Congenital or acquired cardiac malformations associated with high pulmonary flow or pressure should be corrected at an early stage to reverse or avoid remodeling of pulmonary vessels. In some of these patients, as well as in others suffering from severe idiopathic pulmonary hypertension, heart-lung or lung transplantation often represents the only therapeutic option. In the interim period, these groups of patients should be treated with vasodilators since right heart failure is the predominant cause of death. Whereas intravenously infused vasodilators used to be the only pharmacological option available to treat chronic and acute pulmonary hypertension, inhaled NO and aerosolized epoprostenol may now be regarded as promising alternatives, particularly in the treatment of acute pulmonary hypertension.

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