DOES THE EXERCISE STRESS TEST OVER DIAGNOSE LONG QT IN PEDIATRIC PATIENTS?

Abstract Background/Introduction. Genetic abnormalities in heart ion channels can predispose life-threatening arrhythmias. Pathogenic genotype is found in the majority of individuals with phenotype of cardiac channelopathies of which long QT syndrome (LQTS) is the most common. However, incomplete penetrance and substantial heterogeneity in genotype-phenotype relationship results in broad clinical disease spectrum. Purpose. To review data gathered from Vilnius University Hospital Santaros klinikos (VUHSK) probands with LQTS and detect phenotypic differences between groups of individuals according to genetic testing results. Methods. We retrospectively reviewed data from clinical records of study participants with suspected LQTS who were clinically evaluated and treated at VUHSK in 2013-2019 year period. Probands with performed genetic testing were included for the final evaluation. Based on genetic testing results, the participants were divided into group I, involving patients with established genetic diagnosis, and group II, which included patients with no clinically relevant variants identified. Patients with variants of uncertain significance (VUS) were excluded from further analysis. Main characteristics of clinical manifestation (age during first symptoms, syncope, cardiac arrest, etc), results of instrumental examination (ECG, Holter monitoring, exercise stress test), Schwatz & Crotti score were compared between groups I and II. Results. LQTS was suspected for 137 patients; genetic testing was performed for 112 probands: 49 (43.8%) patients had pathogenic and likely pathogenic (P/LP) variant; 12 (10.7%) - VUS; 51 (45.5%) - no clinically relevant variant was determined. Phenotype differences of LQTS were compared between two groups: probands with P/PL variant (group I, n = 49); probands with no alteration found (group II, n = 51) (total of 100 patients). The median age of these two groups was 15 [6] years, 31 (31%) were adults, 56 (56%) were female. Most common P/LP variants among probands were identified in KCNQ1 (Type 1), KCNH2 (Type 2) and CACNA1C (Type 8) genes. Participants with P/PL variant had longer QTc interval on ECG (510 ms [37] vs. 500 ms [41], p = 0.011), 24-hour Holter monitoring (510 ms [34] vs. 500 ms [42], p = 0.013) and during 4th minute of recovery from exercise stress test (485 ms [35] vs. 458 ms [52], p = 0.021), compared to individuals without genetic alterations in LQTS genes. The Schwartz & Crotti score was higher in group I than in group II (3.5 [1.5] vs. 3 [1.25], p = 0.026). Other clinical findings did not differ statistically significantly. Conclusions. Probands with P/PL variants of LQTS had longer QTc intervals and higher Schwartz & Crotti score than patients with no alteration found. Genotype can affect clinical manifestation in patients with LQTS and consequently determine patient’s prognosis and further medical care. Larger scale study is required for more detailed analysis of genotype-driven phenotype differences.

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Assessing the utility of screening electrocardiograms in pediatric patients following COVID-19

Cardiology in the Young ◽

10.1017/s1047951121003012 ◽

2021 ◽

pp. 1-19

Author(s):

Ari J. Gartenberg ◽

Travus J. White ◽

Khoi Dang ◽

Maully Shah ◽

Stephen M. Paridon ◽

...

Keyword(s):

Pediatric Patients ◽

Stress Test ◽

Severe Disease ◽

Retrospective Chart Review ◽

Exercise Stress Test ◽

Primary Care Providers ◽

Exercise Stress ◽

Care Providers ◽

Premature Ventricular Contractions ◽

Small Cohort

Abstract Objective: To determine the utility of screening electrocardiograms after SARS-CoV-2 infection among pediatric patients in detecting myocarditis related to COVID-19. Study Design: A retrospective chart review was performed at a large pediatric academic institution to identify patients with prior SARS-CoV-2 infection who received a screening electrocardiogram by their primary care providers and were subsequently referred for outpatient cardiology consultation due to an abnormal electrocardiogram. The outcomes were the results from their cardiology evaluations, including testing and final diagnoses. Results: Among 46 patients, during their preceding COVID-19 illness, the majority had mild symptoms, four were asymptomatic, and one had moderate symptoms. The median length of time from positive SARS-CoV-2 test to screening electrocardiogram was 22 days, and many electrocardiogram findings that prompted cardiology consultation were normal variants in asymptomatic adolescent athletes. Patients underwent frequent additional testing at their cardiology appointments: repeat electrocardiogram (72%), echocardiogram (59%), Holter monitor (11%), exercise stress test (7%), and cardiac MRI (2%). Five patients were incidentally diagnosed with congenital heart disease or structural cardiac abnormalities, and three patients had conduction abnormalities (premature atrial contractions, premature ventricular contractions, borderline prolonged QTc), although potentially incidental to COVID-19. No patients were diagnosed with myocarditis or ventricular dysfunction. Conclusion: In a small cohort of pediatric patients with prior COVID-19, who were primarily either asymptomatic or mildly symptomatic, subsequent screening electrocardiograms identified various potential abnormalities prompting cardiology consultation, but no patient was diagnosed with myocarditis. Larger multi-center studies are necessary to confirm these results and to evaluate those with more severe disease.

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Risk of cardiac disease and observations on lack of potential predictors by clinical history among children presenting for cardiac evaluation of mid-exertional syncope

Cardiology in the Young ◽

10.1017/s1047951115001481 ◽

2015 ◽

Vol 26 (5) ◽

pp. 894-900 ◽

Cited By ~ 7

Author(s):

Christina Y. Miyake ◽

Kara S. Motonaga ◽

Megan E. Fischer-Colbrie ◽

Liyuan Chen ◽

Debra G. Hanisch ◽

...

