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Real-World Use and Predictors of Response to Disopyramide in Patients with Obstructive Hypertrophic Cardiomyopathy

Background: Although disopyramide has been widely used to reduce left ventricular outflow obstruction (LVOTO) and to improve symptoms in patients with obstructive hypertrophic cardiomyopathy (oHCM), its use in real world as well as patient characteristics associated with a positive treatment respons...

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Autores principales: Maurizi, Niccolò, Chiriatti, Chiara, Fumagalli, Carlo, Targetti, Mattia, Passantino, Silvia, Antiochos, Panagiotis, Skalidis, Ioannis, Chiti, Chiara, Biagioni, Giulia, Tomberli, Alessia, Giovani, Sara, Coppini, Raffaele, Cecchi, Franco, Olivotto, Iacopo
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10095445/
https://www.ncbi.nlm.nih.gov/pubmed/37048808
http://dx.doi.org/10.3390/jcm12072725
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author Maurizi, Niccolò
Chiriatti, Chiara
Fumagalli, Carlo
Targetti, Mattia
Passantino, Silvia
Antiochos, Panagiotis
Skalidis, Ioannis
Chiti, Chiara
Biagioni, Giulia
Tomberli, Alessia
Giovani, Sara
Coppini, Raffaele
Cecchi, Franco
Olivotto, Iacopo
author_facet Maurizi, Niccolò
Chiriatti, Chiara
Fumagalli, Carlo
Targetti, Mattia
Passantino, Silvia
Antiochos, Panagiotis
Skalidis, Ioannis
Chiti, Chiara
Biagioni, Giulia
Tomberli, Alessia
Giovani, Sara
Coppini, Raffaele
Cecchi, Franco
Olivotto, Iacopo
author_sort Maurizi, Niccolò
collection PubMed
description Background: Although disopyramide has been widely used to reduce left ventricular outflow obstruction (LVOTO) and to improve symptoms in patients with obstructive hypertrophic cardiomyopathy (oHCM), its use in real world as well as patient characteristics associated with a positive treatment response are still unclear. Methods: From 1980 to 2021, 1527 patients with HCM were evaluated and 372 (23%) had a LVOTO with active follow-up. The efficacy and safety of disopyramide were assessed systematically during 12 months (2-, 6-, and 12-month visits). Responders were patients with a final NYHA = I and a LVOTO < 30 mmHg; incomplete responders were those patients with NYHA > I and a LVOTO < 30 mmHg; and non-responders were symptomatic patients with no change in functional class NYHA and a LVOT gradient > 30 mmHg. Results: Two-hundred-fifty-four (66%) patients were in functional class NYHA I/II and 118 (34%) in NYHA III/IV. A total of 118/372 (32%, 55 ± 16 years) underwent disopyramide therapy. Twenty-eight (24%) patients responded to therapy, 39 (33%) were incomplete responders, and 51 (43%) did not respond. Responder were mainly patients in functional NYHA class I/II (24/28, 86%), whereas incomplete responders and non-responders were more often in functional NYHA class III/IV (50/54 (93%)). An independent predictor of response to disopyramide treatment was the presence of NYHA I/II at the initiation of therapy (HR 1.5 (95% CI 1.1–4.5), p = 0.03). No major life-threatening arrhythmic events or syncope occurred, despite 19 (16%) patients showing reduced QTc from baseline, 19 (16%) having no difference, while 80 (69%) patients had prolonged QTc interval. Thirty-one (26%) patients experienced side effects, in particular, 29 of the anticholinergic type. Conclusions: Disopyramide was underused in oHCM but effective in reducing LVOTO gradients and symptoms in slightly symptomatic patients with less severe disease phenotype with a safe pro-arrhythmic profile.
