Eisenmenger syndrome

Editor-In-Chief: Prab R Tumpati, MD
Obesity, Sleep & Internal medicine
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Eisenmenger syndrome
Synonyms
Pronounce
Specialty	Cardiology
Symptoms	Cyanosis, dyspnea, fatigue, syncope, chest pain
Complications	Heart failure, stroke, arrhythmias
Onset	Usually in adulthood
Duration	Chronic
Types
Causes	Congenital heart defect with left-to-right shunt
Risks
Diagnosis	Echocardiography, cardiac catheterization
Differential diagnosis
Prevention	Early repair of congenital heart defects
Treatment	Oxygen therapy, phlebotomy, pulmonary vasodilators
Medication	Bosentan, sildenafil, epoprostenol
Prognosis	Variable, depends on severity and management
Frequency	Rare
Deaths

A complex heart condition resulting from long-standing heart defects

Eisenmenger syndrome is a medical condition that arises as a complication of certain congenital heart defects. It is characterized by the reversal of a left-to-right shunt to a right-to-left shunt due to the development of pulmonary hypertension. This condition leads to cyanosis, or a bluish discoloration of the skin, due to decreased oxygenation of the blood.

Pathophysiology

Eisenmenger syndrome develops in patients with congenital heart defects that initially cause a left-to-right shunt, such as a ventricular septal defect, atrial septal defect, or patent ductus arteriosus. Over time, the increased blood flow to the lungs causes damage to the pulmonary vasculature, leading to pulmonary hypertension. As the pressure in the pulmonary arteries rises, it eventually exceeds the systemic blood pressure, causing the shunt to reverse direction (right-to-left), resulting in deoxygenated blood entering the systemic circulation.

Clinical Features

Patients with Eisenmenger syndrome typically present with symptoms such as cyanosis, dyspnea (shortness of breath), and fatigue. The condition may also lead to complications such as hemoptysis (coughing up blood), arrhythmias, and heart failure. One of the characteristic physical findings in Eisenmenger syndrome is digital clubbing, which is the enlargement of the fingertips and toes.

Clubbing of the fingers, a common sign in Eisenmenger syndrome

Diagnosis

The diagnosis of Eisenmenger syndrome is based on clinical evaluation, imaging studies, and cardiac catheterization. Echocardiography is often used to assess the heart's structure and function, while chest X-ray and CT scan can provide additional information about the pulmonary vasculature. Cardiac catheterization is the definitive test to measure pulmonary artery pressures and confirm the presence of a right-to-left shunt.

Management

Management of Eisenmenger syndrome focuses on controlling symptoms and preventing complications. Patients are advised to avoid activities that can exacerbate symptoms, such as strenuous exercise and high altitudes. Medical therapy may include oxygen therapy, diuretics, and anticoagulants. In some cases, pulmonary vasodilators such as bosentan or sildenafil may be used to reduce pulmonary artery pressure. Heart-lung transplantation is considered in severe cases.

Prognosis

The prognosis of Eisenmenger syndrome varies depending on the severity of the condition and the presence of complications. With appropriate management, many patients can live into adulthood, although the condition is associated with a reduced life expectancy compared to the general population.

See also

• Congenital heart defect
• Pulmonary hypertension
• Cyanosis