Pulmonary Endarterectomy in Pediatric Patients: Institutional Experience

Chronic thromboembolic pulmonary hypertension (CTEPH) is rare in children, and published experience with pulmonary endarterectomy (PEA) is limited.

We retrospectively reviewed six patients (<18 years) who underwent seven PEA procedures at our center between December 2011 and September 2025. Clinical characteristics, perioperative findings, hemodynamic outcomes, and follow-up data were analyzed.

Median age was 13 years (range, 2-17), and five patients were female. Five had a history of pulmonary embolism, while one presented with a hydatid cyst. Risk factors for CTEPH were hydrocephalus with ventriculoperitoneal shunt (n = 2), hydatid cyst disease (n = 1), infective endocarditis with left ventricular outflow tract obstruction (n = 1), and chronic kidney disease with prior COVID-19 infection (n = 1). Preoperative mean systolic pulmonary artery pressure (PAP) was 56 ± 23 mm Hg, mean pulmonary artery pressure (mPAP) was 39 ± 15 mm Hg, and mean pulmonary vascular resistance index (PVRi) was 7.51 ± 4.56 U·m². Most patients were in World Health Organization functional class III or IV. All patients underwent PEA, with concomitant procedures required in most cases. Postoperatively, mPAP decreased to 21 ± 4 mm Hg and mean PVRi to 2.36 ± 1.36 U·m². Median intensive care unit and hospital stays were 3.5 and 9 days, respectively. There was one in-hospital mortality; all other patients recovered without major complications.

PEA is feasible and effective in carefully selected pediatric patients, including those with complex comorbidities. Significant hemodynamic and functional improvements were achieved, supporting the role of PEA as a potentially curative therapy in this rare and challenging group.