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[Anatomical repair in transposition of great vessels].

Transposition of the great arteries (TGA) covers a wide range of anatomic variants involving not only the origin of the great arteries, but the intracardiac structures and the aortic arch. TGA can now benefit from complete anatomic repair, namely switch operation, performed in simple forms during the neonatal period. Accurate pre and intraoperative assessment must take into account the main anatomic features: relationship between the aorta and the pulmonary artery and coronary arteries distribution. Over the last sixteen years, 1,063 patients with TGA underwent anatomic repair in our institution. Among 728 patients with simple TGA, 92% were operated on within the first 2 weeks of life. Among 335 patients with complex TGA, all had a large VSD and 154 had additional anomalies. Among these 335 patients, 264 had one-stage complete repair and 71 had two-stage repair, at the beginning of our experience. In complex forms the main associated anomalies involved the ventricles (right ventricle hypoplasia (11), malaligned VSD (90), restrictive VSD (3), multiple VSD (43), the atrioventricular valves (straddling of the mitral or tricuspid valve (15), mitral valve anomalies (cleft or tissue tag, 4) and the aortic arch (coarctation [88], interrupted aortic arch (8)). The hospital mortality was 6.9% for simple TGA, 14.6% for complex TGA. Main causes of early death were failure in coronary artery relocation and pulmonary hypertensive crisis. Mean follow-up (95.5% of patients) was 49 months. Overall 16-year survival was 87.9%, 91.5% for simple forms and 80.4% for complex forms. Anatomic repair is nowadays the treatment of choice for TGA. Long-term results will depend on the evolution of the relocated coronary arteries and of the neo-aortic valve.

Aorta↗

A new operation for d-loop transposition of the great vessels.

Transposition of the great vessels remains a major problem for the cardiac surgeons. Although the Mustard (intraatrial baffle) procedure has provided the means for physiological correction of this anomaly, there are many complications that plague the lives of the survivors. This report describes an operation that is essentially extracardiac and achieves the desired goal of simple switching of the great vessels. Future revisions of the outflow tract prosthesis may become a major problem, but probably less of one than the complications that have been and will be associated with the intraatrial baffle procedure.

Heart Ventricles↗

[Development of a technique for the complete correction of transposition of great vessels].

Complete transposition of the great arteries is one of the most common cardiovascular anomalies. Several surgical methods of treatment have been proposed. Arterial repair theoretically seems a better option since it does not introduce any additional intra cardiac anomaly and it restores the left ventricle to its natival systemic function. The rationale for neonatal arterial repair lies on fetal and neonatal cardiopulmonary physiology. The left ventricle has to eject immediately after surgery a normal cardiac output at systemic pressure in the aorta. This is the case in the neonatal period, because during fetal life pulmonary artery and aortic pressure are equal. For simple TGA, after birth, with the fall in pulmonary vascular resistances and constriction of the ductus arteriosus, pulmonary artery and left ventricular pressures drop dramatically to less than one third (1/3) of systemic pressure. As a result, the left ventricle is not stimulated for growth adaptation and becomes a thin ventricle less contractile and more compliant. However, there is little doubt that during the first 2 to 4 post-natal weeks, the left ventricle is still suitable to sustain a systemic workload. Between april 1984 and april 1992, four hundred and twenty six (426) consecutive neonates underwent an arterial switch operation for various forms of transposition: with 34 hospital deaths. The mean age at operation was 13 days and the mean weight was 3.2 kg. Among patients with TGA-VSD and coarctation, 14 underwent a single stage repair through mid sternotomy. Actuarial survival rates were: 89% for TGA-IVS at 5 years, 90% for TGA-VSD, 85.3% for TGA-VSD and coarctation at 3 years.(ABSTRACT TRUNCATED AT 250 WORDS)

Methods↗

[Physiopathologic findings and surgical treatment in transposition of great vessels: our experience].

Transposition of great vessels (TGV) is the most frequent neonatal cyanotic malformative cardiopathy. The Authors report their experience in surgical anatomical correction of transposition of the great arteries in 55 patients, 44 male and 11 female. Minimum age was 2 days, maximum 6 months. TGV was simple in 38 cases and associated with ventricular defect in 12 cases, with double outlet right ventricle in 3 cases, and with complex cardiopathy in 2 cases. There have been 11 deaths. The cause of death was: cardiac failure in 3 patients, myocardial infarct in 4 patients, respiratory insufficiency in 2 patients, and sepsis in 2 patients. There was one death by myocardial infarction and 1 asymptomatic ostial left coronary stenosis during follow-up (from 1 to 104 months). There were neither anastomotic pulmonary stenosis nor aortic valve incompetence. The authors review the literature on functional and anatomic correction of TGV and underline the importance of precocious anatomic together with early diagnosis, percutaneous atrioseptostomy and pharmacologic (PGE1) therapy in determining further reduction of mortality and to improve late outcome.

