Mid‐ventricular obstruction is associated with non‐sustained ventricular tachycardia in patients with hypertrophic obstructive cardiomyopathy

Nie, Changrong; Zhu, Changsheng; Xiao, Mengsu; Yang, Qiulan; Meng, Yanhai; Wu, Rong; Wang, Shuiyun

doi:10.1002/clc.23575

Cited by 4 publications

(2 citation statements)

References 25 publications

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“…The prevalence of LVMCO has been reported to be as high as 9% to 13% in patients with HCM. 20,21 Notably, as many patients with LVMCO have primary or secondary indications for transvenous ICDs, a trial of PPoP therapy may have a key role earlier in the management of these patients, with progression to other therapies if this fails.…”

Section: Discussionmentioning

confidence: 99%

Distal Ventricular Pacing for Drug-Refractory Mid-Cavity Obstructive Hypertrophic Cardiomyopathy: A Randomized, Placebo-Controlled Trial of Personalized Pacing

Malcolmson,

Hughes,

Husselbury

et al. 2024

Circ: Arrhythmia and Electrophysiology

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BACKGROUND: Patients with refractory, symptomatic left ventricular (LV) mid-cavity obstructive (LVMCO) hypertrophic cardiomyopathy have few therapeutic options. Right ventricular pacing is associated with modest hemodynamic and symptomatic improvement, and LV pacing pilot data suggest therapeutic potential. We hypothesized that site-specific pacing would reduce LVMCO gradients and improve symptoms. METHODS: Patients with symptomatic-drug-refractory LVMCO were recruited for a randomized, blinded trial of personalized prescription of pacing (PPoP). Multiple LV and apical right ventricular pacing sites were assessed during an invasive hemodynamic study of multisite pacing. Patient-specific pacing-site and atrioventricular delays, defining PPoP, were selected on the basis of LVMCO gradient reduction and acceptable pacing parameters. Patients were randomized to 6 months of active PPoP or backup pacing in a crossover design. The primary outcome examined invasive gradient change with best-site pacing. Secondary outcomes assessed quality of life and exercise following randomization to PPoP. RESULTS: A total of 17 patients were recruited; 16 of whom met primary end points. Baseline New York Heart Association was 3±0.6, despite optimal medical therapy. Hemodynamic effects were assessed during pacing at the right ventricular apex and at a mean of 8 LV sites. The gradients in all 16 patients fell with pacing, with maximum gradient reduction achieved via LV pacing in 14 (88%) patients and right ventricular apex in 2. The mean baseline gradient of 80±29 mm Hg fell to 31±21 mm Hg with best-site pacing, a 60% reduction ( P <0.0001). One cardiac vein perforation occurred in 1 case, and 15 subjects entered crossover; 2 withdrawals occurred during crossover. Of the 13 completing crossover, 9 (69%) chose active pacing in PPoP configuration as preferred setting. PPoP was associated with improved 6-minute walking test performance (328.5±99.9 versus 285.8±105.5 m; P =0.018); other outcome measures also indicated benefit with PPoP. CONCLUSIONS: In a randomized placebo-controlled trial, PPoP reduces obstruction and improves exercise performance in severely symptomatic patients with LVMCO. REGISTRATION: URL: https://clinicaltrials.gov ; Unique Identifier: NCT03450252.

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Section: Discussionmentioning

confidence: 99%

Distal Ventricular Pacing for Drug-Refractory Mid-Cavity Obstructive Hypertrophic Cardiomyopathy: A Randomized, Placebo-Controlled Trial of Personalized Pacing

Malcolmson,

Hughes,

Husselbury

et al. 2024

Circ: Arrhythmia and Electrophysiology

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show abstract

“…Prevalence of LVMCO has been reported to be as high as 9-13% of HCM patients. 20, 21 Notably, as many patients with LVMCO have primary or secondary indications for transvenous ICDs, a trial of PPoC therapy may have a key role earlier in the management of these patients, with progression to other therapies if this fails.…”

Section: Discussionmentioning

confidence: 99%

Distal Ventricular Pacing for Drug-Refractory Mid-Cavity Obstructive Hypertrophic Cardiomyopathy: A Randomized, Placebo-Controlled Trial of Personalized Pacing

