{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,5]],"date-time":"2026-10-05T16:00:19Z","timestamp":1791216019258,"version":"4.1.0"},"reference-count":28,"publisher":"Texas Heart Institute Journal","issue":"5","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2022,9,1]]},"abstract":"<jats:p>Patients with Anderson-Fabry disease (AFD) have an elevated incidence of resting arrhythmias and ischemic heart disease, but their exercise arrhythmia burden and ischemic changes are not well understood. In addition, little research has been done on heart rate recovery in these patients.<\/jats:p>\n        <jats:p>We retrospectively reviewed charts of patients with AFD who underwent maximal effort cardiopulmonary exercise testing (CPET) (n=44; 38.2 \u00b1 13.8 yr; 23 men) from 2012 through 2018. Electrocardiographic, Holter monitoring, echocardiographic, cardiac magnetic resonance imaging, and patient demographic data were collected.<\/jats:p>\n        <jats:p>No patient had adverse events that necessitated CPET termination, whereas 25 (57%) had ectopy during CPET, including 3 (7%) with frequent premature atrial contractions and 5 (11%) with frequent premature ventricular contractions. The ectopic burden was higher during resting electrocardiographic monitoring before exercise. In addition, 7 patients (16%) had pathologic ST-segment or T-wave changes on CPET, defined as ST-segment changes \u22652 mm. Among the patients who had concurrent cardiac magnetic resonance findings with their CPET (n=27), ST-segment or T-wave changes were associated with left ventricular myocardial mass (<jats:italic>r<\/jats:italic>=0.43, <jats:italic>P<\/jats:italic>=0.02). Chronotropic incompetence was seen during CPET in 28 patients (64%); however, only 2 patients (4%) had abnormal heart rate recovery at 1 minute.<\/jats:p>\n        <jats:p>This study shows that patients with AFD can safely undergo exercise testing but have a high incidence of exercise-induced arrhythmias and ischemic changes. Ischemic electrocardiographic changes during exercise testing are associated with myocardial mass. Despite the chronotropic incompetence associated with AFD, heart rate recovery appears to be generally preserved in these patients.<\/jats:p>","DOI":"10.14503\/thij-20-7363","type":"journal-article","created":{"date-parts":[[2022,9,7]],"date-time":"2022-09-07T14:10:21Z","timestamp":1662559821000},"source":"Crossref","is-referenced-by-count":5,"title":["Arrhythmia Burden and Heart Rate Response During Exercise in Anderson-Fabry Disease"],"prefix":"10.14503","volume":"49","author":[{"given":"Adam W.","family":"Powell","sequence":"first","affiliation":[{"name":"Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, Ohio"},{"name":"The Heart Institute, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Samuel G.","family":"Wittekind","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, Ohio"},{"name":"The Heart Institute, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Wayne A.","family":"Mays","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, Ohio"},{"name":"The Heart Institute, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sean M.","family":"Lang","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, Ohio"},{"name":"The Heart Institute, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Timothy K.","family":"Knilans","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, Ohio"},{"name":"The Heart Institute, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carlos E.","family":"Prada","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, Ohio"},{"name":"Division of Human Genetics, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Robert J.","family":"Hopkin","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, Ohio"},{"name":"Division of Human Genetics, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Clifford","family":"Chin","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, Ohio"},{"name":"The Heart Institute, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"5628","reference":[{"issue":"2","key":"i1526-6702-49-5-e207363-b1","series-title":"Gene","first-page":"365","article-title":"Novel \u03b1-galactosidase A mutation in patients with severe cardiac manifestations of Fabry disease","volume":"535","year":"2014","unstructured":"Duro\n              G,\n            \n            \n              Musumeci\n              MB,\n            \n            \n              Colomba\n              P,\n            \n            \n              Zizzo\n              C,\n            \n            \n              Albeggiani\n              G,\n            \n            \n              Mastromarino\n              V,\n            \n            \n          \n          Novel \u03b1-galactosidase A mutation in patients with severe cardiac manifestations of Fabry disease.\n\t\t\t\t\tGene2014;535(2):365\u20139."},{"issue":"2","key":"i1526-6702-49-5-e207363-b2","series-title":"Best Pract Res Clin Endocrinol Metab","first-page":"195","article-title":"Fabry disease and the heart","volume":"29","year":"2015","unstructured":"Seydelmann\n              N,\n            \n            \n              Wanner\n              C,\n            \n            \n              St\u00f6rk\n              S,\n            \n            \n              Ertl\n              G,\n            \n            \n              Weidemann\n              F.\n            \n          \n          Fabry disease and the heart.\n\t\t\t\t\tBest Pract Res Clin Endocrinol Metab2015;29(2):195\u2013204."},{"issue":"2","key":"i1526-6702-49-5-e207363-b3","series-title":"Clin Genet","first-page":"158","article-title":"Enzyme replacement therapy with agalsidase beta improves cardiac involvement in Fabry's disease","volume":"66","year":"2004","unstructured":"Spinelli\n              L,\n            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exercise and Holter electrocardiography in Fabry cardiomyopathy","volume":"28","year":"2015","unstructured":"Kr\u00e4mer\n              J,\n            \n            \n              Nordbeck\n              P,\n            \n            \n              St\u00f6rk\n              S,\n            \n            \n              Ritter\n              C,\n            \n            \n              Ertl\n              G,\n            \n            \n              Wanner\n              C,\n            \n            \n              Weidemann\n              F.\n            \n          \n          Electrical changes in resting, exercise and Holter electrocardiography in Fabry cardiomyopathy.\n\t\t\t\t\tJIMD Rep2015;28:19\u201328."},{"issue":"2","key":"i1526-6702-49-5-e207363-b27","series-title":"Am J Cardiol","first-page":"251","article-title":"Arrhythmia and clinical cardiac findings in children with Anderson-Fabry disease","volume":"120","year":"2017","unstructured":"Wilson\n              HC,\n    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