RESUMEN

A 39-year-old woman with intermittent palpitations, psoriasis, and a family history of sudden death presented with dilated right heart chambers and an enlarged coronary sinus. Despite a normal bubble study, further evaluation with transesophageal echocardiography revealed an abnormal pulmonary venous return: the left pulmonary veins drained into the coronary sinus. Cardiac computed tomography confirmed this finding, suggesting a partial abnormal pulmonary venous return as the underlying issue. Cardiac catheterization indicated increased pulmonary artery flow with normal pulmonary vascular resistance. The patient was referred for surgery. In this pathway involving the differential diagnosis of right heart dilatation, despite a confusing history and conflicting findings, echocardiographic clues led to the diagnosis.

Asunto(s)

Ecocardiografía Transesofágica , Humanos , Femenino , Adulto , Diagnóstico Diferencial , Ecocardiografía Transesofágica/métodos , Tomografía Computarizada por Rayos X/métodos , Cardiomiopatía Dilatada/complicaciones , Cardiomiopatía Dilatada/fisiopatología , Cardiomiopatía Dilatada/diagnóstico por imagen , Cardiomiopatía Dilatada/etiología , Venas Pulmonares/diagnóstico por imagen , Venas Pulmonares/anomalías

RESUMEN

INTRODUCTION: Right ventricular (RV) dysfunction is a significant negative prognostic indicator in human dilated cardiomyopathy (DCM). Many RV indices are weight-dependent, and there is a lack of reference values for the right heart in giant breed dogs (over 50 kg), including Great Danes (GDs). This study aimed to compare indices of RV function in echocardiographically normal GDs, those with preclinical DCM (PC-DCM), and those with DCM and congestive heart failure (DCM-CHF). ANIMALS: A total of 116 client-owned adult GDs: 74 normal, 31 with PC-DCM, and 11 with DCM-CHF. METHODS: A retrospective, single-center cohort study assessed RV function using free-wall RV longitudinal strain (RVLS), strain rate, fractional area change (FAC), tricuspid annular plane systolic excursion (TAPSE), and pulsed-wave tissue Doppler imaging-derived systolic myocardial velocity of the lateral tricuspid annulus (TDI S'). Relationships between DCM status and RV function indices were analyzed. RESULTS: RV function, measured by TAPSE (P=0.001), FAC (P<0.001), and TDI S' (P<0.001), decreased in dogs with PC-DCM and DCM-CHF compared to healthy dogs, with FAC being lower in DCM-CHF compared to PC-DCM (P=0.048). RVLS impairment was more significant in the DCM-CHF group than in the PC-DCM group (P=0.048). RVLS had the highest area under the curve (0.899) for differentiating between normal and DCM-CHF dogs. CONCLUSION: As DCM progresses, echocardiographic variables of RV function, including TAPSE, FAC, TDI S', RVLS, and strain rate, worsen, indicating impaired RV systolic function in GDs affected by DCM.

Asunto(s)

Cardiomiopatía Dilatada , Enfermedades de los Perros , Función Ventricular Derecha , Perros , Animales , Cardiomiopatía Dilatada/veterinaria , Cardiomiopatía Dilatada/fisiopatología , Cardiomiopatía Dilatada/diagnóstico por imagen , Enfermedades de los Perros/fisiopatología , Enfermedades de los Perros/diagnóstico por imagen , Estudios Retrospectivos , Masculino , Femenino , Ecocardiografía/veterinaria , Disfunción Ventricular Derecha/veterinaria , Disfunción Ventricular Derecha/fisiopatología , Disfunción Ventricular Derecha/diagnóstico por imagen , Estudios de Cohortes , Insuficiencia Cardíaca/veterinaria , Insuficiencia Cardíaca/fisiopatología , Pueblos Nórdicos y Escandinávicos

RESUMEN

Mid-aortic syndrome (MAS) is a rare vascular disease that usually leads to renovascular hypertension. With the predominant manifestations being intractable arterial hypertension and lower extremity arterial insufficiency, it has rarely been associated with dilated cardiomyopathy. We report a young girl with congestive heart failure, where the cause was initially attributed to dilated cardiomyopathy. A repeated echocardiogram 6 months later brought the physician's suspicion of MAS because of the abnormal colour of Doppler from the subcostal view. Further assessment using CT angiography revealed discrete thoracic coarctation at the level of T10, with the narrowest diameter of 2.1 mm, thus confirming the diagnosis. Her inflammatory markers and connective tissue screening were negative. She underwent successful stenting of coarctation of the aorta, which later caused improvement in her cardiac function. We highlighted the importance of looking for treatable causes of dilated cardiomyopathy and vigilant clinical and echocardiogram assessment with high suspicion to diagnose MAS.

