Left Ventricular Hypertrabeculation and Prognosis in Dilated Cardiomyopathy
Abstract
BACKGROUND: Left ventricular (LV) hypertrabeculation, formerly termed LV noncompaction, is a heterogeneous myocardial entity linked to adverse cardiovascular outcomes. This study evaluated embolic risk in patients with dilated cardiomyopathy (DCM) according to the presence of hypertrabeculation and examined its prevalence and prognostic relevance across DCM genotypes. METHODS: Clinical data from 1160 patients with DCM evaluated by cardiac magnetic resonance imaging and genetic testing (n=997 [86%]) were collected from 22 international centers. End points included embolic events, advanced heart failure events, and major ventricular arrhythmias. RESULTS: LV hypertrabeculation was identified in 354 patients (30.5%) by fractal analysis and in 343 (29.7%) according to Petersen criteria, with good concordance. After a median follow-up of 5.1 years (interquartile range, 2.8–7.4), embolic events occurred in 37 patients (3.2%), advanced heart failure in 62 (5.3%), and major ventricular arrhythmias in 136 (11.7%). Hypertrabeculation was not associated with increased embolic risk (hazard ratio, 1.5 [95% CI, 0.75–3.00]), even among patients in sinus rhythm with LV ejection fraction ≤40% (hazard ratio, 1.89 [95% CI, 0.7–5.5]). In contrast, atrial fibrillation and reduced LV ejection fraction were associated with embolic events (both P <0.01). LV hypertrabeculation was not associated with an increased risk of major ventricular arrhythmias or advanced heart failure; genotype, LV ejection fraction, and late gadolinium enhancement emerged as the main predictors of adverse outcomes. The prevalence of LV hypertrabeculation varied across genotypes, with the highest prevalence observed in patients with sequence variants in motor sarcomeric genes (58%), TTN (38%), and genotype-negative status (33%), and the lowest prevalence observed among those with variants in cytoskeletal/Z-disk (7%) and nuclear envelope (5%) genes. Hypertrabeculation was not associated with adverse outcomes within any genotype. CONCLUSIONS: Although LV hypertrabeculation is common in DCM, it is not associated with worse outcomes and should not prompt differential clinical management. The embolic risk in patients with DCM and hypertrabeculation is low, including in those with reduced LV ejection fraction without atrial fibrillation, and does not support prophylactic anticoagulation in these patients.
Article Details
Authors (41)
Nerea Mora-Ayestarán
Department of Cardiology, Hospital Universitario Puerta de Hierro, Instituto de Investigación Sanitaria Puerta de Hierro Majadahonda (IDIPHIM), Madrid, Spain (N.M.-A., N.R.-L., J.G.M., P.G.-P.).
Noemi Ramos-Lopez
Department of Cardiology, Hospital Universitario Puerta de Hierro, Instituto de Investigación Sanitaria Puerta de Hierro Majadahonda (IDIPHIM), Madrid, Spain (N.M.-A., N.R.-L., J.G.M., P.G.-P.).
Juan Pablo Ochoa
Centro Nacional de Investigaciones Cardiovasculares (CNIC), Madrid, Spain (N.R.-L., J.P.O., A.G.-Á., P.G.-P.).
Amaia Yuan Ibargoyen
Department of Cardiology, Área del Corazón, Hospital Universitario de Navarra, Pamplona, Spain (A.Y.I., M.S., G.L.-L., M.T.B.-E.).
Eduard Claver-Garrido
Bioheart Group, Cardiovascular, Respiratory and Systemic Diseases and Cellular Aging Program, Institut d’Investigació Biomèdica de Bellvitge (IDIBELL; E.C.-G., C.D.-L., A.D.), l’Hospitalet de Llobregat, Spain.
Cristina Gómez-González
Department of Cardiology, Hospital General Universitario Gregorio Marañón, Madrid, Spain (C.G.-G., E.R.G., M.A.E.-C.).
Ahmad S. Amin
Department of Experimental Cardiology, Amsterdam Cardiovascular Sciences, Heart Failure & Arrhythmias, Amsterdam University Medical Centers, University of Amsterdam, The Netherlands (A.S.A., E.P., C.R.B.).
