Atrial Arrhythmia Ablation in Patients With D-Transposition of the Great Arteries and Atrial Switch.

BACKGROUND: Patients with D-transposition of the great arteries and atrial switch have a high incidence of atrial arrhythmias. We sought to analyze the arrhythmia substrate, ablation strategies, and outcomes for catheter ablation in this population. METHODS: An in-depth analysis of all clinical and procedural data in patients with D-transposition of the great arteries, atrial baffles, and atrial arrhythmia ablation was performed. RESULTS: A cohort of 32 patients (72% male, mean age 38±7 years) underwent ablation for non-AV nodal reentrant tachycardia atrial arrhythmias, and 4 patients underwent AV nodal reentrant tachycardia ablation. Cavotricuspid isthmus flutter (CTI-flutter) was the most common arrhythmia, encountered in 75% of patients, followed by scar-related intraatrial reentrant tachycardia (non-CTI intraatrial reentrant tachycardia, 53%) and focal atrial tachycardia (focal atrial tachycardia, 6%). Among the 32 patients, 26 underwent 31 procedures at our institution. For patients with prior outside intervention, the index ablation at our institution revealed CTI-dependent flutter in 3/5 cases. However, redo ablation after an index ablation with demonstrated bidirectional CTI block revealed different/new arrhythmia substrates (80% non-CTI intraatrial reentrant tachycardia, 40% focal atrial tachycardia). Achieving bidirectional block across the CTI often required ablating on both sides of the baffle (retroaortic access, 81%; using a baffle leak, 11.5%; or transbaffle puncture, 7.7%). Combined approaches were necessary in 19% to reach the critical tissue. Acute procedural success was 81%, and recurrence was documented in 58% of patients. Despite recurrence, clinical arrhythmia burden was significantly reduced post-ablation (P<0.001), with rare episodes, amenable to antiarrhythmic therapy. Redo ablation was required in 5 (19%) patients and uncovered new arrhythmia substrates. AV nodal reentrant tachycardia ablation also required transbaffle approaches in 3/4 patients. CONCLUSIONS: CTI-dependent flutter was the most common arrhythmia in patients with Dextro-Transposition of the Great Arteries and atrial switch. Transbaffle approaches were often necessary, and, provided that bidirectional CTI block was achieved at the index ablation, late recurrence was due to different arrhythmia mechanisms. Despite recurrence, ablation was associated with significant clinical improvement.

Adults with congenital heart disease and previous intervention.

Cardiac malformations occur in approximately 1% of live births. Advances in surgery, interventional cardiology, and medical care have translated into increasing numbers of adult patients with congenital heart disease. These patients, even after intervention, have cardiac sequelae that require specialized care by cardiologists and cardiac surgeons with expertise in the management of congenital cardiac disease. We review 8 of the most common lesions encountered after intervention in an adult congenital cardiac practice and discuss longitudinal follow-up, with a focus on appropriate testing, common hemodynamic and electrophysiological issues, and indications for reintervention.

Thrombolytic therapy in the treatment of right-sided prosthetic valve thrombosis in adults with congenital heart disease.

Valve thrombosis is a potentially dangerous complication for right sided prosthetic valves in adults with congenital heart disease. Thrombolytic therapy is an important alternative to surgery and may be of benefit in patients with a history of multiple surgical interventions. We present two cases of successful thrombolytic therapy for right sided valve thrombosis in patients with congenital heart disease.

The use of octreotide to successfully treat protein-losing enteropathy following the Fontan operation.

Octreotide has had limited use for the treatment of protein-losing enteropathy following the Fontan operation. We describe three cases where subcutaneous octreotide was successfully used to treat refractory protein-losing enteropathy following the Fontan operation. Patients received octreotide therapy for a period of 14-28 months. Octreotide was discontinued in one patient due to symptomatic cholelithiasis; this patient died 7 months after treatment was discontinued. Octreotide may be a useful treatment in these patients, but further study on its efficacy and mechanism of action is needed.

Pregnancy and delivery in patients with fontan circulation: a case report and review of obstetric management.

UNLABELLED: The Fontan connection, originally described in 1971, is used to provide palliation for patients with many forms of congenital heart disease that cannot support a biventricular circulation. An increasing number of women who have undergone these connections in childhood are now surviving into adulthood, and some are becoming pregnant. The low flow and fixed cardiac output of a Fontan circulation pose a number of problems during pregnancy. Here, we report a case of a woman who underwent a Fontan procedure at age 7 and experienced significant cardiovascular decline before successfully delivering a viable infant at 33 weeks gestation. In addition, we reviewed the pertinent published data in this area, which suggests that pregnant patients with a Fontan circulation are more likely to face obstetrical, rather than cardiovascular, complications, including preterm labor, intrauterine growth restriction, an increased risk of cesarean section, and the potential need for anticoagulation. The review provides the obstetrician with the information needed to take a prominent role in the appropriate management of this rare, but growing, patient population. TARGET AUDIENCE: Obstetricians & Gynecologists, Family Physicians Leaning Objectives: After completion of this article, the reader will be able to describe the Fontan circulation, describe the importance of the collaborative practice model for patients with Fontan circulation, and identify potential complications in the pregnancy of women with Fontan circulation.