Asunto(s)

Coartación Aórtica , Cardiomiopatía Dilatada , Humanos , Cardiomiopatía Dilatada/diagnóstico , Cardiomiopatía Dilatada/diagnóstico por imagen , Cardiomiopatía Dilatada/complicaciones , Femenino , Coartación Aórtica/complicaciones , Coartación Aórtica/diagnóstico , Coartación Aórtica/diagnóstico por imagen , Angiografía por Tomografía Computarizada , Ecocardiografía , Stents , Diagnóstico Diferencial , Síndrome , Insuficiencia Cardíaca/etiología

RESUMEN

BACKGROUND: Extracting phenotype-representative flow patterns and their associated numerical metrics is a bottleneck in the clinical translation of advanced cardiac flow imaging modalities. We hypothesized that reduced-order models (ROMs) are a suitable strategy for deriving simple and interpretable clinical metrics of intraventricular flow suitable for further assessments. Combined with machine learning (ML) flow-based ROMs could provide new insight to help diagnose and risk-stratify patients. METHODS: We analyzed 2D color-Doppler echocardiograms of 81 non-ischemic dilated cardiomyopathy (DCM) patients, 51 hypertrophic cardiomyopathy (HCM) patients, and 77 normal volunteers (Control). We applied proper orthogonal decomposition (POD) to build patient-specific and cohort-specific ROMs of LV flow. Each ROM aggregates a low number of components representing a spatially dependent velocity map modulated along the cardiac cycle by a time-dependent coefficient. We tested three classifiers using deliberately simple ML analyses of these ROMs with varying supervision levels. In supervised models, hyperparameter grid search was used to derive the ROMs that maximize classification power. The classifiers were blinded to LV chamber geometry and function. We ran vector flow mapping on the color-Doppler sequences to help visualize flow patterns and interpret the ML results. RESULTS: POD-based ROMs stably represented each cohort through 10-fold cross-validation. The principal POD mode captured >80 % of the flow kinetic energy (KE) in all cohorts and represented the LV filling/emptying jets. Mode 2 represented the diastolic vortex and its KE contribution ranged from <1 % (HCM) to 13 % (DCM). Semi-unsupervised classification using patient-specific ROMs revealed that the KE ratio of these two principal modes, the vortex-to-jet (V2J) energy ratio, is a simple, interpretable metric that discriminates DCM, HCM, and Control patients. Receiver operating characteristic curves using V2J as classifier had areas under the curve of 0.81, 0.91, and 0.95 for distinguishing HCM vs. Control, DCM vs. Control, and DCM vs. HCM, respectively. CONCLUSIONS: Modal decomposition of cardiac flow can be used to create ROMs of normal and pathological flow patterns, uncovering simple interpretable flow metrics with power to discriminate disease states, and particularly suitable for further processing using ML.

Asunto(s)

Modelos Cardiovasculares , Humanos , Femenino , Masculino , Persona de Mediana Edad , Adulto , Ventrículos Cardíacos/fisiopatología , Ventrículos Cardíacos/diagnóstico por imagen , Aprendizaje Automático , Cardiomiopatía Dilatada/fisiopatología , Cardiomiopatía Dilatada/diagnóstico por imagen , Fenotipo , Cardiomiopatía Hipertrófica/fisiopatología , Cardiomiopatía Hipertrófica/diagnóstico por imagen , Ecocardiografía Doppler en Color/métodos

RESUMEN

Cardioembolic stroke is one of the most devastating complications of nonischemic dilated cardiomyopathy (NIDCM). However, in clinical trials of primary prevention, the benefits of anticoagulation are hampered by the risk of bleeding. Indices of cardiac blood stasis may account for the risk of stroke and be useful to individualize primary prevention treatments. We performed a cross-sectional study in patients with NIDCM and no history of atrial fibrillation (AF) from two sources: 1) a prospective enrollment of unselected patients with left ventricular (LV) ejection fraction <45% and 2) a retrospective identification of patients with a history of previous cardioembolic neurological event. The primary end point integrated a history of ischemic stroke or the presence intraventricular thrombus, or a silent brain infarction (SBI) by imaging. From echocardiography, we calculated blood flow inside the LV, its residence time (TR) maps, and its derived stasis indices. Of the 89 recruited patients, 18 showed a positive end point, 9 had a history of stroke or transient ischemic attack (TIA) and 9 were diagnosed with SBIs in the brain imaging. Averaged TR, [Formula: see text] performed well to identify the primary end point [AUC (95% CI) = 0.75 (0.61-0.89), P = 0.001]. When accounting only for identifying a history of stroke or TIA, AUC for [Formula: see text] was 0.92 (0.85-1.00) with odds ratio = 7.2 (2.3-22.3) per cycle, P < 0.001. These results suggest that in patients with NIDCM in sinus rhythm, stasis imaging derived from echocardiography may account for the burden of stroke.NEW & NOTEWORTHY Patients with nonischemic dilated cardiomyopathy (NIDCM) are at higher risk of stroke than their age-matched population. However, the risk of bleeding neutralizes the benefit of preventive oral anticoagulation. In this work, we show that in patients in sinus rhythm, the burden of stroke is related to intraventricular stasis metrics derived from echocardiography. Therefore, stasis metrics may be useful to personalize primary prevention anticoagulation in these patients.

