Summary
Criss-cross heart is a rare congenital cardiac anomaly characterized by crossing of the inflow streams of the two ventricles due to an apparent twisting of the heart about its long axis. Hypoplasia of the tricuspid valve and right ventricle is a common feature, like pulmonary stenosis. The genetic or other causes and the developmental mechanisms remain unknown. The frequency of criss-cross heart is no more than 8 per 1,000,000. Most patients present as neonates with cyanosis and a systolic murmur. The diagnosis is readily made using 2-dimensional echocardiography. The diagnostic feature is crossing of the long axes of the atrioventricular valves as seen in a subxiphoid long-axis or coronal plane sweep. Treatment may include initial palliation with a systemic-to-pulmonary shunt to increase pulmonary blood flow and improve cyanosis. This is followed by staged progression toward completion of a Fontan-type operation in the majority. A few patients are candidates for a 2-ventricle repair. Antenatal diagnosis can be made using fetal echocardiography. *Author: Dr S. Sanders (February 2003)*.
