Chronic thromboembolic pulmonary hypertension related to hemangiolymphangioma

Hemangiolymphangioma (HL) is a congenital anomaly and histologically benign tumor, which was composed of both the lymphatic and the blood vessels. We report an adult case of HL complicated by chronic thromboembolic pulmonary hypertension (CTEPH) in a 20-year-old female. Gastrointestinal bleeding scintigraphy and operative findings elucidate a close relationship between CTEPH and residual HL. This case indicates that HL survivors with remaining left-to-right shunt might lead to CTEPH during the long-term follow-up.