2023 年 53 巻 3 号 p. 99-102
In 1978, we reported a case of severe primary pulmonary hypertension (PH) in a 14-year-old girl. Initial cardiac catheterization revealed severe PH and pulmonary arterial pressure (Ppa) of 145/65 (100) mmHg. Twenty-four-hour monitoring revealed a decrease in Ppa with administration of supplemental oxygen and alpha-blockade. After that, she was treated with vasodilating drugs including alpha-blockade and home oxygen therapy. She survived for 45 years with only mild exertional dyspnea, except during her last few years. At 1 month before death, her Ppa was 160/44 (82). A chest X-ray revealed almost the same cardiac configuration as that observed on her initial visit. This is the longest survival seen in a patient with idiopathic pulmonary arterial hypertension and an extremely high Ppa.