María Gallego-Delgado
CIBER Cardiovascular, Instituto de Salud Carlos III, Madrid, Spain (C.G.-G., M.G.-D., G.C.-M., M.N.-P., J.M.G.-P., G.C.-V., A.B.-G., R.S.-B., A.G.-Á., M.B., E.Z., E.R.G., E.V., J.L.-F., M.S.-M., R.B.-V., A.D., M.A.E.-C., J.F.R.-P., J.G.M., P.G.-P.).
Guillem Casas-Masnou
CIBER Cardiovascular, Instituto de Salud Carlos III, Madrid, Spain (C.G.-G., M.G.-D., G.C.-M., M.N.-P., J.M.G.-P., G.C.-V., A.B.-G., R.S.-B., A.G.-Á., M.B., E.Z., E.R.G., E.V., J.L.-F., M.S.-M., R.B.-V., A.D., M.A.E.-C., J.F.R.-P., J.G.M., P.G.-P.).
Marina Navarro-Peñalver
CIBER Cardiovascular, Instituto de Salud Carlos III, Madrid, Spain (C.G.-G., M.G.-D., G.C.-M., M.N.-P., J.M.G.-P., G.C.-V., A.B.-G., R.S.-B., A.G.-Á., M.B., E.Z., E.R.G., E.V., J.L.-F., M.S.-M., R.B.-V., A.D., M.A.E.-C., J.F.R.-P., J.G.M., P.G.-P.).
Jose María Larrañaga-Moreira
Unidad de Cardiopatías Familiares, Servicio de Cardiología, Complexo Hospitalario Universitario A Coruña, Instituto de Investigación Biomédica de A Coruña, Spain (J.M.L.-M., R.B.-V.).
José Manuel García-Pinilla
CIBER Cardiovascular, Instituto de Salud Carlos III, Madrid, Spain (C.G.-G., M.G.-D., G.C.-M., M.N.-P., J.M.G.-P., G.C.-V., A.B.-G., R.S.-B., A.G.-Á., M.B., E.Z., E.R.G., E.V., J.L.-F., M.S.-M., R.B.-V., A.D., M.A.E.-C., J.F.R.-P., J.G.M., P.G.-P.).
Francisco J. Bermudez-Jimenez
Servicio de Cardiología, Hospital Universitario Virgen de las Nieves, Granada, Spain (F.J.B.-J.).
Maria Victoria Mogollón-Jiménez
Department of Cardiology, Complejo Hospitalario Universitario de Cáceres, Spain (M.V.M.-J.).
Gonzalo Cabezón-Villalba
CIBER Cardiovascular, Instituto de Salud Carlos III, Madrid, Spain (C.G.-G., M.G.-D., G.C.-M., M.N.-P., J.M.G.-P., G.C.-V., A.B.-G., R.S.-B., A.G.-Á., M.B., E.Z., E.R.G., E.V., J.L.-F., M.S.-M., R.B.-V., A.D., M.A.E.-C., J.F.R.-P., J.G.M., P.G.-P.).
Antoni Bayés-Genís
Vicente Climent-Payá
Heart Failure and Inherited Cardiac Diseases Unit, Cardiology Department, Dr Balmis General University Hospital, Alicante, Spain (V.C.-P.).
Rafael Salguero-Bodes
CIBER Cardiovascular, Instituto de Salud Carlos III, Madrid, Spain (C.G.-G., M.G.-D., G.C.-M., M.N.-P., J.M.G.-P., G.C.-V., A.B.-G., R.S.-B., A.G.-Á., M.B., E.Z., E.R.G., E.V., J.L.-F., M.S.-M., R.B.-V., A.D., M.A.E.-C., J.F.R.-P., J.G.M., P.G.-P.).
Ana García-Álvarez
Centro Nacional de Investigaciones Cardiovasculares Carlos III, Madrid
María Brion
Ramon Brugada
Cardiology, Cardiac Genetics Clinical Unit, Hospital Universitari Josep Trueta, Hospital Santa Caterina, Girona, Spain (R.B.).
Esther Zorio
CIBER Cardiovascular, Instituto de Salud Carlos III, Madrid, Spain (C.G.-G., M.G.-D., G.C.-M., M.N.-P., J.M.G.-P., G.C.-V., A.B.-G., R.S.-B., A.G.-Á., M.B., E.Z., E.R.G., E.V., J.L.-F., M.S.-M., R.B.-V., A.D., M.A.E.-C., J.F.R.-P., J.G.M., P.G.-P.).
