Joseph Atallah

Neurodevelopmental Outcomes After Cardiac Surgery in Infancy

BACKGROUND: Neurodevelopmental disability is the most common complication for survivors of surgery for congenital heart disease (CHD). METHODS: We analyzed individual participant data from studies of children evaluated with the Bayley Scales of Infant Development, second edition, after cardiac surgery between 1996 and 2009. The primary outcome was Psychomotor Development Index (PDI), and the secondary outcome was Mental Development Index (MDI). RESULTS: Among 1770 subjects from 22 institutions, assessed at age 14.5 ± 3.7 months, PDIs and MDIs (77.6 ± 18.8 and 88.2 ± 16.7, respectively) were lower than normative means (each P < .001). Later calendar year of birth was associated with an increased proportion of high-risk infants (complexity of CHD and prevalence of genetic/extracardiac anomalies). After adjustment for center and type of CHD, later year of birth was not significantly associated with better PDI or MDI. Risk factors for lower PDI were lower birth weight, white race, and presence of a genetic/extracardiac anomaly (all P ≤ .01). After adjustment for these factors, PDIs improved over time (0.39 points/year, 95% confidence interval 0.01 to 0.78; P = .045). Risk factors for lower MDI were lower birth weight, male gender, less maternal education, and presence of a genetic/extracardiac anomaly (all P < .001). After adjustment for these factors, MDIs improved over time (0.38 points/year, 95% confidence interval 0.05 to 0.71; P = .02). CONCLUSIONS: Early neurodevelopmental outcomes for survivors of cardiac surgery in infancy have improved modestly over time, but only after adjustment for innate patient risk factors. As more high-risk CHD infants undergo cardiac surgery and survive, a growing population will require significant societal resources.

Video-Assisted Thoracoscopic Cardiac Denervation: A Potential Novel Therapeutic Option for Children With Intractable Ventricular Arrhythmias

BACKGROUND Left cardiac sympathetic denervation is one of the therapeutic modalities used in the management of patients with medically refractory long QT syndrome. Traditionally, a thoracotomy or cervical incision has been used as the standard surgical approach for performing left cardiac sympathetic denervation. Video-assisted thoracoscopic surgery allows a minimally invasive technique. There is only one published series on the use of video-assisted thoracoscopic surgery for left cardiac sympathetic denervation in patients with long QT syndrome. METHODS We performed a retrospective clinical review of pertinent medical records and report a series including 9 pediatric patients (4 long QT syndrome, 4 catecholaminergic polymorphic ventricular tachycardia, and 1 idiopathic ventricular tachycardia) who underwent a left cardiac sympathetic denervation by means of video-assisted thoracoscopic surgery. RESULTS There were no severe complications, and 6 of 7 symptomatic patients with available follow-up experienced marked improvement in the first month after sympathectomy. CONCLUSIONS This minimally invasive procedure provides a safe novel therapeutic option for children with drug-refractory catecholaminergic polymorphic ventricular tachycardia and other catecholamine-triggered arrhythmias.

Multi-Institutional Study of Implantable Defibrillator Lead Performance in Children and Young Adults Results of the Pediatric Lead Extractability and Survival Evaluation (PLEASE) Study

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: <8 years compared with >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.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: . Unique identifier: [NCT00335036][1]. # Clinical Perspective {#article-title-15} [1]: /lookup/external-ref?link_type=CLINTRIALGOV&access_num=NCT00335036&atom=%2Fcirculationaha%2F127%2F24%2F2393.atom

Presentation, Diagnosis, and Medical Management of Heart Failure in Children: Canadian Cardiovascular Society Guidelines

Pediatric heart failure (HF) is an important cause of morbidity and mortality in childhood. This article presents guidelines for the recognition, diagnosis, and early medical management of HF in infancy, childhood, and adolescence. The guidelines are intended to assist practitioners in office-based or emergency room practice, who encounter children with undiagnosed heart disease and symptoms of possible HF, rather than those who have already received surgical palliation. The guidelines have been developed using the Grading of Recommendations Assessment, Development and Evaluation (GRADE) methodology, and are accompanied by practical Recommendations for their application in the clinical setting, supplemented by online material. This work does not include Recommendations for advanced management involving ventricular assist devices, or other device therapies.

