Deciphering the Mysteries of Crisscross Heart by Transthoracic Echocardiography

BACKGROUND: Accurate diagnosis of crisscross heart and its associated anomalies is important but problematic for cardiologists. This study aimed at identifying unique transthoracic echocardiographic features and common associated lesions of this complex condition. METHOD: Clinical and echocardiographic features of 10 patients with crisscross anatomy were studied. Echocardiographic findings were verified by cardiac magnetic resonance imaging or surgical inspection. RESULTS: Crisscross anatomy (10 patients, age at diagnosis ranged from 1 month to 25 years, five female) was identified in 0.076% of patients with congenital heart diseases from 1985 to 2006. All patients had cyanosis and 80% of them were underweight. Superior-inferior ventricles (SIV) and crossed ventricular inflow streams were seen in 90% and 100% of patients, respectively. All patients had abnormal ventriculo-arterial (VA) connections: five with transposition of great artery (L-type: n = 2; D-type: n = 3) and five with double outlet right ventricle. Commonly associated anomalies included ventricular septal defects (100%), right ventricular outflow tract obstruction (60%), atrioventricular valves straddling or overriding (50%), atrial septal defect (40%), and right ventricular hypoplasia (30%). Seven patients received cardiac surgery for the relief of cyanosis. CONCLUSIONS: SIV and crossed inflow streams are important diagnostic features for crisscross heart by transthoracic echocardiogram. The hemodynamic consequences of abnormal VA connections and associated defects impact surgical management.