Double-outlet right ventricle. Anatomic and angiocardiographic correlations.

A series of 72 patients with proved double-outlet right ventricle was studied with reference to the ability of angiocardiography to determine the location of a ventricular septal defect when it was associated with various great-artery relationships. Only 2 of the 72 patients had an intact ventricular septum. The other 70 patients had 16 possible anatomic variations based on four positions of the ventricular septal defect (subaortic, subpulmonary, subaortic and subpulmonary, and remote type) and four great-artery relationships (normal, side by side, dextromalposition, and levomalposition). Using the hemodynamic information regarding systemic and pulmonary arterial saturations, combined with biplane angiocardiographic data from the right ventricle (and if possible with left ventriculography), one can predict the location of ventricular septal defect. Because the different types of double-outlet right ventricle have different surgical approaches, this information can be important to the surgeon.

Double outlet right ventricle: hemodynamic and anatomic correlations.

There are 16 possible variations of double outlet ventricle with regard to interrelations of the great arteries and to location of the ventricular septal defect. In a series of 62 cases, approximately two thirds of patients had the great arteries in a side by side relation, and most (28 of 41) had the ventricular septal defect in a subaortic position. In double outlet right ventricle with malposition of the great arteries, the ventricular septal defect was either subpulmonary or subaortic. Four of the 13 patients with subpulmonary ventricular septal defect had a supracristal defect with side by side relation of the great arteries (Taussig-Bing anomaly), and 9 patients had malposition of the great arteries with an infracristal ventricular septal defect. In all patients with subpulmonary ventricular septal defect, pulmonary arterial oxygen saturation was greater than systemic arterial saturation regardless of the relation of the great arteries. Forty patients had subaortic ventricular septal defect. In 24 of these patients, including 7 with malposition of the great arteries, systemic arterial oxygen saturation was greater than pulmonary arterial saturation. However, in 9 patients (25 percent) the reverse was true, as seen in complete transposition of the great arteries and in Taussig-Bing anomaly. Thus, pulmonary arterial oxygen saturation greater than systemic arterial saturation is not reliable evidence of a Taussig-Bing anomaly. Of the 25 patients with such saturation, only 4 had the Taussig-Bing anomaly.

Double-outlet right ventricle associated with persistent common atriventricular canal.

A variant of double-outlet right ventricle (DORV) associated with common atrioventricular (A-V) canal was identified. As yet, this syndrome has not been successfully repaired. Of 16 patients with this diagnosis, 14 had the diagnosis confirmed at postmortem examination and two had the diagnosis confirmed by exploratory cardiotomy. The 16 patients were divided into two groups according to the position of the cardiac apex: group 1, ten patients with levocardia, and group 2, six patients with dextrocardia and ventricular inverson. When pulmonary stenosis was present, the clinical presentation was similar to that in tetralogy of Fallot, and when such stenosis was not, the presentation was similar to that in ventricular septal defect with bidirectional shunting. The electrocardiogram had the typical feature of common A-V canal. The right ventricular angiocardiogram had features typical of DORV. The left ventricular angiocardiogram revealed left ventricular outflow deformity typical of A-V canal in patients with levocardia but not in those with dextrocardia. Splenic anomalies were commonly seen (nine of the 16 patients). Asplenia was present in seven, and polysplenia in two. Ten patients had persistent left superior vena cava, seven had common atricum, six had anomalous pulmonary venous connection, and three had subaortic stenosis. Twelve of the 16 patients had pulmonary stenosis. Of the 16, 14 had common A-V canal (Rastelli type C) and two had a ventricular septal defect of the persistent common A-V type.