Objective: Superior-inferior ventricles are characterized by abnormal relationship of the ventricles, in which the ventricles lye one above the other instead of side-by-side. Consequently the interventricular septum is more or less horizontal. The anomaly, that is almost always associated with complex cardiac abnormalities, is very rarely seen and little information exists about it. We aimed to investigate the feasibility of echocardiohraphy in the diagnosis of this complex malformation.
Methods: Patients with superior-inferior ventricles were assessed by segmental and sequential echocardiography study.
Results: 12 patients aged 4 months to 12 years were studied. Two of the patients had in utero diagnosis. Eight patients had usual atrial arrangement (2 of them have isolated dextrocardia), 3 had mirror-imaged arrangement and 1 had left atrial isomerism. Two of the patients had isolated levocardia. Seven patients had discordant atrioventricular connections, and 5 had discordant ventriculoarterial connections. Criss-cross hearts were present in 5 patients. Frequently associated anomalies included ventricular septal defect (10 patients), pulmonary outflow tract stenosis or atresia (7 patients),and double outlet right ventricle (5 patients). One patient had severe left ventricular outflow tract obstruction.
Conclusion: Superior-inferior ventricles are rare anomalies associated with complex cardiac malformations. Correct diagnosis is feasible with echocardiographic study.