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Late follow-up of 1095 patients undergoing operation for complex congenital heart disease utilizing pulmonary ventricle to pulmonary artery conduits.

BACKGROUND: Pulmonary ventricle (PV) to pulmonary artery (PA) conduits have made possible the correction of many complex congenital cardiac anomalies. METHODS: Between April 1964 and January 2001, 1270 patients underwent operation with conduit placement from the PV to PA. The present study evaluates late outcome of 1095 patients (612 males, 483 females) having an operation before July 1992. Mean age was 9.6 +/- 8.2 years old. Diagnoses included pulmonary atresia/tetralogy of Fallot (459), transposition of the great arteries (TGA) (232), truncus arteriosus (193), double outlet right ventricle (DORV) (121), corrected TGA (49), septated univentricular heart (36), and other (5). A porcine-valved Dacron conduit was used in 730, homograft in 239, and non-valved conduit in 126. RESULTS: Early mortality decreased from 23.5% prior to 1980 to 3.7% for the most recent decade. Mean follow-up was 10.9 years (maximum, 29 years). Actuarial survival for early survivors at 10 and 20 years was 77.0% +/- 1.5% and 59.5% +/- 2.6%. On univariate analysis, clinical and hemodynamic factors associated with late mortality were male gender, older age at operation, higher post-repair PV/systemic ventricle (SV) pressure ratio, higher distal PA pressure, and longer bypass time (p < or = 0.01 for all). On multivariate analysis, independent risk factors for late mortality were male gender, older age at operation, diagnosis of TGA, corrected TGA, truncus, or univentricular heart, and PV/SV pressure ratio > or = 0.72 (p < or = 0.03 for all). Freedom from reoperation for conduit failure at 10 and 20 years was 55.5% +/- 2.0% and 31.9% +/- 2.7%. On multivariate analysis, independent risk factors for conduit failure were homograft conduit, diagnosis of TGA, younger age at operation, and smaller conduit size (p < or = 0.007 for all). Reoperation for one conduit replacement was performed in 306 patients, two conduit replacements in 55 patients, three in 6 patients, and four in 3 patients. Overall early mortality for conduit replacement in this series was 4.9%; it was 1.7% for patients operated on from 1989 through 1992. At follow-up, 84% of survivors were in NYHA class I or II. CONCLUSIONS: Operations that include conduit placement and replacement can be performed with low early mortality. Younger age at operation was associated with improved late survival. The diagnosis of TGA was associated with increased risk for conduit failure, and the durability of the homograft, in this series, was inferior to the porcine-valved Dacron conduit. Quality of life was excellent for most patients despite the need for reoperation.

Adolescent↗

Conduit repair for complex congenital heart disease with pulmonary atresia or right ventricular outflow tract obstruction. part I: surgical results.

From 1972 to 1981, 40 valved extracardiac conduits have been implanted to bridge the right ventricular outflow tract (RVOT) in 37 patients for different complex congenital cardiac anomalies. The patients ranged in age from 2 to 23 years (mean 8.5 years). Two Dacron prostheses with incorporated Lillehei-Kaster valves, 1 valveless Dacron conduit, 27 Hancock conduits, and 9 lonescu-Shiley conduits were chosen according to availability and the progress in conduit technology. Thirty-three patients had had up to 4 previous palliative procedure before conduit repair. Although the complication rate appears considerable, the mortality rate seemed to be unaffected by the high percentage of prior surgery. Exchange of the conduit became necessary in 2 children, because of conduit stenosis with calcification of the Hancock valve 5 and 7 years respectively after implantation, and in another patient an outgrown Lillehei-Kaster valve Dacron conduit had to be replaced 9 years after repair. Five children died in the early postoperative course: one in pulmonary failure and kinking of the conduit; one with right heart failure and pulmonary hypertension stage IV; 2 in myocardial failure, and one due to severe bleeding from myocardial failure, and one due to severe bleeding from the prosthesis. Three patients died in the late postoperative course 11 weeks, 4 years and 4 years respectively after conduit repair. Conduit surgery offers new possibilities for repair of complex cyanotic cardiac malformations. At present, however, none of the commercially available conduits is an ideal substitute. Our limited experience with the hemodynamic results of lonescu Shiley conduits is promising although longer observation periods are needed for a definitive judgement. At present, the largest possible conduit should be used whenever possible; problems of compression or kinking can be avoided with proper positioning of the conduit.

Adolescent↗

Low thoracic epidural anaesthesia for elective cholecystectomy in a patient with congenital heart disease and pulmonary hypertension.

A 52-year-old male with pulmonary hypertension secondary to partial anomalous pulmonary venous return unassociated with atrial septal defect was given thoracic epidural anaesthesia for elective cholecystectomy. Partial anomalous pulmonary venous return is a rare congenital anomaly characterized by increased pulmonary blood flow which in severe cases results in pulmonary hypertension subjecting the right ventricle to strain. An epidural catheter was placed at the T11-T12 interspace and anaesthesia was established to the T4 dermatomal level with bupivacaine (180 mg) and lidocaine (100 mg). Central venous pressure, pulmonary artery pressure, radial artery pressure, and cardiac output were monitored. There was minimal change in pulmonary arterial pressure, although there was a significant drop in systemic arterial pressure. Thoracic epidural block is recommended for upper abdominal surgery in clinical situations with pulmonary hypertension.

Anesthesia, Epidural↗