AAP Recommended Cardiac Screening Questions in Preparticipation Physical Evaluation Forms.

To evaluate the utilization of the American Academy of Pediatrics' (AP) cardiovascular screening questions within preparticipation physical evaluation forms from the 50 state high school athletic associations. We found that fewer than half of state forms incorporated all 10 AAP questions; moreover, a subset failed to adhere to criteria recommended by either the AAP or American Heart Association.

Pacing in Pediatric Patients with Postoperative Atrioventricular Block.

Surgery for congenital heart disease may compromise atrioventricular (AV) nodal conduction, potentially resulting in postoperative AV block. In the majority of cases, AV nodal function recovers during the early postoperative period and may only require short-term pacing support, typically provided via temporary epicardial wires. Permanent pacing is indicated when the postoperative AV block persists for more than 7 to 10 days due to the risk of mortality if a pacemaker is not implanted. Although there is a subset of patients who may have late recovery of AV nodal function, those with continued postoperative AV block will need lifelong pacing therapy.

Ventricular Tachycardia in a Pediatric Patient with High-Risk Thrombotic Thrombocytopenia Purpura.

An 8-year-old previously healthy male was diagnosed with thrombotic thrombocytopenic purpura (TTP) and increased serum cardiac troponin I. Telemetry recorded non-sustained ventricular tachycardia, without ST-segment changes or other abnormalities on serial electrocardiogram. This case illustrates that cardiac monitoring by telemetry should be considered in high-risk TTP with elevated cardiac troponin.

Preparticipation State Cardiac Screening Forms for Athletes.

BACKGROUND AND OBJECTIVES: Annual preparticipation physical evaluation (PPE) is used in the United States to screen adolescents for potential causes of sudden cardiac death. The American Heart Association recommends 14 screening elements of history and physical examination. This study sought to define the utilization of these screening elements by each of the 50 states before high school athletics. METHODS: PPE forms were obtained from the public website of the high school athletics governing body in every state. Form content was analyzed to identify which of the 14 screening elements were explicitly fulfilled. Additional PPE forms provided by private/parochial schools, other professional societies, or independent groups were excluded from this study. RESULTS: A total of 48 states (96%) had PPE forms posted online. The remaining 2 states (4%) deferred the specific method of PPE documentation to individual school districts and provided no standardized form. Of the 48 states providing PPE forms, 13 (27%) included all 14 American Heart Association screening elements. The median criteria included by each state was 11 (range 3-14). The 3 criteria most commonly absent were (1) the examination of femoral pulses to exclude coarctation (58%), (2) a family history of specific inherited cardiac disease (31%), and (3) personal history of hypertension (27%). CONCLUSIONS: Annual preparticipation forms are important screening tools. Only a minority of states include all 14 cardiac screening elements recommended by the American Heart Association.

Postoperative Breath-Holding Spells Requiring Cardiopulmonary Resuscitation After Cardiopulmonary Bypass.

Breath-holding spells are common in childhood and can be associated with bradycardia and pulselessness. This report details severe breath-holding spells complicating postoperative management after atrial septal defect closure. The patient required cardiopulmonary resuscitation despite the use of a temporary pacemaker to prevent bradycardia. After multiple episodes of chest compressions, the decision was made to not intervene immediately to pulselessness and the patient was able to recover without further intervention.

Intraventricular Septation in the Context of Dilated Cardiomyopathy Associated With TTN Mutation.

A 6-month-old infant boy presented with symptomatic heart failure. Dilated cardiomyopathy was found in association with a mutation in TTN. Structural heart disease included novel septation of the left ventricle with a fenestrated membrane resulting from aberrant congenital mitral valve apparatus formation. (Level of Difficulty: Advanced.).

Outcomes of Pediatric Patients With Defibrillators Following Initial Presentation With Sudden Cardiac Arrest.

