Nicholas H. Von Bergen

Permanent junctional reciprocating tachycardia in children: A multicenter experience

BACKGROUND Permanent junctional reciprocating tachycardia (PJRT) is an uncommon form of supraventricular tachycardia in children. Treatment of this arrhythmia has been considered difficult because of a high medication failure rate and risk of cardiomyopathy. Outcomes in the current era of interventional treatment with catheter ablation have not been published. OBJECTIVE To describe the presentation and clinical course of PJRT in children. METHODS This is a retrospective review of 194 pediatric patients with PJRT managed at 11 institutions between January 2000 and December 2010. RESULTS The median age at diagnosis was 3.2 months, including 110 infants (57%; aged <1 year). PJRT was incessant in 47%. The ratio of RP interval to cycle length was higher with incessant than with nonincessant tachycardia. Tachycardia-induced cardiomyopathy was observed in 18%. Antiarrhythmic medications were used for initial management in 76%, while catheter ablation was used initially in only 10%. Medications achieved complete resolution in 23% with clinical benefit in an additional 47%. Overall, 140 patients underwent 175 catheter ablation procedures with a success rate of 90%. There were complications in 9% with no major complications reported. Patients were followed for a median of 45.1 months. Regardless of treatment modality, normal sinus rhythm was present in 90% at last follow-up. Spontaneous resolution occurred in 12% of the patients. CONCLUSION PJRT in children is frequently incessant at the time of diagnosis and may be associated with tachycardia-induced cardiomyopathy. Antiarrhythmic medications result in complete control in few patients. Catheter ablation is effective, and serious complications are rare.

Takotsubo-Like Cardiomyopathy in a 17-Year-Old Male with a Pheochromocytoma

We report a case of a 17-year-old adolescent male with Takotsubo-like cardiomyopathy in the setting of pheochromocytoma who presented with hematemesis, hypertension, and pallor. Takotsubo-like cardiomyopathy is rarely reported in the pediatric population, and this is the first report in the pediatric literature of Takotsubo-like cardiomyopathy associated with both pheochromocytoma and an elongated course of the left anterior descending coronary artery.

Mycotic aneurysm in a child with history of coarctation of the aorta repair

A mycotic aneurysm is a rare condition occasionally seen in patients with a history of prior cardiac or vascular surgery. Here we report the presentation of a mycotic aneurysm in a pediatric patient at the site of prior aortic coarctation repair. This patients initial presentation suggested rheumatologic or oncologic disease, and after diagnosis he continued to show evidence of splenic, renal and vascular injury distal to the mycotic aneurysm site while being treated with antibiotics. We discuss the diagnosis, treatment and management of this condition.

Hemodynamic assessment with interventional support should be routine for primary electrophysiology procedures after atrial switch procedure.

OBJECTIVES We sought to review our current philosophy that all primary invasive electrophysiologic (EP) studies in patients with atrial switch procedures (ASPs) should undergo hemodynamic evaluation and have interventional expertise available. BACKGROUND Patients who have undergone an ASP for dextro-transposition of the great arteries have a high incidence of both hemodynamic and EP sequelae. We present our data to support the combined assessment approach for these patients. METHODS Hemodynamic evaluation and interventions performed concurrently during a primary invasive EP procedure in patients with ASP were reviewed. RESULTS A total of 18 patients underwent concurrent EP invasive procedure and cardiac catheterization. The median age was 31 (14-43 years) with the majority being male (67%). Patients underwent a total of 30 concurrent primary invasive EP procedure and cardiac catheterization; 14 (47%) of the catheterization procedure were interventional. Some of the catheterization procedures required more than one intervention with total of 19 separate interventions. There were nine (47%) unexpected interventions. The majority of patients (n = 14, 77.8%) had one or more abnormal hemodynamic finding including baffle obstruction (n = 13, 72%), elevated filling pressures (n = 3, 17%), and secondary pulmonary hypertension (n = 3, 17%). Non-EP-related interventional procedures included systemic or pulmonary venous baffle stenting for significant obstruction (n = 7). EP-related interventions included transbaffle puncture for ablation of left-sided reentry circuits (n = 5), closure of previously undiagnosed baffle leaks prior to pacemaker/implantable cardioverter defibrillator (ICD) placement to prevent paradoxical embolism (n = 3), superior baffle stenting to facilitate pacemaker/ICD lead placement (n = 2), and retrieval of retained transvenous pacemaker/ICD lead (n = 2). CONCLUSION Due to the frequency of abnormal hemodynamics or interventional needs, strong consideration for routine concurrent hemodynamic assessment and availability of interventional expertise is warranted during primary invasive EP procedures in patients post ASP.