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Eisenmenger's syndrome
Last reviewed: 04.07.2025
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Eisenmenger syndrome is a complication of uncorrected heart defects in which blood is shunted from left to right. Often, over time, pulmonary vascular resistance increases, causing a change in the direction of shunting to the right-left. Unoxygenated blood enters the systemic circulation, causing symptoms of hypoxia. Auscultatory data depend on the nature of the primary defect.
Diagnosis is by echocardiography or cardiac catheterization. Treatment of Eisenmenger syndrome is generally supportive, but heart and lung transplantation may be the treatment of choice if manifestations are severe. Endocarditis prophylaxis is recommended.
What causes Eisenmenger syndrome?
Congenital heart defects that lead to Eisenmenger syndrome if untreated include ventricular septal defect, atrioventricular communication, atrial septal defect, truncus arteriosus, and transposition of the great vessels. Its prevalence has decreased significantly in the United States due to early diagnosis and radical treatment of the primary defect.
Right-left shunt in Eisenmenger syndrome results in cyanosis and its complications. Decreased arterial saturation in the systemic circulation leads to clubbing of the fingers and toes, secondary polycythemia, increased viscosity, and sequelae of increased red blood cell breakdown (e.g., hyperuricemia causing gout, hyperbilirubinemia causing cholelithiasis, iron deficiency with or without anemia).
Symptoms of Eisenmenger's syndrome
Symptoms of Eisenmenger syndrome usually do not develop until the age of 20-40 years; they include cyanosis, syncope, dyspnea on exertion, weakness, and jugular venous distension. Hemoptysis is a late symptom. Signs of cerebral embolism or endocarditis may develop.
Secondary polycythemia often has clinical manifestations (eg, rapid speech, vision problems, headache, fatigue, or signs of thromboembolic disorders). Abdominal pain may be a concern as a result of cholelithiasis.
Physical examination reveals central cyanosis and clubbing of the fingers. Rarely, signs of right ventricular failure may be present. A holosystolic murmur of tricuspid regurgitation may be heard in the third or fourth intercostal space to the left of the sternum. An early diastolic decrescendo murmur of pulmonary insufficiency may be heard along the left sternal border. A loud, single second heart sound is a constant change; an ejection click is often heard. Scoliosis is present in about one-third of patients.
Diagnosis of Eisenmenger syndrome
Eisenmenger syndrome is suspected based on a history of unoperated heart defect, taking into account chest X-ray and ECG, and a precise diagnosis is established based on two-dimensional echocardiography with color Doppler.
Laboratory examination reveals polycythemia with a hematocrit greater than 55%. Increased red blood cell breakdown may manifest as an iron deficiency state (eg, microcythemia), hyperuricemia, and hyperbilirubinemia.
The radiograph usually shows prominent central pulmonary arteries, shortening of the peripheral pulmonary arteries, and enlargement of the right heart chambers. The ECG shows hypertrophy of the right ventricle and sometimes hypertrophy of the right atrium.
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Treatment of Eisenmenger's syndrome
Ideally, corrective surgery should have been performed earlier to prevent Eisenmenger syndrome from developing. There is no specific treatment once the syndrome has developed, but drugs that may lower pulmonary artery pressure are being studied. These include prostacyclin antagonists (treprostinil, epoprostenol), endothelin antagonists (bosentan), and nitric oxide enhancers (sildenafil).
Supportive treatment of Eisenmenger syndrome includes avoidance of conditions that may worsen the condition (eg, pregnancy, fluid restriction, isometric exercise, high altitude) and use of oxygen therapy. Polycythemia can be treated with gentle phlebotomy to reduce the hematocrit to 50% to 60%, plus simultaneous volume replacement with normal saline. Hyperuricemia can be treated with allopurinol 300 mg orally once daily. Aspirin 81 mg orally once daily is indicated for thromboprophylaxis.
Life expectancy depends on the type and severity of the primary heart defect and ranges from 20 to 50 years; the average age at death is 37 years. However, low exercise tolerance and secondary complications can significantly limit quality of life.
Heart and lung transplantation may be the treatment of choice, but is reserved for patients with severe manifestations. Long-term survival after transplantation is not promising.
All patients who have Eisenmenger syndrome should receive endocarditis prophylaxis before dental or surgical procedures that may cause bacteremia.