BACKGROUND: Implantable cardioverter defibrillators (ICD) are recommended for secondary prevention after sudden cardiac arrest (SCA). The outcomes of pediatric patients receiving an ICD after SCA remain unclear. The objective of this study is to evaluate outcomes, future risk for appropriate shocks, and identify characteristics associated with appropriate ICD therapy during follow-up. METHODS: Multicenter retrospective analysis of patients (age ≤21 years) without prior cardiac disease who received an ICD following SCA. Patient/device characteristics, cardiac function, and underlying diagnoses were collected, along with SCA event characteristics. Patient outcomes including complications and device therapies were analyzed. RESULTS: In total, 106 patients were included, median age 14.7 years. Twenty (19%) received appropriate shocks and 16 (15%) received inappropriate shocks (median follow-up 3 years). First-degree relative with SCA was associated with appropriate shocks (P<0.05). In total, 40% patients were considered idiopathic. Channelopathy was the most frequent late diagnosis not made at time of presentation. Neither underlying diagnosis nor idiopathic status was associated with increased incidence of appropriate shock. Monomorphic ventricular tachycardia (hazard ratio, 4.6 [1.2-17.3]) and family history of sudden death (hazard ratio, 6.5 [1.4-29.8]) were associated with freedom from appropriate shock in a multivariable model (area under the receiver operating characteristic curve, 0.8). Time from diagnoses to evaluation demonstrated a nonlinear association with freedom from appropriate shock (P=0.015). In patients >2 years from implantation, younger age (P=0.02) and positive exercise test (P=0.04) were associated with appropriate shock. CONCLUSIONS: The risk of future device therapy is high in pediatric patients receiving an ICD after SCA, irrelevant of underlying disease. Lack of a definitive diagnosis after SCA was not associated with lower risk of subsequent events and does not obviate the need for secondary prophylaxis.

Interdisciplinary care of children with trisomy 13 and 18.

Children with trisomy 13 and 18 (previously deemed "incompatible with life") are living longer, warranting a comprehensive overview of their unique comorbidities and complex care needs. This Review Article provides a summation of the recent literature, informed by the study team's Interdisciplinary Trisomy Translational Program consisting of representatives from: cardiology, cardiothoracic surgery, neonatology, otolaryngology, intensive care, neurology, social work, chaplaincy, nursing, and palliative care. Medical interventions are discussed in the context of decisional-paradigms and whole-family considerations. The communication format, educational endeavors, and lessons learned from the study team's interdisciplinary care processes are shared with recognition of the potential for replication and implementation in other care settings.

The QT Interval in Patients With the Turner Syndrome.

Patients with the Turner syndrome (TS) often have longer QT intervals compared with age-matched peers although the significance of this remains unknown. We sought to determine the degree, frequency and impact of QTc prolongation in patients with TS. A chart review of all patients with an electrocardiogram (ECG) and genetically proven TS was performed. Medications at the time of the ECG were reviewed and QTc calculated. Medications were classified according to QTc risk using www.crediblemeds.com. ECG parameters were compared with an age, gender, and cardiac lesion-matched control group. Over the 10-year period of review, 112 TS patients with a mean age of 34 ± 25 years underwent 226 ECGs. At least 1 QTc prolonging medication was prescribed in 81 (74%) patients. Longer QTc interval correlated with absence of y chromosomal material (p = 0.01), older age (p <0.0001), increased number of QTc prolonging and nonprolonging medications (p <0.0001 each). During the 7.0 ± 5.1 years of follow-up, no patient had ventricular arrhythmia or unexplained sudden death. QTc was significantly shorter in matched controls using either Bazett or Hodges formula (424 ± 16 ms vs 448 ± 28 ms, p <0.0001; and 414.8 ± 16 ms vs 424.2 ± 20 ms; p = 0.0002, respectively). However, there was no difference in the frequency of QTc prolongation >460 msec (2.8% vs 2.6%, p = 0.9). In conclusion, despite frequent use of QT-prolonging medications, ventricular arrhythmias are rare in TS.

Williams Syndrome and Neonatal Cardiac Surgery for Congenital Single Ventricle.