Lead extraction in pediatric and congenital heart disease patients.

Background—Transvenous pacemaker and defibrillator implantation is an expanding practice in pediatric and congenital heart disease patients, and given the finite longevity of current lead designs, lead extraction is an eventuality for a significant subset of these patients. Data on the safety and efficacy of different lead extraction techniques in this specific patient population are limited. Methods and Results—We report our experience from a single-center cohort study with a retrospective review of prospectively collected data on all lead extractions performed between January 2002 and December 2008. Lead extraction procedures involved a total of 144 patients and 203 leads. Of these, 61 patients (42%) were female and 86 (60%) had structural heart disease. Successful simple extraction, requiring the use of only a nonlocking stylet, was achieved in 59 (29%) leads. Of the remaining leads, 35 were abandoned and 109 underwent complex extraction techniques, including a radiofrequency-powered sheath used in 78 of 109 leads. Successful extraction was achieved in 80% (162/203) of all leads and 94% (103/109) of leads undergoing a complex extraction. On multivariable analysis, older lead age (odds ratio [OR], 0.63; 95% confidence interval [CI], 0.48 to 0.82; P<0.0001), ventricular lead position (OR, 0.40; 95% CI, 0.20 to 0.79; P=0.015), and polyurethane insulation (OR, 0.34; 95% CI, 0.14 to 0.80; P=0.017) were found to be associated with a decreased likelihood of simple extraction. There were 4 major and 4 minor procedural complications involving 8 patients and no procedure-related deaths. On univariate analysis, lead age (OR, 1.28; 95% CI, 1.09 to 1.50; P=0.002) was the only factor associated with procedural complications. Conclusions—The majority of leads implanted in pediatric and congenital heart disease patients can be extracted successfully; however, the procedure carries a risk of serious complications. Older lead age, ventricular leads, and polyurethane insulation were independent predictors of the decreased likelihood of an extraction by simple traction.

Two-Year Survival and Mental and Psychomotor Outcomes After the Norwood Procedure An Analysis of the Modified Blalock-Taussig Shunt and Right Ventricle–to–Pulmonary Artery Shunt Surgical Eras

Background— The Norwood procedure for stage 1 palliation of hypoplastic left heart syndrome is performed with either the modified Blalock-Taussig (MBTS) or the right ventricle–to–pulmonary artery (RVPA) shunt. In our institution, surgical practice changed from use of the MBTS to use of the RVPA shunt in 2002. We analyzed survival and mental and psychomotor outcomes of the 2 consecutive surgical eras. Methods and Results— Between September 1996 and July 2005, 94 neonates with hypoplastic left heart syndrome underwent the Norwood procedure. Patients were recruited as neonates and followed up prospectively. Health, mental, and psychomotor outcomes (Bayley Scales of Infant Development-II) were assessed at 2 years. The study subjects were from the Norwood-MBTS era (n=62; 1996 to 2002) or the Norwood-RVPA era (n=32; 2002 to 2005). In the MBTS era, early and 2-year mortality rates were 23% (14/62) and 52% (32/62); the mean (SD) mental and psychomotor developmental indices were 79 (18) and 67 (19). In the RVPA era, early and 2-year mortality rates were 6% (2/32) and 19% (6/32); the mean (SD) mental and psychomotor developmental indices were 85 (18) and 78 (18). The 2-year mortality rate (P=0.002) and the psychomotor developmental index (P=0.029) were improved in the more recent surgical era. On multivariable Cox regression analysis, postoperative highest serum lactate independently predicted 2-year mortality in the MBTS and RVPA eras. Conclusions— Analysis of 2 consecutive surgical eras of hypoplastic left heart syndrome patients undergoing the Norwood procedure showed a significant improvement in 2-year survival and psychomotor development in the more recent era. Adverse neurodevelopmental outcome in this patient population remains a concern.

