1 research outputs found
Combination of late gadolinium enhancement and genotype improves prediction of prognosis in non-ischaemic dilated cardiomyopathy
Aims: Genotype and left ventricular scar on cardiac magnetic resonance (CMR) are increasingly recognized as risk markers for adverse outcomes in non-ischaemic dilated cardiomyopathy (DCM). We investigated the combined influence of genotype and late gadolinium enhancement (LGE) in assessing prognosis in a large cohort of patients with DCM. Methods and results: Outcomes of 600 patients with DCM (53.3 ± 14.1 years, 66% male) who underwent clinical CMR and genetic testing were retrospectively analysed. The primary endpoints were end-stage heart failure (ESHF) and malignant ventricular arrhythmias (MVA). During a median follow-up of 2.7 years (interquartile range 1.3â4.9), 24 (4.00%) and 48 (8.00%) patients had ESHF and MVA, respectively. In total, 242 (40.3%) patients had pathogenic/likely pathogenic variants (positive genotype) and 151 (25.2%) had LGE. In survival analysis, positive LGE was associated with MVA and ESHF (both, p < 0.001) while positive genotype was associated with ESHF (p = 0.034) but not with MVA (p = 0.102). Classification of patients according to genotype (G+/Gâ) and LGE presence (L+/Lâ) revealed progressively increasing events across Lâ/Gâ, Lâ/G+, L+/Gâ and L+/G+ groups and resulted in optimized MVA and ESHF prediction (p < 0.001 and p = 0.001, respectively). Hazard ratios for MVA and ESHF in patients with either L+ or G+ compared with those with Lâ/Gâ were 4.71 (95% confidence interval: 2.11â10.50, p < 0.001) and 7.92 (95% confidence interval: 1.86â33.78, p < 0.001), respectively. Conclusion: Classification of patients with DCM according to genotype and LGE improves MVA and ESHF prediction. Scar assessment with CMR and genotyping should be considered to select patients for primary prevention implantable cardioverter-defibrillator placementThis work was supported by grants from the Instituto de Salud
Carlos III (ISCIII) (PI18/0004, PI19/01283, PI20/0320). (Co-funded
by European Regional Development Fund/European Social Fund
âA way to make Europeâ/âInvesting in your futureâ). The Hospital
Universitario Puerta de Hierro Majadahonda, the Hospital Clinic,
the Hospital Vall dâHebron, the Hospital General Universitario
Gregorio Marañón and the Hospital Universitario Virgen de la
Arrixaca are members of the European Reference Network for
rare, low-prevalence, and complex diseases of the heart (ERN
GUARD-Heart). The CNIC is supported by the ISCIII, MCIN,
the Pro-CNIC Foundation, and the Severo Ochoa Centers of
Excellence program (CEX2020-001041-S).
Conflict of interest: none declare