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Subcutaneous implantable cardioverter defibrillators (S-ICDs) are being used with increased frequency in children and patients with congenital heart disease. Vascular access complexities, intracardiac shunts, and specific anatomies make these devices particularly appealing for some of these patients. Alternative screening, implantation, and programming techniques should be considered based on patient size, body habitus, anatomy, procedural history, and preference. Appropriate and inappropriate shock rates are generally comparable to those seen with transvenous devices. Complications such as infection can occur, although their severity is likely to be less than that seen with transvenous devices. Technical advances are likely to further broaden S-ICD applicability. [ABSTRACT FROM AUTHOR]

This study used cardiac magnetic resonance imaging to evaluate flow characteristics and ventricular hemodynamics for children with single right (hypoplastic left heart syndrome) and single left (hypoplastic right heart syndrome) systemic ventricle anatomy after Fontan palliation compared with normal biventricular controls. Twenty children with single ventricle anatomy (hypoplastic left heart syndrome, n = 10; hypoplastic right heart syndrome, n = 10) underwent standardized 4-dimensional flow cardiac magnetic resonance and were compared with age-matched controls (n = 10). End-diastolic volume was partitioned into 4 defined components of variable kinetic energy (direct flow, retained inflow, delayed ejection, and residual volume) and compared between groups. Further, volumetric and functional parameters as defined by cardiac magnetic resonance were evaluated. Children with hypoplastic left heart syndrome had significantly increased indexed end-diastolic and end-systolic volumes compared with both hypoplastic right heart syndrome and control groups. Flow component analysis demonstrated diastolic inefficiency in both hypoplastic left heart syndrome and hypoplastic right heart syndrome groups compared with controls as defined by decreased direct flow and increased residual volumes. Decreased direct flow correlated with decreased ejection fraction and increased end-diastolic and end-systolic volume indices. Increased residual volume correlated with decreased ejection fraction and increased end-systolic volume index. Fontan-palliated patients with single ventricle physiology (hypoplastic left heart syndrome and hypoplastic right heart syndrome) demonstrate altered and inefficient flow patterns in the systemic ventricle as defined by 4-dimensional flow cardiac magnetic resonance compared with normal biventricular controls. Decreased direct flow and increased residual volume indicate that diastolic ventricular dysfunction is prevalent after Fontan palliation. This study provides a foundation for future predictive modeling and cardiac magnetic resonance flow diagnostic studies in this high-risk patient population. Flow component analysis by 4D-flow CMR demonstrates inefficient diastolic inflow partitioning in patients with HLHS and HRHS compared with biventricular control patients as defined by decreased direct flow and increased residual volume. [Display omitted] [ABSTRACT FROM AUTHOR]

The purpose of this study was to determine whether aortic biomechanical properties are abnormal in children with repaired truncus arteriosus (TA) and to concurrently evaluate left ventricular (LV) function post-repair utilizing a novel platform for regional ventricular function. Cardiac magnetic resonance (CMR) studies from 26 children (mean age: 15.6 ± 7.2 years) post-TA repair were compared with 20 normal controls (mean age: 14.7 ± 2.6 years). Parameters of aortic stiffness (pulse wave velocity and relative area change) were measured. Flow hemodynamic metrics (aortic regurgitant fraction, peak systolic flow, and peak systolic velocity) and LV function (volumetric data, ejection fraction, regional wall strain) were also compared. Ascending aortic pulse wave velocity was elevated and relative area change was decreased in TA patients compared with controls. Patients post-TA repair demonstrated elevated end diastolic and end systolic volumes in addition to decreased regional wall strain and increased mechanical dyssynchrony. LV functional changes were independent of aortic biomechanical properties. Children with repaired TA have increased ascending aortic stiffness and altered LV function as measured by CMR imaging. Longitudinal studies and advanced CMR assessments are warranted to better determine the long-term potential for late aortic complications and to optimize both the medical and surgical management of these patients after TA repair. [Display omitted] [ABSTRACT FROM AUTHOR]

Background: Studies have suggested that a fasciculoventricular pathway (FVP) may be the cause of preexcitation in patients with Danon disease, a rare X-linked dominant genetic disorder of hypertrophic cardiomyopathy.Objective: The purpose of this study was to describe the prevalence of ventricular preexcitation on resting 12-lead electrocardiogram (ECG) in patients with Danon disease and the electrophysiological study (EPS) results of those with preexcitation.Methods: Patients with confirmed Danon disease diagnosed with preexcitation (PR ≤120 ms, delta wave, QRS >110 ms) on ECG were included from a multicenter registry. The incidence of arrhythmias, implantable cardioverter-defibrillator (ICD) procedures, ICD shocks, and EPS results were collected.Results: Thirteen of 40 patients (32.5%) with Danon disease were found to have preexcitation (mean age 17.3 years; 38% women). EPS performed in 9 of 13 patients (69%) demonstrated FVP only in 2 (22.2%), extranodal pathway without exclusion of FVP in 2 (22.2%), and both FVP and extranodal pathway in 5 (55.6%). Two patients had malignant accessory pathway (AP) properties. Over median follow-up of 842 days (interquartile range 138-1678), 11 patients (85%) had ICD placement, and 6 (46.1%) underwent heart transplantation. No patients required therapy for ventricular tachycardia, and 2 patients (15%) had paroxysmal atrial fibrillation.Conclusion: In a large multicenter cohort of patients with Danon disease, there was a high prevalence of FVP and extranodal pathways diagnosed on EPS in those with preexcitation. These findings suggest patients with preexcitation and Danon disease should undergo EPS to assess for FVP and potentially malignant extranodal AP. [ABSTRACT FROM AUTHOR]

