Search

Objectives: The purpose of the study was to examine long-term outcome after traditional surgical treatment of corrected transposition of the great arteries to provide a basis for comparison with new procedures, such as the double-switch or Senning-Rastelli procedures., Methods: Patient- and procedure-related variables in 123 patients with corrected transposition and 2 functional ventricles operated on between 1963 and 1996 were analyzed. Patients with intracardiac procedures underwent either a traditional 2-ventricle repair or a Fontan procedure., Results: The 1-, 5-, 10-, and 15-year survivals after the operation were 84%, 75%, 68%, and 61%, respectively. Patients requiring tricuspid valve replacement (27 patients) at any time during follow-up had a significantly worse outcome ( P < .001; hazard ratio, 4.4), whereas the best outcome was seen in patients undergoing the Fontan procedure (17 patients, 0 deaths). Right ventricular end-diastolic pressure of greater than 17 mm Hg before the operation ( P < .0001), complete heart block after the operation ( P = .001), subvalvular pulmonary stenosis ( P = .013), Ebstein malformation of the tricuspid valve ( P = .025), and preoperative systemic (right) ventricular dysfunction ( P = .041) were identified as risk factors for death at any time by means of univariate analysis. Ebstein malformation of the tricuspid valve ( P = .036; hazard ratio, 1.5) was identified as a risk factor for death by multivariate analysis., Conclusions: The long-term outcome of patients with corrected transposition after a classic surgical approach is unsatisfactory. The poorest outcome was seen in patients who required tricuspid valve replacement either at their initial operation or later during follow-up. Alternative surgical approaches, such as the double-switch, Senning-Rastelli, or Fontan procedures, are likely to have better long-term results, especially in the highest risk groups.

Objective: Our purpose was to describe the outcome of the Rastelli repair in D -transposition of the great arteries and to determine the risk factors associated with unfavorable events., Methods: From March 1973 to April 1998, 101 patients with D -transposition of the great arteries and ventricular septal defect underwent a Rastelli type of repair. Median age and weight were 3.1 years (10th to 90th percentiles 0.3-9.9 years) and 12.8 kg (5.9-28.2). Pulmonary stenosis was present in 73 patients and pulmonary atresia in 18; 10 patients had no left ventricular outflow tract obstruction., Results: There were 7 early deaths (7%) and no operative deaths in the last 7 years of the study. Risk factors for early death, by univariable analysis, included straddling tricuspid valve (P =.04) and longer aortic crossclamping times (P =.04). At a median follow-up of 8.5 years, there were 17 late deaths and 1 patient had undergone heart transplantation. Forty-four patients had reoperations for conduit stenosis, 11 for left ventricular outflow tract obstruction, and 28 had interventional catheterization to relieve conduit stenosis. Nine patients had late arrhythmias, and there were 5 sudden deaths. Overall freedom from death or transplantation (Kaplan-Meier) was 82%, 80%, 68%, and 52% at 5, 10, 15, and 20 years, respectively. Freedom from death or reintervention (catheterization or surgical treatment) was 53%, 24%, and 21% at 5, 10, and 15 years of follow-up, respectively., Conclusions: The Rastelli repair can be performed with low early mortality. However, substantial late morbidity and mortality are associated with conduit obstruction, left ventricular outflow tract obstruction, and arrhythmia.

The technique of normothermic caval inflow occlusion was introduced primarily for relief of pulmonary valve stenosis and continues to be used for this purpose with excellent results. However, the technique may also be applied to a number of other lesions such as aortic valve stenosis in neonates and infants, for placement of outflow patches or valve excision for pulmonary atresia with intact ventricular septum, for atrial septectomy in children with restrictive atrial septum, and for other conditions wherein a period of up to 2 minutes of intracardiac exposure is sufficient. This report examines the early and long-term results in 140 children who underwent normothermic caval inflow occlusion at The Children's Hospital, Boston, Massachusetts, over the past 11 years. Ninety-four children underwent pulmonary valvotomy (early mortality 0%), 21 had aortic valvotomy (19%), 10 with pulmonary atresia and intact ventricular septum underwent various procedures (50%), 11 had atrial septectomy (9%), and there were four miscellaneous procedures (50%). Apart from avoiding many of the potential complications of cardiopulmonary bypass, inflow occlusion has provided as good or better short-term and long-term results as those obtained with cardiopulmonary bypass, particularly in infants and neonates, and also has proved more cost effective.

