Corrected Transposition of the Great Vessels, Atrioventricular Heart Block, and Ventricular Septal Defect

Abstract

Three cases with the triad of ventricular septal defect, atrioventricular heart block, and corrected transposition of the great vessels are presented. In each instance the patient successfully underwent open heart surgery on the pump-oxygenator for closure of the ventricular septal defect. A review of the literature suggests a frequent association of these 3 conoenital anomalies. In our experience atrioventricular heart block is rarely encountered with simple ventricular septal defect. The finding of atrioventricular heart block in a patient with an interventricular septal defect warrants consideration of an associated corrected transposition of the great vessels.CORRECTED transposition of the great vessels is a rare anomaly in which the root of the aorta arises anterior to the pulmonary artery, yet each major vessel receives blood from its "correct" ventricle, (the aorta from the left ventricle, the lpulmonary artery from the right). As illustrated in figure 1, the main pulmonary artery fails to arch in front of the aorta in the iiormnal manner, and the left pulmonary artery passes behind the ascending aorta. Figure 2 illustrates the medial displacement of the cardiac catheter as it passes through the main pulmonary artery in corrected transposition. In complete transposition of the great vessels the position of the major trunks is similarly altered but they originate from reversed ventricles, the aorta from the right, and the pulmonary artery from the left ventricle.We are reporting 3 cases that represent combinations of defects that have been reported previously but whose association has not been stressed, namely "corrected tramisposition" of the aorta and pulmonary ar-

Congenital Heart Block

“…The almost invariable presence of heart malformations in children who die with heart block indicates that it is the malformations, not the dysrhythmia, which cause death. Walker et al (1958) report three children with heart block, V.S.D. and corrected transposition of the great vessels.…”

“…The second child was cyanosed only for the first 2 weeks of life and no murmur was heard. Walker et al (1958) report three children with heart block, V.S.D. and corrected transposition of the great vessels.…”

“…Of particular interest is the association of heart block, ventricular septal defect and corrected transposition of the great vessels. Walker, Cooley, McNamara and Moser (1958) operated on 69 children for closure of V.S.D. Three of them had associated corrected transposition and these all showed heart block.…”

The Diagnosis of Corrected Transposition of the Great Vessels

“…Walmsley (1931) and Abbott (1936) reported complete heart block and subsequently this has been observed by Anderson et al (1957), Schaefer andRudolph (1957), Fink et al (1958), Walker et al (1958), Kjellberg et al (1959), Malers et al (1960), Badawi et al (1961), and Lev and Rowlatt (1961), and in 6 out of 29 patients in the Mayo Clinic series (Schiebler et al, 1961). 2:1 A-V block has been reported by Cardell (1956) and by Schiebler et al (1961) in 4 patients.…”

“…Lengthening of the P-R interval beyond the normal for age and rate is even more common: this occurred in 11 of Schiebler's patients (1961), in 10 of the 17 reported by Anderson et al (1957), and in patients reported by Gasul et al (1959), Kjellberg et al (1959), Badawi et al (1961), and Beck et al (1961). Walker et al (1958) had a patient with partial heart block and intermittent dropped beats. The term complete A-V dissociation is sometimes used loosely to include complete heart block but this arrhythmia in the more limited sense of the term was present in two patients (Anderson et al, 1957).…”