Abstract

Primary pulmonary hypertension (PPH) is an uncommon fatal disease. Although this is usually a nonfamilial disease, there are some cases of familial occurrence. We reported two families of PPH ; elder brother and younger sister in one family and elder sister and younger brother in the other. The former siblings died and postmortem examinations revealed plexiform lesion in the younger sister and obliteration of small pulmonary artery by medial hypertrophy in the elder brother.

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