Primary pulmonary hypertension in brother and sister Nariaki Kanemoto 1 , Chiemi Imaoka 1 , Yoshihiro Hayashi 1 , Yuichiro Goto 1 , Shigeo Ubukata 2 , Yoshimichi Hojo 2 1Tokai University, School of Med. Department of Internal Medidne 2Gunma Prefectural Maebashi Hospital, Internal Medicine pp.905-909
Published Date 1983/8/15
DOI https://doi.org/10.11477/mf.1404204286
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The patients with primary pulmonary hyper-tension (PPH) noted in brother and sister were reported. Patient 1 is a 26 year old woman who spent daily usual life until three years previously when she initially noted dyspnea on exertion. At 26 year old, cyanosis of the nail beds and edema of lower legs developed after delivery. Although she was diagnosed as PPH in another hospital and treated properly, she was reffered to this hospital for further evaluation and therapy. Right heart catheterization revealed pulmonary artery pressure of 77/33 (mean 46) mmHg, pulmonary artery capillary wedge pressure of 11 mmHg.

Patient 2 is an older brother (24 year old man) of patient 1. He enjoyed good health until 7 months before entry when he initially noted dyspnea on exertion. He developed right heart failure and died 7 months after he was diagnosed as PPH. Pulmonary artery pressure was 80/36 (mean 50) mmHg.

In both patients, electrocardiogram showed right axis deviation and right ventricular hyper-trophy. Chest roentgenogram showed prominence of the central pulmonary artery trunk and decreased peripheral pulmonary vasculature. Neither patient showed immunological ab-normalities.

In patient 1, the combination therapy of daily dosage of prazosin 4 mg and isosorbide dinitrate 20 mg qid was effective : pulmonary artery pressure decreased from 75/27 (42) to 56/20 (34) mmHg and total pulmonary vascular resistance from 688 to 476 dyne sec cm -5 immediately before and 3 months after the therapy without any adverse effects.

Copyright © 1983, Igaku-Shoin Ltd. All rights reserved.


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