Iqbal El-Assaad

Use of dofetilide in adult patients with atrial arrhythmias and congenital heart disease: A PACES collaborative study

BACKGROUND Arrhythmia management has become the major treatment challenge in adult patients with congenital heart disease (ACHD). OBJECTIVE We sought to investigate the utility and safety profile of dofetilide for atrial arrhythmias in ACHD. METHODS A retrospective chart review was performed. We included patients (age ≥18 years) with congenital heart disease who had atrial fibrillation (AF) or intra-atrial reentrant tachycardia treated with dofetilide. RESULTS We identified 64 patients with a mean age at initiation of 42 ± 14 years. ACHD type included single ventricle (n = 19, 30%), transposition of the great arteries (n = 14, 22%), atrial septal defect (n = 9, 14%), tetralogy of Fallot (n = 8, 12%), atrioventricular canal defect (n = 5, 8%), mitral/aortic stenosis (n = 7, 11%), and other (n = 2, 3%). Thirty-five (55%) had atrial fibrillation, and 29 (45%) had intra-atrial reentrant tachycardia. A total of 3 (4.7%) patients had major inpatient adverse events: torsades de pointes (n = 1, 1.5%), ventricular tachycardia (n = 1, 1.5%), and corrected QT prolongation requiring discontinuation (n = 1, 1.5%). Dofetilide was discontinued in 1 patient because of sinus node dysfunction, and another patient discontinued therapy before discharge because of persistent arrhythmia. Of the patients who were discharged on dofetilide (n = 59, 92%), 40 (68%) had adequate rhythm control and 19 (32%) had partial rhythm control. After a median follow-up of 3 years, 29 (49%) patients remained on dofetilide and 2 (3%) patients died. Reasons for discontinuation included waning effect (n = 16, 57%), side effects (n = 5, 18%), noncompliance (n = 2, 7%), successful ablation (n = 3, 11%), high cost (n = 1, 3.5%), and unknown (n = 1, 3.5%). CONCLUSION Dofetilide remains a viable antiarrhythmic drug option in this challenging population. At 3 years, 49% remained on dofetilide. Close monitoring of renal function, concomitant medications, and corrected QT interval is required.

Pacemaker implantation in pediatric heart transplant recipients: Predictors, outcomes, and impact on survival

BACKGROUND Little is known about the incidence of permanent pacemaker (PPM) implantation after heart transplantation (HTx) in the pediatric population. OBJECTIVE The purpose of this study was to investigate the incidence, predictors, and outcomes of acute need for PPM implantation in pediatric HTx recipients. METHODS We queried the United Network for Organ Sharing (UNOS) database for all pediatric (age <18 years) patients who received HTx (1994-2014). Regression models are reported for prediction of PPM implantation. RESULTS A total of 6156 patients were analyzed, of whom 69 (1.1%) required posttransplant PPM implantation acutely. PPM use decreased over the study period (hazard ratio [HR] 0.95, P = .01). Compared with the non-PPM group, PPM group was more likely to be older (10 vs 5.0 years, P <.001), used antiarrhythmics (35.6% vs 18.3%, P = .006), required intraaortic balloon pump (2.9% vs 0.5%, P = .049), and had undergone biatrial anastomosis (68.1% vs 48.2%, P = .007). In a multivariable model, PPM implantation was predicted by higher donor age (HR 1.05, P = .002), biatrial anastomosis (HR 2.53, P = .04) and antiarrhythmic use (HR 2.12, P = .02). After adjusting for baseline characteristics, PPM recipients were at increased risk for posttransplant infection (47.8% vs 26.4%, P = .001) and dialysis (15.9% vs 6.6%, P = .003). Adjusted graft survival did not differ between the 2 groups (P = .78). CONCLUSION Acute postoperative PPM implantation in pediatric HTx recipients is rare and has decreased over time. Acute PPM use is associated with biatrial anastomosis, antiarrhythmic use, and older donor age. Although PPM recipients had higher incidences of infections and dialysis, PPM implantation did not adversely impact survival.

Implantable cardioverter-defibrillator and wait-list outcomes in pediatric patients awaiting heart transplantation

BACKGROUND Implantable cardioverter-defibrillators (ICDs) reduce the incidence of sudden cardiac death (SCD) in adults with end-stage heart failure; however, their efficacy in pediatric patients awaiting heart transplantation is not well established. OBJECTIVES This study sought to investigate the role of ICDs in preventing SCD and waiting list mortality as well as to determine risk factors for SCD in pediatric patients listed for heart transplantation. METHODS We queried the United Network for Organ Sharing database for all pediatric patients (age ≤18 years) listed for heart transplantation (2005-2014). The Cox proportional hazards model was used to identify risk factors for SCD and all-cause mortality. RESULTS A total of 5072 mostly White (55%) male (55%) patients (mean age 6.2 ± 6.5 years) were identified, of whom 426 (8.3%) had ICD at listing. At 6 months, 65% underwent heart transplantation, 15% died (4% died of SCD), and 20% were alive. In a multivariable model, United Network for Organ Sharing status 1B (hazard ratio [HR] 0.52; 95% confidence interval [CI] 0.29-0.95; P = .03), myocarditis (HR 0.19; 95% CI 0.05-0.77; P = .02), restrictive cardiomyopathy (HR 0.19; 95% CI 0.05-0.76; P = .02), and dilated cardiomyopathy (HR 0.32; 95% CI 0.20-0.52; P < .001) were associated with lower SCD risk, while younger age at listing (HR 0.94 per year; 95% CI 0.90-0.98; P = .003) was associated with higher SCD risk. ICD at listing was not associated with reduced SCD (P = .12), all-cause mortality, or delisting (P = .57). CONCLUSION In pediatric patients listed for heart transplantation, the risk of SCD remains low and does not differ between patients with and without an ICD at listing.

