{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,7,30]],"date-time":"2025-07-30T15:50:10Z","timestamp":1753890610120,"version":"3.41.2"},"reference-count":39,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2024,8,14]],"date-time":"2024-08-14T00:00:00Z","timestamp":1723593600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Cardiovasc. Med."],"abstract":"<jats:sec><jats:title>Background<\/jats:title><jats:p>In obstructive hypertrophic cardiomyopathy (HOCM), disopyramide is used in patients who remain symptomatic despite <jats:italic>\u03b2<\/jats:italic>-blockers or verapamil. However, effectiveness of disopyramide therapy has not been clearly established due to inconsistent definition of responders and the insufficient length of follow-ups reported in literature. To address these shortcomings, we have conducted a retrospective analysis from detailed databases with long follow-up, from two HCM Referral Centers.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>62 symptomatic HOCM patients (43% women, age 52\u2009\u00b1\u200914 years) with left ventricular (LV) outflow tract gradient (LVOTG)\u2009\u2265\u200950 mmHg at rest or during provocation, were recruited from two Italian Centers. Disopyramide was added as second-line therapy in the patients in whom symptoms persisted despite classic pharmacologic treatment. Patients in NYHA class\u2009&amp;gt;\u2009II at baseline who reached NYHA class II or I, and patients in NYHA class II at baseline who reached NYHA class I or symptoms stabilization were defined as responders.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>At follow-up, (mean 4.4 years, IQR 1.1\u20136.6 years), 47 patients (76%) were responders, whereas 15 (24%) were no-responders. Responders showed larger LV diastolic volume index (LVEDVi) at baseline as compared to no-responders (61\u2009\u00b1\u200914 vs. 49\u2009\u00b1\u200916\u2005ml, respectively, <jats:italic>p<\/jats:italic>\u2009=\u20090.018), and, at follow-up, reached lower LVOTG than no-responders (43\u2009\u00b1\u200932 vs. 66\u2009\u00b1\u200928\u2005mmHg, respectively, <jats:italic>p<\/jats:italic>\u2009=\u20090.013), with a LVOTG &amp;lt;50 mmHg more represented in responders than in no-responders (75% vs. 25%, respectively; <jats:italic>p<\/jats:italic>\u2009=\u20090.004). No side effects requiring discontinuation of the therapy were recorded.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>HOCM patients treated with disopyramide as second-line therapy in a quite long-follow-up showed a significant improvement of symptoms, which avoided SRT in up to 70% of them. Moreover, our data suggest that a larger LVEDVi at baseline identify the subgroup of patients who benefit the most from the therapy in terms of symptoms and reduction of LVOTG below 50 mmHg during treatment. We will discuss specific situations where disopyramide may be preferred over myosin inhibition to ensure that effective therapeutic options are fully considered and not prematurely dismissed.<\/jats:p><\/jats:sec>","DOI":"10.3389\/fcvm.2024.1416600","type":"journal-article","created":{"date-parts":[[2024,8,14]],"date-time":"2024-08-14T06:11:01Z","timestamp":1723615861000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":1,"title":["Long-term follow-up study on obstructive hypertrophic cardiomyopathy patients treated with disopyramide: evidences of a notable trend in symptom control within a real-world clinical setting"],"prefix":"10.3389","volume":"11","author":[{"given":"Gaetano","family":"Todde","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Lorenzo Lupo","family":"Dei","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Roberto","family":"Polizzi","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Domenico","family":"Gabrielli","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Grazia","family":"Canciello","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Silvio","family":"Romano","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Felice","family":"Borrelli","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Geza","family":"Halasz","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Leopoldo","family":"Ordine","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Salvatore","family":"Di