[Show abstract][Hide abstract] ABSTRACT: Sudden cardiac death in the young (SCDY) is the leading cause of death in young athletes during sport. Screening young athletes for high-risk cardiac defects is controversial. The purpose of this study was to assess the utility and feasibility of a comprehensive cardiac screening protocol in an adolescent population. Adolescent athletes were recruited from local schools and/or sports teams. Each subject underwent a history and/or physical examination, an electrocardiography (ECG), and a limited echocardiography (ECHO). The primary outcome measure was identification of cardiac abnormalities associated with an elevated risk for sudden death. We secondarily identified cardiac abnormalities not typically associated with a short-term risk of sudden death. A total of 659 adolescent athletes were evaluated; 64% men. Five subjects had cardiac findings associated with an elevated risk for sudden death: prolonged QTc >500 ms (n = 2) and type I Brugada pattern (n = 1), identified with ECG; dilated cardiomyopathy (n = 1) and significant aortic root dilation; and z-score = +5.5 (n = 1). History and physical examination alone identified 76 (11.5%) subjects with any cardiac findings. ECG identified 76 (11.5%) subjects in which a follow-up ECHO or cardiology visit was recommended. Left ventricular mass was normal by ECHO in all but 1 patient with LVH on ECG. ECHO identified 34 (5.1%) subjects in whom a follow-up ECHO or cardiology visit was recommended. In conclusion, physical examination alone was ineffective in identification of subjects at elevated risk for SCDY. Screening ECHO identified patients with underlying cardiac disease not associated with immediate risk for SCDY. Cost of comprehensive cardiac screening is high.
The American Journal of Cardiology 09/2014; 114(11). DOI:10.1016/j.amjcard.2014.09.011 · 3.28 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Medium-chain acyl-coenzyme A dehydrogenase (MCAD) deficiency is the most common disorder of mitochondrial fatty acid oxidation. We report a term male infant who presented at 3 days of age with hypoglycemia, compensated metabolic acidosis, hypocalcemia, and prolonged QTc interval. Pregnancy was complicated by maternal premature atrial contractions and premature ventricular contractions. Prolongation of the QTc interval resolved after correction of metabolic derangements. The newborn screen was suggestive for MCAD deficiency, a diagnosis that was confirmed on genetic analysis that showed homozygosity for the disease-associated missense A985G mutation in the ACADM gene. This is the first report of acquired prolonged QTc in a neonate with MCAD deficiency, and it suggests that MCAD deficiency should be considered in the differential diagnoses of acute neonatal illnesses associated with electrocardiographic abnormality. We review the clinical presentation and diagnosis of MCAD deficiency in neonates.
[Show abstract][Hide abstract] ABSTRACT: Background
In attempts to detect diseases that may place adolescents at risk for sudden death, some have advocated for population-based screening. Controversy exists over electrocardiography (ECG) screening due to the lack of specificity, cost, and detrimental effects of false positive or extraneous outcomes.Objectives
Analyze the relationship between precordial lead voltage on ECG and left ventricle (LV) mass by echocardiogram in adolescent athletes.Methods
Retrospective cohort analysis of a prospectively obtained population of self-identified adolescent athletes during sports screening with ECG and echocardiogram. Correlation between ECG LV voltages (R wave in V6 [RV6] and S wave in lead V1 [SV1]) was compared to echocardiogram-based measurements of left ventricular mass. Potential effects on ECG voltages by body anthropometrics, including weight, body mass index (BMI), and body surface area were analyzed, and ECG voltages indexed to BMI were compared to LV mass indices to analyze for improved correlation.ResultsA total of 659 adolescents enrolled in this study (64% male). The mean age was 15.4 years (14–18). The correlations between LV mass and RV6, SV1, and RV6 + SV1 were all less than 0.20. The false positive rate for abnormal voltages was relatively high (5.5%) but improved if abnormal voltages in both RV6 and SV1 were mandated simultaneously (0%). Indexing ECG voltages to BMI significantly improved correlation to LV mass, though false positive findings were increased (12.9%).Conclusion
There is poor correlation between ECG precordial voltages and echocardiographic LV mass. This relationship is modified by BMI. This finding may contribute to the poor ECG screening characteristics.
[Show abstract][Hide abstract] ABSTRACT: Purpose: Sudden cardiac death in the young (SCDY) is the leading cause of death in young athletes during sport. Screening young athletes for cardiac defects that put at them at risk for SCDY is controversial. The purpose of this study was to assess the utility of a unique screening protocol, utilizing a limited echocardiogram, in identification of cardiac abnormalities and prevent SCDY.