Keyword(s):

Cardiac Disease ◽

Stress Test ◽

Exercise Stress Test ◽

Clinical History ◽

Exercise Stress ◽

Tilt Test ◽

Long Qt ◽

Paediatric Cardiologist ◽

Cardiac Syncope ◽

Cardiac Evaluation

AbstractObjectiveThis study aimed to evaluate the incidence of cardiac disorders among children with mid-exertional syncope evaluated by a paediatric cardiologist, determine how often a diagnosis was not established, and define potential predictors to differentiate cardiac from non-cardiac causes.Study designWe carried out a single-centre, retrospective review of children who presented for cardiac evaluation due to a history of exertional syncope between 1999 and 2012. Inclusion criteria included the following: (1) age ⩽18 years; (2) mid-exertional syncope; (3) electrocardiogram, echocardiogram and an exercise stress test, electrophysiology study, or tilt test, with exception of long QT, which did not require additional testing; and (4) evaluation by a paediatric cardiologist. Mid-exertional syncope was defined as loss of consciousness in the midst of active physical activity. Patients with peri-exertional syncope immediately surrounding but not during active physical exertion were excluded.ResultsA total of 60 patients met the criteria for mid-exertional syncope; 32 (53%) were diagnosed with cardiac syncope and 28 with non-cardiac syncope. A majority of cardiac patients were diagnosed with an electrical myopathy, the most common being Long QT syndrome. In nearly half of the patients, a diagnosis could not be established or syncope was felt to be vasovagal in nature. Neither the type of exertional activity nor the symptoms or lack of symptoms occurring before, immediately preceding, and after the syncopal event differentiated those with or without a cardiac diagnosis.ConclusionsChildren with mid-exertional syncope are at risk for cardiac disease and warrant evaluation. Reported symptoms may not differentiate benign causes from life-threatening disease.

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Exercise stress test amplifies genotype-phenotype correlation in the LQT1 and LQT2 forms of the long-QT syndrome

ACC Current Journal Review ◽

10.1016/s1062-1458(03)00202-2 ◽

2003 ◽

Vol 12 (3) ◽

pp. 81 ◽

Cited By ~ 1

Author(s):

K. Takenaka ◽

A. Tomohiko ◽

W. Shimizu

Keyword(s):

Long Qt Syndrome ◽

Stress Test ◽

Exercise Stress Test ◽

Exercise Stress ◽

Phenotype Correlation ◽

Long Qt ◽

Genotype Phenotype Correlation ◽

Qt Syndrome

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Abstract 15780: The Utility of Exercise Stress Testing in the Evaluation of Ischemia Among Pediatric Patients With Exertional Chest Pain

Circulation ◽

10.1161/circ.142.suppl_3.15780 ◽

2020 ◽

Vol 142 (Suppl_3) ◽

Author(s):

Nicholas Robles ◽

Shailendra Upadhyay

Keyword(s):

Chest Pain ◽

Coronary Artery ◽

Pediatric Patients ◽

Stress Test ◽

Retrospective Chart Review ◽

Exercise Stress Test ◽

Exercise Stress ◽

Z Score ◽

Coronary Artery Abnormalities ◽

Myocardial Perfusion Study

Introduction: Exertional chest pain (CP) regularly prompts performing an exercise stress test (EST) out of concern for exercise-induced ischemia. We aimed to study the diagnostic utility of EST in the evaluation of ischemia among pediatric patients without previously diagnosed congenital heart disease (CHD) who present with exertional CP and to correlate EST results with available cardiac imaging findings. Methods: Retrospective chart review of all pediatric patients who underwent EST for exertional CP from Jan 2015 to Dec 2019 at our institution. Exercise stress test was performed on treadmill utilizing Bruce protocol. Analysis included demographic medical records and all available cardiac testing. Results: A total of 106 patients (56 males; 53%) were included. Cohort mean age, height, and weight were 15.1 ± 2.6 years (range: 7.3 - 19.6yr), 165 ± 12.7 cm, and 67.5 ± 26.2 kg. ST-T wave changes were noted in 8 patients (7.5%), 7 of which were non-specific and read as normal ESTs. One (0.94%) EST was suggestive of ischemic changes. His echocardiogram (echo) showed mild thickening of papillary muscles and ventricular septum. A follow-up exercise myocardial perfusion study showed no abnormality. Echo was performed on 86/106 (81%) patients. Of these, 23/86 (27%) had incidental abnormalities on echo; none contributed to the patients’ CP. Five patients (5/86; 5.8%) had notable coronary artery abnormalities on echo; all had normal ESTs. Two of these were confirmed by Cardiac CTA: 1) Left Main Coronary Artery (LMCA) arising from non-coronary cusp, and 2) dilated LMCA (Z-score 4.67) with slight dilation of Left Anterior Descending (Z-score 2.54). In the other 3 of 5 ( prominent LMCA, possible CA fistula, RCA with more leftward origin ) further coronary evaluations were not performed. Conclusions: Incidental non-specific echo abnormalities are common among pediatric patients who undergo EST for exertional CP. EST was negative in patients with coronary artery abnormalities. ESTs have a low yield in identifying cardiac abnormalities among pediatric patients without previously diagnosed CHD undergoing evaluation for exertional CP. Larger studies are needed to better evaluate the utility of EST in patients with coronary artery anomalies.

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