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PubMed2000

[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↗
PubMed1996

-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↗
PubMed1993

[Correction of transposition of great vessels with and without associated congenital defects. Description of two clinical cases].

We describe two cases of corrected transposition of great vessel (L Transposition). The first case was diagnosed in a man of 70 years old and was not associated with other congenital defects. The second case regards a young man of 16 years old and congenital cardiopathy was associated with interventricular defect and left Ebstein disease. We discuss the different evolution of L Transposition according to the presence or not of other congenital defects and we analyse right ventricle behaviour when it is submitted systemic pressure. In the literature only 26 cases of L Transposition were described in patients over 40 years old. The first case represents a rarity and it shows how in the absence of associated congenital defects, the right ventricle is able to adapt to systemic pressure. Bidimensional echocardiography in the method of choice to diagnose and evaluate congenital cardiopathy in adult age.

Abnormalities, Multiple↗
PubMedDate not supplied

[Cognitive development of children and adolescents after correction of transposition of great vessels].

BACKGROUND: To study the effect of transposition of the great arteries on later cognitive functioning. PATIENTS: Twentyeight children and adolescents underwent psychometric testing 3.5 to 13.7 years following operation. METHODS: A battery of intelligence, attention and visual memory tasks as well as the Draw-a-Man-Test were administered. Parents completed standardized questionnaires on developmental milestones. RESULTS: Both, mean verbal (93 +/- 15) and performance IQs (97 +/- 21) were normal. The length of interval between operation and psychologic test but not age at repair was a significant predictor of intelligence quotient. Half of the children (9/18) showed attention deficits, 7/11 (64%) were identified as suspect of brain dysfunction. Performance on the Draw-a-Man-Test disclosed 12 (43%) as slightly mentally retarded. CONCLUSIONS: (1) Cognitive functions in children with TGA are more impaired than suggested by the results of intelligence tests. (2) Psychological and neurologic follow-up should be mandatory from early on. (3) Previous studies have overestimated children's intelligence due to unrecognized changes of test norms.

Adolescent↗
PubMed1993

[Transposition of great vessels in Cantrell syndrome].

A case is presented of complete transposition of great vessels with atrial and ventricular septum defect and coarctation of the pulmonary artery in Cantrell syndrome. The Cantrell syndrome consists of: congenital heart disease, defect of pericardium, diaphragm, sternum, and anterior abdomen wall. In all cases of Cantrell syndrome described as yet ventricular septum defect was present alone or in combination with other intracardiac defects. The presented case is the first report of congenital abnormality in the from of d-TGA in Cantrell syndrome.

Constriction, Pathologic↗
PubMed1989

[Anatomic study of the coronary arteries in transposition of great vessels].

The authors studied 64 hearts presenting transposition of the great arteries, in order to evaluate the pattern of distribution and origin of the main coronary arteries. Regarding the origin in each sinus of Valsalva, there was no significant difference with the data from the literature. It was also evaluated the precise site of origin inside the sinuses, which were divided in three thirds: the medial and those adjacent to the valvar commissures. We observed that, although the coronary ostia took origin from the medial third in more than half of the cases, there was some variation when considering the different patterns of distribution. This observations is relevant, since, when Jatene's procedure is considered, the surgical disinsertion of a coronary artery may put at risk the valvar leaflet, when the ostium is near the commissure.

Child↗
PubMed1975

[Palliative surgery of transposition of great vessels associated with pulmonary stenosis].

Palliative treatment for transposition of the great vessels with pulmonary stenosis (combined or not with ventricular septal defect) was studied in relation with 24 patients operated upon at Laennec's Hospital in Professor Mathey's section between January 70 and January 74. Three therapeutic attitudes are possible according to whether or not an atrial septal defect is created (Blalock-Hanlon's operation) in combination with the systemic-pulmonary artery anastomosis, and according to whether the operations are performed in one or many steps. It seems that for the badly-tolerated forms of the new-born, combination of both operations seems to be the best attitude, the curative treatment being impossible at this age.

Blood Pressure↗
PubMed1997

[Angioplasty of residual lesions after correcting surgery of transposition of great vessels].

