急激な進行をみた原発性肺高血圧症の1女子例

A case of rapidly progressive primary pulmonary hypertension in a 14-year-old girl

　原発性肺高血圧症（Primary Pulmonary Hyperten-sion：以下PPHと略す）は，肺実質あるいは心臓に明らかな基礎疾患が証明されずに前毛細管性の著しい肺高血圧をきたす原因不明の疾患と定義され，予後は極めて不良である。症例は蛋白尿，浮腫にて発症し，特徴的な所見を欠くため初期診断に苦慮し，症状の出現より8カ月という急激な経過にて死亡した。本稿ではその臨床所見，経過，剖検所見について報告する。

A 14-year-old girl was admitted with chief com-plaints of edema and chest pain. She had hepato-megaly, but did not have heart murmur and accen-tuation of the pulmonary component of the second heart sound. The electrocardiogram showed right axis deviation, negative T wave in V_3,4 and ST depression in II aVF. But right ventricular hyper-trophy was not dominant. Chest radiography show-ed a cardiothoracic ratio of 54% and a slight pro-minence of proximal pulmonary arteries.

The edema was soon diminished only by the diu-retics, but it appeared again without the diuretics. At the cardiac catheterization 3 months after the onset of symptoms, the pulmonary arterial pressure was 150/85 mmHg and the pulmonary resistance was 3,232 dyn/sec/cm⁵. The right atrial pressure was 9.5 mmHg and oxygen saturation at the pulmonary artery was 31.0%. Prostaglandin E1 reduced the pulmonary artery pressure only a little, but raised the systemic pressure. The patient was treated with several vasodilaters, but her condition deteriorated rapidly and she developed severe right ventricular failure. She died only 8 months after the onset of symptoms and 5 months after the catheterization.

At autopsy, histological examination demonstrated intimal fibrotic thickening of the small-sized pulmo-nary arteries and organizing thrombus. But there was not plexiform lesion.

Heart failure was easily improved when she was first admitted. But after 3 months the cardiac cathe-terization revealed that her condition was already severe. Several vasodilators was not effective to such a rapidly progressive primary pulmonary hyper-tension.