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Sequential diagnosis of coronary arterial anatomy in congenitally corrected transposition of the great arteries.

BACKGROUND: The objective of this study was to analyze coronary arteries (CA) in congenitally corrected transposition (CCT) and to determine the influence of aortopulmonary rotation on its pattern systematically. Precise CA anatomy is surgically needed in the current era of double switch for CCT. METHODS: We collected data on 62 patients who had CCT with situs solitus or inversus between 1981 and 1999. Coronary artery anatomy was analyzed as it related to apical position, atrial situs, ventricular looping, and aortopulmonary rotation. Five main types with similar variants of epicardial configuration at the base of the heart were categorized into five central patterns (patterns X, O, I, II, and IV). RESULTS: The right CA coursed to the left in CCT with situs solitus, and to the right in CCT with situs inversus; and to the more posterior atrioventricular groove in both without apicocaval ipsilaterality. However, in CCT with more apicocaval ipsilaterality, the left circumflex might shift posterior to the right CA. With the same aortopulmonary rotation, the two groups had similar central patterns, and eta-square analysis showed that the evolution from patterns X, O, I, II, toward IV (n = 1, 36, 15, 9 to 1) was dependent on clockwise aortopulmonary rotation (p < 0.00000). CONCLUSIONS: Peripheral CA pattern in the atrioventricular groove was dictated by apicocaval ipsilaterality anteroposteriorly and ventricular looping dextrosinistrally, irrespective of atrial situs. The central CA pattern near the aortic sinus depended on aortopulmonary rotation due to "marriage of convenience" between them, and thus was predictable from arterial relations irrespective of its disease category.

Adolescent↗

The arterial switch operation. An eight-year experience.

Sixty-six patients (23 neonates with transposition of the great arteries and intact ventricular septum, 33 infants and children with transposition and a large ventricular septal defect, and 10 with double-outlet right ventricle with a subpulmonary ventricular septal defect have received an arterial switch repair since 1977. Eight (one with transposition and intact ventricular septum, six with transposition and ventricular septal defect, and one with double-outlet right ventricle with subpulmonary ventricular septal defect) died in the hospital. All other patients have had follow-up as of June, 1985. Including the hospital deaths, the 11 month actuarial survival rate for the entire group was 81%, and no deaths have occurred among the 33 patients alive at that time and traced as long as 8 years. The hazard function for death has only a single early phase, and its 70% confidence limits overlap the hazard function of a matched general population by 12 months after the operation. Incremental risk factors for death included low birth weight (but not weight or age at operation), transposition of the great arteries with large ventricular septal defect, double-outlet right ventricle with subpulmonary ventricular septal defect, and presence of a patent ductus arteriosus. Coronary artery morphology and position of the great arteries were not risk factors. Long aortic cross-clamp time was possibly (p = 0.11) a risk factor. Early date of operation was a risk factor (p = 0.004); thus, predicted 1 year survival rate, including hospital deaths, after the arterial switch operation in 1985 is 99.9% (70% confidence limits 99.0%-100%) for neonates with transposition and intact ventricular septum and 99.7% (98.4%-99.9%) for those with transposition and a large ventricular septal defect or double-outlet right ventricle. The late functional status was excellent, and the rhythm was sinus in 96% of the 55 surviving patients. A formal comparison with the results of the atrial switch repair indicates that the arterial switch repair is superior.

Aorta↗

Left ventricular and biventricular extracardiac conduits.

The insertion of an extracardiac conduit from the right ventricle has gained extensive success during the past decade. This report concerns the application of such a conduit from the left ventricle. Its use in four types of anomalies is described: (1) left ventricular outflow hypoplasia, (2) transposition of the great arteries with ventricular inversion and associated pulmonary stenosis, (3) complex anomalies involving double-outlet right ventricle wherein construction of an intracardiac tunnel is not feasible, and (4) transposition of the great arteries with intact ventricular septum and severe pulmonary stenosis. The correction of the last two mentioned anomalies involves the insertion of two parallel conduits, one from each ventricle to its appropriate great artery. The transposition anomaly may alternatively be corrected by combined Mustard repair and a left ventricle-to-pulmonary artery extracardiac conduit.

Adolescent↗

[Echocardiography as an imaging procedure in pediatrics].

