Transposition of the great vessels in infancy.
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We reported on the autopsy of a 35-year-old man who had mixed levocardia with situs inversus including atrial inversion, transposition of the great vessels, and other complex cardiac malformations. Levocardia was discussed.
BACKGROUND: A new operation to relieve pulmonary obstruction is proposed for patients with corrected transposition of the great vessels and pulmonary stenosis (PS). A right transatrial approach involves excising or detaching the right-sided atrioventricular valve (AV). Next, the pulmonary outflow tract is opened wide with an incision extending from the right-sided ventricle upward across the AV valve annulus. This incision extends into the main trunk of the pulmonary artery located behind the right atrium. A patch, with or without implantation of a pulmonary valve prosthesis, widens the outflow tract, thus avoiding use of an extracardiac conduit. METHOD: We describe this operation performed in a 51-year-old man who had previously undergone correction with an extracardiac conduit that had become obstructed. The patient had severe right-sided AV valve insufficiency and complete heart block with a functioning transvenous pacemaker. We replaced the right-sided AV valve and positioned the permanent pacemaker lead outside of the prosthetic skirt. The PS was corrected as described above and a pulmonary prosthetic valve implanted. Use of an extracardiac conduit was avoided altogether. CONCLUSION: This technique may be applicable even without excising the right-sided AV valve. The clinical result for our patient is still optimal 4 years after surgery.
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Calf blood flow was measured by venous occlusion plethysmography using a mercury in rubber strain gauge in infants with transposition of the great vessels. (TGV), and in comparable infants free from cardiopulmonary disease. Resting calf blood flow in the infants with TGV was 3.6 +/- 0.8 ml/100 ml/min, while in the control group flow was 6.8 +/- 2.3 ml/100 ml/min, a highly significant difference. We postulate that newborns with TGV decrease their resting calf flow in response to chronic hypoxia.
The arterial switch operation has become the preferred surgical procedure for transposition of the great arteries worldwide. The low operative mortality at "low-risk" institutions has been well documented. The advantages of the arterial switch compared with atrial-level repairs include a lower incidence of arrhythmias and the likelihood of normal systemic ventricular function over the long term. However, the long-term sequelae of this operation must be continually evaluated, including the fate of the supravalvular pulmonary and aortic anastomoses, growth of the aortic root, competency of the neoaortic valve, patency of the coronary arteries, effects on the conduction system, and adequacy of ventricular function. These anatomic results, as well as the neurodevelopmental outcomes of these patients, are summarized in this review. Copyright 1998 by W.B. Saunders Company
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We report the observation of a female patient with a corrected transposition of the great arteries, ventricular septal defect and complete atrio-ventricular block. This complex cardiac disease originated heart failure and was discovered at the age of 4 months. This baby has been operated at the age of 7 months, in April 1998. She had a complete repair by the double switch, closure of the ventricular septal defect and implantation of a permanent pace-maker. Short term results are favorable. Total repair of the corrected transposition of the great arteries is currently possible thanks to the recent technique of the double switch. It remains a difficult operation but with satisfactory short term results.
Sinus node dysfunction after intra-atrial repair of transposition of the great arteries by a Mustard or Senning procedure is well known. We undertook this study to evaluate the frequency, the nature, the severity and evolution of these dysrhythmias by performing Holter monitoring in 123 children followed up over 5 years; 302 Holter recordings were reviewed. The patients were divided into 3 groups of increasing severity: I = no sinus node dysfunction, II = moderate sinus node dysfunction, III = severe sinus node dysfunction with bradycardia of less than 30/min and/or pauses of over 2000 ms. The association of atrial hyperexcitability was classified in 3 subgroups: A = no extrasystoles, B = at least 4 extrasystoles per 24 hours, C = atrial tachycardia (focal tachycardia or flutter) after the first six postoperative months. There were only 15% of normal recordings (IA) and the majority of children (58%) were classified in the intermediary groups (IB, IIA and IIB). Sinus node dysfunction tended to become more severe with time in nearly 30% of the 69 cases followed up sequentially. The bradycardia tended to become more severe and associated with episodes of atrial tachycardia: the frequency of type B and C increased to 30% in Group I, to 68% in Group II and to 91% in Group III. The attacks were severe, especially in patients with a mediocre postoperative haemodynamic result. This explains the global mortality of 3%, the morbidity of 15% and the pacemaker implantation rate of 12%.(ABSTRACT TRUNCATED AT 250 WORDS)
The specialized atrioventricular (A-V) conduction system was electrophysiologically delineated during open-heart surgery in four patients with congenitally corrected transposition of the great vessels and associated ventricular septal defect and one patient with single ventricle. Two consistent observations were made: 1) In no case was the specialized A-V conduction system found in the right atrium, whether or not there was a coronary sinus ostium present. 2) Specialized A-V conduction system electrograms were never delineated posterior to the ventricular septal defect, in contradistinction to this usual location in hearts having ventricular septal defects associated with other congenital lesions. In three of five patients, the initial course of the A-V conduction system of the ventricles was delineated between the anterior aspect of the ventricular septal defect and the pulmonary artery. In one patient the proximal portion of the A-V conduction system was delineated on the anterior aspect of the pulmonary conus. The course and extent of the A-V conduction system delineated in the morphological left ventricle suggests it is a left bundle branch. The surgical implications of the ectopic location of the A-V conduction system anterior to the ventricular septal defect, and the variability of the more proximal portion of the A-V conduction system are discussed.
Less than 60 cases of bilateral chylothorax have been previously reported, and only two of these involve complicated Mustard procedures. We describe herein a patient in whom severe bilateral chylothorax developed three weeks after Mustard repair of D transposition. Complete reversal of this condition was obtained with revision of the constricted interatrial baffle and ligation of the thoracic duct. This cases is compared clinically with previously reported instances of chylothorax, and the role of played by obstruction of the superior vena cava after a Mustard procedure for transposition of the great vessels is emphasized.
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