Abstract
Chronic thromboembolic pulmonary hypertension (CTEPH) in pediatric population is rare. We present a case of five-year-old female child, diagnosed as CTEPH on warfarin and home oxygen for 6 months. Imaging showed extensive thrombus in distal right pulmonary artery (RPA) than left pulmonary artery (LPA). She clinically deterioration inspite of maximal medical management and was referred for surgical option. Considering lack of literature evidence on surgical outcomes in pediatric CTEPH, decision to perform surgery was made after multi-disciplinary team discussion between adult and paediatric pulmonary hypertension and cardiothoracic surgical teams across two units in UK. We performed pulmonary thromboendarterectomy (PEA) using cardiopulmonary bypass under deep hypothermic circulatory arrest (DHCA). The surgical procedure was uneventful with good clinical recovery. Our report highlights the need for earlier and prompt diagnosis with a need for referral to a specialized surgical center at an earlier stage before intractable vascular resistance ensues. Surgery can be safely performed for CTEPH in pediatric populations.
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