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

Ayaka Kobashi, Michihiro Suwa, Tomomi Nakamura, Takahide Ito, Taiko Horii, Tadashi Isomura, Hisayoshi Suma
2003 Circulation Journal  
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
more » ... loplasty 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 changed at 1 month after the operation (Table 1, Fig 1) . A thickened pericardium and pericardial effusion were noted behind the systemic ventricle after the operation. On contrast-enhanced electron-beam computed tomograms, the end-diastolic volume of the systemic ventricle was decreased from 213 ml to 180 ml after systemic ventriculectomy, but stroke volume was not reduced (38 ml to 41 ml) and the EF increased from 18% to 23% (Table 2) . On cardiac catheterization performed 2 months after the operation, pulmonary wedge pressure and right-side pressures were decreased, but cardiac output (CO) was somewhat reduced (Table 2) . Nine months after the operation, CO returned to the preoperative level (Table 2 ) and the EF had increased from 15% to 38% on radionuclide angiography (Table 3) , but pulmonary wedge pressure and rightside pressures were re-elevated ( Table 2) . The patient's symptoms had clearly improved after the Congenitally corrected transposition of the great arteries (CCTGA) is a rare congenital heart disease characterized by atrioventricular (AV) and ventriculo-arterial discordance; 1 that is, the left ventricle supports the pulmonary circulation and the right ventricle supports the systemic circulation. The most common cardiac anomalies in CCTGA include ventricular septal defect, pulmonary outflow tract obstruction and abnormalities of the systemic AV valve. 1 The dysfunction of the systemic ventricle occurs with increasing frequency in older patients with CCTGA, independent of their commonly associated structural defects. [2] [3] [4] We report a patient with CCTGA undergone the partial ventriculectomy for his systemic ventricular dysfunction. (Circ J 2003; 67: 354 -356)
doi:10.1253/circj.67.354 pmid:12655168 fatcat:uwuobbyifbevxpc4ud232vw25u