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PubMed1970

Electrocardiogram in corrected transposition of the great vessels of the bulbo-ventricular inversion type.

Twenty cases of corrected transposition of the great vessels of the bulbo-ventricular inversion type, either lone or combined with other intracardiac anomalies, were analysed. Rhythm and/or atrio-ventricular conduction disturbances were common to all groups of cases. QRS pattern changes were found to be related both to ventricular inversion and to ventricular hypertrophy. Isolated corrected transposition and corrected transposition with systemic ventriculo-atrial regurgitation give rise to tracings suggestive of systemic ventricular hypertrophy.Corrected transposition of the great vessels with pulmonary stenosis or pulmonary artery hypertension is usually accompanied by the electrocardiographic signs of a venous-ventricular hypertrophy, with a characteristic inversion of the normal praecordial pattern. The conventional criteria of ventricular hypertrophy may be applied in corrected transposition of the great vessels but are less reliable than in cases without ventricular inversion. The so-called electrocardiographic pattern of ;ventricular inversion' in this anomaly is related not only to the inverted position of the ventricles but to a greater extent to the predominant, anatomically left, venous-ventricular hypertrophy which re-establishes the normal weight ratio between the anatomically right and anatomically left ventricles.

Adolescent↗
PubMed1983

[His-bundle electrographic localization of a complete intra-His AV block in a patient with congenital corrected transposition of the great vessels].

Patients with corrected congenital transposition of the large arteries (CCT) often develop atrioventricular (AV) conduction disturbances, which may lead to syncope or sudden death. This report is concerned with our findings in a 24-year-old man with a CCT accompanied by a small ventricular septal defect and a complete AV block. While published case reports of electrophysiologically investigated third-degree AV blocks show conduction disturbances proximal to the bundle of His, we found the block to be localized in the intra-His region, which makes the case a rarity.

Adult↗
PubMed1996

[32 years of Senning's correction for transposition of the great vessels].

Between 1962 and 1994 342 patients with transposition of the great arteries (TGA) were treated by atrial correction. Since 1992 the atrial switch operation is the treatment of choice for TGA. We reviewed our 32 year experience. Average age of the patients at operation was 69 months (7 days--8.5 years). 177/342 (52%) patients had a complex TGA: 74 patients with ventricular septal defect (VSD), 49 patients with pulmonary stenosis (PS) and 54 with both (VSD and PS). The 30 day mortality was for the whole series 15.7%. In the last 4 years 7.5%. The actuarial survival rate for all patients was 88% after 10 years and 82% after 20 years. For simple TGA 91% after 10 years and 83% after 20 years, for complex TGA 84% and 81%. The most important cause of death during our longterm observation were heart failure (19 patients) and sudden death (7 patients). Average follow-up for the whole group was 13.4 years. Most of the survivors are functionally symptom free (66% NYHA I) or they have slight symptoms (29% NYHA II). Only 5% were NYHA III or IV. Arterial switch operation has replaced the atrial correction for TGA. Nevertheless the longterm results after atrial correction remains encouraging. The main threat to the patients is the failure of the systemic ventricle.

Cardiac Surgical Procedures↗
PubMed1975

Correction of transposition of the great vessels with previous Baffes procedure.

Twenty-four patients with transposition of the great vessels who underwent a Baffes procedure in infancy were totally corrected by a modified Mustard procedure. There were three postoperative deaths and one major complication. All survivors have been followed from 2 to 8 yr and have done well except for one patient who required a tricuspid valve replacement because of tricuspid insufficiency. It is our conclusion that this group of patients can be corrected with a relatively low mortality and a favorable outcome.

Adolescent↗
PubMed1987

Surgical management of subaortic stenosis in patients with single ventricle and transposition of the great vessels.

Subaortic stenosis can develop in patients with single ventricle and transposition of the great vessels. Its presence increases the risk of septation or Fontan repair, and reports of surgical management have indicated high mortality. This report describes the results of an approach that includes direct resection of the stenosis via an aortotomy, and delay of the Fontan repair to a later date. Nine children with single ventricle underwent surgery for subaortic stenosis from 1968 to 1984. Three deaths occurred postoperatively from ventricular dysfunction in patients who underwent either conduit bypass (one patient) or repair via ventriculotomy (two patients). Of the six long-term survivors, one infant underwent ventriculotomy and subaortic resection, and the last five patients had subaortic resection via an aortotomy without any other simultaneous procedure. Avoidance of ventriculotomy and decreased cardiopulmonary bypass time lowers the risk of stenosis repair in the presence of a hypertrophied ventricle. Fontan repair after some regression of the ventricular hypertrophy is recommended, and has been accomplished in one patient to date. All patients repaired via aortotomy are alive and well 1 to 6 years postoperatively.

