Primary pulmonary hypertension. An analysis of 28 cases and a review of the literature.

The diverse potential etiologies and varying degrees of severity at the time of presentation suggest that primary pulmonary hypertension is a spectrum of disease states, all of which share the hemodynamic and pathologic correlates of pulmonary hypertension without a demonstrable cause. The course is variable, but manifestations of a low cardiac output or right ventricular failure imply a poor prognosis; the median survival is 2 to 3 years from the time of diagnosis. Although several systemic vasodilators have been demonstrated to improve pulmonary hemodynamics and ameliorate symptoms in some cases, these drugs may produce undesirable adverse effects, including death, in unresponsive patients. Additionally, the impact of vasodilator therapy on mortality from PPH has not been assessed. Combined heart-lung transplantation should be considered in patients who are unresponsive to vasodilators and who manifest the indicators of a poor prognosis.