BACKGROUND: Surgical correction in infants born with transposition of the great arteries, venous rerouting procedure (Mustard or Senning operation) or an arterial switch operation, can be complicated by vascular narrowings. Balloon dilatation angioplasty may be the treatment for these stenotic lesions and is attempted to avoid a reoperation of these patients. MATERIAL AND METHODS: Angioplasty was attempted in nine patients after the surgical correction of transposition of the great arteries. Senning procedure was performed in sixty-one patients; two patients (3.2%) were successfully balloon-dilated for systemic venous obstruction: superior vena caval obstruction (case 1) and inferior vena caval obstruction (case 2). Arterial switch operation was performed in forty patients, balloon angioplasty was attempted in seven patients (17.5%) who developed supravalvar pulmonary artery stenosis. Mean age at dilation was 32.5 +/- 20.7 months (range 11 to 67) in the switch arterial group. Age at dilation in the Senning group was 8 and 106 months. The balloon/stenosis diameter was 2 to 3 in the switch arterial operation and 3 to 8 in the Senning procedure. RESULTS: Balloon dilatation angioplasty was successful in three patients with supravalvar pulmonary stenosis postarterial switch operation (mean age was 16.6 months) and reduced the peak-to-peak gradient from 78.6 +/- 36.6 mmHg to 44 +/- 15 mmHg. There was no success after angioplasty in three patients and it was not possible to cross the stenosis with the guidewire in one (mean age was 46 months). Balloon dilatation angioplasty was successful in the obstruction following Senning procedure. CONCLUSIONS: These data indicate that although the success rate of balloon angioplasty for supravalvar pulmonary stenosis after the arterial switch operation is low, balloon angioplasty can be the first therapeutic choice owing to its low complication rate and the potential benefit of the procedure. The success rate should be higher if angioplasty is performed at a younger age after operating and a balloon of adequate size is used. Postoperative systemic venous obstructions can be successfully managed with balloon dilatation.

Catheterization↗
PubMed1983

[Prognosis for patients following surgical correction of transposition of great vessels].

Without surgical help, 90% of patients with transposition of the great arteries (TGA) will die during the first year of life. After repair of TGA, 90% of patients reach adult life. A number of hemodynamic complications and arrhythmias may occur and must be recognized, because some require reoperation or pacemaker implantation. Nearly 8 years postoperatively the vast majority of patients were symptom-free and over 90% were attending an ordinary school or pursuing a profession. Dysfunction of the right (systemic) ventricle and its atrio-ventricular valve may become progressive in the late follow-up and could limit the reported success-rate in the future.

Arrhythmias, Cardiac↗
PubMed1997

[Has prenatal diagnosis of transposition of great vessels changed its prognosis?].

Neonatal anatomical correction of transposition of the great arteries (TGA) has transformed the prognosis of this condition but the diagnosis must be made rapidly. The aim of this retrospective study was to evaluate the benefits of antenatal diagnosis on the outcome of TGA. The cases of 50 consecutive neonates with TGA with or without ventricular septal defect hospitalised between 1989 and 1996 were reviewed. All these children underwent anatomical correction of their malformation in the neonatal period. In seventeen of the children the diagnosis was made in the antenatal period at a gestational age of 28.7 +/- 5 weeks of amenorrhea and the other 33 had a postnatal diagnosis at 6.2 +/- 13 days. The clinical and echographic features were identical in the two groups. The risk factors of mortality for the whole population were a Yacoub type B or C coronary disposition, an intramural coronary course, difficulties in reimplantation of the coronary arteries and/or peroperative haemodynamic failure. In the authors' experience, the time of diagnosis (antenatal or postnatal) did not had on the management and prognosis of TGA. Studies with larger population groups are probably necessary to demonstrate the possible benefits of antenatal diagnosis.

Cardiac Surgical Procedures↗
PubMed1988

[Late results following Rastelli corrective operations in transposition of great vessels].

From 1974 to 1985, 19 patients with transposition of the great arteries, ventricular septal defect and pulmonary valve stenosis or atresia underwent Rastelli correction. The mean age of the patients at operation was 9.7 years. Four patients died on the day of operation (early mortality: 21%). One patient died 7 years later due to dysrhythmias (overall mortality: 26%). Cardiac catheterization was performed on average 1.8 and 6.7 years after the operation. Gradients above 40 mm Hg were registered in 70% of the conduits, 6 years after the operation. Five patients underwent six reoperations for conduit stenosis. The actuarial survival rate was 75%; the event-free survival rate was only 20% after 12 years.