Aortic Valve Stenosis↗
PubMed1994

New approach to great-vessel reconstruction in transposition complexes with interrupted aortic arch.

Interrupted aortic arch can be associated with transposition of the great arteries with ventricular septal defect and with the Taussig-Bing anomaly. This usually results in a marked disparity between the hypoplastic ascending aorta and the dilated main pulmonary artery. Currently, standard arterial switch and arch repair is a widely applied approach to the surgical management of these lesions. Though the morphology of the great vessels in these lesions does not preclude this approach, the great-vessel mismatch can result in difficulties at and beyond the proximal neo-aortic to ascending aortic suture line that cause excessive bleeding, obstruction, tension, distortion, and the need of patch augmentation of the ascending aorta or arch with nonviable material. We present an alternative surgical approach used in 2 patients, 1 with Taussig-Bing anomaly and interrupted aortic arch and the other with transposition of the great arteries, ventricular septal defect, and interrupted aortic arch, that greatly simplifies the reconstruction of the neo-aorta and repair of the interrupted arch and avoids these potential hazards.

Anastomosis, Surgical↗
PubMed2002

[Outcome of operated transposition of the great vessels].

The object of this report is to describe the long-term outcome of patients operated for transposition of the great vessels. Understanding what we mean by transposition of the great vessels, the surgical options with their advantages, limitations and complications, helps the cardiologist decide on the mode of follow-up, the investigations and even the reoperations that these patients may need. The authors review the results of the literature and their experience over the years with children and adults with congenital heart disease. Although considerable progress has been made in the management of a condition considered to be constantly and often rapidly fatal, most of the procedures which allow patients to have a normal or quasi-normal quality of life have not resolved all the problems and require maintenance of long-term follow-up.

Adult↗
PubMed1977

[Echocardiographic diagnosis of d-transposition of the great vessels by deductive analysis].

The process of diagnosis by echocardiography of transposition of the great vessels is based fundamentally on the recognition of the position and relative orientation of the two ventricles and of the two vessels of the base of the heart. Traditional two-dimensional echocardiography allows us to establish the position of the left ventricle because of the atrio-ventricular (mitral) valve and its direct continuity with a sigmoid valve. If this continuity is absent, the mitral valve may be recognised because it closes after the tricuspid valve. It the tricuspid valve is on the right of and/or in front of the mitral valve, the aorta will in principle be found on the right of the pulmonary artery ("loop rule"). The orientation of the great vessels may then be determined. Two-dimensional systems are particularly useful in diagnosis of vascular disorders. If these are not available, traditional echocardiography may lead us to suspect this abnormality: 1. Provided that the orientation of the transducer is known at all times during the recording; 2. Provided the recording demonstrates simultaneously the two great vessels with their respective valves. Two other criteria are important if Van Praagh's rule or the "loop rule" is unhelpful: the diameter of the two vascular trunks, and identification of the valve by a study of the ejection times.

Aorta, Thoracic↗
PubMed1979

Pulmonary vascular disease in transposition of the great vessels and intact ventricular septum.

Eight of 135 (6%) children with d-transposition of the great vessels and with intact ventricular septum and no patent ductus arteriosus had evidence of progressive pulmonary vascular disease. Seven of 101 (7%) patients for whom histologic data was available, had Heath-Edwards grades IV or V pulmonary vascular disease, six had grade II, and 88 had either normal or grade I findings. One of 34 patients for whom histologic data was not available had hemodynamic evidence of pulmonary vascular disease at cardiac catheterization after the Mustard operation. When infants younger than 3 months old were excluded, eight of 85 (9%) had advanced pulmonary vascular disease. Twenty-three patients had microthrombi in their pulmonary arteries and arterioles, and in one patient thrombi were observed before the development of pulmonary vascular disease. Clinically unrecognized pulmonary microthrombi are suggested as a possible etiologic agent in the development of pulmonary vascular disease in patients with transposition of the great vessels. Progressive pulmonary vascular disease can first be discovered after the Mustard operation, even in patients without preoperative evidence of pulmonary hypertension or elevated pulmonary vascular resistance.