Asunto(s)

Cardiomiopatía Dilatada , Humanos , Cardiomiopatía Dilatada/diagnóstico por imagen , Cardiomiopatía Dilatada/fisiopatología , Cardiomiopatía Dilatada/complicaciones , Masculino , Femenino , Persona de Mediana Edad , Anciano , Estudios Transversales , Estudios Retrospectivos , Infarto Encefálico/diagnóstico por imagen , Infarto Encefálico/etiología , Infarto Encefálico/fisiopatología , Ecocardiografía , Función Ventricular Izquierda , Factores de Riesgo , Estudios Prospectivos , Accidente Cerebrovascular/etiología , Accidente Cerebrovascular/diagnóstico por imagen , Accidente Cerebrovascular/prevención & control , Accidente Cerebrovascular Embólico/etiología , Accidente Cerebrovascular Embólico/prevención & control , Accidente Cerebrovascular Embólico/diagnóstico por imagen , Enfermedades Asintomáticas , Volumen Sistólico

RESUMEN

BACKGROUND: Carney syndrome is an uncommon autosomal disorder closely linked to mutations in the PRKAR1A gene. Skin lesions are the most pronounced feature of Carney syndrome, affecting over 80% of individuals with this condition. This syndrome is characterized by a triad of myxomas, skin pigmentation, and endocrine hyperfunction, featuring multiple endocrine neoplasms with skin and cardiac involvement. Dilated cardiomyopathy, a primary cardiomyopathy, is defined as the dilation and impaired systolic function of the left or both ventricles. Its clinical presentation varies from being asymptomatic to heart failure or sudden cardiac death, making it a leading global cause of heart failure. Currently, Dilated cardiomyopathy has an estimated prevalence of 1/2500-1/250 individuals, predominantly affecting those aged 30-40 years, with a male-to-female ratio of 3:1. This case report describes a heart failure patient with cardiac myxoma caused by Carney syndrome combined with dilated cardiomyopathy. The patient was successfully treated for heart failure by heart transplantation. CASE PRESENTATION: Herein, we report a case of heart failure due to Carney syndrome that resulted in cardiac myxoma combined with dilated cardiomyopathy. A 35-year-old male was admitted to the hospital three years ago because of sudden chest tightness and shortness of breath. Echocardiography indicated myxoma, and a combination of genetic screening and physical examination confirmed Carney syndrome with cardiac myxoma. Following symptomatic management, he was discharged. Surgical interventions were not considered at the time. However, the patient's chest tightness and shortness of breath symptoms worsened, and he returned to the hospital. A New York Heart Association grade IV heart function was confirmed, and echocardiography indicated the presence of dilated cardiomyopathy accompanied by cardiac myxoma. Ultimately, the patient's heart failure was successfully treated with heart transplantation. CONCLUSIONS: Cardiac myxoma caused by Carney syndrome combined with heart failure caused by dilated cardiomyopathy can be resolved by heart transplantation.

Asunto(s)

Cardiomiopatía Dilatada , Complejo de Carney , Insuficiencia Cardíaca , Neoplasias Cardíacas , Trasplante de Corazón , Mixoma , Humanos , Cardiomiopatía Dilatada/cirugía , Cardiomiopatía Dilatada/etiología , Cardiomiopatía Dilatada/diagnóstico , Cardiomiopatía Dilatada/diagnóstico por imagen , Masculino , Complejo de Carney/genética , Complejo de Carney/diagnóstico , Complejo de Carney/cirugía , Complejo de Carney/complicaciones , Adulto , Mixoma/complicaciones , Mixoma/cirugía , Mixoma/diagnóstico por imagen , Mixoma/diagnóstico , Mixoma/genética , Insuficiencia Cardíaca/etiología , Insuficiencia Cardíaca/diagnóstico , Insuficiencia Cardíaca/cirugía , Neoplasias Cardíacas/cirugía , Neoplasias Cardíacas/complicaciones , Neoplasias Cardíacas/diagnóstico por imagen , Neoplasias Cardíacas/diagnóstico , Neoplasias Cardíacas/genética , Resultado del Tratamiento , Subunidad RIalfa de la Proteína Quinasa Dependiente de AMP Cíclico/genética

RESUMEN

AIM: Liver damage frequently occurs in patients with cardiovascular (CV) disease and is associated with adverse clinical outcomes. The associations of liver damage with cardiac structure/function measures and the risk of adverse CV events in patients with dilated cardiomyopathy (DCM) are poorly known. METHODS: We retrospectively enrolled consecutive patients with DCM undergoing cardiac magnetic resonance imaging (MRI). In addition to standard cardiac assessment, iron-corrected T1 mapping was also assessed in the liver. Cross-sectional associations between hepatic T1-time and cardiac structure and function were examined accounting for potential confounders. Longitudinal associations between hepatic T1-time and the risk of hospitalization for HF or CV death were also assessed. RESULTS: Overall, 120 stable patients with established DCM were included in the study (mean age 54.7 years, 26 % women). The mean hepatic iron-corrected T1-time was 563±73 ms. In linear regression analyses, measures of left atrial structure (LA maximal volume, p = 0.035, LA minimal volume=0.012), interventricular septum thickness (p = 0.026), and right ventricular ejection fraction (p = 0.005) were significantly associated with greater hepatic T1-time. Over a mean follow-up of 4.5 ± 1.8 years, 32 (27 %) died or were hospitalized for HF at a rate of 6.7 per 100 person-year. Higher hepatic iron-corrected T1-time was independently associated with a higher risk of adverse events (adjusted-hazard ratio 1.71, 95 % confidence interval: 1.14-2.56, p = 0.009). Patients with a hepatic T1-time ≥563 ms had a higher risk of CV events (log-rank p = 0.03). CONCLUSION: Among stable patients with DCM, higher hepatic iron-corrected T1-time is associated with worse cardiac size and function and with higher rates of hospitalization for HF or CV death. CONDENSED ABSTRACT: Limited data exist regarding the clinical value of hepatic T1-time in patients with dilated cardiomyopathy (DCM) undergoing cardiac Magnetic Resonance imaging (MRI). We found that higher hepatic iron-corrected T1-time is associated with worse cardiac size and function, even after accounting for clinical confounders. Over a mean follow-up of 4.5 ± 1.8 years, higher hepatic iron-corrected T1-time was independently associated with a higher risk of hospitalization for heart failure or cardiovascular death. Among stable patients with DCM, the evaluation of liver tissue by cardiac MRI may provide useful clinical information for CV risk stratification.