Methods: We recruited adolescent athletes from local schools and sports teams using fliers sent to schools and athletic trainers. Subjects underwent a history and physical examination by a pediatrician or pediatric nurse practitioner, an electrocardiogram and a limited echocardiogram, both reviewed on site by a pediatric cardiologist. Providers completing each station of the study (history and physical examination, electrocardiogram, and echocardiogram) were blinded to the outcome of other stations. A separate pediatric cardiologist reviewed the results of all studies on-site and recommendations for follow up were made if needed. The primary outcome measure was the identification of cardiac abnormalities associated with a risk for sudden death. We evaluated the following: the number of subjects with cardiac concerns based on history and physical examination alone; the diagnostic utility added by the electrocardiogram; and the additional diagnostic benefit of the echocardiogram. We secondarily looked at the identification of cardiac abnormalities not typically associated with a short-term risk of sudden death.
Results: 659 adolescent athletes aged 13-18 years enrolled in this study; 64% male. Four patients had cardiac findings associated with a risk for sudden death: prolonged QTc>500 ms (n=2) and type I Brugada pattern (n=1) identified by electrocardiogram, and significant aortic root dilation with a z-score = +5.5 (n=1) identified by echocardiogram. History and physical examination alone identified 76 (12%) subjects in which cardiology referral was recommended by the pediatrician/nurse practictioner. The four patients with conditions associated with risk for sudden death had normal history and physical examination. Electrocardiogram identified 68 (10%) subjects for which a follow-up echocardiogram or cardiology visit was recommended by the reading cardiologist: including left ventricular hypertrophy (LVH) by voltage criteria (n=28), prolonged QTc greater than 460ms (n=14), and type I Brugada pattern (n=1). Left ventricular mass was normal by echocardiogram in all patients with LVH on EEG. Echocardiogram additionally identified the following: dilated aortic root (z-score = +2 to +2.5)(n=7), left ventricular non-compaction (n=5), and atrial septal defect (n=2).
Conclusion: History and physical examination alone was ineffective in identification of individuals at risk for SCDY. Screening electrocardiogram and echocardiogram identified different types of cardiac disease that may put an individual at risk for SCDY. The addition of a limited screening echocardiogram also identified patients with underlying cardiac disease that may not pose immediate risk for sudden death but do require cardiac follow-up.
2013 American Academy of Pediatrics National Conference and Exhibition; 10/2013
[Show abstract][Hide abstract] ABSTRACT: "Purpose ": Syncope is common in older children and adolescents and is typically due to neurocardiogenic syncope. Pediatric patients with syncope have low health-related quality of life (HRQOL) scores. The purpose of this study was to examine the impact of comorbidities on HRQOL scores in patients presenting with the chief complaint of syncope to a pediatric Syncope Clinic.
"Methods ": A retrospective case series of patients seen in a tertiary care cardiac center Syncope Clinic with initial visits between 6/2010-4/2013 was performed. Patients were included if they were 8-18 years of age and completed the Pediatric Quality of Life Inventory (PedsQL) HRQOL tool at the time of clinic presentation. Comorbidities were documented on each patient. The Wilcoxon Rank Sum test was used to compare PedsQL scores (Total, Physical, Psychosocial, Emotional, Social, and School) between healthy pediatric historical controls, syncope patients with no other medical illnesses, and syncope patients with chronic comorbid conditions. To account for multiple comparisons a Bonferroni correction was applied and a p-value < 0.0027 was considered significant.
"Results ": 468 patients were included for analysis. The patients were predominantly Caucasian (91%) and female (66%) with a mean age 14.82.7 years. Referrals to the Syncope Clinic were by primary care physicians (61%), the emergency department (13%), neurology (9%), the patient themselves (6%) or other provider types (11%). These patients had experienced a median of 2 (1-10) syncopal events and were experiencing a median of 2 (0-7) presyncopal episodes per week at the time of their clinic visit. There were 214 patients (46%) with no other reported medical problems. 254(54%) of these patients reported having syncope and other medical problems. Of those with other medical problems, 144 (57%) reported having one or more chronic diagnoses commonly seen as comorbidities with syncope including migraine headaches (37%), anxiety (28%), depression (20%), chronic fatigue (11%) and fibromyalgia (4%). The mean PedsQL scores for patients presenting with syncope with no other medical illnesses (n=214) were significantly lower in all domains (p<0.001) except for the Social Subscale compared to healthy control patients. Syncope patients with other chronic comorbid diagnoses (migraine headaches, anxiety, depression, chronic fatigue or fibromyalgia) had lower PedsQL scores across all domains (p<0.001) when compared to healthy controls and when compared to syncope patients with no other medical illnesses. (Figure 1)
"Conclusion ": Pediatric syncope patients commonly have other comorbid conditions. While patients with syncope have lower than normal HRQOL scores, those with comorbid chronic conditions have even worse HRQOL. Because of common comorbid conditions in this population, and the negative effect on HRQOL, this group of patients would be best served by a multidisciplinary approach, rather than cared for by a single provider, regardless of subspecialty.
