Congenitally Corrected Transposition of the Great Arteries Treated by Partial Systemic Ventriculectomy.

Abstract

42-year-old man was admitted to hospital with worsening dyspnea. At the age of 18, he underwent annuloplasty of the systemic AV valve for severe valvular regurgitation related to CCTGA at another hospital and did not have any problems on follow-up until age 33 when he visited hospital with paroxysmal nocturnal dyspnea. Because the systemic AV valve regurgitation had recurred to a severe degree and systemic ventricular contraction had decreased, he underwent systemic AV valve replacement and annuloplasty of the pulmonary AV valve. At age 39, atrial fibrillation with a rapid heart rate developed and could not be controlled with medications, so the His-bundle was cryoablated to block AV conduction and a permanent rate-adaptive pacemaker inserted intravenously. Although he was controlled in all pacemaker rhythm without tachycardia, his systemic ventricular function decreased and he was in New York Heart Association (NYHA) functional class 4 even after receiving digitalis, diuretics, angiotensin-converting enzyme inhibitor (ACEI) and other oral inotropic agents.His height was 185 cm and body weight was 75 kg. His blood pressure was 116/60 mmHg and the pulse rate was 60 beats/min and regular. Auscultation showed no significant murmur. Electrocardiograms revealed all pacemaker rhythm (VVI). Chest X-rays showed a cardiothoracic ratio of 58% and systemic atrial and ventricular enlargement. Echocardiography revealed enlargement of the systemic atrium and ventricle, and reduction of the systemic ventricular systolic function (the left atrial dimension was 4.7 cm, the end-diastolic dimension of the systemic ventricle was 8.6 cm and the fractional shortening was 14%) ( Table 1, Fig 1). Pulmonary wedge pressure and right-side pressures were elevated ( Table 2). The systemic ventriculogram showed diffused hypokinesis, but myocardial perfusion imaging using technetium-99m tetrofosmin indicated a severe perfusion defect in the lateral wall and the apex at rest.Because his symptoms could not be relieved by medical treatment, he was transferred to Shonan Kamakura General Hospital to undergo partial systemic ventriculectomy in February 1998. Under cardiopulmonary bypass (CPB) with heart beating and without aortic cross-clamping, the systemic ventricle was incised laterally from the apex to the base. There was neither mitral apparatus nor recognizable papillary muscle because of the previous valve replacement. Much of the anterolateral wall that was thin and akinetic on intraoperative palpation, and which corresponded to the defect on perfusion imaging, was excised in a tear-drop shape (7 cm wide and 12 cm long, 66 g in weight). Closure of the ventricle was done in 2 layers. The duration of CPB was 73 min and ventricular function recovered well without mechanical support. The resected myocardium had severe interstitial fibrosis (28.5%) and mild degeneration and hypertrophy of the myocardial cells.On echocardiography, the end-diastolic dimension of the systemic ventricle decreased to 5.7 cm, but the ejection fraction (EF) had not cha...

Prenatal Diagnosis of Congenitally Corrected Transposition of the Great Arteries

“…This can worsen with time and eventually lead to congestive heart failure. 6,7 The physiologic approach to treating symptomatic CCTGA is to correct the associated abnormalities such as tricuspid or pulmonic valve problems or a VSD. This classic approach leaves the right ventricle as the systemic ventricle.…”