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| Pàgina inicial > Articles > Articles publicats > Late gadolinium enhancement distribution patterns in non-ischaemic dilated cardiomyopathy : |
(Hospital Universitario Puerta de Hierro Majadahonda (Madrid)) (Hospital Universitario Puerta de Hierro Majadahonda (Madrid)) (Instituto de Investigación Biomédica de Salamanca (Salamanca, Castilla y León)) (Hospital Universitari Vall d'Hebron. Institut de Recerca) (Hospital Universitario de Navarra) (Hospital Clínico Universitario de Valladolid) (Hospital General Universitario de Alicante (Alacant, País Valencià)) (Hospital Clínic i Provincial de Barcelona) (Complejo Hospitalario Universitario de Santiago de Compostela) (Hospital Universitari de Girona Doctor Josep Trueta) (Instituto de Investigacion Biosanitaria de Granada) (Institut Germans Trias i Pujol. Hospital Universitari Germans Trias i Pujol) (Universidad Francisco de Vitoria) (Complejo Asistencial Universitario de Salamanca) (Universidad Francisco de Vitoria)| Data: | 2023 |
| Resum: | Late gadolinium enhancement (LGE) is frequently found in patients with dilated cardiomyopathy (DCM); there is little information about its frequency and distribution pattern according to the underlying genetic substrate. We sought to describe LGE patterns according to genotypes and to analyse the risk of major ventricular arrhythmias (MVA) according to patterns. Cardiac magnetic resonance findings and LGE distribution according to genetics were performed in a cohort of 600 DCM patients followed at 20 Spanish centres. After exclusion of individuals with multiple causative gene variants or with variants in infrequent DCM-causing genes, 577 patients (34% females, mean age 53. 5 years, left ventricular ejection fraction 36. 9 ± 13. 9%) conformed to the final cohort. A causative genetic variant was identified in 219 (38%) patients, and 147 (25. 5%) had LGE. Significant differences were found comparing LGE patterns between genes (P < 0. 001). LGE was absent or rare in patients with variants in TNNT2, RBM20, and MYH7 (0, 5, and 20%, respectively). Patients with variants in DMD, DSP, and FLNC showed a predominance of LGE subepicardial patterns (50, 41, and 18%, respectively), whereas patients with variants in TTN, BAG3, LMNA, and MYBPC3 showed unspecific LGE patterns. The genetic yield differed according to LGE patterns. Patients with subepicardial, lineal midwall, transmural, and right ventricular insertion points or with combinations of LGE patterns showed an increased risk of MVA compared with patients without LGE. LGE patterns in DCM have a specific distribution according to the affected gene. Certain LGE patterns are associated with an increased risk of MVA and with an increased yield of genetic testing. |
| Ajuts: | Instituto de Salud Carlos III PI18/0004 Instituto de Salud Carlos III PI19/01283 Instituto de Salud Carlos III PI20/0320 Instituto de Salud Carlos III CM20/00101 |
| Drets: | Aquest document està subjecte a una llicència d'ús Creative Commons. Es permet la reproducció total o parcial, la distribució, la comunicació pública de l'obra i la creació d'obres derivades, sempre que no sigui amb finalitats comercials, i sempre que es reconegui l'autoria de l'obra original.  |
| Llengua: | Anglès |
| Document: | Article ; recerca ; Versió publicada |
| Matèria: | Dilated cardiomyopathy ; Late gadolinium enhancement ; Cardiac magnetic resonance ; Genetics ; Sudden cardiac death |
| Publicat a: | European Heart Journal Cardiovascular Imaging, Vol. 25 (august 2023) , p. 75-85, ISSN 2047-2412 |