Napoli","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Daniela","family":"Pacella","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Raffaella","family":"Lombardi","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Giovanni","family":"Esposito","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Federica","family":"Re","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Maria-Angela","family":"Losi","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1965","published-online":{"date-parts":[[2024,8,14]]},"reference":[{"key":"B1","doi-asserted-by":"publisher","first-page":"3503","DOI":"10.1093\/eurheartj\/ehad194","article-title":"2023 ESC guidelines for the management of cardiomyopathies","volume":"44","author":"Arbelo","year":"2023","journal-title":"Eur Heart J"},{"key":"B2","doi-asserted-by":"publisher","first-page":"e159","DOI":"10.1016\/j.jacc.2020.08.045","article-title":"2020 AHA\/ACC guideline for the diagnosis and treatment of patients with hypertrophic cardiomyopathy: a report of the American College of Cardiology\/American Heart Association joint committee on clinical practice guidelines","volume":"76","author":"Ommen","year":"2020","journal-title":"J Am Coll Cardiol"},{"key":"B3","doi-asserted-by":"publisher","first-page":"2232","DOI":"10.1161\/CIRCULATIONAHA.106.644682","article-title":"Hypertrophic cardiomyopathy is predominantly a disease of left ventricular outflow tract obstruction","volume":"114","author":"Maron","year":"2006","journal-title":"Circulation"},{"key":"B4","doi-asserted-by":"publisher","first-page":"295","DOI":"10.1056\/NEJMoa021332","article-title":"Effect of left ventricular outflow tract obstruction on clinical outcome in hypertrophic cardiomyopathy","volume":"348","author":"Maron","year":"2003","journal-title":"N Engl J Med"},{"key":"B5","first-page":"194","article-title":"Contemporary treatment of hypertrophic cardiomyopathy","volume":"36","author":"Marian","year":"2009","journal-title":"Tex Heart Inst J"},{"key":"B6","doi-asserted-by":"publisher","first-page":"847","DOI":"10.1161\/01.CIR.35.5.847","article-title":"Amelioration of angina pectoris in idiopathic hypertrophic subaortic stenosis with beta-adrenergic blockade","volume":"35","author":"Cohen","year":"1967","journal-title":"Circulation"},{"key":"B7","doi-asserted-by":"publisher","first-page":"846","DOI":"10.1161\/01.CIR.38.5.846","article-title":"Muscular subaortic stenosis. Prevention of outflow obstruction with propranolol","volume":"38","author":"Flamm","year":"1968","journal-title":"Circulation"},{"key":"B8","doi-asserted-by":"publisher","first-page":"804","DOI":"10.1136\/hrt.32.6.804","article-title":"Long-term propranolol therapy in muscular subaortic stenosis","volume":"32","author":"Adelman","year":"1970","journal-title":"Br Heart J"},{"key":"B9","doi-asserted-by":"publisher","first-page":"488","DOI":"10.1136\/hrt.44.5.488","article-title":"Effects of propranolol on myocardial oxygen consumption, substrate extraction, and haemodynamics in hypertrophic obstructive cardiomyopathy","volume":"44","author":"Thompson","year":"1980","journal-title":"Br Heart J"},{"key":"B10","doi-asserted-by":"publisher","first-page":"352","DOI":"10.1016\/j.jmb.2009.06.077","article-title":"Uropathogenic E. coli adhesin-induced host cell receptor conformational changes: implications in transmembrane signaling transduction","volume":"392","author":"Wang","year":"2009","journal-title":"J Mol Biol"},{"key":"B11","doi-asserted-by":"publisher","first-page":"715","DOI":"10.1016\/j.amjcard.2012.04.051","article-title":"\u03b2 blockers for prevention of exercise-induced left ventricular outflow tract obstruction in patients with hypertrophic cardiomyopathy","volume":"110","author":"Nistri","year":"2012","journal-title":"Am J Cardiol"},{"key":"B12","doi-asserted-by":"publisher","first-page":"35","DOI":"10.1136\/hrt.42.1.35","article-title":"Treatment of hypertrophic obstructive cardiomyopathy with verapamil","volume":"42","author":"Kaltenbach","year":"1979","journal-title":"Br Heart J"},{"key":"B13","doi-asserted-by":"publisher","first-page":"1208","DOI":"10.1161\/01.CIR.60.6.1208","article-title":"Verapamil therapy: a new approach to the pharmacologic treatment of hypertrophic cardiomyopathy. II. Effects on exercise capacity and symptomatic status","volume":"60","author":"Rosing","year":"1979","journal-title":"Circulation"},{"key":"B14","doi-asserted-by":"publisher","first-page":"437","DOI":"10.1161\/01.CIR.64.3.437","article-title":"Verapamil: its potential for causing serious complications in patients with hypertrophic cardiomyopathy","volume":"64","author":"Epstein","year":"1981","journal-title":"Circulation"},{"key":"B15","doi-asserted-by":"publisher","first-page":"701","DOI":"10.1536\/ihj.27.701","article-title":"Comparable effects of oral diltiazem and verapamil in the treatment of hypertrophic cardiomyopathy. Double-blind crossover study","volume":"27","author":"Toshima","year":"1986","journal-title":"Jpn Heart J"},{"key":"B16","doi-asserted-by":"publisher","first-page":"59","DOI":"10.1002\/ejhf.3120","article-title":"Real-world candidacy to mavacamten in a contemporary hypertrophic obstructive cardiomyopathy population","volume":"26","author":"Bertero","year":"2024","journal-title":"Eur J Heart Fail"},{"key":"B17","doi-asserted-by":"publisher","first-page":"1190181","DOI":"10.3389\/fcvm.2023.1190181","article-title":"Comparing the efficacy and safety of medications in adults with hypertrophic cardiomyopathy: a systematic review and network meta-analysis","volume":"10","author":"Mi","year":"2023","journal-title":"Front Cardiovasc Med"},{"key":"B18","doi-asserted-by":"publisher","first-page":"695247","DOI":"10.3389\/fcvm.2021.695247","article-title":"Non-pharmaceutical interventions for hypertrophic cardiomyopathy: a mini review","volume":"8","author":"He","year":"2021","journal-title":"Front Cardiovasc Med"},{"key":"B19","doi-asserted-by":"publisher","first-page":"1251","DOI":"10.1016\/j.jacc.2005.01.012","article-title":"Multicenter study of the efficacy and safety of disopyramide in obstructive hypertrophic cardiomyopathy","volume":"45","author":"Sherrid","year":"2005","journal-title":"J Am Coll Cardiol"},{"key":"B20","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1016\/j.echo.2014.10.003","article-title":"Recommendations for cardiac chamber quantification by echocardiography in adults: an update from the American Society of Echocardiography and the European Association of Cardiovascular Imaging","volume":"28","author":"Lang","year":"2015","journal-title":"J Am Soc Echocardiogr"},{"key":"B21","doi-asserted-by":"publisher","first-page":"1261","DOI":"10.1053\/euhj.1998.0978","article-title":"Pattern of left ventricular filling in hypertrophic cardiomyopathy. Assessment by Doppler echocardiography and radionuclide angiography","volume":"19","author":"Losi","year":"1998","journal-title":"Eur Heart J"},{"key":"B22","doi-asserted-by":"publisher","first-page":"2733","DOI":"10.1093\/eurheartj\/ehu284","article-title":"2014 ESC guidelines on diagnosis and management of hypertrophic cardiomyopathy: the task force for the diagnosis and management of hypertrophic cardiomyopathy of the European Society of Cardiology (ESC)","volume":"35","author":"Elliott","year":"2014","journal-title":"Eur Heart J"},{"key":"B23","doi-asserted-by":"publisher","first-page":"7","DOI":"10.1186\/1476-7120-8-7","article-title":"Echocardiography in patients with hypertrophic cardiomyopathy: usefulness of old and new techniques in the diagnosis and pathophysiological assessment","volume":"8","author":"Losi","year":"2010","journal-title":"Cardiovasc Ultrasound"},{"key":"B24","doi-asserted-by":"publisher","first-page":"2313","DOI":"10.1016\/j.jacc.2011.08.040","article-title":"Long-term survival in patients with resting obstructive hypertrophic cardiomyopathy comparison of conservative versus invasive treatment","volume":"58","author":"Ball","year":"2011","journal-title":"J Am Coll Cardiol"},{"key":"B25","doi-asserted-by":"publisher","first-page":"795","DOI":"10.1016\/j.jacbts.2019.06.004","article-title":"Electrophysiological and Contractile effects of disopyramide in patients with obstructive hypertrophic cardiomyopathy: a translational study","volume":"4","author":"Coppini","year":"2019","journal-title":"JACC Basic Transl Sci"},{"key":"B26","doi-asserted-by":"publisher","first-page":"741","DOI":"10.7326\/M18-3016","article-title":"Mavacamten treatment for obstructive hypertrophic cardiomyopathy: a clinical trial","volume":"170","author":"Heitner","year":"2019","journal-title":"Ann Intern Med"},{"key":"B27","doi-asserted-by":"publisher","first-page":"968","DOI":"10.1001\/jamacardio.2023.3342","article-title":"Mavacamten in patients with hypertrophic cardiomyopathy referred for septal reduction: week 56 results from the VALOR-HCM randomized clinical