Successful heart lung transplantation in patient with pulmonary capillary hemangiomatosis: case presentation

Our observation demonstrates a case of a 40-year-old female with pulmonary arterial hypertension World Health Organization functional class III, who was admitted to NMRC of Cardiology repeatedly due to disease progression including dyspnea worsening, onset of desaturation and chest pain during exertion on riociguat (7.5 mg daily), bosentan (115 mg daily) and selexipag (400 mcg daily) therapy. Computed tomography scan revealed pulmonary capillary hemangiomatosis pattern and the diagnosis was revealed. We performed PAH-specific therapy escalation which resulted in selexipag withdrawal, bosentan dose titration to 250 mg daily with good treatment tolerability. Due to unreleased treatment goals, high-risk status and heart failure progression, the patient was referred to Shumakov National Medical Research Center of Transplantology and Artificial Organs, where successful heart lung transplant was performed in may 2022. The postoperative period elapsed without complication, the patient discharged from hospital and is staying alive in stable condition. Pulmonary capillary hemangiomatosis is a rare cause of pulmonary arterial hypertension with pure prognosis. PAH-specific treatment should be avoided due to increased risk of rapid disease progression and pulmonary oedema. The only one definitive treatment is lung or heart lung transplantation.