Objective: To evaluate the early and medium term results of operations for congenitally corrected transposition of the great arteries (CCTGA) wherein the left ventricle becomes the systemic ventricle. Methods: Fourteen patients with CCTGA who underwent anatomic repair from 1994 to 1998 were placed in one of two groups: those without pulmonic stenosis (PS) (N = 7) and those with PS (N = 7). Main associations in the group without PS were: severe left atrioventricular (AV) valve regurgitation ( N = 4), ventricular septal defect (VSD) (N = 4), criss-cross AV connection (N = 1), and hypoplasia of the morphologic RV (N = 1). Main associations in the group with PS were: VSD (N = 7), anomalous pulmonary venous drainage (N = 2) and major aorto-pulmonary collaterals (N = 1). All patients in the group without PS underwent an arterial switch operation and all except one had a modified senning repair for atrial rerouting for anatomic correction. All seven patients with PS underwent a Rastelli repair and all except one needed a modified senning repair. Results: Early survivorship in both groups was 6/7. Follow-up ranges from 1 month to 48 months. The mean left ventricular ejection fraction (LVEF) in the arterial switch and senning patients was 65% and in the Rastelli and atrial rerouting was 52%. None of the survivors except one have any significant mitral regurgitation (MR). The one exception resulted from iatrogenic damage to a chorda during VSD closure. All except two patients are class I symptomatic and all are in normal sinus rhythm. Conclusion: Satisfactory early and mid term results of anatomic repair support the double switch option as the procedure of choice for patients of CCTGA amenable to biventricular repair. © 1999 Elsevier Science B.V. All rights reserved.

/pdf/double-switch-for-congenitally-corrected-transposition-of-5qo1r52c0s.pdf

Double switch for congenitally corrected transposition of the great arteries.

The purpose of this review/editorial is to discuss how and when to treat the most common cyanotic congenital heart defects (CHDs); the discussion of acyanotic heart defects was presented in a previous editorial. By and large, the indications and timing of intervention are decided by the severity of the lesion. While some patients with acyanotic CHD may not require surgical or transcatheter intervention because of spontaneous resolution of the defect or mildness of the defect, the majority of cyanotic CHD will require intervention, mostly surgical. Total surgical correction is the treatment of choice for tetralogy of Fallot patients although some patients may need to be palliated initially by performing a modified Blalock-Taussig shunt. For transposition of the great arteries, arterial switch (Jatene) procedure is the treatment of choice, although Rastelli procedure is required for patients who have associated ventricular septal defect (VSD) and pulmonary stenosis (PS). Some of these babies may require Prostaglandin E1 infusion and/or balloon atrial septostomy prior to corrective surgery. In tricuspid atresia patients, most babies require palliation at presentation either with a modified Blalock-Taussig shunt or pulmonary artery banding followed later by staged Fontan (bidirectional Glenn followed later by extracardiac conduit Fontan conversion usually with fenestration). Truncus arteriosus babies are treated by closure of VSD along with right ventricle to pulmonary artery conduit; palliative banding of the pulmonary artery is no longer recommended. Total anomalous pulmonary venous connection babies require anastomosis of the common pulmonary vein with the left atrium at presentation. Other defects should also be addressed by staged correction or complete repair depending upon the anatomy/physiology. Feasibility, safety and effectiveness of treatment of cyanotic CHD with currently available medical, transcatheter and surgical methods are well established and should be performed at an appropriate age in order to prevent damage to cardiovascular structures.

Consensus on Timing of Intervention for Common Congenital Heart Diseases: Part II - Cyanotic Heart Defects

The morphologic variations of mixed totally anomalous pulmonary venous connection are many and varied. In this review, we give an account of all cases previously described as mixed totally anomalous pulmonary venous connection, analyzing in detail those cases where an accurate anatomical description was provided. We identified 182 suitable cases, from 54 investigations, and reviewed the clinical presentation, anatomic variations, diagnostic features, and management of the patients described.Cross-sectional echocardiography, and cardiac catheterization, provided the necessary diagnostic information, and defined the anatomy before surgery in 139 patients. Magnetic resonance imaging and computerized tomographic angiography had been used for further clarification of the pulmonary venous anatomy. An obstructive pattern of drainage, involving one or more pulmonary veins, had been described in over half of the patients. We then grouped the lesions into categories that have a bearing on the appropriate surgical approach, discussing the appropriate repair for each group. For the overall group, the operative mortality remains high, at 22.9%. We submit that an increased appreciation of various types of mixed totally anomalous pulmonary venous connection may well contribute to improved future surgical management.

A suggested new surgical classification for mixed totally anomalous pulmonary venous connection

Total anomalous pulmonary venous connection (TAPVC) is a rare congenital cardiac anomaly. Death usually occurs in the first 3 months of life in about 50 % of neonates with this disease, and no more than 20 % of patients survive to the age of 1 year. The patient with the longest recorded survival is 49 years old. Here, we report successful surgical treatment of this disease in a 56-year-old female patient. This patient was discharged in good health about 1 month after operation, with no evidence of recurrence of heart failure at 13 months' follow-up.

Surgical treatment of a 56-year-old woman with an intracardiac type of total anomalous pulmonary venous connection.

Professor IM Rao, from All India Institute of Medical Sciences, New Delhi, was a pioneering cardiac surgeon who, against overwhelming odds, developed congenital heart surgery in India. He made many contributions to the evolution of his specialty, spanning more than four decades and three countries. This is a brief report of his professional life and accomplishments.

Developing congenital heart surgery in India: The travails and triumphs of a pioneer.

Fourteen infants ranging in age from 18 days to 10 months (median age = 4 months) underwent surgical repair of total anomalous pulmonary venous connection (TAPVC) between September, 1988 and December, 1989. The anomalous drainage was supracardiac in nine, cardiac in two, infracardiac in one and mixed in one. One patient had a complex type of TAPVC. There were two hospital deaths, one a critically ill three week old infant with obstructed infracardiac TAPVC and the other with complex TAPVC. There was no late death. All twelve survivors are in NYHA functional Class 1. Postoperative echocardiography revealed unobstructed pulmonary blood flow in all of them. Surgical correction of TAPVC in infancy has been performed with gratifying results. A high index of suspicion, early diagnosis, prompt referral and an aggressive surgical approach are essential for success in this otherwise lethal condition.

Surgical treatment of total anomalous pulmonary venous connection.

Surgical treatment of major intracardiac lesions associated with discordant atrioventricular connexion

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Surgical treatment of total anomalous pulmonary venous connection.

Surgical treatment of major intracardiac lesions associated with discordant atrioventricular connexion