Ex vivo cryoablation of Wolff-Parkinson-white in a donor heart prior to pediatric heart transplantation.

This report describes the use of a donor heart with ventricular pre-excitation for pediatric orthotopic heart transplantation and the successful surgical cryoablation of the donor heart prior to transplantation. The issues related to the preoperative evaluation and surgical management of the donor heart with Wolff-Parkinson White syndrome are discussed.

Left coronary artery arising from the non-coronary sinus: a rare congenital coronary anomaly.

Anomalous origin of the left coronary artery is a cause of sudden death in adolescents and young adults. Most commonly, it originates from the right coronary sinus or the pulmonary artery. Origin of the left main coronary artery from the non-coronary sinus of Valsalva is extremely rare. We report a case of a child with anomalous origin of the left main coronary artery from the non-coronary sinus diagnosed during the evaluation of a ventricular arrhythmia.

Ultrasound-assisted cannulation of the right internal jugular vein during electrophysiologic studies in children.

Percutaneous access of the right internal jugular vein for coronary sinus cannulation in pediatric patients undergoing electrophysiologic studies may be technically difficult. We report the use of an ultrasound-guided technique for obtaining jugular venous access. Forty-five pediatric patients who underwent electrophysiologic study were analyzed. Access was obtained in 100 % of the patients using this technique with no major complications. Ultrasound guidance for access of the internal jugular vein for coronary sinus cannulation during electrophysiologic studies in pediatric patients, may increase the success rate and prevent the development of complications.

Bulboventricular foramen resection: hemodynamic and electrophysiologic results.

BACKGROUND: The two major surgical approaches to the relief of bulboventricular foramen (BVF) obstruction in patients with single left ventricle (LV) are the Damus-Kaye-Stansel (DKS) procedure or direct BVF resection. Theoretical advantages of the DKS include better out-flow gradient relief, lower potential incidences of postoperative heart block and lower incidences of reoperation. Potential disadvantages of this approach include increased semilunar valvar insufficiency, lack of feasibility when attempting septation-type operations for univentricular hearts, and a technically more difficult operation. We report the results of direct surgical BVF resection. METHODS: From June 1990 to June 1999, 9 patients had direct BVF resection performed at our institution. The median age at surgery was 16.5 years (range 1 month to 27 years). Diagnoses in these patients were [S,L,L] single LV (n = 8) and [S,D,D] single LV tricuspid atresia (n = 1). Eight of 9 patients had pulmonary artery bands placed either before BVF resection or at the same time as this procedure. Three patients required reoperation for reobstruction at the BVF (12 total operations in 9 patients). RESULTS: Median preoperative peak systolic gradient across the BVF measured at cardiac catheterization was 47 mm Hg (range 10 to 63 mm Hg). The median peak gradient measured by Doppler echocardiography was 44 mm Hg (range 5 to 125 mm Hg). Eight of 9 patients survived the operation to discharge from the hospital and 7 of 9 are alive at follow-up. At a median follow-up of 22 months (range 5 to 76 months), 8 of 8 surviving patients had an unobstructed BVF as determined by qualitative two-dimensional echocardiography and Doppler color flow imaging. There was one perioperative and one late death 5 months postoperatively (secondary to fungal sepsis). No patient developed new or worsened aortic insufficiency after BVF resection. Eight of 9 patients had no change in AV nodal conduction after surgery. One patient developed Mobitz II heart block requiring postoperative implantation of a pacemaker. CONCLUSIONS: Direct resection of an obstructive BVF can be performed with total relief of obstruction although reoperation may be required. Atrioventricular nodal function can be preserved in most patients with this operative approach, including those with [S,L,L] segmental anatomy.

Torsade de pointes in a child with acute rheumatic fever.

Ventricular arrhythmias are uncommon in acute rheumatic carditis. We report the case of a child who presented with rheumatic carditis, prolonged corrected QT interval, and torsade de pointes. The episodes of torsade were controlled with beta-blockade and cardiac pacing. The child subsequently died as a result of brain injury; the autopsy revealed classic findings of acute rheumatic carditis.

Continuous intravenous diltiazem infusion for short-term ventricular rate control in children.

Intravenous diltiazem was administered to 10 pediatric patients with primary atrial tachyarrhythmias with rapid ventricular response. Rapid, consistent, and safe temporary ventricular rate control was obtained in all patients given this medication.

A new technique for endomyocardial biopsy in infants and small children.

Endomyocardial biopsy (EMB) is usually performed from the femoral veins in infants and small children. This requires the use of a long vascular sheath to direct a bioptome to the ventricular septum. We describe a technique of EMB in this population that obviates the need for the use of a long vascular sheath. A 5 Fr 0.038" internal diameter, no-sideholes Cobra catheter (Cook, Bloomington, IN) introduced via a 5 Fr short sheath was used to direct a 3 Fr, 60-cm-length Cook bioptome to the ventricular septum. From January 1998 to August 1999, this technique was used 22 times in seven patients for EMB (median, age 13 months; range, 1-72 months). Indications for EMB in this group were posttransplantation surveillance (21 times) and evaluation for cardiomyopathy/myocarditis (1). Median number of EMB specimens obtained per procedure was 6 (range, 5-9). There was one pneumopericardium seen post-EMB with this technique without hemodynamic embarrassment. EMB in infants and small children can be performed successfully, safely, and repeatedly from the femoral veins without the need for a long sheath.

Radiofrequency ablation of an accessory pathway in a surgically created atrioventricular Fontan anastomosis: case report and review of previous published cases.

Supraventricular tachyarrhythmias following the Fontan procedure can be life-threatening. Though most are commonly due to intraatrial reentry, orthodromic reentrant tachycardia may also be present. Atrioventricular accessory pathways may develop across suture lines following right atrial to right ventricular anastomosis in patients with tricuspid atresia. We report a case of a patient who underwent this type of Fontan who developed orthodromic reentrant tachycardia and heart failure. An electrophysiological study revealed the presence of an atrioventricular accessory pathway traversing the Fontan anastomosis suture line. Successful radiofrequency ablation of the accessory pathway led to control of the tachyarrhythmia and improvement of heart failure.