Echocardiographic evaluation of hearts with twisted atrioventricular connections (criss-cross heart).

An echocardiographic study was performed on seven patients with twisted atrioventricular connections (criss-cross heart) relative to the usual atrial arrangement. The atrioventricular connections were concordant in five patients and discordant in two. The ventriculoarterial junction was either double-outlet right ventricle (n = 6) or discordant connections (n = 1). Associated cardiac defects were ventricular septal defect (n = 7), pulmonary stenosis (n = 5), straddling tricuspid valve (n = 2), and straddling mitral valve (n = 1). Malalignment between the atrial and ventricular septum was the most important feature of this disease and resulted in altered spatial orientation of the trabecular ventricular septum, and recesses in the ventricles and the right atrium. When assessed by echocardiography, these anatomical features were not invariably recognized in every case, but they were dependent upon the type of atrioventricular connections and the presence of straddling of the atrioventricular valves. Left ventricular recess was the most consistent feature and was present in every patient, regardless of the atrioventricular connections. Right atrial recess was found in four of five patients with concordant atrioventricular connections but was not present in patients with discordant atrioventricular connections. Right ventricular recess was recognized as such in a single patient with concordant atrioventricular connection and straddling mitral valve. The trabecular septum was oriented in a horizontal plane in hearts with concordant atrioventricular connections and without atrioventricular valve straddling. The trabecular septum was oriented semivertically in all other hearts.(ABSTRACT TRUNCATED AT 250 WORDS)