Eisenmenger syndrome

Eisenmenger syndrome

Initial paper by Victor Eisenmenger was published in 1897 and was named: “Congenital defects of the ventricular septum.” The first description was of a thirty year old man who had features of Eisenmenger syndrome in 1894 who succumbed to massive hemoptysis later. Eisenmenger syndrome occurs as a consequence of large left to right shunt which results in severe pulmonary vascular disease and finally shunt reversal. Initially the pulmonary vascular changes are reversible if the shunt is closed. The pulmonary vascular changes include medial hypertrophy and intimal proliferation. As the pulmonary vascular disease progresses, irreversible changes like plexiform lesions and necrotizing arteritis develop. When the pulmonary vascular resistance exceeds the systemic vascular resistance, reversal of shunt occurs, causing systemic desaturation and cyanosis. This will be associated with exertional dyspnea and impaired exercise tolerance in most persons. Palpitations can occur in more than half the of patients, of which forty percent can have atrial flutter or fibrillation while one tenth can have ventricular tachycardia. Hemoptysis is common and can occur in about one fifth of cases. Anginal type of chest pain and syncope can occur due to the pulmonary hypertension and right ventricular hypertrophy. Endocarditis is also rare complication.

Clinical signs of pulmonary hypertension are remarkable in Eisenmenger syndrome and include a left parasternal right ventricular heave, palpable second heart sound (pulmonary component) and sometimes right sided S3. Pulmonary ejection click (vascular, non phasic) and a soft ejection systolic murmur can be heard due to the dilated pulmonary trunk. High pitched decrescendo diastolic murmur of pulmonary regurgitation (Graham-Steell murmur) can also be heard in many. Peripheral edema can be noted with onset of right heart failure, which is usually quite late in the natural history of Eisenmenger syndrome.

ECG may show right atrial enlargement, right axis deviation and right ventricular hypertrophy. Atrial arrhythmias may also be noted. Chest x-ray shows prominent central pulmonary arteries and decreased peripheral lung vasculature (peripheral pruning). Large end on vessels may also be seen (five or more).

There is a large variation in the survival of adults with Eisenmenger syndrome. Survival rates have been reported as 80% at 10 years, 77% at 15 years, 42% at 25 years. Independent predictors of mortality described have been the age at presentation, supraventricular arrhythmias and poor NYHA (New York Heart Association) functional class (III or IV).

A maternal mortality of about fifty percent and fetal loss of about sixty percent makes pregnancy a formidable option in Eisenmenger syndrome. Cerebrovascular accidents and cerebral abscesses due to paradoxical embolism are a feature of Eisenmenger physiology. Volume depletion predisposes to thrombosis in the polycythemic state and has to be avoided. Heavy exertion and exposure to high altitudes (due to low oxygen concentration in atmospheric air) are to be avoided. Intravenous epoprostenol may be beneficial in decreasing the high pulmonary vascular resistance. Venesection with isovolumic replacement is recommended for patients with moderate to severe symptoms of hyperviscosity and a packed cell volume above sixty five percent. Prevention of iron deficiency is important as microcytosis which occurs in the context of iron deficiency leads to renal ischemia, further rise in erythropoietin secretion and decompensated erythrocytosis as the microcytes are less deformable and hence less able to negotiate the renal microcirculation. Supplemental oxygen can reduce episodes of dyspnea and help in reducing the elevated pulmonary vascular resistance. Single lung transplantation with repair of cardiac defect or heart/lung transplantation are the final options in Eisenmenger syndrome, though with its on problems.

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