Background: The efficacy of cascade screening for the inherited heart conditions long QT syndrome (LQTS) and hypertrophic cardiomyopathy (HCM) is incompletely characterized.Objective: The purpose of this study was to examine the use of genetic testing and yield of cascade screening across diverse regions in the United States and to evaluate obstacles to screening in multipayer systems.Methods: An institutional review board-approved 6 United States pediatric center retrospective chart review of LQTS and HCM patients from 2008-2014 was conducted for (1) genetic test completion and results and (2) family cascade screening acceptance, methods, results, and barriers.Results: The families of 315 index patients (mean age 9.0 ± 5.8 years) demonstrated a 75% (254) acceptance of cascade screening. The yield of relative screening was 39% (232/601), an average of 0.91 detected per family. Genetic testing was less utilized in HCM index patients and relatives. Screening participation was greater in families of gene-positive index patients (88%) (P <.001) compared to gene-negative patients (53%). Cascade method utilization: Cardiology-only 45%, combined genetic and cardiology 39%, and genetic only 16%. Screening yield by method: combined 57%, genetic-only 29%, and cardiology-only 20%. Family decisions were the leading barriers to cascade screening (26% lack of followthrough and 26% declined), whereas insurance (6%) was the least cited barrier.Conclusion: Family participation in cascade screening is high, but the greatest barriers are family mediated (declined, lack of followthrough). Positive proband genetic testing led to greater participation. Cardiology-only screening was the most utilized method, but combined cardiology and genetic screening had the highest detection. [ABSTRACT FROM AUTHOR]

Ross and Ross-Konno operations are associated with the inherent risk of residual ventricular septal dysfunction and injury to the conduction system. However, comprehensive biventricular functional outcomes on magnetic resonance imaging after Ross and Ross-Konno procedures are unknown. The purpose of this study was to evaluate and compare the degree of electrical and mechanical dyssynchrony using cardiac magnetic resonance imaging in patients late after Ross and Ross-Konno operations. Patients following Ross operation (n = 16), Ross-Konno operation (n = 13), and healthy controls (n = 12) underwent cardiac magnetic resonance imaging strain deformation analysis to quantify left ventricular (LV) intraventricular dyssynchrony and right ventricular (RV)–LV interventricular dyssynchrony. Mechanical dyssynchrony indices were correlated with the degree of electrical dyssynchrony as assessed by QRS duration, as well as with magnetic resonance imaging-derived biventricular and autograft regurgitation parameters. Patients in the Ross and Ross-Konno groups had reduced LV global longitudinal strain when compared with controls (both P values <.01). Both Ross (P <.05) and Ross-Konno (P <.01) groups demonstrated increased RV–LV interventricular dyssynchrony compared with controls. Patients in the Ross-Konno group also had increased septal LV intraventricular dyssynchrony when compared with control and Ross groups (both P values <.01). The global LV intramechanical dyssynchrony strongly correlated with QRS duration (R = 0.716; P <.001). There was no association between mechanical dyssynchrony and aortic regurgitation fraction. Long-term follow-up of patients who underwent Ross and Ross-Konno operations demonstrates reduced LV global longitudinal strain suggestive of chronically increased afterload independent of autograft function. Additionally, Ross-Konno operation is associated with impaired LV intraventricular mechanics and electrical dyssynchrony. [ABSTRACT FROM AUTHOR]