2013 American Academy of Pediatrics National Conference and Exhibition; 10/2013
[Show abstract][Hide abstract] ABSTRACT: Traditional imaging for ablation of supraventricular tachycardia has been fluoroscopy, although 3-dimensional electroanatomic mapping (3D) has been demonstrated to reduce radiation exposure. This study compares a technique for the reduction of radiation, low-dose fluoroscopy (LD), with standard-dose fluoroscopy (SD) and 3D with SD (3D-SD). This was a single institutional retrospective cohort study. All patients undergoing initial ablation for atrioventricular reentrant tachycardia (AVRT) or atrioventricular nodal reentrant tachycardia (AVNRT) from 2009 to 2012 were reviewed and divided into 3 groups: (1) SD, (2) 3D (CARTO or NavX) with SD, or (3) LD. LD uses the same equipment as SD but includes customized changes to the manufacturer's lowest settings by decreasing the requested dose to the detector. Primary outcomes were fluoroscopy time and dose area product exposure. One hundred eighty-one patients were included. The median age was 15.0 years (3.3-20.8); 59% had AVRT, 35% had AVNRT, and 6% had both AVRT and AVNRT. LD decreased the dose area product (DAP) compared with SD (637.0 vs 960.1 cGy*cm(2), p = 0.01) with no difference in fluoroscopy time. 3D-SD decreased fluoroscopy time compared with SD (9.9 vs 18.3 minutes, p <0.001) with DAP of 570.1.0 versus 960.1 cGy*cm(2) (p = 0.16). LD and 3D-SD had comparable DAP (637.0 vs 570.1 cGy*cm(2), p = 0.67), even though LD had significantly longer fluoroscopy time (19.9 vs 9.9 minutes, p <0.001). In conclusion, LD during catheter ablation of AVRT and AVNRT significantly reduced the DAP compared with SD and had similar radiation exposure compared with 3D with SD.
The American journal of cardiology 04/2013; 112(1). DOI:10.1016/j.amjcard.2013.02.059 · 3.28 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Accessory pathways with "high-risk" properties confer a small but potential risk of sudden cardiac death. Pediatric guidelines advocate for either risk stratification or ablation in patients with ventricular pre-excitation but do not advocate specific methodology. We sought to compare the cost of differing risk-stratification methodologies in pediatric patients with ventricular pre-excitation in this single institutional, retrospective cohort study of asymptomatic pediatric patients who underwent risk stratification for ventricular pre-excitation. Institutional methodology consisted of stratification using graded exercise testing (GXT) followed by esophageal testing in patients without loss of pre-excitation and ultimately ablation in high-risk patients or patients who became clinically symptomatic during follow-up. A decision analysis model was used to compare this methodology with hypothetical methodologies using different components of the stratification technique and an "ablate all" method. One hundred and two pediatric patients with asymptomatic ventricular pre-excitation underwent staged risk stratification; 73% of patients were deemed low risk and avoided ablation and the remaining 27% ultimately were successfully ablated. The use of esophageal testing was associated with a 23% (p ≤0.0001) reduction in cost compared with GXT stratification alone and a 48% (p ≤0.0001) reduction compared with the "ablate all" model. GXT as a lone stratification method was also associated with a 15% cost reduction (p ≤0.0001) compared with the "ablate all" method. In conclusion, risk stratification of pediatric patients with asymptomatic ventricular pre-excitation is associated with reduced cost. These outcomes of cost-effectiveness need to be combined with the risks and benefits associated with ablation and risk stratification.