Adolescent↗
PubMed1977

[Corrected transposition of the great vessels and preexcitation syndrome (apropos of 2 cases)].

Two cases with treated transposition of the great vessels and incompetence of the left atrioventricular valve showed a type B preexcitation syndrome. In one case, this consisted of a typical W.P.W. syndrome in which the second PR interval was not shortened, but rather consisted of a delta wave and a widened QRS complex. Post mortem examination showed an abnormal connection between the bundle of His and the ventricular septum, and a low insertion of the inverted tricuspid valve. The published cases of W.P.W. syndrome in cases with treated transposition are reviewed, and the mechanism of preexciation discussed in the light of the anatomical peculiarities of the malformation and of the abnormalities which are a feature of Ebstein's syndrome.

Child↗
PubMed2000

[Preoperative mortality in transposition of the great vessels].

UNLABELLED: The aim of the study was to report the incidence and causes of preoperative deaths in isolated transposition of the great vessels and to describe the clinical findings in these neonates. PATIENTS AND METHODS: In five French centers of pediatric cardiology, data of all the neonates with isolated transposition of the great vessels who died before arterial switch operations between January 1986 and June 1996 were obtained from reviewing hospital files, echocardiography records and autopsy reports. RESULTS: Among 199 neonates with transposition of the great vessels, 20 (9.9%) died before surgery. The death was related to intracranial haemorrhage in one premature neonate, severe and early hypoxemia in 13 full-term patients (group A) and later sudden collapse in six patients (group B). In group A, the symptoms occurred within 20 minutes after the birth and included cyanosis (n = 12), acute respiratory distress (n = 8), and shock (n = 4). Despite assisted ventilation (n = 13), bicarbonate infusion (n = 12), prostaglandin E1 (n = 7), inotropic drugs (n = 5) and balloon atrioseptostomy (n = 7), death occurred at the median age of five hours. The patent foramen ovale was absent or tiny in ten patients, normal in one patient and not specified in two patients. The ductus arteriosus was patent in ten patients and not specified in three patients. In group B, the neonates were initially in a good hemodynamic condition. Unexplained death occurred between two and five days after the birth: one infant with a large patent foramen ovale did not receive prostaglandin E1, four patients died a few hours after an angiographic study or a balloon atrioseptostomy was performed in a catheterization laboratory, and one child suffered from a cerebral anoxia due to a tightened cord. CONCLUSION: We conclude that the high preoperative mortality rate in isolated transposition of the great vessels is mainly due to absent or small atrial shunt. These findings suggest that only prenatal diagnosis of transposition of the great vessels with immediate balloon atrioseptostomy could avoid a fatal outcome.

Angioplasty, Balloon↗
PubMed1977

[Long term results (5 years) of the first 50 consecutive operations following Mustard's technique for simple transposition of the great vessels].

The long-term results of Mustard's operation for simple transposition of the great vessels have been studied in a homogenous series of the first 50 consecutive cases operated on by G. Lemoine. The overall mortality was 20% over the 5 year follow-up period. Serious arrhythmias or conduction defects can usually be picked up immediately postoperatively; on the other hand, stenosis of the venae cavae or of the pulmonary venous channels may come on very late and unexpectedly as growth takes place. Reoperation must be carried out to remove these stenoses when they are troublesome.

Adolescent↗
PubMed1975

An assessment of Mustard's operation as a palliative procedure for transposition of the great vessels.

Ten patients in whom palliative Mustard operations have been performed for transposition of the great vessels with ventricular septal defect and elevated pulmonary vascular resistance are reported. There has been early or late mortality in this group of patients, and postoperative morbidity has been minimal. Five of them have been recatheterized, and the results indicate substantial improvement in postoperative arterial oxygen saturation with change in pulmonary vascular resistance. The operation is reserved for those patients in whom peripheral desaturation is the major cause of symptomatology. Clinical improvement has been gratifying, and the continued use of the operation in selected patients seen indicated.

Adolescent↗