CASE REPORT Anomalies Associated With Congenitally Corrected Transposition of Great Arteries: Expect the Unexpected

Abstract

Objective: Congenitally corrected transposition of great arteries (CCTGA) is characterized by atrioventricular and ventriculoarterial discordance. Characterizations of these anomalies are important because they may influence surgical approach and management.

Methods: We present a case of newly diagnosed CCTGA at the age of 50. He presented with sudden onset of shortness of breath for the first time and was diagnosed with CCTGA. Echocardiogram, magnetic resonance imaging, and cardiac catheterization were utilized to elucidate the pathology.

Results: Intraoperatively, patient's CCTGA and ventricularization of the right ventricle were confirmed. The severe systemic atrioventricular valve regurgitation was replaced with a bioprosthetic valve (Medtronic Mosaic No. 29) with placement of epicardial ventricular leads for possible future placement of automatic implantable cardioverter defibrillators. Pathology report confirmed a degeneration of the systemic atrioventricular valve.

Conclusions: Significant coronary artery anomalies have also been described in literature with CCTGA. The variances encountered in this case are excellent examples of the intricacies associated in diagnosis and surgical care in patients with CCTGA.

Figure 1

Transesophageal echocardiogram showing hypertrophied SV and dilated LA. LA indicates left atrium; SV, systemic ventricle.

Figure 2

Transesophageal echocardiogram color Doppler showing severe systemic atrioventricular valve regurgitation.

Figure 3

MRI cine SSFP short axis showing a bileaflet valve (white arrows) with in a morphologic left ventricle. This ventricle is noted to be anterior and supports the pulmonary circulation. MRI indicates magnetic resonance imaging.

Figure 4

Double-oblique SSFP from the left side showing (1) infundibulum (circle), which conformed the right ventricular morphology. (2) White arrows show 2 of the 3 valve leaflets in the left-sided tricuspid valve. Ao indicates Aorta; LA, left atrium; PA, pulmonary artery; RV, right ventricle. White arrows indicate tricuspid leaflets and white oval indicate infundibulum.

Figure 5

Double-oblique SSFP showing no infundibulum and fibrous continuity of atrioventricular and ventriculoarterial valves consistent with left ventricular morphology. LV indicates left ventricle; PA, pulmonary artery; RA, right atrium. White oval indicates infundibulum asterisk (*) indicates liver.

Figure 6

MRI SSFP showing trileaflet valve between left atrium and morphological right ventricle. LV indicates left ventricle; MRI, magnetic resonance imaging. White arrows indicate trileaflet valve.

Figure 7

Coronary angiogram showing origins of left circumflex (LCx) and ramus intermedius from different ostia on left coronary cusp.

Figure 8

Schematic diagram showing anomalous origin in our case. RCA and LAD originating from an ostium on right coronary cusp, while Ramus Intermedius and LCx originating from separate ostia on left coronary cusp. LAD, left anterior descending; LCx, left circumflex; RCA, right coronary artery.

Figure 9

Coronary angiogram showing origin of right coronary artery, RV marginal, and LAD artery from right coronary cusp ostium. LAD indicates left anterior descending; RCA, right coronary artery; RV, right ventricle.