the patients’ age, weight, and hemodynamic compromise preceding the LuTx. Even in end-stage pediatric PAH with poor RV function and low cardiac output, LuTx should be preferred over heart-lung transplantation.