Tomas Ripoll-Vera
Maria Luisa Peña-Peña
General Hospital of H.U. Virgen Del Rocio, Seville, Spain
Ana Royuela
Miriam Sánchez
Department of Cardiology, Área del Corazón, Hospital Universitario de Navarra, Pamplona, Spain (A.Y.I., M.S., G.L.-L., M.T.B.-E.).
Carles Diez-Lopez
Bioheart Group, Cardiovascular, Respiratory and Systemic Diseases and Cellular Aging Program, Institut d’Investigació Biomèdica de Bellvitge (IDIBELL; E.C.-G., C.D.-L., A.D.), l’Hospitalet de Llobregat, Spain.
Elena Rodríguez González
Department of Cardiology, Hospital General Universitario Gregorio Marañón, Madrid, Spain (C.G.-G., E.R.G., M.A.E.-C.).
Edwin Poel
Department of Experimental Cardiology, Amsterdam Cardiovascular Sciences, Heart Failure & Arrhythmias, Amsterdam University Medical Centers, University of Amsterdam, The Netherlands (A.S.A., E.P., C.R.B.).
Eduardo Villacorta
CIBER Cardiovascular, Instituto de Salud Carlos III, Madrid, Spain (C.G.-G., M.G.-D., G.C.-M., M.N.-P., J.M.G.-P., G.C.-V., A.B.-G., R.S.-B., A.G.-Á., M.B., E.Z., E.R.G., E.V., J.L.-F., M.S.-M., R.B.-V., A.D., M.A.E.-C., J.F.R.-P., J.G.M., P.G.-P.).
Javier Limeres-Freire
CIBER Cardiovascular, Instituto de Salud Carlos III, Madrid, Spain (C.G.-G., M.G.-D., G.C.-M., M.N.-P., J.M.G.-P., G.C.-V., A.B.-G., R.S.-B., A.G.-Á., M.B., E.Z., E.R.G., E.V., J.L.-F., M.S.-M., R.B.-V., A.D., M.A.E.-C., J.F.R.-P., J.G.M., P.G.-P.).
María Sabater-Molina
Roberto Barriales-Villa
Complexo Hospitalario Universitario A Coruña, Instituto de Investigación Biomédica de A Coruña, CIBERCV–Instituto de Salud Carlos III, A Coruña, Spain
Gemma Lacuey-Lecumberri
Department of Cardiology, Área del Corazón, Hospital Universitario de Navarra, Pamplona, Spain (A.Y.I., M.S., G.L.-L., M.T.B.-E.).
Andrea Di Marco
Bioheart Group, Cardiovascular, Respiratory and Systemic Diseases and Cellular Aging Program, Institut d’Investigació Biomèdica de Bellvitge (IDIBELL; E.C.-G., C.D.-L., A.D.), l’Hospitalet de Llobregat, Spain.
María Angeles Espinosa-Castro
Department of Cardiology, Hospital General Universitario Gregorio Marañón, Madrid, Spain (C.G.-G., E.R.G., M.A.E.-C.).
Connie R. Bezzina
José F. Rodríguez-Palomares
CIBER Cardiovascular, Instituto de Salud Carlos III, Madrid, Spain (C.G.-G., M.G.-D., G.C.-M., M.N.-P., J.M.G.-P., G.C.-V., A.B.-G., R.S.-B., A.G.-Á., M.B., E.Z., E.R.G., E.V., J.L.-F., M.S.-M., R.B.-V., A.D., M.A.E.-C., J.F.R.-P., J.G.M., P.G.-P.).
María Teresa Basurte-Elorz
Department of Cardiology, Área del Corazón, Hospital Universitario de Navarra, Pamplona, Spain (A.Y.I., M.S., G.L.-L., M.T.B.-E.).
Jesus G. Mirelis
Department of Cardiology, Hospital Universitario Puerta de Hierro, Instituto de Investigación Sanitaria Puerta de Hierro Majadahonda (IDIPHIM), Madrid, Spain (N.M.-A., N.R.-L., J.G.M., P.G.-P.).
Pablo Garcia-Pavia
Department of Cardiology, Hospital Universitario Puerta de Hierro, Instituto de Investigación Sanitaria Puerta de Hierro–Segovia de Arana, Centro de Investigación Biomédica en Red Enfermedades Cardiovaculares, and Centro Nacional de Investigaciones Cardiovasculares, Madrid