Eisenmenger syndrome refers to any untreated congenital cardiac defect with intracardiac communication that leads to pulmonary hypertension, reversal of flow, and cyanosis. Lesions in Eisenmenger syndrome, such as large septal defects, are characterized by high pulmonary pressure and/or a high pulmonary flow state. Eisenmenger syndrome occurs in patients with large, congenital cardiac or surgically created extracardiac left-to-right shunts. These shunts initially cause increased pulmonary blood flow.
The symptoms of the disease are as follows:
Diagnosis and Treatment
The doctor may note a change or increase in intensity of a cardiac murmur (a blowing or rasping sound heard while listening to the heart that may or may not indicate problems within the heart or circulatory system) during a routine physical examination. Blood test , chest x-rays Electrocardiogram etc are performed. Medical treatment for Eisenmenger syndrome is based on symptoms. Phlebotomy (removal of blood) should not be done frequently, but may be recommended when erythrocytosis causes hyperviscosity (extreme thickness) of the blood.
Eisenmenger syndrome usually develops before puberty but may develop in adolescence and early adulthood. Tolerance and prognosis are better than with idiopathic pulmonary hypertension. However, patients follow-up is often performed in inappropriate non-specialized care centres which makes the collection of epidemiological data difficult.