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[Total transposition of the great vessels associated to a peculiar preexcitation syndrome].

Following the historical introduction concerning the evolution of our present knowledge of the W-P-W syndrome, we discuss a unique case of complete transposition of the great vessels with an unusual type of pre-excitation. We attributed this excitation to an uncommonly placed bundle of Kent, which, to our knowledge, has not been previously reported as coexisting with the aforementioned congenital heart malformation. A review of the literature, a discussion of other congenital heart diseases associated with disturbances of A-V conduction, as well as the clinical and para-clinical data upon which we base our diagnosis are presented.

Adult↗
PubMed1982

[Surgery in complex forms of transposition of the great vessels: long-term results in a series of 100 cases].

A series of 100 patients with complex forms of transposition of the great arteries (TGA) were operated upon over a 10 year period. Group 1 consisted of 13 TGA with pulmonary stenosis (PS), usually treated by an atrial baffle and direct repair of the stenosis. There was no operative or late mortality in this group and the long-term results were generally good (10/13). Group II comprised 29 TGA with ventricular septal defect (VSD) and PS. Seven Rastelli procedures gave 4 good results. Twenty two operations, associating atrial baffle, repair of VSD and PS (17 direct procedures, 5 left ventricle-pulmonary artery tube) were associated with a high mortality (5 operative and 4 late deaths) and 10 good long-term results. Group III comprised 58 TGA with VSD. Up to 1977 (n = 33) treatment consisted of atrial baffle + repair of VSD +/- removal of previous banding. Mortality was high (11 operative and 8 late deaths) with only 8 good long-term results. Since 1977, these patients have been treated by complete anatomical repair, the operative mortality of which is higher in the period under study (9/23) but the long-term results are much better. The surgical indications in our Department are based on the analysis of these results. In Group I only very significant PS is treated, either by left ventricle-pulmonary artery tube or direct repair depending on the form of the stenosis. In Group II, where the results based on atrial baffle are poor, a Rastelli procedure is preferred and especially its variants which avoid the use of prosthetic materials on the pulmonary trunk. In Group III, anatomical correction at the level of the great arteries is the routine procedure, the only point of discussion being the possibility of prior banding.

Adolescent↗
PubMed1997

[Complete D-transposition of the great vessels diagnosed late in a 10-month infant. Rashkind manoeuvre].

We report a first documented case in Senegal with simple transposition of the great arteries diagnosed in a 2 months old girl treated by Rashkind atrioseptostomy. Our patient benefited from clinical examination, ECG (15 derivations), chest X ray and standard laboratory tests. Pulsed-Doppler, two dimensional and TM echocardiography have been performed with an ATL MK 600 echocardiograph. Cardiac catheterism, angiocardiography and Rashkind procedure have been realized in our Department. These data are discussed and compared to the literature. At admission this patient presents with major cyanosis and polypnea. At examination, there is a 3/6 murmur at the left sternal border and a subclavicular continuous murmur. Laboratory tests showed metabolic acidosis and severe hypoxemia. Chest x-ray showed a cardio-thoracic ratio at 0.64 with increased pulmonary vascular markings. ECG showed right ventricular hypertrophy. Echocardiography-Doppler revealed ventriculo-arterial discordance with restrictive atrial septal defect and persistent ductus arteriosus. Rashkind procedure was followed by an increased aortic saturation. After 6 weeks there was an improvement of cyanosis and cardiac failure. Diagnosis of transposition of the great arteries is actually easier with development of ultrasonography which is useful when performed by experienced cardiologist. Spontaneous prognosis of this malformation is very poor. Rashkind atrioseptostomy is an important step for the initial treatment of transposition of the great arteries in terms of survival before open heart surgery.

Cardiac Catheterization↗
PubMed1988

[Anesthesiologic management of cesarean section in a patient with transposition of the great vessels].

