Eisenmenger syndrome

[Marquetta]

Eisenmenger syndrome is a complication of uncorrected large intracardiac left-to-right shunts. Increased pulmonary resistance may develop over time, eventually leading to bidirectional shunting and then to right-to-left shunting. Deoxygenated blood enters the systemic circulation, causing symptoms of hypoxia. Murmurs and heart sounds depend on the underlying anomaly. Diagnosis is by echocardiography or cardiac catheterization. Treatment is generally supportive, but heart and lung transplantation may be an option when symptoms are severe. Endocarditis prophylaxis is recommended.
(See also Overview of Congenital Cardiovascular Anomalies.)
Congenital heart anomalies that, if untreated, may result in Eisenmenger syndrome include

Ventricular septal defect (VSD)
Atrioventricular septal defect (AVSD)
Atrial septal defect (ASD)
Patent ductus arteriosus (PDA)
Truncus arteriosus
Aortopulmonary window
Partial anomalous venous return (PAPVR, whereby some of the pulmonary veins connect to the right atrium instead of the left atrium)

Rare patients with single ventricle variants or with transposition of the great arteries with a persistent patent ductus arteriosus and/or ventricular septal defect may develop Eisenmenger syndrome if untreated, though the vast majority of these patients die early in infancy. In the US, the incidence has markedly decreased because of early diagnosis and definitive repair of the causative anomaly.
Right-to-left shunting due to Eisenmenger syndrome results in cyanosis and its complications. Systemic oxygen desaturation leads to clubbing of fingers and toes, secondary polycythemia, hyperviscosity, hemoptysis, CNS events (eg, brain abscess or stroke), and sequelae of increased RBC turnover (eg, hyperuricemia causing gout, hyperbilirubinemia causing cholelithiasis, iron deficiency with or without anemia).

[Lizzy Liz]

a heart problem present at birth that affects the blood flow from the heart to the lungs: