Adaptation of the Systemic Right Ventricle in a Congenitally Corrected Transposition of the Great Arteries

A 50-year-old woman with a history of mild exertional dyspnea for several years was consulted for preoperative cardiovascular evaluation as a result of an abnormal ECG. Physical examination revealed a blood pressure of 154/90 mm Hg and a regular pulse rate of 84 beats per minute. Cardiac auscultation noted a grade 2/6 systolic murmur at the apex and the lower left sternal border. No peripheral cyanosis or clubbing of fingers was noted. Her resting ECG showed a sinus rhythm with a normal PR interval, left axis deviation, Q waves in the inferior leads, and left ventricular hypertrophy with ST-T change (Figure 1). Transthoracic echocardiographic segmental analysis of apical 4-chamber view revealed left atrium entered a morphological right ventricle and right atrium entered a morphological left ventricle (atrioventricular discordance; Movie I in the online-only Data Supplement). There was a moderate-to-severe tricuspid regurgitation from morphological right ventricle into left atrium (Movie II in the online-only Data Supplement). The long axis view showed the morphological left ventricle entering into a pulmonary artery with its left and right branches (Movie III in …