Key result
Familial primary pulmonary hypertension causes rapidly fatal right-sided heart failure in affected siblings despite treatment.
Case Report (n=2)
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. We diagnosed them as primary pulmonary hypertension as based on hemodynamic and histopathological findings. Regardless of the treatment, both patients died of right-sided heart failure with a short time course. We examined their family members, but there were no other members with primary pulmonary hypertension.
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Kodama et al. (1991) conducted a case report in Familial primary pulmonary hypertension (n=2). Familial primary pulmonary hypertension was evaluated on Right-sided heart failure and death. Familial primary pulmonary hypertension in two siblings resulted in rapid fatal right-sided heart failure in both cases despite treatment, with no other family members found to be affected.
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