Several medical conditions have been reported to be involved in the development of CTEPH, including splenectomy, ventriculo-atrial shunt, inflammatory bowel disease and previous cancers. 1 These conditions increase the risk of a poor outcome in CTEPH patients. Systemic dysfunctions are also associated with pulmonary arterial hypertension (PAH) and PAH-associated death, including insulin resistance, dyslipidemia and renal hronic thromboembolic pulmonary hypertension (CTEPH) is characterized by chronic and mechanical thromboembolic obstruction of large and small pulmonary arteries, with high mortality without intervention. 1,2 Although pulmonary endarterectomy (PEA) is recommended for the treatment of CTEPH, 37% of the cases are considered inoperable. 3 Recently, we and others have demonstrated that balloon pulmonary angioplasty (BPA) markedly improves the pulmonary hemodynamics and the long-term prognosis in CTEPH patients. Background: Pulmonary arterial hypertension with systemic dysfunctions, including metabolic disorders and renal dysfunction, has a poor prognosis. However, it remains to be elucidated whether chronic thromboembolic pulmonary hypertension (CTEPH) is also associated with systemic dysfunctions, and if so, whether balloon pulmonary angioplasty (BPA) improves them.