Lone Pediatric Atrial Fibrillation in the United States: Analysis of Over 1500 Cases

Little is known about lone atrial fibrillation (AF) in pediatrics and its risk factors due to low prevalence. We sought to determine risk factors and estimate recurrence rates in children with lone AF using a large clinical database. Using the Explorys clinical database, we retrospectively identified patients who were below 20 years of age at the time of their AF diagnosis. Patients with congenital heart disease, cardiomyopathy, prior open heart surgery, or thyroid disease were excluded. Out of 7,969,230 children identified, 1910 had AF and 1570 met the definition of lone AF. The prevalence of lone AF was 7.5 per 100,000 children. In comparison to young children (0–4 years), risk for lone AF increased with age (adjusted odds ratio (aOR) 1.2 [95% CI 0.9–1.5, P = 0.21] in those 5–9 years, aOR 1.7 [95% CI 1.3–2.1, P < 0.001] in those 10–14 years, and aOR 10.7 [95% CI 8.7–13.2, P < 0.001] in those 15–19 years). Risk of lone AF was also higher in males than females (aOR 1.7 [95% CI 1.5–1.9, P < 0.001]), and was higher in obese children (BMI ≥ 95th percentile) versus children with normal BMI (aOR 1.3 [95% CI 1.1–1.5], P < 0.001), but there was no difference between overweight (BMI = 85th–94th percentile) and normal (P = 0.14). One-month recurrence rate was 15%, and increased with age. In this large pediatric cohort, the prevalence of lone AF was low, but risk was higher in males and increased with age and obesity. Older children with lone AF had higher rates of recurrence.

Trends of Out-of-Hospital Sudden Cardiac Death Among Children and Young Adults

By using death certificate reports from the Centers for Disease Control and Prevention’s Wide-ranging Online Data for Epidemiologic Research database, we examine in this article age and racial and ethnic trends of sudden cardiac death in children and young adults. BACKGROUND AND OBJECTIVES: Previous estimates of sudden cardiac death in children and young adults vary significantly, and population-based studies in the United States are lacking. We sought to estimate the incidence, causes, and mortality trends of sudden cardiac death in children and young adults (1–34 years). METHODS: Demographic and mortality data based on death certificates for US residents (1–34 years) were obtained (1999–2015). Cases of sudden death and sudden cardiac death were retrieved by using the International Classification of Diseases, 10th Revision codes. RESULTS: A total of 1 452 808 subjects aged 1 to 34 years died in the United States, of which 31 492 (2%) were due to sudden cardiac death. The estimated incidence of sudden cardiac death is 1.32 per 100 000 individuals and increased with age from 0.49 (1–10 years) to 2.76 (26–34 years). During the study period, incidence of sudden cardiac death declined from 1.48 to 1.13 per 100 000 (P < .001). Mortality reduction was observed across all racial and ethnic groups with a varying magnitude and was highest in children aged 11 to 18 years. Significant disparities were found, with non-Hispanic African American individuals and individuals aged 26 to 34 years having the highest mortality rates. The majority of young children (1–10 years) died of congenital heart disease (n = 1525, 46%), whereas young adults died most commonly from ischemic heart disease (n = 5075, 29%). CONCLUSIONS: Out-of-hospital sudden cardiac death rates declined 24% from 1999 to 2015. Disparities in mortality exist across age groups and racial and ethnic groups, with non-Hispanic African American individuals having the highest mortality rates.

Automated External Defibrillator Application Before EMS Arrival in Pediatric Cardiac Arrests

Using pediatric data from CARES, we examine in this article patient- and neighborhood-level characteristics associated with pre-EMS AED application. BACKGROUND: Little is known about the predictors of pre–emergency medical service (EMS) automated external defibrillator (AED) application in pediatric out-of-hospital cardiac arrests. We sought to determine patient- and neighborhood-level characteristics associated with pre-EMS AED application in the pediatric population. METHODS: We reviewed prospectively collected data from the Cardiac Arrest Registry to Enhance Survival on pediatric patients (age >1 to ≤18 years old) who had out-of-hospital nontraumatic arrest (2013–2015). RESULTS: A total of 1398 patients were included in this analysis (64% boys, 45% white, and median age of 11 years old). An AED was applied in 28% of the cases. Factors associated with pre-EMS AED application in univariable analyses were older age (odds ratio [OR]: 1.9; 12–18 years old vs 2–11 years old; P < .001), white versus African American race (OR: 1.4; P = .04), public location (OR: 1.9; P < .001), witnessed status (OR: 1.6; P < .001), arrests presumed to be cardiac versus respiratory etiology (OR: 1.5; P = .02) or drowning etiology (OR: 2.0; P < .001), white-populated neighborhoods (OR: 1.2 per 20% increase in white race; P = .01), neighborhood median household income (OR: 1.1 per

Differential expression of TAR DNA-binding protein (TDP-43) in the central nervous system of horses afflicted with equine motor neuron disease (EMND): a preliminary study of a potential pathologic marker

20 000 increase; P = .02), and neighborhood level of education (OR: 1.3 per 20% increase in high school graduates; P = .006). However, only age, witnessed status, arrest location, and arrests of presumed cardiac etiology versus drowning remained significant in the multivariable model. The overall cohort survival to hospital discharge was 19%. CONCLUSIONS: The overall pre-EMS AED application rate in pediatric patients remains low.