Cardiopulmonary Bypass Strategy for a Cyanotic Child With Hemoglobin SC Disease.

Hemoglobin SC (HbSC) disease is a hemoglobinopathy that may produce sickling under conditions of hypoxemia, dehydration, and acidosis. We present a case of HbSC disease and tricuspid atresia, type IB. We describe management by cardiopulmonary bypass CPB using exchange transfusion at initiation of bypass and fractionation of collected blood, allowing platelet and plasma apheresis, as an option for patients unable to undergo this procedure off pump.

Slow pathway modulation in a patient with tricuspid valve atresia.

Owing to increased life expectancy, patients with grown-up congenital heart disease nowadays present various types of arrhythmias. We report treatment of a 27-year-old patient with tricuspid and pulmonary atresia who was referred to our department with symptomatic tachycardia. During electrophysiologic study, a diagnosis of typical AV-nodal re-entrant tachycardia was made, and he was successfully treated despite the described anatomic malformation.

Ablation of atrioventricular nodal reentrant tachycardia in a patient with tricuspid atresia guided by electroanatomic mapping.

We describe a case of ablation of atrioventricular nodal reentrant tachycardia in a patient with tricuspid atresia and L-malposition of great vessels using an electroanatomical mapping system integrated with cardiac magnetic resonance imaging. Atrial activation mapping during tachycardia identified the retrograde fast pathway proximal to the His bundle, observed in the left interatrial septum. Ablation was successfully completed below this area. Map integration with the patient's anatomy allowed a safe, individualized procedure.

Transvenous right atrial and left ventricular pacing after the Fontan operation: long-term hemodynamic and electrophysiologic benefit of early atrioventricular resynchronization.

We report a case of long-term, successful, endocardial atrioventricular pacing in a 32-year-old man who had severe heart failure and ascites after having undergone a Fontan procedure for tricuspid atresia 9 years earlier. The patient was referred to our hospital for Fontan revision. However, electroanatomic mapping of the right atrium revealed viable tissue at the interatrial septum above the os of the coronary sinus, and it appeared that the left ventricle could be paced from a coronary sinus branch. Therefore, instead of Fontan revision, an endocardial atrioventricular pacemaker was implanted transvenously. On 5-year follow-up, the patient remained in New York Heart Association functional class I and had not been readmitted to the hospital for congestive heart failure or arrhythmias. His atrial and ventricular leads continued to show excellent pacing and sensing results.

Ablation of atrioventricular nodal reentrant tachycardia in tricuspid atresia.

A 31-year-old woman with tricuspid atresia and a palliative Waterston shunt presented with intractable poorly tolerated supraventricular tachycardia. Electrophysiologic evaluation was consistent with AV nodal reentrant tachycardia. The fast pathway region was mapped electroanatomically during tachycardia and with constant rate ventricular pacing. Successful radiofrequency ablation was achieved by placement of lesions in an area adjacent and inferior to the His-bundle electrogram on the left side of the septum.

The failing Fontan circulation: successful conversion of atriopulmonary connections.

OBJECTIVES: Symptoms from low cardiac output or refractory atrial arrhythmias are complicating atriopulmonary (classical) Fontan connections. We present our experience of converting such patients to total cavopulmonary connections with and without arrhythmia surgery. METHODS: Between 1997 and 2002, 15 patients (mean age, 19.7 +/- 7.0 years) underwent conversion operations 12.7 +/- 3.5 years after atriopulmonary Fontan operations. Preoperative New York Heart Association functional class was I in 2 patients, II in 2 patients, III in 6 patients, and IV in 5 patients. Four patients underwent intracardiac lateral tunnel conversion alone, and 11 received extracardiac total cavopulmonary connection, right atrial reduction, and cryoablation. RESULTS: No mortality occurred. One patient had conduit obstruction in the immediate postoperative period requiring replacement, and another required a redo operation for endocarditis. Average hospitalization was 17.9 +/- 9.38 days; chest drains were removed on median day 4 (range, 1-29; mean, 7.4 +/- 7.58 days). At follow-up (mean, 42.6 +/- 22.1 months), late atrial arrhythmias had recurred in 3 of 4 patients with intracardiac total cavopulmonary connections (without ablation) and 1 of 11 patients with extracardiac total cavopulmonary connections with ablation. All patients are in New York Heart Association class I or II. Exercise ability (Bruce protocol) improved 69% from a mean of 6.18 +/- 4.01 minutes to 10.45 +/- 2.11 minutes (P <.05). Need for antiarrhythmic agents decreased postoperatively (patients receiving < or =1 antiarrhythmic: 9 preoperatively vs 15 at long-term follow-up, P <.05). No patient has required transplantation. Protein-losing enteropathy, which was present in 1 patient, improved transiently with conversion. There was 1 late death from gastrointestinal hemorrhage. CONCLUSIONS: Fontan conversion can be achieved with low mortality and improvement in New York Heart Association class and exercise ability. Concomitant arrhythmia surgery reduces the incidence of late arrhythmias.

Recent advances in pediatric cardiology--electrophysiology, transcatheter and surgical advances.

Whereas the medical advances were reviewed in the previous paper, electrophysiological, transcatheter and surgical advances will be the focus in this review. Greater understanding of the arrhythmias, development of non-pharmacological treatment, namely catheter ablation and internal cardioverter-defibrillator (ICD) and miniaturization pacemakers and IDs have occurred in the last decade so that the methods could be applied to smaller and more complex patient population. Surgery has been the traditional treatment option for palliation and correction of congenital and acquired heart defects in infants and children. During the last one to one and one-half decades, a remarkable number of transcatheter methods were developed and refined. These developments during the last decade were reviewed and include long-term results of balloon dilatation procedures, transcatheter closure of atrial septal defects, patent ductus arteriosus, and ventricular septal defect, percutaneous valve replacement, intravascular stents to manage vascular obstructive lesions that can not be satisfactorily balloon-dilated, catheter completion of Fontan procedure, myocardial reduction in hypertrophic cardiomyopathy and other miscellaneous procedures. Recent advances in the transcatheter modes of therapy have added a new dimension to the management of neonates, infants and children with heart disease. They should now be added to the armamentarium available to the Pediatrician and Pediatric Cardiologist in the management of cardiac problems in the pediatric patient. Surgical methods and concepts have been greatly refined such that surgery can be undertaken even in the sickest and most complex patient. The majority of congenital heart defects can be corrected by open heart surgery; some require prior palliation and others can be operated without prior palliation. Recent advances in various defects were reviewed.