Integrated role of cardiac magnetic resonance and genetics in predicting left ventricular reverse remodelling in dilated and non‐dilated cardiomyopathy

射血分数 扩张型心肌病 医学 心脏病学 内科学 LMNA公司 心力衰竭 心源性猝死 心脏磁共振成像 心肌病 磁共振成像 放射科 拉明 精神科 核心
作者
Martina Setti,Manuela Iseppi,Job A.J. Verdonschot,Jacopo Giulio Rizzi,Alessia Paldino,Carola Pio Loco Detto Gava,Giulia Barbati,Matteo Dal Ferro,Max F.G.H.M. Venner,Anne G. Raafs,Marta Gigli,Davide Stolfo,Antonio De Luca,Giulia De Angelis,T Capovilla,Sharon Graw,Flavio Ribichini,Matthew R.G. Taylor,Luisa Mestroni,Stéphane Heymans
出处
期刊:European Journal of Heart Failure [Elsevier BV]
标识
DOI:10.1002/ejhf.3671
摘要

Abstract Aims Left ventricular reverse remodelling (LVRR) is a prognostic marker in patients with dilated (DCM) and non‐dilated left ventricular cardiomyopathy (NDLVC). The utility of combining late gadolinium enhancement (LGE) and genetic testing in predicting LVRR in DCM/NDLVC remains a knowledge gap. This study aimed to assess an integrated approach including LGE data and genetics to predict LVRR in DCM/NDLVC patients. Methods and results This multicentre observational study included DCM/NDLVC patients with: (i) baseline echocardiographic left ventricular ejection fraction (LVEF) <50%; (ii) genetic testing; (iii) baseline cardiac magnetic resonance (CMR); (iv) 12‐month follow‐up echocardiographic data. LVRR was defined as LVEF increase ≥10% or LVEF ≥50% (if baseline LVEF <45%) at 12 months. Outcome measures were: (i) all‐cause mortality, heart transplant, or left ventricular assist device implantation (D/HT/LVAD); (ii) sudden cardiac death or major ventricular arrhythmias (SCD/MVA). Arrhythmogenic genes studied were LMNA , DSP , FLNC, and RBM20 . Among 1757 DCM/NDLVC with genetic data, 616 met eligibility (462 DCM, 154 NDLVC; age 51 ± 14 years, 34% female). LVRR occurred in 314 patients (51%): 251 (54%) in DCM and 63 (41%) in NDLVC ( p = 0.004). Independent predictors of LVRR within 1 year included titin truncating variants, absence of arrhythmogenic genes, and absence of LGE ring‐like pattern. In patients with LVEF <35%, only the presence of LGE ring‐like pattern and arrhythmogenic genes remained independently related to a lower rate of LVRR and increased SCD/MVA risk. Conclusion In a large genetically and CMR characterized DCM/NDLVC cohort, arrhythmogenic genotypes and LGE ring‐like pattern were inversely related to LVRR, particularly in patients with LVEF <35%.

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