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spelling pubmed-100954452023-04-13 Real-World Use and Predictors of Response to Disopyramide in Patients with Obstructive Hypertrophic Cardiomyopathy Maurizi, Niccolò Chiriatti, Chiara Fumagalli, Carlo Targetti, Mattia Passantino, Silvia Antiochos, Panagiotis Skalidis, Ioannis Chiti, Chiara Biagioni, Giulia Tomberli, Alessia Giovani, Sara Coppini, Raffaele Cecchi, Franco Olivotto, Iacopo J Clin Med Article Background: Although disopyramide has been widely used to reduce left ventricular outflow obstruction (LVOTO) and to improve symptoms in patients with obstructive hypertrophic cardiomyopathy (oHCM), its use in real world as well as patient characteristics associated with a positive treatment response are still unclear. Methods: From 1980 to 2021, 1527 patients with HCM were evaluated and 372 (23%) had a LVOTO with active follow-up. The efficacy and safety of disopyramide were assessed systematically during 12 months (2-, 6-, and 12-month visits). Responders were patients with a final NYHA = I and a LVOTO < 30 mmHg; incomplete responders were those patients with NYHA > I and a LVOTO < 30 mmHg; and non-responders were symptomatic patients with no change in functional class NYHA and a LVOT gradient > 30 mmHg. Results: Two-hundred-fifty-four (66%) patients were in functional class NYHA I/II and 118 (34%) in NYHA III/IV. A total of 118/372 (32%, 55 ± 16 years) underwent disopyramide therapy. Twenty-eight (24%) patients responded to therapy, 39 (33%) were incomplete responders, and 51 (43%) did not respond. Responder were mainly patients in functional NYHA class I/II (24/28, 86%), whereas incomplete responders and non-responders were more often in functional NYHA class III/IV (50/54 (93%)). An independent predictor of response to disopyramide treatment was the presence of NYHA I/II at the initiation of therapy (HR 1.5 (95% CI 1.1–4.5), p = 0.03). No major life-threatening arrhythmic events or syncope occurred, despite 19 (16%) patients showing reduced QTc from baseline, 19 (16%) having no difference, while 80 (69%) patients had prolonged QTc interval. Thirty-one (26%) patients experienced side effects, in particular, 29 of the anticholinergic type. Conclusions: Disopyramide was underused in oHCM but effective in reducing LVOTO gradients and symptoms in slightly symptomatic patients with less severe disease phenotype with a safe pro-arrhythmic profile. MDPI 2023-04-06 /pmc/articles/PMC10095445/ /pubmed/37048808 http://dx.doi.org/10.3390/jcm12072725 Text en © 2023 by the authors. https://creativecommons.org/licenses/by/4.0/Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/).
spellingShingle Article
Maurizi, Niccolò
Chiriatti, Chiara
Fumagalli, Carlo
Targetti, Mattia
Passantino, Silvia
Antiochos, Panagiotis
Skalidis, Ioannis
Chiti, Chiara
Biagioni, Giulia
Tomberli, Alessia
Giovani, Sara
Coppini, Raffaele
Cecchi, Franco
Olivotto, Iacopo
Real-World Use and Predictors of Response to Disopyramide in Patients with Obstructive Hypertrophic Cardiomyopathy
title Real-World Use and Predictors of Response to Disopyramide in Patients with Obstructive Hypertrophic Cardiomyopathy
title_full Real-World Use and Predictors of Response to Disopyramide in Patients with Obstructive Hypertrophic Cardiomyopathy
title_fullStr Real-World Use and Predictors of Response to Disopyramide in Patients with Obstructive Hypertrophic Cardiomyopathy
title_full_unstemmed Real-World Use and Predictors of Response to Disopyramide in Patients with Obstructive Hypertrophic Cardiomyopathy
title_short Real-World Use and Predictors of Response to Disopyramide in Patients with Obstructive Hypertrophic Cardiomyopathy
title_sort real-world use and predictors of response to disopyramide in patients with obstructive hypertrophic cardiomyopathy
topic Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10095445/
https://www.ncbi.nlm.nih.gov/pubmed/37048808
http://dx.doi.org/10.3390/jcm12072725
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