Asunto(s)

Cardiomiopatía Dilatada , Hígado , Imagen por Resonancia Magnética , Humanos , Cardiomiopatía Dilatada/diagnóstico por imagen , Cardiomiopatía Dilatada/fisiopatología , Cardiomiopatía Dilatada/mortalidad , Cardiomiopatía Dilatada/complicaciones , Femenino , Masculino , Persona de Mediana Edad , Estudios Retrospectivos , Anciano , Hígado/diagnóstico por imagen , Hígado/patología , Adulto , Estudios Transversales , Hospitalización/estadística & datos numéricos , Modelos Lineales , Volumen Sistólico , Insuficiencia Cardíaca/fisiopatología , Insuficiencia Cardíaca/mortalidad

RESUMEN

BACKGROUND: The growth of mitral leaflets (MLs) adaptive to left ventricluar (LV) remodeling has been observed. However, the elasticity of MLs upon mechanical stimuli would be supposed if it shrinks with LV reverse remodeling (LVRR). MATERIAL AND METHODS: Patients with idiopathic recent-onset dilated cardiomyopathy (RODCM) (n = 82) and 50 matched normal controls (NC) were prospectively enrolled. Echocardiography was performed at baseline and 6 months of follow-up for the anterior and posterior mitral leaflet (AML and PML) length, mitral annular dimension (MAD), and tenting height (TH). LVRR was measured as a ≥ 15% reduction in LV end-diastolic volume (LVEDV). RESULTS: After 6 months, LVRR was achieved in 69.5% of patients. The AML (28 ± 3 vs. 26 ± 3 mm, p = 0.004) and PML (19 ± 4 vs. 17 ± 3 mm, p < 0.001) decreased in length, as well as the MAD (31 ± 5 vs. 28 ± 5 mm, p = 0.001) and TH (10 ± 3 vs. 8 ± 2 mm, p < 0.001). Compared with the NC group, the AML and PML of the RODCM group were 16.7% and 35.7% longer at baseline and remained 8.3% and 21.2% longer at follow-up, respectively. The change in AML or PML correlated moderately with that in LVEDV (r = 0.487, p < 0.001; r = 0.516, p < 0.001, respectively). The AML and PML length decreased in the LVRR (+) subgroup (AML, 28 ± 3 vs. 26 ± 3 mm, p = 0.001; PML, 20 ± 4 vs. 16 ± 3 mm, p < 0.001), but remained the same in the LVRR (-) subgroup (27 ± 4 vs. 28 ± 4 mm, p = 0.318; 17 ± 3 vs. 17 ± 3 mm, p = 0.790). CONCLUSIONS: Enlarged MLs could reverse accompanied by LV reverse remodeling. This study provided the other facet of ML plasticity adaptive to mechanical stretching.

Asunto(s)

Cardiomiopatía Dilatada , Válvula Mitral , Función Ventricular Izquierda , Remodelación Ventricular , Humanos , Femenino , Masculino , Cardiomiopatía Dilatada/fisiopatología , Cardiomiopatía Dilatada/tratamiento farmacológico , Cardiomiopatía Dilatada/diagnóstico por imagen , Estudios Prospectivos , Válvula Mitral/diagnóstico por imagen , Válvula Mitral/fisiopatología , Persona de Mediana Edad , Función Ventricular Izquierda/fisiología , Adulto , Factores de Tiempo , Resultado del Tratamiento , Estudios de Seguimiento , Volumen Sistólico/fisiología , Ecocardiografía

RESUMEN

OBJECTIVE: This study investigated the feasibility and prognostic relevance of threshold-based quantification of myocardial delayed enhancement (MDE) on CT in patients with nonischemic dilated cardiomyopathy (NIDCM). MATERIALS AND METHODS: Forty-three patients with NIDCM (59.3 ± 17.1 years; 21 male) were included in the study and underwent cardiac CT and MRI. MDE was quantified manually and with a threshold-based quantification method using cutoffs of 2, 3, and 4 standard deviations (SDs) on three sets of CT images (100 kVp, 120 kVp, and 70 keV). Interobserver agreement in MDE quantification was assessed using the intraclass correlation coefficient (ICC). Agreement between CT and MRI was evaluated using the Bland-Altman method and the concordance correlation coefficient (CCC). Patients were followed up for the subsequent occurrence of the primary composite outcome, including cardiac death, heart transplantation, heart failure hospitalization, or appropriate use of an implantable cardioverter-defibrillator. The Kaplan-Meier method was used to estimate event-free survival according to MDE levels. RESULTS: Late gadolinium enhancement (LGE) was observed in 29 patients (67%, 29/43), and the mean LGE found with the 5-SD threshold was 4.1% ± 3.6%. The 4-SD threshold on 70-keV CT showed excellent interobserver agreement (ICC = 0.810) and the highest concordance with MRI (CCC = 0.803). This method also yielded the smallest bias with the narrowest range of 95% limits of agreement compared to MRI (bias, -0.119%; 95% limits of agreement, -4.216% to 3.978%). During a median follow-up of 1625 days (interquartile range, 712-1430 days), 10 patients (23%, 10/43) experienced the primary composite outcome. Event-free survival significantly differed between risk subgroups divided by the optimal MDE cutoff of 4.3% (log-rank P = 0.005). CONCLUSION: The 4-SD threshold on 70-keV monochromatic CT yielded results comparable to those of MRI for quantifying MDE as a marker of myocardial fibrosis, which showed prognostic value in patients with NIDCM.

