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Eisenmenger syndrome
Last reviewed: 23.04.2024
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Eisenmenger syndrome is a complication of uncorrected heart defects, in which there is a discharge of blood from left to right. Often over time, the increase in vascular resistance in the lungs, causing a change in the direction of discharge to the right. Non-oxygenated blood enters a large circle of blood circulation, leading to the appearance of symptoms of hypoxia. Auscultative data depend on the nature of the primary defect.
Diagnosis is based on echocardiography or cardiac catheterization. Treatment of Eisenmenger syndrome is generally supportive, however, heart and lung transplantation can be a method of choice if the manifestations are severe. It is recommended to prevent endocarditis.
What causes Eisenmenger syndrome?
Congenital heart defects, which lead to Eisenmenger syndrome in the absence of treatment, include an interventricular septal defect, atrioventricular communication, an atrial septal defect, a common arterial trunk, and a transposition of the main vessels. In the US, its prevalence has significantly decreased due to early diagnosis and radical treatment of primary malformation.
Rightful discharge of blood in the Eisenmenger syndrome leads to cyanosis and its complications. Reduction of saturation of arterial blood in a large circle of blood circulation leads to the formation of fingers and toes in the form of tympanic sticks, secondary polycythemia, increased viscosity and the consequences of increased erythrocyte decay (eg, hyperuricemia, causing gout, hyperbilirubinemia, causing cholelithiasis, iron deficiency with or without anemia).
Symptoms of Eisenmenger syndrome
Symptoms of Eisenmenger syndrome usually do not develop until the age of 20-40 years; they include cyanosis, syncopal conditions, dyspnoea with physical exertion, weakness, swelling of the jugular veins). Hemoptysis is a late symptom. Signs of the phenomenon of embolism of cerebral vessels or endocarditis may develop.
Secondary polycythemia often manifests clinically (for example, accelerated speech, vision problems, headache, fatigue or signs of thromboembolic disorders). As a result, cholelithiasis can be troubled by abdominal pain.
In the physical examination, central cyanosis is detected, fingers in the form of drumsticks. Seldom there may be signs of a lack of right ventricle. The holosystolic regurgitation noise on the tricuspid valve can be heard in the 3rd-4th intercostal space to the left of the sternum. Early diastolic diminishing pulmonary artery failure may be heard along the left edge of the sternum. Loud single tone II is a constant change; a click of exile is often heard. Scoliosis is present in about 1/3 of the patients.
Diagnosis of Eisenmenger syndrome
Eisenmenger's syndrome is suspected from a history of unoperated heart disease, taking into account the radiography of the chest and ECG, an accurate diagnosis is established based on two-dimensional echocardiography with color Dopplercardiography.
Laboratory examination reveals polycythemia with a hematocrit above 55%. Increased decay of erythrocytes can manifest as an iron deficiency state (eg, microcythemia), hyperuricemia and hyperbilirubinemia.
On the roentgenogram, it is usually noted protruding central pulmonary arteries, shortening of the peripheral pulmonary arteries, an increase in the right heart. ECG shows right ventricular hypertrophy and sometimes right atrial hypertrophy.
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Treatment of Eisenmenger syndrome
Ideally, corrective operations should have been performed earlier in order to prevent the development of Eisenmenger syndrome. Specific treatment does not exist when the syndrome has already developed, however, drugs that can reduce the pressure in the pulmonary artery are being studied. They include prostacyclin antagonists (treprostinil, epoprostenol), endothelin antagonists (bosentan), and nitric oxide enhancers (sildenafil).
Supportive treatment for Eisenmenger syndrome includes avoiding conditions that can trigger deterioration (eg, pregnancy, fluid restriction, isometric loading, high altitude exposure), and the use of oxygen therapy. Polycythemia can be treated with cautious phlebotomy to reduce the hematocrit to 50-60%, plus simultaneous volume replacement with physiological saline. Hyperuricemia can be treated with allopurinol 300 mg orally once a day. Aspirin 81 mg orally once a day is indicated for the prophylaxis of thrombosis.
The expected life expectancy depends on the type and severity of the primary heart defect and varies from 20 to 50 years; the average age at which the patient dies is 37 years. However, low tolerance of physical exertion and secondary complications can significantly limit the quality of life.
Transplantation of the heart and lungs can be a method of choice, but 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 in which bacteraemia may develop.