Utility of Exercise Testing in Children and Teenagers With Arrhythmogenic Right Ventricular Cardiomyopathy
Department of Paediatrics and Child Health, University of Manitoba, Winnipeg, Manitoba, Canada.The American journal of cardiology (Impact Factor: 3.28). 09/2009; 104(3):411-3. DOI: 10.1016/j.amjcard.2009.03.056
Arrhythmogenic right ventricular cardiomyopathy (ARVC) is increasingly recognized as an important cause of exertional sudden death in otherwise healthy young individuals and athletes. Graded exercise testing is routinely included in the evaluation of patients with suspected ARVC, but its diagnostic utility has not been systematically assessed. Using a single-center retrospective design, the rhythm response to graded exercise testing was examined in 33 tests performed in 16 young (aged <18 years) patients with established diagnosis of ARVC. Ventricular premature complexes (VPCs) were classified as absent (graded 0), as being isolated or in couplets (graded 1), or as comprising nonsustained ventricular tachycardia (graded 2) during pretest rest, at peak exercise, and during postexercise recovery. VPCs were absent at rest in 21 of 33 studies, subsequently appearing at peak exercise in 4 studies and during recovery in 2 studies. Isolated VPCs and couplets were present at rest in 9 of 33 studies, with subsequent exercise provoking higher grade ectopic activity in 2 instances at peak exercise and in 1 case during recovery, while VPCs decreased or remained unchanged in all other cases. In all 3 instances in which ventricular tachycardia was observed during pretest rest, there was either suppression (3 at peak exercise, 2 during recovery) or no change (1 case during recovery) in VPC grade. In conclusion, the exercise response of ventricular ectopic activity is highly variable in young patients with ARVC. The diagnostic utility of graded exercise testing is thus questionable in young patients with suspected ARVC, and the absence or suppression of VPCs during exercise should not be considered reassuring in terms of its diagnostic exclusion.
Full-textDOI: · Available from: Robert Murray Hamilton, Oct 03, 2015
Data provided are for informational purposes only. Although carefully collected, accuracy cannot be guaranteed. The impact factor represents a rough estimation of the journal's impact factor and does not reflect the actual current impact factor. Publisher conditions are provided by RoMEO. Differing provisions from the publisher's actual policy or licence agreement may be applicable.
- [Show abstract] [Hide abstract]
ABSTRACT: The authors summarize the present knowledge on arrhythmogenic right ventricular cardiomyopathy/dysplasia. Limited data are available about natural history of asymptomatic patients with arrhythmogenic right ventricle cardiomyopathy/dysplasia, who have a ventricular tachycardia during exercise test. A 25-year old female patient was treated with osteosynthesis because of ankle injury. Cardiology consultation was performed because of an abnormal ECG. Physical examination was normal. ECG showed a normal sinus rhythm, left axis deviation, negative T waves in leads II, III, aVF and V2-V6. Chest X-ray and laboratory findings were normal. Echocardiography showed normal left ventricular ejection fraction along with inferior akinesis and dilated right ventricle. Bicycle exercise test revealed a good exercise tolerability (9 MET), and after sporadic ventricular extra systoles ventricular tachycardia developed lasting for 3 minutes, which spontaneously stopped after aborting the test and performed abdominal strain. MRI was performed which has shown normal left ventricular size, wall motion and ejection fraction and depressed right ventricle function (ejection fraction 31.6%) enlarged right ventricular end-systolic and diastolic volumes, hypo-akinetic regions without aneurysm and bulging. No contrast enhancement was seen in the thin right ventricular wall. According to abnormal ECG and MRI findings arrhythmogenic right ventricle cardiomyopathy/dysplasia was diagnosed. No ICD implantation was indicated because the patient was asymptomatic, and no sudden cardiac death occurred in the family. Three month later the patient was found dead. At autopsy the right ventricular chamber was markedly enlarged, with multiple translucent areas of fatty accumulation accompanied with extended myocytes loss. There was a characteristic triangle dysplasia: the inflow, outflow tracts and apical areas. The coronaries were free of atherosclerosis. Mallory's phosphotungstic acid-hematoxilin stain demonstrated the presence of fibrosis within the scattered myocardium. Conclusion: malignant ventricular arrhythmia provoked by exercise test in an asymptomatic arrhythmogenic right ventricle cardiomyopathy/dysplasia patient with negative family history should be an indication for ICD implantation.Orvosi Hetilap 12/2010; 151(52):2145-9. DOI:10.1556/OH.2010.28995