Asunto(s)

Cardiomiopatía Dilatada , Medios de Contraste , Estudios de Factibilidad , Fibrosis , Imagen por Resonancia Magnética , Tomografía Computarizada por Rayos X , Humanos , Masculino , Cardiomiopatía Dilatada/diagnóstico por imagen , Femenino , Persona de Mediana Edad , Pronóstico , Tomografía Computarizada por Rayos X/métodos , Fibrosis/diagnóstico por imagen , Imagen por Resonancia Magnética/métodos , Miocardio/patología , Adulto , Anciano

RESUMEN

BACKGROUND: Current cardiovascular magnetic resonance sequences cannot discriminate between different myocardial extracellular space (ECSs), including collagen, noncollagen, and inflammation. We sought to investigate whether cardiovascular magnetic resonance radiomics analysis can distinguish between noncollagen and inflammation from collagen in dilated cardiomyopathy. METHODS: We identified data from 132 patients with dilated cardiomyopathy scheduled for an invasive septal biopsy who underwent cardiovascular magnetic resonance at 3 T. Cardiovascular magnetic resonance imaging protocol included native and postcontrast T1 mapping and late gadolinium enhancement (LGE). Radiomic features were computed from the midseptal myocardium, near the biopsy region, on native T1, extracellular volume (ECV) map, and LGE images. Principal component analysis was used to reduce the number of radiomic features to 5 principal radiomics. Moreover, a correlation analysis was conducted to identify radiomic features exhibiting a strong correlation (r>0.9) with the 5 principal radiomics. Biopsy samples were used to quantify ECS, myocardial fibrosis, and inflammation. RESULTS: Four histopathological phenotypes were identified: low collagen (n=20), noncollagenous ECS expansion (n=49), mild to moderate collagenous ECS expansion (n=42), and severe collagenous ECS expansion (n=21). Noncollagenous expansion was associated with the highest risk of myocardial inflammation (65%). Although native T1 and ECV provided high diagnostic performance in differentiating severe fibrosis (C statistic, 0.90 and 0.90, respectively), their performance in differentiating between noncollagen and mild to moderate collagenous expansion decreased (C statistic: 0.59 and 0.55, respectively). Integration of ECV principal radiomics provided better discrimination and reclassification between noncollagen and mild to moderate collagen (C statistic, 0.79; net reclassification index, 0.83 [95% CI, 0.45-1.22]; P<0.001). There was a similar trend in the addition of native T1 principal radiomics (C statistic, 0.75; net reclassification index, 0.93 [95% CI, 0.56-1.29]; P<0.001) and LGE principal radiomics (C statistic, 0.74; net reclassification index, 0.59 [95% CI, 0.19-0.98]; P=0.004). Five radiomic features per sequence were identified with correlation analysis. They showed a similar improvement in performance for differentiating between noncollagen and mild to moderate collagen (native T1, ECV, LGE C statistic, 0.75, 0.77, and 0.71, respectively). These improvements remained significant when confined to a single radiomic feature (native T1, ECV, LGE C statistic, 0.71, 0.70, and 0.64, respectively). CONCLUSIONS: Radiomic features extracted from native T1, ECV, and LGE provide incremental information that improves our capability to discriminate noncollagenous expansion from mild to moderate collagen and could be useful for detecting subtle chronic inflammation in patients with dilated cardiomyopathy.

Asunto(s)

Cardiomiopatía Dilatada , Matriz Extracelular , Humanos , Cardiomiopatía Dilatada/diagnóstico por imagen , Cardiomiopatía Dilatada/patología , Matriz Extracelular/patología , Matriz Extracelular/metabolismo , Femenino , Masculino , Persona de Mediana Edad , Adulto , Colágeno/metabolismo , Miocardio/patología , Anciano , Fibrosis , Imagen por Resonancia Magnética/métodos , Biopsia , Análisis de Componente Principal , Radiómica

RESUMEN

BACKGROUND: Nondilated left ventricular cardiomyopathy (NDLVC) has been recently differentiated from dilated cardiomyopathy (DCM). A comprehensive characterization of these 2 entities using cardiac magnetic resonance (CMR) and genetic testing has never been performed. OBJECTIVES: This study sought to provide a thorough characterization and assess clinical outcomes in a large multicenter cohort of patients with DCM and NDLVC. METHODS: A total of 462 patients with DCM (227) or NDLVC (235) with CMR data from 4 different referral centers were retrospectively analyzed. The study endpoint was a composite of sudden cardiac death or major ventricular arrhythmias. RESULTS: In comparison to DCM, NDLVC had a higher prevalence of pathogenic or likely pathogenic variants of arrhythmogenic genes (40% vs 23%; P < 0.001), higher left ventricular (LV) systolic function (LV ejection fraction: 51% ± 12% vs 36% ± 15%; P < 0.001) and higher prevalence of free-wall late gadolinium enhancement (LGE) (27% vs 14%; P < 0.001). Conversely, DCM showed higher prevalence of pathogenic or likely pathogenic variants of nonarrhythmogenic genes (23% vs 12%; P = 0.002) and septal LGE (45% vs 32%; P = 0.004). Over a median follow-up of 81 months (Q1-Q3: 40-132 months), the study outcome occurred in 98 (21%) patients. LGE with septal location (HR: 1.929; 95% CI: 1.033-3.601; P = 0.039) was independently associated with the risk of sudden cardiac death or major ventricular arrhythmias together with LV dilatation, older age, advanced NYHA functional class, frequent ventricular ectopic activity, and nonsustained ventricular tachycardia. CONCLUSIONS: In a multicenter cohort of patients with DCM and NDLVC, septal LGE together with LV dilatation, age, advanced disease, and frequent and repetitive ventricular arrhythmias were powerful predictors of major arrhythmic events.