Background: Subcutaneous implantable cardioverter-defibrillator (SICD) shows promise for select patients at risk of sudden cardiac death. However, patients need to pass an electrocardiographic (ECG) screening (ECG-S) test before they can receive an SICD. Predictors of ECG-S failure in children are unclear.Objective: The purpose of this study was to identify the incidence and predictive factors for failure of ECG-S in children.Methods: Patients 18 years and younger with a preexisting ICD underwent ECG-S for SICD. ECG and demographic data were analyzed for factors predictive of failure.Results: Seventy-three patients (mean age 14.2 ± 3.3 years; range 5-18 years) with hypertrophic cardiomyopathy (n = 24, 33%), long QT syndrome (n =18, 25%), other inherited arrhythmia syndromes (n = 20, 27%), congenital heart disease (n = 9, 12%), and miscellaneous conditions (n = 2) with an existing transvenous ICD underwent prospective ECG-S. Nineteen (26%) failed ECG-S. Failed patients had a longer corrected QT (QTc) interval (457 ms vs 425 ms; P = .03), a longer QRS duration (120 ms vs 98 ms; P = .04), and a lower ratio of R-wave to T-wave amplitudes (R:T ratio) in lead aVF (4 vs 5; P = .001). Multivariable logistic regression identified QTc interval (odds ratio [OR] 4.31; P = .04), QRS duration (OR 4.93; P = .03), R:T ratio in lead aVF (OR 3.13; P = .08) as predictors of failure. A risk score with 1 point each for QTc interval >440 ms, QRS duration >120 ms, and R:T ratio <6.5 in lead aVF was associated with probability of failure of 15.4% (1 point), 47.4% (2 points), and 88.6% (3 points), respectively.Conclusion: ECG-S failure for SICD occurred in 26% of children, which is higher than the reported incidence in adults. Factors predicting ECG-S failure included longer QTc interval, longer QRS duration, and lower R:T ratio in lead aVF. [ABSTRACT FROM AUTHOR]

Objectives: To identify the clinical presentation of children and adolescents affected by 1 of 4 cardiac conditions predisposing to sudden cardiac arrest: hypertrophic cardiomyopathy, long QT syndrome (LQTS), catecholaminergic polymorphic ventricular tachycardia (CPVT), and anomalous origin of the left coronary artery from the right sinus of Valsalva (ALCA-R).Study Design: This was a retrospective review of newly diagnosed pediatric patients with hypertrophic cardiomyopathy, LQTS, CPVT, and ALCA-R referred for cardiac evaluation at 6 US centers from 2008 to 2014.Results: A total of 450 patients (257 male/193 female; median age 10.1 years [3.6-13.8 years, 25th-75th percentiles]) were enrolled. Patient age was ≤13 years for 70.4% of the cohort (n = 317). Sudden cardiac arrest was the initial presentation in 7%; others were referred on the basis of abnormal or suspicious family history, personal symptoms, or physical findings. Patients with LQTS and hypertrophic cardiomyopathy were referred most commonly because of family history concerns. ALCA-R was most likely to have abnormal signs or symptoms (eg, exercise chest pain, syncope, or sudden cardiac arrest). Patients with CPVT had a high incidence of syncope and the greatest incidence of sudden cardiac arrest (45%); 77% exhibited exercise syncope or sudden cardiac arrest. This study demonstrated that suspicious or known family history plays a role in identification of many patients ultimately affected by 1 of the 3 genetic disorders (hypertrophic cardiomyopathy, LQTS, CPVT).Conclusion: Important patient and family history and physical examination findings may allow medical providers to identify many pediatric patients affected by 4 cardiac disorders predisposing to sudden cardiac arrest. [ABSTRACT FROM AUTHOR]

An abstract of the article "Comparative assessment of pediatric right ventricular volumes and function by MRI: right horizontal long axis versus short axis," by Brian D. Soriano and colleagues is presented.

Background Electrical and right ventricular (RV) mechanical dyssynchrony has been previously described in pediatric pulmonary arterial hypertension (PAH), but less is known about the relationship between electrical dyssynchrony and biventricular function. In this study we applied cardiac magnetic resonance (CMR) imaging to evaluate biventricular size and function with a focus on left ventricular (LV) strain mechanics in pediatric PAH patients with and without electrical dyssynchrony. Methods Fifty-six children with PAH and comprehensive CMR evaluation were stratified based on QRS duration z -score, with electrical dyssynchrony defined as z -score ≥2. Comprehensive biventricular volumetric, dyssynchrony, and strain analysis was performed. Results Nineteen PAH patients had or developed electrical dyssynchrony. Patients with electrical dyssynchrony had significantly reduced RV ejection fraction (35% vs 50%, p = 0.003) and greater end-diastolic (168 vs 112 ml/m 2 , p = 0.041) and end-systolic (119 vs 57, ml/m 2 , p = 0.026) volumes. Patients with electrical dyssynchrony had reduced RV longitudinal strain (–14% vs –19%, p = 0.007), LV circumferential strain measured at the free wall (–19% vs –22%, p = 0.047), and the LV longitudinal strain in the septal region (–10% vs –15%, p = 0.0268). LV mechanical intraventricular dyssynchrony was reduced in patients with electrical dyssynchrony at the LV free wall (43 vs 19 ms, p = 0.019). Conclusions The electrical dyssynchrony is associated with the reduced LV strain, enlarged RV volumes, and reduced biventricular function in children with PAH. CMR assessment of biventricular mechanical function with respect to QRS duration may help to detect pathophysiologic processes associated with progressed PAH. [ABSTRACT FROM AUTHOR]