Williams syndrome (WS) is an arteriopathic derangement associated with supravalvular aortic stenosis and branch pulmonary stenosis. We describe double-outlet right ventricle with mitral atresia and aortic arch hypoplasia in an infant with WS. This case demonstrates the difficulty in managing patients with WS with complex cardiac defects. To our knowledge, this is the first reported single-ventricle physiology in a patient with WS. (Level of Difficulty: Advanced.).

Can noninvasive testing identify benign patterns of suggested pre-excitation on electrocardiogram?

BACKGROUND: The presence of anterograde conduction through an accessory pathway (AP) has been linked to sudden cardiac death. Unfortunately, pre-excitation associated with classic pathways can be difficult to differentiate from benign APs such as nodofascicular fibers. OBJECTIVE: Identifying characteristics on electrocardiogram (ECG) and exercise that differentiate classic and benign AP connections in suggested pre-excitation patterns. METHODS: Retrospective review of patients presenting between 1995 and 2017 with ventricular pre-excitation on ECG, determined to have either typical left-lateral AP during electrophysiology study (EPS), or benign, or no AP determined by either transesophageal electrophysiology study (TEP), or EPS. RESULTS: A total of 96 patients were included, 14.2 years (4-24), 45% female, 90% Caucasian. Of these, 60 (63%) had a classic APs identified on EPS and 58 (97%) underwent successful ablation. Conversely, 36 (37%) had benign pathways identified. ECG findings differed between the groups: PR-interval 102 versus 120 ms (P < .0001), QRS-duration 110 versus 102 ms (P < .0001), QRS-axis 74 versus 59 degrees (P = .0005), and QRS onset to peak R/S in limb leads 64 versus 42 ms (P < .0001), and precordial leads 66 versus 46 ms (P < .0001). Change in QRS duration during exercise differed between the groups: 25 versus 2 ms (P < .0001) and ECG characteristics identified the presence of an AP with 97% sensitivity and 94% negative predictive value. CONCLUSION: Classic and benign APs exhibit different ECG characteristics, though clinical overlap does not allow for absolute differentiation. These data may help with risk stratification decision making though does not obviate the need for additional invasive testing.

Ambulatory Arrhythmia Detection with ZIO® XT Patch in Pediatric Patients: A Comparison of Devices.

Ambulatory electrocardiogram monitoring devices can be used for 24-72 h to detect arrhythmias. A new device, the ZIO® XT Patch has cardiac monitoring capabilities that can be utilized for up to 14 days. The purpose of this study is to describe duration of ZIO use by age, and to compare its time to arrhythmia detection with the Holter monitor in a pediatric population. A single-center, retrospective review of patients < 18 years of age who underwent clinical investigation with ZIO from October 2014 to February 2016 was performed. An age-matched cohort was utilized to compare ZIO to Holter monitor results. Demographic and diagnostic data, time to first arrhythmia, and arrhythmia burden were analyzed. A total of 406 ZIO were prescribed; median age 12.7 years and 50% male subjects. Median duration of ZIO monitoring significantly increased with age (p < 0.001). 499 Holter monitors were prescribed on a statistically different age group. Arrhythmia detection rates were similar between groups, 10% (n = 42) by ZIO and 9% (n = 45) by Holter (p = NS). The majority of arrhythmias (57%) detected by ZIO were after 24 h (p < 0.0001). All arrhythmias detected by Holter monitor occurred within 24 h (p < 0.0001), mean duration of wear was 24.1 h, range 0.5-48 h. The ZIO® XT Patch may be considered as an ambulatory ECG monitor to diagnose arrhythmia in pediatric patients of all ages. Increasing patient age resulted in increasing duration of ZIO monitoring. Majority of arrhythmias detected with ZIO were identified after 24 h, which would have been missed by other short-term monitors.

Tilt table testing to diagnose pseudosyncope in the pediatric population.

