Repair of aortic-left atrial fistula following the transcatheter closure of an atrial septal defect.

We describe the surgical treatment of an aortic-left atrial fistula that appeared 6 months after the placement of an Amplatzer septal occluder for closure of a large secundum atrial septal defect. Successful repair of the fistula was accomplished by a combined transatrial-transaortic approach similar to that used to repair the rupture of a sinus of Valsalva aneurysm.

Surgery for congenital heart disease in low-birth weight neonates: a comprehensive statewide Connecticut program to improve outcomes.

Low-birth weight (LBW) remains a significant risk factor in surgery for congenital heart disease (CHD). The timing of surgery and the choice of complete repair vs palliative measures are controversial issues. Delay of surgery to achieve weight gain may result in poorer outcomes. The results of a statewide, cardiac surgery protocol in LBW infants using specific selection criteria for repair vs palliation or delay over a 42-month period are reviewed. From September 1999 through February 2003, 32 low-birth weight infants (1,320 to 2,500 grams) underwent surgery for congenital heart disease in Connecticut. The congenital heart surgery programs in the two major pediatric centers in the state (Yale-New Haven Hospital and Connecticut Children's Medical Center) were amalgamated to form a joint program, with the same surgical team involved in all cases. Median gestational age was 34 weeks with 18 (60%) premature (< or = 37 wks). Median age at operation was eight days. Fourteen infants (44%) had recognized syndromes. Primary diagnoses included variant of single ventricle (8), ventricular septal defect (VSD) (4), tetralogy of Fallot (TOF) (2), pulmonary atresia (PA) with VSD (3), simple transposition of the great arteries (TGA) (1), TGA with VSD (TGA/VSD) (2), atrioventricular canal (AVC) (4), double outlet right ventricle (DORV) (3), aortic coarctation (CoA) (2), and interrupted aortic arch with VSD (IAA/VSD) (3). Eighteen patients (56%) were able to undergo procedures resulting in normal biventricular physiology. Eight patients (25%) had palliation for single ventricle, including two Norwood procedures. Seven patients potentially suitable for biventricular repair underwent palliative surgery because of contraindications to complete repair that were unrelated to weight. Follow-up (100%) ranged from two to 41 months. There were two early deaths (6%), one cardiac related (Norwood with ischemia), and one with sepsis. There was one early reintervention (PA stenosis following arterial switch). There were seven late deaths (22%), two cardiac related (one sudden death following Blalock-Taussig (BT) shunt, one sepsis related to low output), and four noncardiac. In three patients who received pulmonary artery banding (PAB), delay of surgery may have contributed to death due to progressive pulmonary disease. Of 23 long-term survivors, five have undergone biventricular repair following palliation, and two have had further palliation for univentricular heart physiology. A strategy of early surgical intervention favoring primary repair, or surgical palliation for those patients with single ventricle, results in good overall survival in symptomatic low-birth weight neonates with congenital heart disease. Delay in surgery due to LBW may not be beneficial in most cases and could result in lower overall survival and increased cost. A program utilizing statewide resources results in excellent outcomes for this challenging group of patients.

Effect of enalaprilat on postoperative hypertension after surgical repair of coarctation of the aorta.

OBJECTIVE: Hypertension in pediatric patients after surgical repair of coarctation of the aorta can be difficult to control and may lead to morbidity. The renin-angiotensin system mediates at least part of this hypertension. Enalaprilat, the only intravenous angiotensin-converting enzyme inhibitor, is used to treat hypertension in pediatric patients in other settings. However, its effect on postoperative hypertension during the early postoperative period in patients undergoing surgical repair of coarctation of the aorta is unknown. DESIGN: Prospective, randomized, double-blind study. SETTING: Operating room and the pediatric intensive care unit. PATIENTS: Fourteen consecutive pediatric patients between the ages of 1 and 18 yrs scheduled to undergo surgical repair of coarctation of the aorta. INTERVENTIONS: Patients were randomized to receive enalaprilat or saline placebo. Infusions were begun intraoperatively within 15 mins of aortic repair and repeated every 6 hrs. MEASUREMENTS AND MAIN RESULTS: Plasma renin activity was measured at baseline and on postoperative day 1. Blood pressure was determined at 30 mins and at 2, 4, and 6 hrs after infusion and scored relative to the preoperative blood pressure. The blood pressure in the enalaprilat group was consistently lower at 30 mins, 2 hrs, and 4 hrs after infusion (p <.05), but not at 6 hrs. Plasma renin activity was significantly lower in the placebo group on postoperative day 1. Length of stay in the pediatric intensive care unit trended shorter in the treated group. CONCLUSIONS: Conclusions are limited by a small cohort. Angiotensin-converting enzyme inhibitor therapy resulted in improved blood pressure control after coarctation repair. Further improvement of blood pressure control may be achievable by use of a larger dose of enalaprilat or a 4-hr enalaprilat-dosing interval.

Intraoperative radiofrequency ablation of the atrium: effectiveness for treatment of supraventricular tachycardia in congenital heart surgery.

BACKGROUND: Supraventricular tachycardia (SVT) is common in surgical patients with congenital heart disease. Ablation and maze operations have been shown to be effective in treating SVT, but these procedures can be complex and time-consuming because of variable anatomy and a thickened right atrium. To simplify and shorten these procedures, we used a long, flexible radiofrequency probe capable of producing long ablation lines quickly and effectively. We report the initial results with this procedure. METHODS: Six patients aged 6 weeks to 40 years with refractory SVT were referred for reoperation for repair of complex congenital heart disease (transposition of the great vessels, Ebstein's anomaly, single ventricle, tetralogy of fallot). Intraoperative radiofrequency ablation was performed in the right atrium for refractory SVT as an adjunct to surgical reconstruction (redo Fontan, right atrial reduction plasty, right ventricular outflow tract reconstruction, tricuspid repair). Lesions were made with a radiofrequency probe using temperatures of 70 degrees C for 60 seconds. Lesions were placed between the coronary sinus and the tricuspid valve, between the tricuspid valve and the inferior vena cava, between the atrial septal defect and the superior and inferior vena cava in patients with intraatrial reentry tachycardia/atrial flutter, and at the location of the accessory pathway in a patient with Wolff-Parkinson-White syndrome. The long, flexible probe has multiple independently controlled segments allowing ablation lesions that conform to the atrial morphology. RESULTS: An average of five intraoperative radiofrequency ablation lesions per patient were made. Average time for ablation was 14 minutes. With up to 25 months' follow-up, 5 patients are in sinus rhythm, and 1 is in a paced atrial rhythm. The patient with Wolff-Parkinson-White syndrome showed no preexcitation after operation. No complications resulting from intraoperative radiofrequency ablation were encountered. CONCLUSIONS: Intraoperative radiofrequency ablation in the atrium is a safe, effective, and expeditious procedure for control of SVT in patients undergoing reoperation for congenital heart disease with refractory SVT.