Pulmonary arterial hypertension in adults with congenital heart disease: The Eisenmenger syndrome

Despite the early surgical repair of congenital cardiac defects in most institutions of developed countries, advanced pulmonary vasculopathy associated with unrepaired shunts remains a major problem, particularly in underserved areas of developing nations. The so called Eisenmenger syndrome (reversed right-to-left shunting caused by heightened pulmonary vascular resistance, sometimes above the systemic level) is a multisystemic disorder associated with progressive right cardiac failure and hypoxemia. Management of this disorder is far beyond the simple administration of vasodilators. Rather, in the era of the "new drugs" for treatment of pulmonary hypertension, general therapeutic measures remain central in patient care. These include monitoring of blood viscosity and prevention of hyperviscosity states, prophylaxis against pulmonary and systemic infections and thrombotic events, monitoring of renal function and systemic blood pressure, contraception in females, and management of specific cardiac complications such as myocardial ischemia and arrhythmias.