OBJECTIVE: Pseudosyncope can be difficult to distinguish from true syncope. Often, pediatric patients with pseudosyncope undergo multiple tests and referrals before the appropriate diagnosis is reached. The purpose is to describe the utility of the head-up tilt table test to elicit the diagnosis of pseudosyncope in the pediatric population. DESIGN: Retrospective chart review from November 2012 to December 2015 of patients age ≤23 years referred for 30-minute, 80-degree tilt table test. Pretest probability for pseudosyncope was high if there was no response to traditional management, atypical episodes, occurrence during undesirable exercise, or prolonged episode duration. Inductive techniques were utilized to persuade patients of the likelihood of experiencing an episode during the procedure. Pseudosyncope was confirmed when a patient had normal vital signs during their event and had reflex responses to disruptive maneuvers. RESULTS: Tilt table testing was performed on 89 patients [median age 16 years (5-23); 26% male] with the majority (60%) being negative for pseudosyncope, including 51 true negatives and 2 false-negatives. Of the 36 patients with syncope during tilt table testing, 28 were diagnosed with vasovagal syncope and 8 with pseudosyncope [median age 16 years (15-21); 38% male]. Pseudosyncope episodes were observed immediately in 2 patients. All patients with late-onset pseudosyncope required inductive techniques prior to the recorded episode. CONCLUSIONS: Pseudosyncope can be identified during tilt table testing if inductive techniques are utilized in patients with a high index of suspicion. Disruptive maneuvers are excellent adjunctive methods to confirm the diagnosis. Tilt table testing is an effective means to identify pseudosyncope and allow appropriate diagnosis and treatment.

Adolescent Presents With Altered Mental Status and Elevated Anion Gap After Suicide Attempt by Ethylene Glycol Ingestion.

We report the case of a 16-year-old healthy adolescent male who presented to the local emergency department with altered mental status. En route to a tertiary care facility, he began to decompensate and was found to be markedly acidotic. Further investigation revealed an elevated anion gap, and physical examination showed only abdominal pain and decreased level of consciousness. A broad differential diagnosis was considered at the time of the patient's presentation at the tertiary care center including ingestion of a volatile alcohol, sepsis, and an abdominal catastrophe. Although fomepizole and emergent dialysis were being initiated, laboratory tests confirmed ethylene glycol poisoning. This case demonstrates the importance of early recognition of potential ingestions in patients with altered mental status and supportive laboratory findings.

Breath Holding Spells in Children with Long QT Syndrome.

BACKGROUND: Long QT syndrome (LQTS) is a genetic heart rhythm disorder that may present with syncope, seizures, or sudden cardiac death. Breath holding spells (BHS) occur in 5% of all children and have been noted in children with LQTS anecdotally. The purpose of this study was to determine the frequency of BHS in children diagnosed with LQTS at ≤5 years of age. DESIGN: A retrospective review was performed to identify children diagnosed with LQTS who were ≤5 years old at initial presentation to our LQTS clinic from August 1999 to November 2013. The mean length of follow-up was 6.4 ± 2.8 years. The electronic medical records were reviewed for clinical presentation of BHS, as well as LQTS-associated symptoms, diagnostic tests, and treatment. RESULTS: The study cohort consisted of 115 children with LQTS (58% male; median age at diagnosis, 11 months [range, birth to 5 years]; mean corrected QT interval (QTc), 478 ± 60 milliseconds). At presentation, 80% of patients were asymptomatic. Genetic testing revealed type 1 LQTS (LQT1) in 48%. Overall, 5 of 115 patients (4.3%) had BHS (2 of 5 [40%] male, mean QTc: 492 ± 14 milliseconds, 4 [80%] with family history of LQTS). BHS were the presenting symptom in 1 of 23 symptomatic patients (4.3%). All BHS occurred in patients with LQT1 (P = .02). CONCLUSIONS: Although BHS among children with LQTS are relatively rare and occur at similar frequency as the general population, they can be the presenting symptom for a heart rhythm disorder. Careful attention to BHS is important to distinguish an innocent BHS from a potential LQTS-triggered cardiac event so that proper treatment is initiated.

Duration and magnitude of vasopressor support predicts poor outcome after infant cardiac operations.

