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PubMed1988

[Supravalvular pulmonary stenosis following anatomic correction of transposition of great vessels: report of a successful balloon dilatation].

Primary anatomical repair of TGA has several theoretical advantages over conventional atrial baffle techniques. However, one of the most common postoperative complications of the new technique is the development of an obstruction at the arterial anastomosis to the pulmonary artery (PA). A supravalvular pulmonary stenosis developed progressively over the first months after an arterial switch operation of a simple TGA in a newborn infant. The diagnosis was easily performed by Doppler-echocardiographic follow-up studies. Hemodynamic assessment by cardiac catheterization performed when the patient was five months old revealed a systolic gradient of 76 mm Hg at the arterial anastomosis. Percutaneous balloon angioplasty was successfully performed.

Catheterization↗
PubMed1975

[Transposition of great vessels].

Venous inflow stenoses either pulmonary or systemic represents a major complication after Mustard's operation. In order to eliminate such stenoses two modified methods of atrial in version have been developed using no or a minimum of foreign material. The indication for each method is different. Both methods have the incorporation of the remaining atrial septum and of a part of the left atrial wall into the new septum in common. The midportion is completed using a small Dacron patch in cases with fragile atrial tissue (method I) or a flap of the rightatrial wall in cases with normal tissue (method II). The incorporation of growing tissue into the new atrial septum minimizes the risk of secondary stenoses, due to shrinkage or excessive apposition of fibrous pannus.

Child↗
PubMed1996

[Treatment and prognosis of tachyarrhythmia after atrial surgical repair of transposition of great vessels].

The Mustard and Senning procedures may be complicated by atrial tachyarrhythmias. In order to determine their prognosis, the authors undertook a review of the 288 patients operated and followed up in their department. Thirty-six (12.5%) had documented atrial tachycardias. In 13 cases, Holter monitoring showed bursts of atrial tachycardia. In the other 23 cases, the tachycardia was sustained and often caused severe symptoms (syncope). Reduction of the sustained forms was obtained by rapid atrial pacing, cardioversion or amiodarone therapy. After restoration of sinus rhythm, several therapeutic protocols were used, often in the same patient: abstention, leading to 5 recurrences in 6 cases; treatment with betablockers in 12 patients with well tolerated or exercise-induced atrial tachycardia with 11 successes; amiodarone, with 4 relapses out of 5 when the dosage was less than 200 mg/m2/day and 13 successes out of 18 when the dosage was 200-250 mg/m2/day. During a follow-up of 1 to 19 years, 6 severe complications (neurological sequellae, cardiac failure), and 6 deaths, were observed. All these patients were poorly controlled and continued to be have tachyarrhythmias. The authors conclude that tachyarrhythmias are neither rare nor always symptomatic, so justifying their systematic investigation. They should be reduced by oral amiodarone or rapid atrial pacing depending on the clinical urgency, never by intravenous antiarrhythmics which can be dangerous. The poor prognosis associated with tachyarrhythmias makes their control essential either with betablockers or good doses of amiodarone, eventually covered by permanent cardiac pacing.

Adolescent↗
PubMed1975

[Balloon atrioseptostomy (B.A.S.) in the management of neonates and infants with transposition of great vessels (author's transl)].

From June 1969 to April 1973, B.A.S. was carried out in 2-day to 6-month-old 65 infants at the Institute of Paediatrics of the Academy of Medicine in Warsaw. There were 16 infants up to one week old, 39 infants--up to one month, and 10 infants more than one month old. There were 43 boys and 22 girls among them. Cardiac catheterization and B.A.S. were carried out in most children up to 24 hours following hospitalization under local anaesthesia and premedication with robenzperidol and dolantin. In 11 of the 65 infants after B.A.S. the saturation with oxygen in the right atrium under-went no significant changes; in 54 cases it increased by 10 to 49 per cent. Of the 65 infants in whom B.A.S. was performed, 37 are alive, 28 had died. In 20 children under constant outpatient cardiological follow up the observation period has amounted from 6 months to 3 years. Their motoric development and growth is retarded, there is moderate cyanosis, but no symptoms of congestive failure were found. All these children are administered digitalis in chronic maintenance doses. Respiratory infections occured frequently in these patients. As mentioned above, 28 infants died at the age of 2 days to 6 months. Post mortem examination revealed that the B.A.S. was unsufficient in 14 cases. However, 14 infants died in spite of the satisfactority performed atrioseptostomy. Pulmonary oedema or haemorrhagic-and-inflammatory changes in the lungs as well as generalized thrombosis were the most frequent causes of death. On the basis of their own experience the authors elaborated indications and instructions for B.A.S. in neonates and infants with congenital heart diseases. These directives are based on the Team Work of cardiologists, anaesthesiologists, cardiac surgeons and paediatric radiologists. Because ever greater numbers of neonates are being sent to the Institute of Paediatric of the Academy of Medicine from all over Poland, the authors organized continuous cardiological emergency service to carry out B.A.S. procedures as soon as possible, without delay.