Age Factors↗

Successful closure of ventricular septal defect through a left-sided ventriculotomy in corrected transposition of the great vessels.

Corrected transposition of the great vessels is often associated with other cardiac anomalies. Ventricular septal defect (VSD) is the most common among them. Closure of a VSD is usually performed through a right-sided ventriculotomy. We had previously closed the VSD through the right-sided ventricle in 2 patients with corrected transposition of the great vessels and in 2 others with double-outlet right ventricle with ventricular inversion, which resulted in complete heart block. Recently we have closed the VSD through the left-sided ventricle in 3 patients with corrected transposition, and no block resulted.

Adolescent↗

[Intraventricular conduit for complex forms of transposition of great vessels (author's transl)].

Complex forms of transposition of great vessels necessitate different approaches to correction. In a case of double outlet right ventricle with d-transposition and atrioventricular concordance and a subvalvular pulmonary stenosis an intraventricular conduit with a 16 mm Dacron vascular prosthesis was used. This was anastomosed in an acute angular manner with the muscular margins of the VSD and routed to the base of the aorta. Surgical and anatomic implications are discussed.

Cardiac Catheterization↗

[Prenatal diagnosis of transposition of great vessels reduces neonatal morbidity and mortality].

Transposition of the great arteries (TGA) is a common malformation which sometimes has a dramatic presentation at birth but which is completely curable with early and appropriate initial management. Antenatal diagnosis of this condition may change the neonatal prognosis. The authors compared morbidity and mortality in the pre- and postoperative periods of 68 neonates with an antenatal diagnosis of TGA (foetal diagnosis) with that of 250 neonates in whom the diagnosis was made after birth (neonatal diagnosis). The delay before admission to the department was 2 +/- 2.8 hours in the foetal group and 73 +/- 210 hours in the neonatal group (p < 0.01). Severe haemodynamic distress (metabolic acidosis, multi-organ failure) were more common in the neonatal group (p < 0.01). Management on admission was identical in the two groups (p > 0.05). The preoperative mortality was 15/250 in the neonatal group (6%, 95% CI = 3-9%) compared with 0/68 in the foetal group (p < 0.05). The postoperative morbidity was comparable in the two groups (25/235 and 6/68) but the hospital stay was longer in the neonatal group (30 +/- 17 versus 24 +/- 11 days, p < 0.01). Finally, postoperative mortality was significantly higher in the neonatal group (20/235 compared with 0/68, p < 0.01) although the risk factors of death at arterial switch surgery were identical in the two groups. Therefore, antenatal diagnosis of TGA reduces neonatal morbidity and mortality in this condition. Antenatal diagnosis must be developed by the education of obstetricians. The transfer of mothers with a foetus affected by TGA to centres capable of assuming the initial management, sometimes during labour, is essential.

Female↗

[Causes of preoperative mortality in transposition of great vessels. 2 cases].

The prognosis of transposition of the great arteries improved tremendously with the development of an early medico-surgical strategy including balloon atrioseptostomy, prostaglandin infusion and the arterial switch operation within the first days of life. Nevertheless, some patients still die preoperatively. We report on two newborn infants whose fatal outcome was promoted by an inadequate intercirculatory mixing. Since the diagnosis was not immediately made, the restrictive foramen ovale resulted very quickly in deep metabolic acidosis and balloon atrioseptostomy performed yet in the first hours of life could not prevent death. We emphasize the importance of prenatal echographic detection of this defect, only way to plan a balloon septostomy immediately after delivery in those infants suffering from inadequate atrial mixing.

Acidosis↗

-Aneurysm of the pulmonary artery and pulmonary artery hypertension 22 years after Mustard reversal operation in transposition of great vessels-.

An increasing number of patients with transposition of great arteries reaches adult age after an atrial redirection operation. The well known late sequelae of the Mustard and Senning operations include supraventricular brady-tachyarrhythmias, dilatation and failure of the systemic ventricle, severe tricuspid valve incompetence, obstruction of either the systemic or pulmonary venous return as well as left ventricular outflow tract obstruction, and baffle leaks. The case of a 26-year-old woman with a rarer postoperative course, namely severe aneurysmal dilatation of the pulmonary artery and pulmonary arterial hypertension 22 years after a Mustard operation is described. The pros and contras of the available therapeutic alternatives are discussed.

Adult↗