BACKGROUND: The vasoactive inotrope score (VIS) is a sum of the total vasopressor dose at a single point in time. Incorporating duration and magnitude of vasopressor requirements during the postcardiac surgical period could improve VIS sensitivity for predicting poor outcome. METHODS: This is a retrospective review of 244 infants (aged ≤365 days) who underwent cardiopulmonary bypass during congenital cardiac operations from 2002 to 2011. The VIS was calculated hourly for the first 72 hours. Poor outcome was defined as prolonged mechanical ventilation (≥6 days) or intensive care length of stay (≥12 days). First, the association between the maximum VIS (maxVIS) in the first 48 postoperative hours and poor outcome was confirmed for our study population. Next, postoperative intervals and VIS values that were significantly associated with poor outcome were identified and incorporated into a formula, termed the VISindex, which was compared with the traditional maxVIS. RESULTS: The VISindex demonstrated improved sensitivity for predicting prolonged mechanical ventilation (VISindex: area under the curve [AUC], 0.85; 95% confidence interval [CI], 0.79 to 0.90; maxVIS: AUC, 0.80; 95% CI, 0.75 to 0.86) and intensive care unit length of stay (VISindex: AUC, 0.84; 95% CI, 0.79 to 0.89; maxVIS: AUC, 0.77; 95% CI, 0.71 to 0.83) after cardiac operations in infants. CONCLUSIONS: Incorporating magnitude and duration of postoperative vasopressor support into the VIS improves its sensitivity for predicting poor outcome.

Cardiac and multiorgan transplantation for end-stage congenital heart disease.

OBJECTIVE: To report our single-center experience with patients who had cardiac and multiorgan transplantation for end-stage congenital heart disease (CHD). PATIENTS AND METHODS: We reviewed records for all patients with CHD who had undergone heart transplantation at Mayo Clinic, Rochester, Minnesota, from November 1, 1990, through June 30, 2012. Patients with cardiomyopathy were excluded, unless CHD was present. RESULTS: Overall, 45 patients had cardiac transplantation for end-stage CHD (mean age, 26.1±18.4 years; range, 1 month to 65 years). Two patients (4%) had combined heart/liver transplantation; 1 (2%) had heart/kidney transplantation. Six patients (13%) had no previous cardiac operation; the remaining 39 patients had a mean of 3 (range, 1-8) previous cardiac operations. Patient survival (95% CI) at 1, 5, and 10 years was 89% (80%-98%), 89% (80%-98%), and 72% (56%-87%), respectively, while graft survival at 1, 5, and 10 years was 89% (80%-98%), 89% (80%-98%), and 61% (44%-78%), respectively. During the same era, the International Society for Heart & Lung Transplantation reported that survival in patients undergoing transplant for non-congenital diagnoses was 85%, 72%, and 56%, respectively. Over a mean follow-up of 8.7±6.2 years, rejection requiring treatment was documented in 35 patients (78%). Eleven patients (24%) have been diagnosed with neoplasia (8 skin, 1 blood, 1 lymph, and 1 other), and 3 patients (7%) have required retransplantation. Four patients (9%) have developed significant coronary vasculopathy; 1 successfully underwent retransplantation, and 3 died 6, 8, and 14 years after transplantation. CONCLUSION: With appropriate patient selection and posttransplant monitoring, survival has improved for patients with complex end-stage CHD. Multiorgan transplantation is an option for selected patients with CHD.

Pitfalls in diagnosis: suspected anomalous origin of the right coronary artery from the pulmonary artery.

Anomalous coronary arteries are rare in the general population. We report the case of a term neonate who underwent an echocardiogram to evaluate a possible patent ductus arteriosus. Unexpectedly, an apparent anomalous origin of the right coronary artery from the main pulmonary artery was detected by surface 2-dimensional transthoracic echocardiography and color-flow Doppler imaging. Because ventricular size and function were normal, the patient ultimately underwent cardiac catheterization to verify the anatomy before proposed surgery. Angiograms showed that the right coronary artery arose from the left anterolateral portion of the mid-ascending aorta. The patient did not require surgery. This case report illustrates pitfalls that can occur in the diagnosis of coronary artery anomalies.