Familial Primary Pulmonary Hypertension. Report of Two Siblings

Koji KODAMA, Mareomi HAMADA, Yuji SHIGEMATSU, Makoto SUZUKI, Kunio HIWADA, Yutaka HAYASHI, Hiroyuki HASHIMOTO, Haruhisa HASHIMOTO, Atsuro SUGITA
1991 Japanese Journal of Medicine  
Familial primary pulmonary hypertension was found in two siblings (sister and brother). The woman noted exertional dyspnea at the age of 28 yr. The younger brother noticed exertional dyspnea, cough with bloody sputum and pretibial edema at the age of 38 yr. Wediagnosed them as primary pulmonary hypertension as based on hemodynamicand histopathological findings. Regardless of the treatment, both patients died of right-sided heart failure with a short time course. Weexamined their family members,
more » ... but there were no other members with primary pulmonary hypertension.
doi:10.2169/internalmedicine1962.30.273 fatcat:y6la2qu3xrelrhuyhb5ufnsjze