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[Tricuspid atresia with hypoplasia of aortic isthmus in absence of transposition of great vessels (author's transl)].

A case of tricuspid atresia with a concordant ventriculoarterial connection associated with hypoplasia of the aortic isthmus and malformation of left atrioventricular valve is presented. Hyoplasia of aortic isthmus in absence of transposition of great vessels was explained by a posterior deviation of infundibular septum that reduced subaortic outflow tract and created an ample bulboventricular orifice. In the left atrioventricular valve an anterolateral papillary muscle that was prolonged in a thick chorda tendineaee fused to ventricular face of the cusp up to its insertion in the atrioventricular ring was observed. A systolic murmur of high tonality compatible with regurgitation of the left atrioventricular valve and a notch that was registered in postmortem angiography were attributed to this anomalous chorda tendinea. In the posterior wall of the heart there were two descending coronary arteries. One descended fro the crux cordis; the other, to its right, adjacent ot the acute margin, followed the insertion of the trabecular septum, delimited the outlet chamber, and was anastomosed to the anterior descending coronary artery.

Aortic Coarctation↗

[Development of surgical treatment of transposition of great vessels in the last 15 years. Methods and early results].

Between 1978 and June 1992, 213 patients were operated for transposition of the great arteries at our institution. 148 patients underwent atrial repair, 41 patients an anatomic repair and 24 patients only palliation. Since 1986 the anatomic repair has progressively replaced the arterial repair: 1978-1986 95-100% atrial repair, 1992 100% anatomic repair. At operation the average age of the patients treated by atrial repair was 22 months, resp. 10 months from 1986 to 1991, for anatomic repair 9 months, resp. 46 days in 1991/1992. The 30-day mortality for atrial repair was 11% (17/148), for anatomic repair 22% (9/41), resp. 12% (3/24) in 1991/1992. Potentially superior long-term results for anatomic repair are possible, but cannot be demonstrated at present.

Child↗

[Intensive care for newborns and infants after anatomic correction of transposition of great vessels].

A retrospective study was made in 48 babies undergone an arterial switching operation at the Bakulev Research Center of Cardiovascular Surgery in January 2004 to September 2005. Their age at surgery was 5 days to 11 months; body weight, 3.9 +/- 1.1 kg (2.4-8.7 kg). According to the course of a postoperative period, the patients were divided into 2 groups: 1) 28 (58.3%) neonates with simple transposition of great vessels (TGV) and older babies (above 3 weeks of life) with simple TGV; 2) 20 (41.6%) patients with TGV + ventricular septal defect. The postoperative management of all the patients was based on the delayed bringing the sternum together in babies at risk for close mediastinum syndrome, on the reduction in left and right ventricular afterload with the mean blood pressure (BPmean) minimally sufficient to maintain adequate coronary blood flow and diuresis, by using inodilatators and nitric oxide (II) and by limiting the volume of administered fluid. The sternum was primarily brought together in 20 (41.6%) patients, in 13 (27.1%) on day 2. Sternal separation was required in 1 patient from Group 1 on postoperative day 2. The duration of artificial ventilation was 3.3 +/- 5.2 days (0.7-15 days); the length of stay in an intensive care unit was 4.8 +/- 8.2 days (1-20 days). There were 9 (18.7%) deaths. Left ventricular failure, high pulmonary hypertension, and coronary insufficiency were causes of death in 2 (4.8%), 1 (2.4%), and 2 (4.8%) patients, respectively. After arterial switching, the optimum management of patients with low cardiac output is to maintain low peripheral resistance and to restrict preload and to use FDE III inhibitors as the drugs of choice.

Assisted Circulation↗

[Mono-dimensional and bi-dimensional echocardiographic diagnosis of corrected transposition of the great vessels].

Three patients with corrected transposition of the great arteries (CTGA) have been studied by means of single plane (M-mode) and two-dimensional (2-D) echocardiography: the first study was performed after surgical closure of a VSD and the remaining two before cardiac catheterization. The following M-mode findings can suggest the diagnosis in CTGA: the interventricular septum (IVS) may not be visualized, there is lack of continuity between the posterior a-v valve (tricuspid) echoes and the anterior great artery (aorta), the posterior a-v valve leaflets may show some abnormalities in shape. The measurement of systolic time intervals of both semilunar valves can help in distinguishing the pulmonary artery from the aorta; further information can be obtained by means of peripheral venous contrast injection, which, in absence of right-to-left shunt, opacifies the posterior great vessel (pulmonary). The 2-D view of the heart allows the identification of the morphological type of ventricle and of the spatial great arteries relationship. The posteriorly and left located ventricle is recognized as morphologically right, because of the tricuspidal shape of its a-v valve: its attachment to IVS is lower than that of the mitral valve (4-chambers view) and three leaflets may be evident (short-axis); 3 papillary muscles and gross trabeculations can be identified (short axis and 4-chambers views, respectively). In long-axis the anterior vessel (aorta) runs parallel to the sternum; in short-axis both vessels are imaged as adjacent circles; by means of peripheral contrast injection, in conditions without right-to-left-shunt, the right and posterior vessel is recognized as a pulmonary artery. The differential diagnosis is discussed with d-transposition, Fallot's tetralogy, Taussig-Bing anomaly, common trunk, univentricular heart. According to our experience, the diagnosis of CTGA and associated defects can be made by M-mode and 2-D echocardiography.

Adult↗