System survival of nontransvenous implantable cardioverter-defibrillators compared to transvenous implantable cardioverter-defibrillators in pediatric and congenital heart disease patients

BACKGROUND Nontransvenous (NTV) implantable cardioverter-defibrillator (ICD) systems with pericardial and/or subcutaneous coils are used in select pediatric and congenital heart disease patients who are not candidates for transvenous ICD leads. Outcomes with these hybrid configurations are not well understood. OBJECTIVE The purpose of this study was to compare survival of NTV ICD systems to standard transvenous (TV) ICD systems. METHODS We conducted a retrospective single-center study in which the TV group was matched to the NTV group 2:1 by type of cardiac disease and implant date. RESULTS There were 39 patients in the NTV group and 78 matched in the TV group. Compared to the TV group, the NTV group was younger (median 7 vs 20 years) with a smaller body surface area at implant (0.9 vs 1.8 m(2); both P <.001). Median follow-up in the NTV group was 17 months. System survival at 12, 24, and 36 months was 73%, 55%, and 49% in the NTV group versus 91%, 83%, and 76% in the TV group (P = .003). A multivariable Cox proportional hazards model including group, body surface area, and age at implant revealed the NTV group to be an independent predictor of system failure (hazard ratio 2.9, P = .04). Rate of total unanticipated interventions in the NTV group was 18 versus 6 per 1,000 person-months in the TV group. In patients with NTV systems, 23% received appropriate shocks and 18% received inappropriate shocks. CONCLUSION Survival of ICD systems using NTV defibrillation coils is significantly shorter than with TV ICD systems. Although NTV systems provide protection for this unique subset of patients, more durable options are needed.

Nonfluoroscopic imaging systems reduce radiation exposure in children undergoing ablation of supraventricular tachycardia

BACKGROUND The current standard of care for imaging during supraventricular tachycardia (SVT) ablation uses fluoroscopy, which exposes otherwise healthy children to the potential harmful effects of radiation. OBJECTIVE The purpose of this study was to determine whether the adjunct use of nonfluoroscopic imaging reduces radiation exposure during SVT ablation among children. METHODS This was a prospective, controlled, single-center study of patients age ≥8 years, weight ≥25 kg, with SVT and normal cardiac anatomy. Patients were randomized to control (fluoroscopy only) or study group (fluoroscopy + AcuNav intracardiac ultrasound + NavX electroanatomic mapping), stratified by operator to one of five electrophysiologists. Fluoroscopy times (minutes) and radiation doses (mGy) were recorded, and outcomes and adverse events were noted. RESULTS Seventy-four patients were enrolled (37 control, 37 study). Median age was 14.7 years (range 8.6-22.3 years); 61% had accessory pathways and 39% had atrioventricular nodal reentrant tachycardia. Nonfluoroscopic imaging reduced median fluoroscopy time by 59% (18.3 minutes vs 7.5 minutes, P <.001) and radiation exposure by 72% (387 vs 110 mGy, P <.001). In the study group, 26 of 37 had ≤10 minutes of fluoroscopy, including 2 with no fluoroscopy exposure and 2 with <30 seconds. Electrophysiologic procedure time was not affected by use of nonfluoroscopic imaging, but total case times were prolonged by 31 minutes (P <.001). Acute success was 97% in control and 100% in study patients, with no difference in adverse events. CONCLUSION Use of nonfluoroscopic imaging during SVT ablation in children resulted in substantial and immediate reductions in fluoroscopy time and radiation exposure without change in acute success or adverse event rates but did increase overall procedural time.