Asunto(s)

Cardiomiopatía Dilatada , Imagen por Resonancia Cinemagnética , Humanos , Masculino , Femenino , Cardiomiopatía Dilatada/diagnóstico por imagen , Cardiomiopatía Dilatada/fisiopatología , Persona de Mediana Edad , Estudios Retrospectivos , Imagen por Resonancia Cinemagnética/métodos , Adulto , Anciano , Muerte Súbita Cardíaca/epidemiología , Muerte Súbita Cardíaca/etiología , Estudios de Seguimiento

RESUMEN

Myocardial work (MW) derived from pressure-strain loops is a novel non-invasive tool to assess left ventricular (LV) function, incorporating global longitudinal strain (GLS) by speckle tracking echocardiography and non-invasively assessed blood pressure. Studies on the role of MW in dilated cardiomyopathy (DCM) are still limited. Therefore, the aim of this study was to evaluate the potential value of MW for predicting adverse outcomes in patients with DCM. 116 consecutive patients with DCM who underwent heart catheterization were retrospectively recruited from June 2009 to July 2014. 34 patients (30%) met the composite endpoints for major adverse cardiac events (MACE) of cardiac transplantation, need for implantable cardioverter-defibrillator (ICD) therapy, heart failure hospitalization and all-cause mortality. Patients with DCM were followed up for a mean of 5.1 years (IQR: 2.2-9.1 years). Global work index (GWI) and global constructive work (GCW) were not only independent predictors but also provided incremental predictive values (Integrated discrimination improvement [IDI] > 0) of MACE in multivariate Cox models. Furthermore, Patients with GWI < 788 mm Hg% (HR 5.46, 95%CI 1.66-17.92, p = 0.005) and GCW < 1,238 mm Hg% (HR 4.46, 95%CI 1.53-12.98, p = 0.006) had higher risks of MACE. GWI and GCW assessed by strain imaging echocardiography may have an additional value beyond LV-EF and GLS for predicting adverse outcomes in DCM.

Asunto(s)

Cardiomiopatía Dilatada , Valor Predictivo de las Pruebas , Función Ventricular Izquierda , Humanos , Cardiomiopatía Dilatada/fisiopatología , Cardiomiopatía Dilatada/diagnóstico por imagen , Cardiomiopatía Dilatada/mortalidad , Cardiomiopatía Dilatada/terapia , Cardiomiopatía Dilatada/complicaciones , Masculino , Femenino , Persona de Mediana Edad , Estudios Retrospectivos , Factores de Riesgo , Factores de Tiempo , Pronóstico , Anciano , Adulto , Cateterismo Cardíaco/efectos adversos , Trasplante de Corazón , Cardioversión Eléctrica/instrumentación , Cardioversión Eléctrica/efectos adversos , Desfibriladores Implantables , Insuficiencia Cardíaca/fisiopatología , Insuficiencia Cardíaca/mortalidad , Insuficiencia Cardíaca/terapia , Insuficiencia Cardíaca/diagnóstico por imagen , Contracción Miocárdica , Medición de Riesgo , Reproducibilidad de los Resultados , Ecocardiografía

RESUMEN

RATIONALE AND OBJECTIVES: This study aims to evaluate the capability of machine learning algorithms in utilizing radiomic features extracted from cine-cardiac magnetic resonance (CMR) sequences for differentiating between ischemic cardiomyopathy (ICM) and dilated cardiomyopathy (DCM). MATERIALS AND METHODS: This retrospective study included 115 cardiomyopathy patients subdivided into ICM (n = 64) and DCM cohorts (n = 51). We collected invasive clinical (IC), noninvasive clinical (NIC), and combined clinical (CC) feature subsets. Radiomic features were extracted from regions of interest (ROIs) in the left ventricle (LV), LV cavity (LVC), and myocardium (MYO). We tested 10 classical machine learning classifiers and validated them through fivefold cross-validation. We compared the efficacy of clinical feature-based models and radiomics-based models to identify the superior diagnostic approach. RESULTS: In the validation set, the Gaussian naive Bayes (GNB) model outperformed the other models in all categories, with areas under the curve (AUCs) of 0.879 for IC_GNB, 0.906 for NIC_GNB, and 0.906 for CC_GNB. Among the radiomics models, the MYO_LASSOCV_MLP model demonstrated the highest AUC (0.919). In the test set, the MYO_RFECV_GNB radiomics model achieved the highest AUC (0.857), surpassing the performance of the three clinical feature models (IC_GNB: 0.732; NIC_GNB: 0.75; CC_GNB: 0.786). CONCLUSION: Radiomics models leveraging MYO images from cine-CMR exhibit promising potential for differentiating ICM from DCM, indicating the significant clinical application scope of such models. CLINICAL RELEVANCE STATEMENT: The integration of radiomics models and machine learning methods utilizing cine-CMR sequences enhances the diagnostic capability to distinguish between ICM and DCM, minimizes examination risks for patients, and potentially reduces the duration of medical imaging procedures.