Malcolmson,

Hughes,

Husselbury

et al. 2023

Preprint

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BackgroundPatients with refractory symptomatic left ventricular (LV) mid-cavity obstructive (LVMCO) hypertrophic cardiomyopathy (HCM) have few therapeutic options. Right ventricular (RV) pacing is associated with modest hemodynamic and symptomatic improvement, and LV pacing pilot data suggest therapeutic potential. We hypothesized site-specific-pacing would reduce LVMCO gradients and improve symptoms.MethodsPatients with symptomatic-drug-refractory LVMCO were recruited for a randomized blinded trial of personalized prescription of pacing (PPoP). Multiple LV and apical RV pacing sites were assessed during invasive hemodynamic study of multisite pacing. Patient-specific pacing-site and atrioventricular (AV) delays, defining PPoP, were selected on basis of LVMCO gradient reduction and acceptable pacing parameters. Patients were randomized to 6-months of active PPoP or back-up pacing in a cross-over design. The primary outcome examined invasive gradient change with best-site pacing. Secondary outcomes assessed quality of life and exercise following randomization to PPoP.ResultsA total of 17 patients were recruited; 16 of whom met primary endpoints. Baseline NYHA was 3±0.6 despite optimal medical therapy. Hemodynamic effects were assessed during pacing at the RV apex and at a mean of 8 LV sites. The gradients in all 16 patients fell with pacing, with maximum gradient reduction achieved via LV pacing in 14 (88%) patients and RV apex in 2. The mean baseline gradient of 80±29 mmHg, fell to 31±21 mmHg with best-site pacing, a 60% reduction (p<0.0001). One cardiac vein perforation occurred in one case, and 15 subjects entered cross-over; 2 withdrawals occurred during cross-over. Of the 13 completing cross-over, 9 (69%) chose active pacing in PPoP configuration as preferred setting. PPoP was associated with improved 6-minute walking test performance (328.5±99.9 vs 285.8±105.5 meters, p=0.018); other outcome measures also indicated benefit with PPoP.ConclusionsIn a randomized placebo-controlled trial, LV pacing reduces obstruction and improves exercise performance in severely symptomatic LVMCO patients.RegistrationNCT03450252.Clinical PerspectiveWhat is Known?Patients with refractory, symptomatic LVMCO present a significant challenge for clinical management, with very few treatment options.Data on the use of right ventricular (RV) pacing in patients with refractory, symptomatic LVMCO indicate suboptimal therapeutic responses whilst pilot data indicate a potential therapeutic role for LV pacing.What the Study Adds?Personalized prescription of pacing (PPoP) therapy guided by invasive hemodynamics significantly reduced LVMCO gradients and improved exercise performance in the first randomized, placebo-controlled trial in symptomatic LVMCO.This study provides the basis for a multicenter trial of PPoP for LVMCO and for the use of site-specific pacing in managing other forms of HCM.Graphical Abstract

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Definitional Challenges in Understanding Hypertrophic Cardiomyopathy

Federspiel,

Pfeifer,

Ramsthaler

et al. 2024

Diagnostics

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Hypertrophic cardiomyopathy (HCM) is the most common hereditary cardiomyopathy. It is often caused by mutations of genes encoding for sarcomeric or sarcomere-associated proteins. Despite its clinical importance, divergent definitions are published by major cardiology societies. Some regard HCM as a specific genetic disease, whereas others define it as a broad ‘spectrum of the thick heart’. The present narrative review aimed to assess both definitions from a pathoanatomical perspective. As a conjoint interdisciplinary and translational approach is needed to further increase knowledge and improve the understanding of HCM, the PubMed database was searched using several advanced search algorithms to explore the perspectives of the (forensic) pathologist, clinician, and basic researcher regarding the difference between the definitions of HCM. This discrepancy between definitions can impact critical data, such as prevalence and mortality rate, and complicate the understanding of the disease. For example, due to the different definitions, research findings regarding molecular changes from studies applying the narrow definition cannot be simply extended to the ‘spectrum’ of HCM.

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Mid‐ventricular obstruction is associated with non‐sustained ventricular tachycardia in patients with hypertrophic obstructive cardiomyopathy

Cited by 4 publications

References 25 publications

Distal Ventricular Pacing for Drug-Refractory Mid-Cavity Obstructive Hypertrophic Cardiomyopathy: A Randomized, Placebo-Controlled Trial of Personalized Pacing

Distal Ventricular Pacing for Drug-Refractory Mid-Cavity Obstructive Hypertrophic Cardiomyopathy: A Randomized, Placebo-Controlled Trial of Personalized Pacing

Distal Ventricular Pacing for Drug-Refractory Mid-Cavity Obstructive Hypertrophic Cardiomyopathy: A Randomized, Placebo-Controlled Trial of Personalized Pacing

Definitional Challenges in Understanding Hypertrophic Cardiomyopathy

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