Infusion of prostaglandin E1 into the main pulmonary artery of an infant with interruption of the aortic arch and a closing ductus arteriosus resulted in dilation of the ductus arteriosus and improved systemic perfusion. Treatment with prostaglandin E1 is recommended for infants with interruption of the aortic arch, critical coarctation of the aorta, and other lesions in which systemic perfusion is limited by a restrictive ductus arteriosus.

To determine the accuracy and reproducibility of cardiac output determination by thermodilution (COT) in children, simultaneous outputs were measured by the Fick technique (COF) (using measured oxygen consumption) and thermodilution in 26 children, ranging in age from 8 to 86 months, who were undergoing cardiac catheterization. There was excellent correlation between mean output by thermodilution and by the Fick technique: COT = 1.10 COF -- 0.2 l/minute, R = 0.91. In three-quarters of the patients with COT differed by 15% or less and in none differed by more than 25%. Serial values of thermodilution outputs were reproducible in each patient with a SD of 5.5%. Our observations indicate that COT is accurate, reproducible, and valuable in the care of critically ill infants and children.

A total of 466 neonates with transposition of the great arteries, all less than 15 days of age, have been entered into a 20-institution study between Jan. 1, 1985, and June 1, 1987. Seventy-three percent were less than 48 hours old when entered into the study. Two hundred twelve of these underwent an arterial switch repair, and the 1-week, 1-year, and 2 1/2-year survival rates were 82%, 79%, and 78%, respectively. The usual coronary anatomy was present in 67% of the patients. The arterial switch repair was performed in 16 of the institutions. Six among these were shown to be "low-risk" institutions, with the prevalence of the demographic and surgical variables seeming to be the same in these as in the other institutions. The 1-week, 1-year, and 2 1/2-year survival rates after the arterial switch repair in low-risk institutions for simple transposition were 96%, 91%, and 90%, respectively; for transposition with ventricular septal defect they were 84%, 83%, and 83%. Only older age at operation (over 14 days of age), in the case of simple transposition, and transposition with ventricular septal defect were risk factors for death in these six institutions. Among the patients as a whole, freedom from reoperation for pulmonary outflow obstruction at 1 week and 1 year was 99% and 89%, respectively. A previous pulmonary artery banding and, possibly, one institution were identified as risk factors for reoperation. Inferences: In low-risk institutions, the arterial switch repair can be accomplished with good early results, which suggests the possibility that the late results will be better than after the atrial switch repair. Since young age was not a risk factor for the arterial switch repair of transposition and ventricular septal defect, this type of repair for this anomaly, as well as for simple transposition, may be more advantageously performed early in life than at 2 to 3 months of age.

Twenty-five children and young adults with complete heart block and 50 normal control subjects were maximally exercised on a treadmill and their electrocardiograms were analyzed for the presence and degree of ventricular ectopy activity. Sixty-eight % (17 of 25) of the heart block patients and significant ventricular ectopy (frequent unifocal ectopy or worse) on exercise compared to 2% (one of 50) of the controls (p = less than 0.01). In the patients with block there was a significant trend toward more frequent and more severe ectopy with increasing age (p = less than 0.05) independent of heart rate. More severe ventricular ectopy was also seen in those patients with QRS prolongation (p = less than 0.05). No significant differences were found between the 14 patients with isolated "congenital" heart block, the seven with associated defects, and the four with surgically acquired block. We believe that severe ventricular ectopy is common at exercise in patients with complete heart block and they may have an increased risk for sudden death.

The course and prognosis of 208 patients with an ascending aorta to pulmonary artery anastomosis is reviewed. Mortality rate during, or within one month, of surgery was 24 per cent (50/208) and late mortality rate, prior to repair, was 10 per cent (21/208). An additional 5 per cent (10/208) died during subsequent intracardiac repair. Congestive heart failure developed in 25 per cent (53/208), pulmonary artery hypertension in 17 per cent (12/72), and pulmonary vascular obstruction in 6 per cent (4/72). An increase in orifice size of the stoma with time was documented in eight patients. Additional subsequent palliative surgery was required in 22 per cent (45/208). Mortality rate was directly related to age at operation and was highest in neonates less than one week of age. In infants with tetralogy of Fallot, a preliminary comparison of mortality rate between palliative surgery and primary repair clearly suggests that the latter is the preferred method of treatment.