Pulmonary Hypertension

Our pulmonary hypertension program spans mechanism, diagnosis and intervention, with the right ventricle as a central determinant of outcome. Single-nucleus transcriptomic studies across patient samples and complementary models have identified fibroblast AXL signal as a driver of maladaptive right ventricular fibrosis through PI3K–AKT–NFIC activation and extracellular-matrix production, nominating a target for preserving right ventricular function. To improve phenotyping and risk assessment, we develop video-based artificial intelligence methods that automate beat-to-beat right ventricular segmentation, fractional area change and pulmonary hypertension classification across multicenter pediatric echocardiography cohorts.

We also translate physiological insights into treatment strategies for advanced disease. These include multidisciplinary, imaging-guided selection and timing of Potts shunt, rescue pathways combining reverse Potts shunt with venopulmonary extracorporeal membrane oxygenation for refractory pulmonary hypertension crises, and a portable electrochemical nitric oxide generator designed for affordable in-home inhalation. Together, these efforts connect molecular targets, standardized imaging and pragmatic devices or surgical palliation to enable earlier recognition, better stratification and more accessible, individualized care for children with pulmonary hypertension and progressive right ventricular failure.