Congenital Heart Disease

Eisenmenger's Syndrome

Signs and Symptoms
Diagnosis
Treatment

Signs and Symptoms

Eisenmenger's syndrome, which is named after the doctor who first described it, is a combination of two conditions. First, it involves having a communication, or hole, between two cardiac chambers that allows oxygenated blood to recirculate back into the right ventricle and to the lungs instead of flowing out of the left ventricle to the rest of the body. Over time, this extra blood flow to the lungs damages their vessels, causing high pressures or pulmonary hypertension that reverses the flow of blood, so the deoxygenated blood goes out to the rest of the body. Eisenmenger's syndrome refers to this combination of reversed blood flow with pulmonary hypertension. It results in cyanosis or low oxygen content in the blood and may eventually result in failure of the right ventricle. However, many patients live well into adulthood with the proper care.

The hole may be a ventricular septal defect, an atrial septal defect or a patent ductus arteriosus. A baby born with a single ventricle also may develop this condition.

Signs and symptoms of Eisenmenger's syndrome include:

• Cyanosis, a blue tinge to the skin resulting from lack of oxygen
• High red blood cell count
• Swollen or clubbed finger tips
• Fainting, called syncope
• Heart failure
• Arrhythmia or irregular heart rhythms
• Bleeding disorders
• Coughing up blood
• Iron deficiency
• Kidney problems
• Stroke
• Gout
• Gallstones

Reviewed by health care specialists at UCSF Medical Center.
Last updated December 18, 2008