- [Show abstract] [Hide abstract]
ABSTRACT: Determining safe levels of physical activity for children and adolescents with electrophysiologic and structural congenital heart disease is a challenging clinical problem. The body of evidence for making these recommendations is limited and likely based on expert opinion, medicolegal concerns, and perceived risks of sudden cardiac death (SCD) with activity. The Bethesda Conference has established consensus guidelines for determining the eligibility of athletes with cardiovascular abnormalities for competitive sports and their disqualification from them. However, literature on guidelines for noncompetitive physical activity is not available. A survey was designed to determine practice patterns for patients with electrophysiologic and structural congenital heart disease. Between July 2011 and December 2011, approximately 350 health care providers working with this group of patients were recruited by email or while attending professional meetings. The survey received 81 responses, primarily from pediatric cardiologists (70 %). The findings indicate that the majority of Canadian cardiac care providers surveyed are only partially implementing current recommendations. Areas of variance included physical activity recommendations for hypertrophic cardiomyopathy, long QT syndrome, catecholaminergic polymorphic ventricular tachycardia, and heart transplantation, among others. The development of comprehensive consensus guidelines for activity recommendations was supported by 96 % of the respondents. The heterogeneity of responses may be attributable to conflicting and poorly evidenced information in the literature, a lack of emphasis on recreational activity, an entrenched tendency toward bed rest in the cardiology community, and a lack of awareness by cardiac care providers regarding the actual risk associated with physical activity in electrophysiologic and structural congenital heart disease. A balanced discussion is required in considering both the significant benefit of physical activity in reducing cardiovascular risk factors and the small possibility of SCD in children and young adults with electrophysiologic and structural congenital heart disease.Pediatric Cardiology 02/2013; 34(6). DOI:10.1007/s00246-013-0654-z · 1.31 Impact Factor
- [Show abstract] [Hide abstract]
ABSTRACT: We sought to determine if exercise testing could expose a latent electrical substrate of arrhythmogenic right ventricular cardiomyopathy (ARVC) in asymptomatic gene carriers. Management of asymptomatic ARVC gene carriers is challenging due to variable penetrance of disease and the recognition that sudden cardiac death may be the first clinical manifestation. Exercise induced abnormalities during exercise treadmill testing (ETT) were initially compared in 60 subjects - 30 asymptomatic ARVC gene carriers and 30 healthy controls. In phase 2 of the study, we evaluated the ETT of 25 ARVC patients with a history of sustained ventricular arrhythmia or cardiac arrest to determine if ETT abnormalities in asymptomatic gene carriers were common to individuals with a malignant electrical form of the disease. Depolarization abnormalities were found to develop more frequently in asymptomatic gene carriers compared to healthy controls: epsilon waves appeared in 4/28(14%) vs. 0/30(0%)(p=0.048), premature ventricular contractions (PVCs) in 17/30(57%) vs. 3/30(10%)(p=0.0003), and new QRS terminal activation duration (TAD) ≥55 ms in 7/22(32%) vs. 2/29(7%)(p=0.03). Superior axis PVCs only occurred in gene carriers. In the second phase of the study, the frequency of these abnormalities was found to be high in symptomatic ARVC patients: new epsilon waves appeared in 3/18(17%), superior axis PVCs in 21/25(84%), and new TAD ≥55ms in 8/12(67%). Exercise testing exposes a latent electrical substrate in asymptomatic ARVC gene carriers that is shared by ARVC patients with a history of ventricular arrhythmia. ETT may be useful in guiding treatment decisions, exercise prescription and prioritizing medical surveillance in asymptomatic ARVC gene carriers.Journal of the American College of Cardiology 06/2013; 62(19). DOI:10.1016/j.jacc.2013.04.084 · 16.50 Impact Factor