Efficacy of implantable cardioverter defibrillators in young patients with catecholaminergic polymorphic ventricular tachycardia: Success depends on substrate

Background—The effectiveness of implantable cardioverter-defibrillator (ICD) therapy for the management of catecholaminergic polymorphic ventricular tachycardia (VT) in young patients is not known. ICD discharges are not always effective and inappropriate discharges are common, both resulting in morbidity and mortality. Methods and Results—This is a multicenter, retrospective review of young patients with catecholaminergic polymorphic VT and ICDs from 5 centers. ICD discharges were evaluated to determine arrhythmia mechanism, appropriateness, efficacy of therapy, and complications. A total of 24 patients were included. Median (interquartile range) ages at onset of catecholaminergic polymorphic VT symptoms and ICD implant were 10.6 (5.0–13.8) years and 13.7 (10.7–16.3) years, respectively. Fourteen patients received 140 shocks. Ten patients (42%) experienced 75 appropriate shocks and 11 patients (46%) received 65 inappropriate shocks. On actuarial analysis, freedom from appropriate shock at 1 year after ICD implant was 75%. Of appropriate shocks, only 43 (57%) demonstrated successful primary termination. All successful appropriate ICD discharges were for ventricular fibrillation. No episodes of polymorphic VT or bidirectional VT demonstrated successful primary termination. The adjusted mean (95% confidence interval) cycle length of successful discharges was significantly shorter than unsuccessful discharges (168 [152–184] ms versus 245 [229–262] ms; adjusted P=0.002). Electrical storm occurred in 29% (4/14) and induction of more malignant ventricular arrhythmias in 36% (5/14). There were no deaths. Conclusions—ICD efficacy in catecholaminergic polymorphic VT depends on arrhythmia mechanism. Episodes of ventricular fibrillation were uniformly successfully treated, whereas polymorphic and bidirectional VT did not demonstrate successful primary termination. Inappropriate shocks, electrical storm, and ICD complications were common.

Long-Term Follow-Up of a Pediatric Cohort With Short QT Syndrome

OBJECTIVES The purpose of this study was to define the clinical characteristics and long-term follow-up of pediatric patients with short QT syndrome (SQTS). BACKGROUND SQTS is associated with sudden cardiac death. The clinical characteristics and long-term prognosis in young patients have not been reported. METHODS This was an international case series involving 15 centers. Patients were analyzed for electrocardiography characteristics, genotype, clinical events, Gollob score, and efficacy of medical or defibrillator (implantable cardioverter-defibrillator [ICD]) therapy. To assess the possible prognostic value of the Gollob score, we devised a modified Gollob score that excluded clinical events from the original score. RESULTS Twenty-five patients 21 years of age or younger (84% males, median age: 15 years, interquartile range: 9 to 18 years) were followed up for 5.9 years (interquartile range: 4 to 7.1 years). Median corrected QT interval for heart rate was 312 ms (range: 194 to 355 ms). Symptoms occurred in 14 (56%) of 25 patients and included aborted sudden cardiac death in 6 patients (24%) and syncope in 4 patients (16%). Arrhythmias were common and included atrial fibrillation (n = 4), ventricular fibrillation (n = 6), supraventricular tachycardia (n = 1), and polymorphic ventricular tachycardia (n = 1). Sixteen patients (84%) had a familial or personal history of cardiac arrest. A gene mutation associated with SQTS was identified in 5 (24%) of 21 probands. Symptomatic patients had a higher median modified Gollob score (excluding points for clinical events) compared with asymptomatic patients (5 vs. 4, p = 0.044). Ten patients received medical treatment, mainly with quinidine. Eleven of 25 index cases underwent ICD implantation. Two patients had appropriate ICD shocks. Inappropriate ICD shocks were observed in 64% of patients. CONCLUSIONS SQTS is associated with aborted sudden cardiac death among the pediatric population. Asymptomatic patients with a Gollob score of <5 remained event free, except for an isolated episode of supraventricular tachycardia, over an average 6-year follow-up. A higher modified Gollob score of 5 or more was associated with the likelihood of clinical events. Young SQTS patients have a high rate of inappropriate ICD shocks.