Age Factors↗
PubMed1998

[5-year results of arterial correction in transposition of great vessels].

A prospective study was performed on in-hospital patients between June 1985 and July 1992 to assess the 5 year results of surgical detransposition of the great arteries. Clinical examination, electrocardiography, echocardiography, right and left heart catheterisation with selective coronary angiography, isotopic right and left ventricular ejection fractions at rest and with infusion of dobutamine and SestaMibi myocardial perfusion scintigraphy at rest and with dipyridamole, were performed during the 5th year after surgery. Twenty-six children underwent this protocol: eight others did not come for examination because they had moved from the region, one of whom had suffered regressive postoperative myocardial infarction. All patients were asymptomatic and had only minor electrocardiographic changes. Stenosis of the pulmonary tract was observed in 38.5% but only one case of stenosis at the origin of the right pulmonary artery required percutaneous angioplasty, which was successful. Pulmonary regurgitation was a common echocardiographic finding (65.4% of cases) but rarely severe (1/26: 3.9%). Aortic regurgitation was also observed commonly (53.8%), nearly always mild, grade I (13/14 cases). No significant stenosis of the aortic anastomosis was observed. The right and left ventricular ejection fractions were normal at rest except in one case and all values improved with dobutamine. Myocardial scintigraphy did not show any perfusion defect and there was no stenosis or occlusion of the coronary arteries at coronary angiography. The authors conclude that the results of arterial detransposition at 5 years are satisfactory in this series, with no cases of major obstructive lesions, major ventriculo-arterial regurgitation, ventricular dysfunction or coronary lesions. However, longer term trials with larger numbers of patients are required to determine the real incidence of coronary lesions and the long-term outcome of the pulmonary valve in the systemic position.

Cardiac Catheterization↗
PubMedDate not supplied

[Total transposition of the great vessels: Surgical treatment].

One hundred nine cases of complete transposition of great vessels alone or associated to other abnormalities are presented. In 91 cases a palliative treatment is given and in 18 cases there is a total correction done, depending upon the technique. Surgical indication and the technique used in every case is commented, making special reference to transpositions with ductus arteriosus or associated to coarctation of the aorta. In the 18 cases where we have done a total correction, we comment the surgical procedure of one of them who had a subpulmonary stenosis of the fibromuscular type, which we resolved through left ventriculotomy done on the apex of the ventricle and resecting the stenosis with an electroscalpel, which is a technique we have devised.

Age Factors↗
PubMed1964

THE ROLE OF SURGERY IN THE TREATMENT OF TRANSPOSITION OF THE GREAT VESSELS.

In 28 infants and children with complete transposition of the great vessels, atrial septal defects were created utilizing an open technique with inflow caval occlusion and moderate hypothermia. Of the 12 infants for whom operation was necessary during the first two weeks of life only two survived, suggesting that this technique is not adequate for infants at this age. Only four of the 16 children operated on between the ages of two weeks and three years failed to survive. Two of these died because of pre-existing non-cardiac conditions. Seven children with associated systemic-to-pulmonary shunts survived; the open technique may be preferable in this particular group. Despite the initial improvement afforded by this procedure, three sudden late deaths occurred. For this reason, and because of the danger of early development of pulmonary vascular disease, total operative correction of the malformation should be performed early.