The American journal of cardiology 04/2013; 112(2). DOI:10.1016/j.amjcard.2013.03.017 · 3.28 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Patients with congenital heart disease (CHD) are at increased risk of cardiac arrhythmias. The utility of ambulatory (Holter) monitoring in predicting these arrhythmias remains unclear. We sought to evaluate the clinical utility and cost effectiveness of Holter monitoring in patients with CHD. A retrospective study of patients with CHD who had undergone Holter monitoring was performed. The Holter data from patients with tetralogy of Fallot (TOF), d-transposition of the great arteries (d-TGA) after an atrial switch operation, and patients with a single ventricle after Fontan palliation were reviewed. The Holter indication included evaluation of clinical symptoms or as a part of routine screening. The Holter results were deemed clinically significant if they resulted in a change in patient treatment. Sudden cardiac events included death or aborted sudden death and appropriate defibrillator therapies. A total of 589 Holter studies were performed in 189 patients (100 with TOF, 38 with d-TGA, and 51 with Fontan). The results of Holter monitoring performed for clinical symptoms had a low positive predictive value (0.08) for clinically significant changes in management. On routine monitoring, the sensitivity was low (0.40) but the negative predictive value was high (0.96) for future clinically significant arrhythmias. The frequency of clinically significant findings and associated cost-effectiveness improved with older patient age and Fontan and d-TGA CHD type. Nonsustained ventricular tachycardia was associated with sudden cardiac events in patients with TOF but not in those with d-TGA or Fontan palliation. In conclusion, Holter monitoring is generally inefficient for symptomatic evaluation; however, within specific age and CHD type subgroups, such as patients with repaired TOF >25 years old, it could be useful in clinical management and risk assessment as a part of routine care.
The American journal of cardiology 12/2012; 111(5). DOI:10.1016/j.amjcard.2012.11.021 · 3.28 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Pallid breath-holding (PBH) is a childhood condition that presents with recurrent syncope. Although typically benign, severe cases can lead to asystole and anoxic seizures. Previous studies have advocated pacemaker placement to abbreviate symptoms. This was a retrospective study of patients treated with fluoxetine for PBH spells. Clinical response, side effects and avoidance of pacemaker implantation were reviewed in six patients (12-60 months) treated with fluoxetine for PBH. Patients were referred because of concern of arrhythmia and failed medical treatment strategies. Two patients had previously implanted loop recorders, 5 patients had documented episodes of asystole, and 2 patients had generalized seizures. Fluoxetine resulted in alleviation of syncope in 5 of 6 patients. Time to symptomatic improvement symptoms ranged from 2 days to 1 month (median, 2 weeks). Median duration of treatment with fluoxetine was 12 months (12-24 months). One patient demonstrated no improvement and had a pacemaker implanted. There were no reported side effects to fluoxetine. Fluoxetine can be used to treat childhood PBH spells and may obviate the need for permanent pacing in a significant subset of this population. Considering its safe side-effect profile it is a worthwhile first-line agent to treat this disorder.
[Show abstract][Hide abstract] ABSTRACT: Introduction:
Cardiac conduction system injury is a cause of postoperative cardiac morbidity following repair of congenital heart disease (CHD). The national occurrence of postoperative complete heart block (CHB) following surgical repair of CHD is unknown. We sought to describe the occurrence of and costs related to postoperative CHB following surgical repair of common forms of CHD using a large national database.
Methods and results:
Retrospective, observational analysis performed over a 10-year period (2000-2009) using the Kids' Inpatient Database (KID). Visits for patients ≤24 months of age were identified who underwent surgical repair of ventricular septal defects (VSD), atrioventricular canal defects (AVC), and tetralogy of Fallot (TOF). Patients were identified who were diagnosed with postoperative CHB, further identifying those requiring a new pacemaker placement during the same hospitalization. Costs associated with visits were calculated. There were 16,105 surgical visits: 7,146 VSD, 3,480 AVC, and 5,480 TOF. There was a decrease in postoperative mortality (P = 0.0001) with no significant change in postoperative CHB. Hospital stay and cost were higher with CHB and placement of a permanent pacemaker. Repair of AVC (OR 1.77; [1.32-2.38]) was associated with a higher rate of postoperative CHB. Length of hospital stay and total cost were significantly increased with the development of postoperative CHB and increased further with placement of a permanent pacemaker.
There has been little change over time in the frequency of postoperative CHB in patients undergoing repair of VSD, AVC, and TOF. Postoperative CHB results in major added cost to the healthcare system.
[Show abstract][Hide abstract] ABSTRACT: Syncope is a common problem in children and adolescents. The diagnostic yield for most tests commonly used in the evaluation of pediatric patients with syncope is low.
