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Background— Implantable cardioverter-defibrillator (ICD) therapy in children and congenital heart disease patients is hampered by poor long-term lead survival. Lead extraction is technically difficult and carries substantial morbidity. We sought to determine the outcomes of ICD leads in pediatric and congenital heart disease patients. Methods and Results— The Pediatric Lead Extractability and Survival Evaluation (PLEASE) is a 24-center international registry. Pediatric and congenital heart disease patients with ICD lead implantations from 2005 to 2010 were eligible. Study subjects comprised 878 ICD patients (44% congenital heart disease). Mean±SD age at implantation was 18.6±9.8 years. Of the 965 total leads, 54% were thin (≤7F), of which 57% were Fidelis, and 23% were coated with expanded polytetrafluoroethylene. There were 139 ICD lead failures (14%) in 132 patients (15%) at a mean lead age of 2.0±1.4 years, causing shocks in 53 patients (40%). Independent predictors of lead failure included younger implantation age and Fidelis leads. Actuarial analysis showed an incremental risk of lead failure with younger age at implantation: 18 years ( P =0.015). The actuarial yearly failure rate was 2.3% for non-Fidelis and 9.1% for Fidelis leads. Extraction was performed on 143 leads (80% thin, 7% expanded polytetrafluoroethylene coated), with lead age as the only independent predictor for advanced extraction techniques. There were 6 major extraction complications (4%) but no procedural mortality. Conclusions— This study demonstrates that ICD leads in children and congenital heart disease patients have an age-related suboptimal performance, further compounded by a high failure rate of Fidelis leads. Advanced extraction techniques were common and correlated with older lead age. Clinical Trial Registration— URL: http://www.clinicaltrials.gov . Unique identifier: NCT00335036.

Introduction: Patients with congenital heart disease (CHD) frequently require implantable cardiac devices. Device infection or malfunction may necessitate lead extraction. Extraction may be challenging due to long lead dwell times, anatomic abnormalities, and prior cardiac surgery. Little is known about extraction outcomes in the CHD population. Methods and Results: This retrospective study included 41 CHD patients and 82 age and gender matched controls that underwent lead extractions at two centers between 2001-2014. Only patients with leads older than 12 months were included. There were 79 leads in CHD patients and 150 in controls. Patients with CHD had a mean age of 39±17 years at extraction and on average their leads had been implanted for 81±84 months, this was not significantly different from the control group. Eighty eight percent of CHD patients had 1 or more cardiac surgeries as compared to only 22% of controls (p Conclusions: Lead extraction can be safely performed in patients with CHD. Despite anatomic abnormalities and previous cardiac surgery, the outcome of lead extraction in patients with CHD is comparable to controls. Prior cardiac surgery may lower the risk for extraction complications.

A 12 year-old girl with corrected transposition (L-transposition of the great arteries with ventricular inversion) and congenital complete atrioventricular block underwent epicardial pacemaker implantation with a single ventricular lead at 1 week of life. Subsequently, she presented for routine follow-up care in the electrophysiology clinic with no symptomatic concerns. She denied chest pain. Her physical examination was unremarkable, with the pacemaker generator palpable in the left subcostal region. ECG at that time demonstrated ventricular pacing with 100% capture and no evidence of atrioventricular conduction. Her chest x-ray revealed that the pacemaker lead completely encircled the cardiac silhouette and prompted concern for cardiac strangulation (Figure 1). A subsequent computed tomography scan showed a likely restrictive effect on both ventricles caused by the pacemaker lead encircling the heart with concern …

