Successful Pulmonary Thromboendarterectomy for Malignancy-Associated Septic Chronic Thromboembolic Disease in...

Chronic thromboembolic pulmonary hypertension is exceptionally rare in children and is typically associated with major acquired prothrombotic conditions.

We report a 14-year-old girl with acute lymphoblastic leukemia who developed chronic thromboembolic pulmonary artery obstruction following necrotizing pneumonia and septic thromboembolic disease. Computed tomography, ventilation-perfusion scintigraphy, and pulmonary angiography demonstrated complete right pulmonary arterial occlusion with absent perfusion to the right middle and lower lobe territories. Despite mildly elevated pulmonary artery pressures (mean pulmonary artery pressure 23 mm Hg), the patient presented with severe functional limitation, oxygen dependence, and surgically accessible disease. Bilateral pulmonary thromboendarterectomy was successfully performed, achieving restoration of pulmonary perfusion and favorable postoperative recovery. Molecular analysis of the resected thrombotic material identified Mycobacterium tuberculosis.

This case highlights the feasibility of pulmonary thromboendarterectomy in pediatric chronic thromboembolic pulmonary hypertension and emphasizes that surgical candidacy should integrate symptom burden, anatomical operability, and multidisciplinary assessment beyond hemodynamic severity alone.

Pulmonary thromboendarterectomy is feasible and associated with favorable outcomes in pediatric patients and remains the only potentially curative treatment. Early multidisciplinary evaluation is essential to identify operable disease, integrating anatomical burden, clinical and hemodynamic status, and the risk of disease progression.