To examine the epidemiology of pediatric patients presenting to United States (US) emergency departments (EDs) with a complaint of syncope and compare their initial management to published guidelines.
ED visits from the National Hospital Ambulatory Medical Care Survey for 2003-2007 for patients aged 7-18 years were analyzed. Outcome variables were diagnostic tests and management of patients presenting with syncope.
There were 627,489 (95% confidence interval [CI] 527,237-727,722) ED visits for syncope (0.9% of all ED visits for patients aged 7-18 years). Patients presenting to the ED for syncope were more commonly female (p<0.01), adolescent (13-18 years) (p<0.01), covered by private insurance (p=0.01), and more likely to arrive to the ED by ambulance (p<0.01), compared to those presenting with other complaints. Only 58.1% (95% CI 50.3-66.0%) of syncope patients received an electrocardiogram, and 26.5% (95% CI 18.2-34.7%) received a computed tomography (CT) or magnetic resonance imaging (MRI) scan as part of their diagnostic work-up.
When evaluating pediatric patients presenting with syncope, there should be an increased use of the electrocardiogram to screen for underlying cardiac abnormalities. There should also be a tempered use of CT/ MRI imaging in this population.
Journal of Emergency Medicine 03/2012; 43(4):575-83. DOI:10.1016/j.jemermed.2012.01.020 · 0.97 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Syncope is common in children and adolescents and most commonly represents neurocardiogenic syncope. No information has been reported regarding the effect of syncope on health-related quality of life in children.
This was a retrospective cohort study of patients seen in the Heart Institute Syncope Clinic at Cincinnati Children's Hospital Medical Center between July, 2009 and June, 2010. Health-related quality of life was assessed using the PedsQL™ tool. PedsQL™ scores were compared with both healthy historical controls and historical controls with chronic illnesses.
A total of 106 patients were included for analysis. In all, 90% were Caucasian and 63% were girls. The median age was 15.1 years (8.2-21.6). Compared with healthy controls, patients had lower PedsQL™ scores: Total score (75.2 versus 83.8, p < 0.0001); Physical Health Summary (78.8 versus 87.5, p < 0.0001); Psychosocial Health Summary (73.9 versus 81.9, p < 0.001), Emotional Functioning (68.9 versus 79.3, p < 0.001); and School Functioning (66.4 versus 81.1, p < 0.001). No difference was seen in Social Functioning (86.2 versus 85.2, p = 0.81). Patients also had lower PedsQL™ Total scores than patients with diabetes mellitus (p < 0.0001) and similar scores to patients with asthma, end-stage renal disease, obesity, and structural heart disease. Conclusion Children with syncope, although typically benign in aetiology, can have low health-related quality of life.
Cardiology in the Young 02/2012; 22(5):583-8. DOI:10.1017/S1047951112000133 · 0.84 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Cardiac rhythm devices are important in the management of pediatric patients with rhythm abnormalities, although factors driving utilization are poorly understood.
This study sought to evaluate utilization trends, complication rates, and cost associated with device implantation in the pediatric population.
Device implantation was analyzed using the Kids' Inpatient Database from 1997 to 2006. The type of device implantation, patient demographics, hospital characteristics, acute in-hospital complications, cost, and length of stay (LOS) were analyzed. χ(2) tests were used to test association between categorical variables, and logistic regression analysis was performed to evaluate risk factors associated with complications.
There were 5788 hospitalizations with device implantations. Although there was a significant increase in defibrillator implantation, there was no significant increase in the number of pacemaker implantations over this time period. Patient- and device-related complications were relatively common in all device cohorts (pacemaker 11.2%, 7.2%; defibrillator 5.9%, 11.5%; and biventricular device 19.4%, 26.7%). Type of complication was dependent on device type. Increased risk of complication was evident in the pacemaker cohort, patients with congenital heart disease, cardiomyopathy, previous cardiac arrest, and other heart operations. Patient-related complications increased cost and LOS regardless of patient or procedural characteristics. Device implantation in patients <5 years old was associated with increased LOS and cost but was not associated with increased risk of complication.
Device utilization in pediatrics is increasing due to escalating defibrillator implantation and biventricular pacing. Cost and LOS are significantly increased by patient complications. Reduction in these complications would improve patient care and lower medical costs.
Heart rhythm: the official journal of the Heart Rhythm Society 09/2011; 9(2):199-208. DOI:10.1016/j.hrthm.2011.09.004 · 5.08 Impact Factor