Angiocardiography↗
PubMed1989

[Experience in the use of the Mustard operation in the surgical treatment of transposition of the great vessels].

In the 1980-1988 period, Mustard's operation was used to treat transposition of great vessels at the Department of Younger Children, A. N. Bakulev Institute of Cardiovascular Surgery. Seventy nine patients were operated on: 56 with simple transposition, 9 (16%) patients died; 23 with complex transpositions of great vessels (18 with concurrent ventricular septum defect, 5 with Taussig-Bing's disease), 5 (22%) patients died. Rhythm disturbances and obstruction of the venae cavae were most common postoperative complications. Six patients underwent Mustard's operation with closure of ventricular septal defect. In 18 patients this operation was performed as a palliative procedure due to high pulmonary hypertension. Lung biopsy showed the majority of patients with concurrent transposition and interventricular septal defect to have grade III-IV changes (by Hit-Edwards' classification) in the pulmonary vessels. Long-term hemodynamic studies showed good effect of palliative Mustard's operation.

Child↗
PubMed1976

[Reconstruction of the pulmonary outflow tract with a valved conduit (author's transl)].

The authors describe 25 patients who underwent open heart surgery for reconstruction of the pulmonary outflow tract with a valved conduit. The following anomalies were observed: six cases of great vessels transposition, five cases of pulmonary artery atresia, five cases of truncus, three cases of tetralogy of Fallot and six cases of single ventricle. Two cases of single ventricle were treated in order to be transformed into a systemic ventricle. The pulmonary outflow was reconstructed with a valved conduit anastomosed between right atrium and pulmonary artery. This study includes pre- and postoperative hemodynamic values, surgical evaluation and a 5-months to 3-years follow-up. The authors emphasize that it is important that the surgical indications are closely related to the morphology and hemodynamics of pulmonary circulation for a better result.

Adolescent↗
PubMed1975

[Clinical diagnosis of corrected transposition of the great vessels by bulbo-ventricular inversion].

29 cases of corrected transposition of great vessels (CTGV), of which 3 cases were isolated and 26 cases were associated with various malformations were studied. The aim was to make the diagnosis of CTGV were made evident, to differentiate those which came from the associated malformations. The specific signs for CTGV are as follows: 1) a slight systolic ejection murmur at the 2nd or 3rd left intercostal space along the sternal margin; 2) a loud 2nd tone in the same focal zone. Specific radiographic signs are that: 1) the aortic arch comes up to sternal-costal articulation; 2) the inferior left margin has a convex form; 3) in the right oblique-anterior projection it is possible to see 2 imprints on the opaque esophagus, which is represented by the aorta above and pulmonary artery below...

Adolescent↗
PubMedDate not supplied

[Congenitally corrected transposition of the great vessels throughout ventricular inversion (author's transl)].

Congenitally corrected transposition of the great vessels is a cardiac anomaly in which the pulmonary artery and the aorta are transposed in relation to one another but in which the flow of blood is maintained in the physiologic direction. In this condition there is a concomitant inversion of the ventricular chambers. 8 patients--5 adults and 3 children--with this condition have been reviewed with special attention to clinical symptoms and electrocardiographic and roent-genologic features. The anomaly is of importance because it is much more common than formerly believed. Frequently associated cardiac defects are correctable by surgical procedures. Therefore a preoperatively firmly established disgnosis is necessary by angiocardiography and heart catheterization.

Adolescent↗
PubMed1978

Sickle cell anemia and transposition of the great vessels.

A child with homozygous sickle cell disease and transposition of the great vessels had erythrocytosis associated with markedly increased plasma erythropoietin activity. Her clinical course was complicated by neurologic manifestations but not by recurrent sickle cell vasooculsive episodes. The fetal hemoglobin level which had been greater than 25% during the first two years of life gradually decreased to less than 10%. She died at 3 years of age of congestive heart failure and severe anemia. The only sickle cell painful crisis occurred during her terminal illness. It is likely that the high levels of fetal hemorglobin decreased sickling and thus allowed erythrocytosis to develop. Fetal hemoglobin may also have prevented frequent vaso-occlusive events despite the high hematocrit level.

Anemia, Sickle Cell↗