Abstract
A 10-year-old boy, with multiple comorbidities presented with fever, exertional dyspnea, fatigue and an obliterated brachiocephalic and inferior caval vein. Chronic thromboembolic pulmonary hypertension (CTEPH) was diagnosed. Nadroparine, antibiotics and supplemental oxygen were successfully started. Aged 12 years, supplemental oxygen was permanently needed with progressive exertional dyspnea and fatigue. In the country of residence the patient was considered as inoperable. The right ventricle was severely dilated, hypocontractile and hypertrophic. Mean pulmonary artery pressure (mPAP) was 79 mmHg and cardiac output 2.2 L/min. Pulmonary endarterectomy was uneventful. Four days later, mPAP was 33 mmHg and cardiac output 6.4 L/min. Three months later the boy restarted his education without supplemental oxygen. Six months after surgery right ventricular size and function and mPAP (14 mmHg) were normal. We demonstrated that pulmonary endarterectomy in young aged children is feasible and well-tolerated, even in the presence of severe co-morbidities. CTEPH should be an important diagnostic consideration in symptomatic children with a known hypercoaguable state, a history of thrombo-embolism or venous catheter placement, and/or a diagnosis of pulmonary hypertension. Hesitating to refer children for surgical consideration, or attempting to treat them by medication, only postpones the single potentially curable treatment and may worsen their prognosis.