trial","volume":"8","author":"Desai","year":"2023","journal-title":"JAMA Cardiol"},{"key":"B28","doi-asserted-by":"publisher","first-page":"759","DOI":"10.1016\/S0140-6736(20)31792-X","article-title":"Mavacamten for treatment of symptomatic obstructive hypertrophic cardiomyopathy (EXPLORER-HCM): a randomised, double-blind, placebo-controlled, phase 3 trial","volume":"396","author":"Olivotto","year":"2020","journal-title":"Lancet"},{"key":"B29","doi-asserted-by":"publisher","first-page":"694","DOI":"10.1161\/CIRCHEARTFAILURE.112.000122","article-title":"Treatment of obstructive hypertrophic cardiomyopathy symptoms and gradient resistant to first-line therapy with beta-blockade or verapamil","volume":"6","author":"Sherrid","year":"2013","journal-title":"Circ Heart Fail"},{"key":"B30","doi-asserted-by":"publisher","DOI":"10.3390\/jcm12072725","article-title":"Real-world use and predictors of response to disopyramide in patients with obstructive hypertrophic cardiomyopathy","volume":"12","author":"Maurizi","year":"2023","journal-title":"J Clin Med"},{"key":"B31","doi-asserted-by":"publisher","first-page":"1691","DOI":"10.1016\/0002-8703(93)90760-7","article-title":"Acute dose-response effects of intravenous disopyramide in hypertrophic obstructive cardiomyopathy","volume":"125","author":"Kimball","year":"1993","journal-title":"Am Heart J"},{"key":"B32","doi-asserted-by":"publisher","first-page":"547","DOI":"10.1016\/S0002-9149(98)00911-4","article-title":"Influence of left ventricular cavity size on clinical presentation in hypertrophic cardiomyopathy","volume":"83","author":"Manganelli","year":"1999","journal-title":"Am J Cardiol"},{"key":"B33","doi-asserted-by":"publisher","first-page":"75","DOI":"10.1016\/j.ijcard.2019.09.066","article-title":"Markers of responsiveness to disopyramide in patients with hypertrophic cardiomyopathy","volume":"297","author":"Habib","year":"2019","journal-title":"Int J Cardiol"},{"key":"B34","doi-asserted-by":"publisher","first-page":"162","DOI":"10.1016\/S0002-9149(00)00854-7","article-title":"Utility of cardiopulmonary exercise in the assessment of clinical determinants of functional capacity in hypertrophic cardiomyopathy","volume":"86","author":"Sharma","year":"2000","journal-title":"Am J Cardiol"},{"key":"B35","doi-asserted-by":"publisher","DOI":"10.1093\/eurjpc\/zwae198","article-title":"The impact of disopyramide on exercise capacity among patients with obstructive hypertrophic cardiomyopathy: beyond LVOT gradient","author":"Halasz","year":"2024","journal-title":"Eur J Prev Cardiol"},{"key":"B36","doi-asserted-by":"publisher","first-page":"775","DOI":"10.1056\/NEJM199703133361107","article-title":"The management of hypertrophic cardiomyopathy","volume":"336","author":"Spirito","year":"1997","journal-title":"N Engl J Med"},{"key":"B37","doi-asserted-by":"publisher","first-page":"308","DOI":"10.1152\/physiol.00021.2012","article-title":"No rest for the weary: diastolic calcium homeostasis in the normal and failing myocardium","volume":"27","author":"Louch","year":"2012","journal-title":"Physiology (Bethesda)"},{"key":"B38","doi-asserted-by":"publisher","first-page":"e011307","DOI":"10.1161\/CIRCHEARTFAILURE.123.011307","article-title":"Diastolic tone-friend and foe of cardiac performance","volume":"16","author":"Meyer","year":"2023","journal-title":"Circ Heart Fail"},{"key":"B39","doi-asserted-by":"publisher","first-page":"1243","DOI":"10.1378\/chest.07-1188","article-title":"Oral disopyramide for the acute treatment of severe outflow obstruction in hypertrophic cardiomyopathy in the ICU setting","volume":"133","author":"Sirak","year":"2008","journal-title":"Chest"}],"container-title":["Frontiers in Cardiovascular Medicine"],"original-title":[],"link":[{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fcvm.2024.1416600\/full","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,8,14]],"date-time":"2024-08-14T06:11:03Z","timestamp":1723615863000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fcvm.2024.1416600\/full"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,8,14]]},"references-count":39,"alternative-id":["10.3389\/fcvm.2024.1416600"],"URL":"https:\/\/doi.org\/10.3389\/fcvm.2024.1416600","relation":{},"ISSN":["2297-055X"],"issn-type":[{"type":"electronic","value":"2297-055X"}],"subject":[],"published":{"date-parts":[[2024,8,14]]},"article-number":"1416600"}}