Introduction: Although the vast majority of mitral valve prolapse (MVP) is benign, women with bileaflet MVP (biMVP), complex ventricular ectopy (VE), and abnormal T waves may comprise the recently described malignant biMVP syndrome. The mechanism of ventricular arrhythmia is unknown. To further characterize the arrhythmic substrate, we reviewed our center’s ablation experience in 6 biMVP patients with prior cardiac arrest and recurrent ICD shocks for drug refractory ventricular fibrillation (VF). Methods and Results: Six women with biMVP (median age 31.5 [range 24.2 - 58.7] years, EF 65 [45 - 67]%, all ≤moderate mitral regurgitation) experienced 6 (3 - 25) appropriate ICD shocks over 4.8 (2.8 - 10.7) years and underwent index ablation between 2/2007 - 10/2013. All had multiple VE morphologies (median 7 [3 - 24]) with variable coupling intervals but with a predominant VE trigger for the VF. A median 2 (1 - 4) VE foci were ablated. Sites of successful ablation of VF-triggering and other dominant VE were left ventricular papillary muscles [PM] (1 anterior, 1 posterior, 1 both), fascicles (1 anterior, 1 posterior), or both (1 both PM and posterior fascicle). Outflow tract VE was also present and targeted (1 left, 1 right)i. Two underwent repeat ablation (288 and 312 days) for recurrent complex VE without shocks, with different foci to the index ablation (1 posterior fascicle, 1 both fascicles). The VF-triggering VE in all patients was confirmed as originating from within the left fascicular system, which in 3/6 was at a papillary muscle. Acute procedural success was seen in all with no complications to date. A VF storm occurred within 24 hours of ablation in a single patient. At follow-up of a mean 662 (47 - 2099) days, 1 patient received a single shock (p=0.03 vs. preablation). Symptomatic VE was reduced in all; while 3/6 continue Class 1c antiarrhythmics and 5/6 have beta blockade. Conclusion: Malignant biMVP syndrome is characterized by fascicular and papillary muscle PVCs that trigger ventricular fibrillation, yet in all patients, the VE is multifocal. Ablation of at least one focus appears to improve symptoms and reduce shocks.

Baruteau and colleagues1 present findings that further the potential for a genetic basis for nonimmune isolated atrioventricular (AV) block. Their observations warrant further consideration in the evaluation and management of patients with no definitive cause of their cardiac conduction defect. Article see p 1469 Congenital complete AV block (CAVB) affects ≈1 in 20 000 live-born infants2 and is commonly associated with an immune-related cause associated with maternal collagen vascular disease or structural cardiac disease.3 Other causes of CAVB have been described, including infections, myopathies, and genetic disorders such as the Hunter and Hurler syndromes. Still, the specific cause of CAVB remains elusive for a significant number of patients, raising the possibility that a portion of idiopathic CAVB stems from CAVB-susceptibility genes. Currently, mutations in transcription factors and cardiac channels yield electrocardiographic phenotypes that include cardiac conduction abnormalities. For example, patients with NKX2.5 mutations can have cardiac conduction defects, cardiomyopathy, and atrial septal defects.4 Long-QT syndrome has also been associated with both 2:1 AV block ad CAVB, but this is usually in the presence of overt QT prolongation on the ECG.5 Mutations in the SCN5A- encoded Nav1.5 sodium channel have an ever-expansive …

A 4-year-old girl with a history of a moderate-sized secundum atrial septal defect and mild mitral valve prolapse underwent transcatheter closure with a 22-mm Amplatzer septal occluder (ASO) (AGA Medical Corporation, Golden Valley, Minn) device. Approximately 12 months later, she presented to the emergency department in septic shock with a 5-day history of fevers up to 105°F and was transferred to the pediatric intensive care unit. Her blood and urine cultures grew methicillin-sensitive Staphylococcus aureus within 24 hours. Multiple septic emboli were found on a brain magnetic resonance imaging scan. A transthoracic echocardiogram revealed vegetations on the mitral and tricuspid valves (Figures 1 and 2⇓ and Movies I and II), with a perforation in the mitral valve and severe mitral insufficiency. The patient was taken to the cardiovascular operating room, and severe mitral and tricuspid valve endocarditis was …

