Primary pulmonary hypertension (PPH) is progressive, and no effective cure is currently available. Patients with PPH usually die within several years after onset. We report a 23 years old man who was treated as PPH for 11 years finally revealed as multiple peripheral pulmonary stenosis (PS).
Case report: This male patient was referred to our hospital when he was 12 years old because of right ventricular hypertrophy. His right ventricular hypertrophy was noticed by the electrocardiogram examination routinely performed during the first-year of junior high school in our prefecture. The cardiac catheterization showed high pulmonary artery pressure [93/75 (82) mm Hg], and the imaging view of the pulmonary artery showed proximal artery dilation and progressive narrowing suitable for PPH. We diagnosed him as PPH, and treated using belaprost sodium, ACE-I, and aspirin. Though 11 years passed since his PPH was diagnosed, he did not show rapid aggravation of either his physical condition (NYHA I-II) or pulmonary pressure. Therefore, we referred him to the cardiovascular division of another hospital that specially deals with adult PPH patients. Cardiac catheter examination conducted in the hospital showed that pulmonary artery pressure was high [96/33 (56) mm Hg]. Cardiovascular angiography revealed he had multiple pulmonary artery stenosis in peripheral area. He was finally diagnosed not as PPH but as multiple peripheral PS.
Our case suggested that we should seek another cause of PH whenever patients with PPH whose symptoms do not progress rapidly.