Since the introduction of echocardiography into paediatrics by Lundström at the end of the 1960's, this noninvasive technique has come a long way. The original m-mode technique was followed by sector-echocardiography, which permits two-dimensional visualization of the heart. By this means cardiac and great vessel anatomy can be studied in real time. Even complex congenital heart defects can be analyzed and, in contrast to angiocardiography, the atrioventricular valves are well visualized. The addition of doppler-echocardiography enables one to examine direction and velocity of blood flow, which helps to reveal shunts within the heart and between the great vessels as to detect valvular stenosis and insufficiency. The principal advantage of echocardiography lies in this ability to rapidly diagnose congenital and acquired heart disease without harm to the patient. In addition, size and dynamics of the cardiac chambers and the great vessels including valves can be adequately studied.

Child↗

[The conduit application in surgical treatment of corrected transposition of the main vessels].

Experience of surgical treatment of 45 patients with corrected transposition of the main vessels (CTMV) is summarized. Indications and tactic of surgical intervention for CTMV with the pulmonary trunk (PT) stenosis using conduit as a parallel exit from venous ventricle are substantiated. Original construction of autopericardial conduit is adduced. Basing on analysis of results of surgical treatment an optimal type of conduit for treatment of CTMV with the PT stenosis is proposed.

Blood Vessel Prosthesis↗

Heart transplantation in repaired transposition of the great arteries.

Cardiac transplantation was carried out in a 40-year-old man with the diagnosis of repaired transposition of the great arteries and right-sided aortic arch who had end-stage cardiac failure due to myopathy of the ventricles. Because of several previous surgical repairs and the orientation of the great vessels, the operation presented some technical problems. Therefore, modifications of operative procedures were used, including recipient hypothermia, circulatory arrest, and changes in the donor heart implantation. The removal of the donor heart was modified in such a way that the graft included the aortic arch and both pulmonary arteries. With the extra length of ascending aorta and transverse arch, the innominate, left carotid, and left subclavian vessels were excised as a button, thereby leaving the distal orifice of the aorta in the superior portion of the transverse arch. For the recipient, the operation was performed using hypothermic total circulatory arrest to dissect free the huge pulmonary artery and the short right-sided aortic arch to place the clamp. Implantation of the donor heart was modified accordingly. The technical results were confirmed one and a half months later on a control digital angiogram. Thirty-five days postoperatively the patient was discharged. Six months after operation, the patient is doing better than ever before in his life. Our findings suggest that a complicated conotruncal development does not preclude cardiac transplantation.

Adult↗

Surgical palliation of subaortic stenosis in the univentricular heart.

Seventeen patients with a univentricular atrioventricular connection and subaortic stenosis at the outlet foramen level were seen from 1974 until 1983. The ventricular anatomy was of a single left ventricle with an outlet chamber, and the great arteries were transposed. This condition often followed pulmonary artery banding (15 cases) but also occurred with pulmonic stenosis (one) or pulmonary atresia (one). The median interval to the detection of subaortic stenosis after banding was 2.3 years (range 11 days to 15 years). Thirteen patients had a resting gradient to 118 mm Hg and three a gradient only with isoprenaline infusion. The following surgical procedures have been performed: (1) insertion of a conduit between the left ventricle and aorta (one patient); (2) creation of an aorticopulmonary window proximal to the band and tightening of the band (three patients); (3) enlargement of the ventricular septal defect (three patients); (4) Fontan procedure (five patients); and (5) Fontan procedure plus arterial switch (two patients). All of the procedures but the last one have met with significant mortality in this difficult group of patients.

Abnormalities, Multiple↗

Left ventricular outflow tract obstruction in TGA: treatment with LV-to-PA valved conduit.

Progressive or recurrent left ventricular outflow tract obstruction after a previous Mustard or Senning operation represents a rare but challenging problem. The obstruction can be resected in some patients, but abnormal attachment of the mitral valve or a long fibromuscular tunnel represents a difficult surgical problem. Between 1979 and 1993, we encountered this type of left ventricular outflow tract obstruction in 10 patients, 4 to 13 years after the atrial repair. They ranged in age from 5 to 15 years (mean, 8.8 years) and weighed between 11.5 and 47 kg (mean, 25.3 kg). Operations were performed through a left thoracotomy with the patient on hypothermic cardiopulmonary bypass but without aortic cross-clamping. The left atrial appendage and descending aorta were cannulated. Good relief of the gradient was obtained in all patients (mean residual gradient, 14.8 mm Hg). All patients survived the operation. One patient died suddenly at home 6 months later; 2 patients required conduit replacement. All 9 long-term survivors are asymptomatic as of 6 months to 8 years after their conduit placement or replacement. We recommend the placement of a left ventricle-to-pulmonary artery valved conduit for the relief of severe left ventricular outflow tract obstruction arising after a Senning or Mustard operation that cannot be managed by other means.

Adolescent↗