Asunto(s)

Cardiomiopatía Dilatada , Aprendizaje Automático , Imagen por Resonancia Cinemagnética , Isquemia Miocárdica , Humanos , Cardiomiopatía Dilatada/diagnóstico por imagen , Femenino , Imagen por Resonancia Cinemagnética/métodos , Masculino , Persona de Mediana Edad , Estudios Retrospectivos , Isquemia Miocárdica/diagnóstico por imagen , Diagnóstico Diferencial , Anciano , Algoritmos , Adulto , Interpretación de Imagen Asistida por Computador/métodos , Radiómica

RESUMEN

INTRODUCTION: Early diagnosis of patients with dilated cardiomyopathy (DCM) remains challenging. Cardiac MR can correlate myocardial changes with their pathological basis. There have been some previous studies on the effect of T1 mapping in DCM, but there is limited data on the incremental value of T2 mapping for DCM in routine clinical practice. This study will examine whether the combination of MRI T1 and T2 mapping offers greater advantages in the diagnosis of DCM. METHODS: The study included 28 patients with DCM and 21 healthy controls. CMR evaluation included late gadolinium enhancement (LGE), T1 mapping, extracellular volume (ECV) fraction and T2 mapping. The DCM group was divided into LGE (+) and LGE (-) subgroups. The main modes of LGE are subendocardial, midwall, subepicardial, or transmural. T1 values, T2 values, and ECV in the 16 segments myocardial levels were measured by post-processing software. Student's t-tests or Mann-Whitney U test was used to compare between two groups, and one-way ANOVA or Kruskal-Wallis H test was used to compare between multiple groups, with p values corrected by Bonferroni. The difference was considered statistically significant at P < 0.05. ROC curve analysis was used to compare the area under the curve (AUC) of each index and its combined value, and the cut-off value, sensitivity and specificity were determined by Jordan's index. RESULTS: Mean native myocardial T1, ECV and T2 were significantly higher in the DCM group compared to controls (p ≤ 0.001, respectively). The best cut-off values for T1, T2 and ECV to discriminate DCM from controls were 1184 ms, 40.9 ms and 29.2%, respectively. The AUC of T1, ECV and T2 were 0.87, 0.89, and 0.83, respectively. The combined AUC of the three values was 0.96. CONCLUSION: Native T1 value and ECV overcome some of the limitations of LGE, and the T2 helps to understand the extent of myocardial damage. The combination of T1 and T2 mapping techniques can reveal fibrotic and oedematous changes in the early stages of DCM, providing a more comprehensive assessment of DCM and better guidance for individualised clinical management of patients. IMPLICATIONS FOR PRACTICE: We suggest that the addition of T2 mapping to the routine CMR examination of patients with suspected DCM, and the combined assessment of T1mapping and T2 mapping can provide complementary information about the disease and improve the early diagnosis of DCM.

Asunto(s)

Cardiomiopatía Dilatada , Medios de Contraste , Humanos , Cardiomiopatía Dilatada/diagnóstico por imagen , Femenino , Masculino , Persona de Mediana Edad , Adulto , Estudios de Casos y Controles , Imagen por Resonancia Magnética/métodos , Imagen por Resonancia Cinemagnética/métodos , Sensibilidad y Especificidad

RESUMEN

BACKGROUND: Careful interpretation of the relation between phenotype changes of the heart and gene variants detected in dilated cardiomyopathy (DCM) is important for patient care and monitoring. OBJECTIVE: We sought to assess the association between cardiac-related genes and whole-heart myocardial mechanics or morphometrics in nonischemic dilated cardiomyopathy (NIDCM). METHODS: It was a prospective study consisting of patients with NIDCM. All patients were referred for genetic testing and a genetic analysis was performed using Illumina NextSeq 550 and a commercial gene capture panel of 233 genes (Systems Genomics, Cardiac-GeneSGKit®). It was analyzed whether there are significant differences in clinical, two-dimensional (2D) echocardiographic, and magnetic resonance imaging (MRI) parameters between patients with the genes variants and those without. 2D echocardiography and MRI were used to analyze myocardial mechanics and morphometrics. RESULTS: The study group consisted of 95 patients with NIDCM and the average age was 49.7 ± 10.5. All echocardiographic and MRI parameters of myocardial mechanics (left ventricular ejection fraction 28.4 ± 8.7 and 30.7 ± 11.2, respectively) were reduced and all values of cardiac chambers were increased (left ventricular end-diastolic diameter 64.5 ± 5.9 mm and 69.5 ± 10.7 mm, respectively) in this group. It was noticed that most cases of whole-heart myocardial mechanics and morphometrics differences between patients with and without gene variants were in the genes GATAD1, LOX, RASA1, KRAS, and KRIT1. These genes have not been previously linked to DCM. It has emerged that KRAS and KRIT1 genes were associated with worse whole-heart mechanics and enlargement of all heart chambers. GATAD1, LOX, and RASA1 genes variants showed an association with better cardiac function and morphometrics parameters. It might be that these variants alone do not influence disease development enough to be selective in human evolution. CONCLUSIONS: Combined variants in previously unreported genes related to DCM might play a significant role in affecting clinical, morphometrics, or myocardial mechanics parameters.