The maternal mortality associated with cesarean section in the presence of congenital cyanotic heart disease is high. We report the anesthetic management of a 26-year-old pregnant patient with transposition of the great vessels and a functional single ventricle whose child was delivered by elective cesarean section under continuous epidural anesthesia. There were no hemodynamic problems and the outcome was successful for both mother and child, who could be discharged from the hospital on the 17th postoperative day. Elective cesarean section may be an acceptable method of delivery, and lumbar epidural block proved to be an appropriate procedure for this patient. The cooperation of cardiologists, anesthesiologists, and obstetricians was necessary to assure maternal and fetal survival. Continuous invasive hemodynamic monitoring and use of small epidural top-up doses (2-3 ml) of local anesthetic were of utmost importance in maintaining the hemodynamic stability.

Adult↗
PubMed1981

[Anatomic correction of transposition of the great vessels with interventricular communication. A series of 24 consecutive operations].

The results of surgical correction at arterial level in 24 cases of transposition of the great arteries with ventricular septal defect are reported. The majority of children (19/24) had undergone previous pulmonary artery bonding between the ages of fifteen days and 3 months (average 1,5 months). Surgical correction was performed on average 14 months later. This consisted of repair of the ventricular septal defect and arterial shift to reestablish ventriculoarterial concordance with reimplantation of the coronary arteries. In the last 9 cases no prosthetic materials were used on the right outflow tract. The operative mortality was 9/24 (6 of the first 12, and 3 of the last 12 cases). The cause of death was due to associated lesions, congenital (coarctation of the aorta, mitral valve malformation), or iatrogenic related to previous surgery (stenosis of the right ventricular infundibulum). Left ventricular failure was common despite preparation with pulmonary artery banding and merited systematic treatment mainly with arterial vasodilator therapy and prolonged ventilatory assistance. The surviving children are asymptomatic without treatment. Control catheter studies did not show any significant abnormality except in one case of pulmonary hypertension. The exceptional quality of these results, the absence of prosthetic material and the hope of reducing avoidable mortality justify this approach to surgical cure of the malformation. Preparation by pulmonary artery banding and the use of this method in "simple" forms of transposition are questions which remain open to discussion.

Child, Preschool↗
PubMed1984

Surgical approach to corrected transposition of the great vessels and situs inversus [I, D, D] with ventricular septal defect and systemic atrioventricular valve regurgitation.

Successful surgical repair of a 4-year-old boy with situs inversus [I, D, D] and corrected transposition of the great vessels (TGV) as well as hemodynamically significant ventricular septal defect (VSD), systemic atrioventricular (tricuspid) valve regurgitation, and atrial septal defect is described. Unique technical aspects of VSD and tricuspid valve repair in association with corrected TGV and situs solitus or inversus are discussed and clarified.

Child, Preschool↗
PubMed1986

[Corrected transposition of the great vessels in aged adults. Apropos of 2 patients aged 72 and 80].

The authors report two unusual new cases of corrected transposition of the great arteries diagnosed in patients 72 and 80 years of age respectively, presenting with symptoms of cardiac failure of recent onset. Both patients had mild or moderate regurgitation of the systemic atrio-ventricular valve, associated in one case with aortic regurgitation. This association would appear to be fortuitous although already reported in six occasions. The diagnosis was made by echocardiography and confirmed by catheterisation in one case, and by digitised intravenous angiography in the other. These cases are of interest for three reasons: They are very rare: there have been only 20 comparable cases reported in patients over 40 years of age, the eldest being 73. This is probably explained by the high frequency of associated malformations. They illustrate the decisive role of echocardiography in the diagnosis of congenital heart disease, even in the adult. They constitute a "natural experimental model" of a right ventricle submitted to systemic pressures and tend to show that, in the absence of other abnormalities giving rise to an additional pressure or volume overload, the right ventricle is capable of long term adaptation to this situation. These observations indicate a favourable long-term prognosis for children who have undergone "corrective" surgery at atrial level for simple transposition and in whom the right ventricle is required to assume the function of the systemic ventricle for the rest of their life.

Aged↗