Introduction : Annual mortality in children with hypertrophic cardiomyopathy (HCM) has been reported to be 1– 6%. Risk factors for death in adult HCM patients have been characterized; their application to pediatric HCM is unknown. The purpose of this study was to correlate adult risk factors with outcomes in our pediatric population using a standard management strategy. Methods : A retrospective cohort study of children with HCM was performed. Death and cardiac transplant were the primary outcomes. Diagnosis was based on asymmetric septal or concentric hypertrophy as determined by echocardiography. Exclusion criteria included: genetic syndrome, mitochondrial or metabolic disorder, infants of diabetic mothers, congenital heart disease, systemic hypertension, diagnosis >18 years of age, or follow-up Results : From 1/1/85 to 10/1/06, 96 patients met inclusion criteria. Mean age at diagnosis was 10.6 ± 5.4 years. Mean follow-up was 6.5 ± 5.2 years. 11 patients had an adverse outcome (7 deaths, 4 transplants). Kaplan-Meier analysis predicts an 82% survival over 20 years. Evidence of left ventricular outflow tract obstruction (LVOTO) occurred in =6% of patients, syncope in 23% and a family history for malignant HCM in 17%. Aborted sudden death occurred in 6%. 10% had non-sustained ventricular tachycardia. 58% of patients underwent at least one exercise treadmill test. All patients were restricted from strenuous activity. 95% of patients were on a β-blocker or calcium channel blocker, with 10% on both. Additional intervention depended on symptoms, family history, and degree of LVOTO. 17% had an implantable defibrillator (ICD). 10% were given a pacemaker due to LVOTO, and 5% underwent left ventricular myectomy. Only extreme LVH (>6 z-scores for BSA) and a blunted blood pressure response to exercise were statistically significant for worse outcomes (both p Conclusions : A low mortality/transplantation rate occurred in children with isolated HCM whose management consisted of exercise restriction and medication, with or without an ICD. Management that included myectomy was uncommon. Patients with extreme LVH and a blunted blood pressure response to exercise are high-risk individuals.

This study was performed to determine whether a preoperative hemodynamic evaluation with oxygen and inhaled nitric oxide identifies patients with pulmonary hypertension who are appropriate candidates for corrective cardiac surgery or transplantation more accurately than an evaluation with oxygen alone.At 10 institutions, 124 patients with heart disease and severe pulmonary hypertension underwent cardiac catheterization to determine operability. The ratio of pulmonary and systemic vascular resistance (Rp:Rs) was determined at baseline while breathing approximately 21% to 30% oxygen, and in approximately 100% oxygen and approximately 100% oxygen with 10 to 80 parts per million nitric oxide to evaluate pulmonary vascular reactivity. Surgery was performed in 74 patients. Twelve patients died or developed right heart failure secondary to pulmonary hypertension following surgery. Rp:Rs0.33 and a 20% decrease in Rp:Rs from baseline were chosen as 2 criteria for operability to determine, in retrospect, the efficacy of preoperative testing in patient selection. In comparison to an evaluation with oxygen alone, sensitivity (64% versus 97%) and accuracy (68% versus 90%) were increased by an evaluation with oxygen and nitric oxide when Rp:Rs0.33 was used as the criterion for operability. Specificity was only 8% when a 20% decrease in Rp:Rs from baseline was used as the criterion for operability.By using a combination of oxygen and inhaled nitric oxide, a greater number of appropriate candidates for corrective cardiac surgery or transplantation can be identified during preoperative testing when a specific value of Rp:Rs is used as a criterion for operability.

Background This study was performed to determine whether a preoperative hemodynamic evaluation with oxygen and inhaled nitric oxide identifies patients with pulmonary hypertension who are appropriate candidates for corrective cardiac surgery or transplantation more accurately than an evaluation with oxygen alone. Methods and Results At 10 institutions, 124 patients with heart disease and severe pulmonary hypertension underwent cardiac catheterization to determine operability. The ratio of pulmonary and systemic vascular resistance (Rp:Rs) was determined at baseline while breathing ∼21% to 30% oxygen, and in ∼100% oxygen and ∼100% oxygen with 10 to 80 parts per million nitric oxide to evaluate pulmonary vascular reactivity. Surgery was performed in 74 patients. Twelve patients died or developed right heart failure secondary to pulmonary hypertension following surgery. Rp:Rs Conclusion By using a combination of oxygen and inhaled nitric oxide, a greater number of appropriate candidates for corrective cardiac surgery or transplantation can be identified during preoperative testing when a specific value of Rp:Rs is used as a criterion for operability.