Adolescent↗
PubMed1976

[Corrected transposition of great vessels in adult age].

Two adult cases of corrected transposition of the great arteries are presented. One of the two males had associated ventricular septal defect, the other one presented as an isolated anomaly. The characteristic clinical features are: loud second heart sound with audible pulmonic component of the left sternal border in the absence of pulmonary hypertension. Abnormal precordial impulse upon palpation. Chest X-ray shows absence of pulmonic segment and of the aortic knob with the contour of the ascending aorta to the left of the mediastinum. Ecg anomalies may be multiple; typically anomalous septal Q-waves are consistently absent. Corrected transposition is well tolerated even into adulthood. Prognosis is dominated by assiciated anomalies.

Adolescent↗
PubMed1992

[Doppler echocardiography after anatomical repair of transposition of great vessels].

Forty seven patients who underwent anatomical repair of transposition of the great arteries were assessed by Doppler Echocardiography on average 16 months after surgery. Thirty three had transposition alone and 14 had an associated ventricular septal defect. Abnormal left ventricular function was observed in 4 patients (8%) and was associated with a preoperative left to right ventricular systolic pressure ratio less than that of patients with normal left ventricular function (0.76 +/- 0.22 vs 0.90 +/- 0.14 respectively, p = 0.10). Aortic regurgitation was detected in 19 children (40%). It was minimal in 18 cases and mild in the other case, in which a ventricular septal defect has been approached via the original pulmonary valves. Previous pulmonary banding before detransposition was a predisposing factor for postoperative aortic regurgitation. Thirteen patients had pulmonary pressure gradients of over 20 mmHg. The site of obstruction was usually the main pulmonary artery. The 3 cases in which the coronary orifices were closed with two patches instead of one had significant stenosis of the main pulmonary artery. Pulmonary regurgitation was detected in 38 patients (81%); it was minimal in 29 cases, mild in 5 cases and severe in 4 cases. Five patients (11%) had minimal mitral regurgitation and 16 (34%) tricuspid regurgitation. In the 22 cases who had serial echocardiographic evaluation with an average follow-up of 18 months, the postoperative abnormalities were characterised by their stability, with no significant progression of the regurgitant lesions. A reduction in pulmonary pressure gradient was observed in 5 patients and an increase in 1 patient (from 28 to 40 mmHg).(ABSTRACT TRUNCATED AT 250 WORDS)

Aortic Valve Insufficiency↗
PubMed1996

[A case of corrected transposition of great vessels in an adult. Value of magnetic resonance].

Corrected transposition of the great arteries is an unusual congenital heart defect, particularly in adults. Echocardiography and angiography are still cornerstones for its diagnosis. However, other techniques, as magnetic resonance imaging, may constitute a noinvasive diagnostic alternative to cardiac catheterization. We present one case of corrected transposition of the great arteries in an adult patient in which this method was used.

Adult↗
PubMed1996

[Cardiac output evaluation during exercise in children treated with atrial surgery for transposition of great vessels].

The long-term physiopathological consequences of atrial surgery (Senning or Mustard procedures) for transposition of the great vessels with respect to exercise capacity are not well known. We measured the cardiac index by the technique of CO2 rebreathing at two submaximal levels of exercise corresponding to a stable oxygen consumption of 20 (E20) and 30 (E30) ml/min/kg in 7 patients successfully operated for transposition of the great vessels and in 7 control children paired for age, gender and body surface area. Despite an identical chronotropic response to exercise in the two groups, the increase in cardiac index was not as great in the children operated for transposition (from 6.86 +/- 0.51 to 7.71 +/- 0.78 l/min/m2) as in the control population (from 7.71 +/- 0.78 to 10.2 +/- 0.51 l/min/m2; p < 0.02). The stroke volume index was therefore significantly lower in the transposition group at both levels of exercise (52 +/- 3.2 vs 63 +/- 4.1 ml/m2; p < 0.04 at E20; and 46.4 +/- 4.3 vs 66 +/- 5.1 ml/m2 at E30). The main cause of this reduction of the stroke volume index is probably a lack of adaptation of right ventricular systolic function on exercise but it is not possible to exclude diastolic dysfunction due to reduce compliance secondary to the intraatrial patch. The conditions of preload are in fact instrumental in increasing stroke volume index at submaximal exercise levels.

Adaptation, Physiological↗