Asunto(s)

Cardiomiopatía Dilatada , Predisposición Genética a la Enfermedad , Fenotipo , Función Ventricular Izquierda , Humanos , Cardiomiopatía Dilatada/genética , Cardiomiopatía Dilatada/fisiopatología , Cardiomiopatía Dilatada/diagnóstico por imagen , Persona de Mediana Edad , Masculino , Femenino , Adulto , Estudios Prospectivos , Función Ventricular Izquierda/genética , Volumen Sistólico , Remodelación Ventricular/genética , Imagen por Resonancia Magnética , Fenómenos Biomecánicos , Variación Genética , Ecocardiografía , Contracción Miocárdica/genética , Estudios de Asociación Genética , Valor Predictivo de las Pruebas

Asunto(s)

Cardiomiopatía Dilatada , Distrofia Miotónica , Humanos , Distrofia Miotónica/diagnóstico , Distrofia Miotónica/complicaciones , Cardiomiopatía Dilatada/diagnóstico , Cardiomiopatía Dilatada/etiología , Cardiomiopatía Dilatada/diagnóstico por imagen , Cardiomiopatía Dilatada/complicaciones , Masculino , Adulto , Femenino , Persona de Mediana Edad

RESUMEN

Background Right atrial (RA) function strain is increasingly acknowledged as an important predictor of adverse events in patients with diverse cardiovascular conditions. However, the prognostic value of RA strain in patients with dilated cardiomyopathy (DCM) remains uncertain. Purpose To evaluate the prognostic value of RA strain derived from cardiac MRI (CMR) feature tracking (FT) in patients with DCM. Materials and Methods This multicenter, retrospective study included consecutive adult patients with DCM who underwent CMR between June 2010 and May 2022. RA strain parameters were obtained using CMR FT. The primary end points were sudden or cardiac death or heart transplant. Cox regression analysis was used to determine the association of variables with outcomes. Incremental prognostic value was evaluated using C indexes and likelihood ratio tests. Results A total of 526 patients with DCM (mean age, 51 years ± 15 [SD]; 381 male) were included. During a median follow-up of 41 months, 79 patients with DCM reached the primary end points. At univariable analysis, RA conduit strain was associated with the primary end points (hazard ratio [HR], 0.82 [95% CI: 0.76, 0.87]; P < .001). In multivariable Cox analysis, RA conduit strain was an independent predictor for the primary end points (HR, 0.83 [95% CI: 0.77, 0.90]; P < .001). A model combining RA conduit strain with other clinical and conventional imaging risk factors (C statistic, 0.80; likelihood ratio, 92.54) showed improved discrimination and calibration for the primary end points compared with models with clinical variables (C statistic, 0.71; likelihood ratio, 37.12; both P < .001) or clinical and imaging variables (C statistic, 0.75; likelihood ratio, 64.69; both P < .001). Conclusion CMR FT-derived RA conduit strain was an independent predictor of adverse outcomes among patients with DCM, providing incremental prognostic value when combined in a model with clinical and conventional CMR risk factors. Published under a CC BY 4.0 license. Supplemental material is available for this article.

Asunto(s)

Cardiomiopatía Dilatada , Adulto , Humanos , Masculino , Persona de Mediana Edad , Cardiomiopatía Dilatada/diagnóstico por imagen , Función del Atrio Derecho , Estudios Retrospectivos , Imagen por Resonancia Magnética , Radiografía

RESUMEN

BACKGROUND: To prospectively examine the dynamic evolution of fibrotic processes within a one-year in patients with dilated cardiomyopathy (DCM). METHODS: Between May 2019 and September 2020, 102 DCM patients (mean age 45.2 ± 11.8 years, EF 29.9 ± 11.6%) underwent cardiac magnetic resonance (CMR-1). After 13.9 ± 2.9 months, 92 of these patients underwent a follow-up CMR (CMR-2). Replacement fibrosis was assessed via late gadolinium enhancement (LGE), quantified in terms of LGE mass and extent. Interstitial fibrosis was evaluated via T1-mapping and expressed as extracellular volume fraction (ECV). This data, along with left ventricular (LV) mass, facilitated the calculation of LV matrix and cellular volumes. RESULTS: At CMR-1, LGE was present in 45 patients (48.9%), whereas at CMR-2 LGE was detected in 46 (50%) (p = 0.88). Although LGE mass remained stable, LGE extent increased from 2.18 ± 4.1% to 2.7 ± 4.6% (p < 0.01). Conversely, ECV remained unchanged [27.7% (25.5-31.3) vs. 26.7% (24.5-29.9); p = 0.19]; however, LV matrix and cell volumes exhibited a noteworthy regression. During a subsequent follow-up of 19.2 ± 9 months (spanning from CMR-2 to April 30th, 2023), the composite primary outcome (all-cause mortality, HTX, LVAD or heart failure worsening) was evident in 18 patients. Only the LV matrix volume index at follow-up was an independent predictor of outcome (OR 1.094; 95%CI 1.004-1.192; p < 0.05). CONCLUSIONS: In optimally managed DCM patients, both replacement and interstitial fibrosis remained stable over the course of one year. In contrast, LV matrix and cell volumes displayed significant regression. LV matrix volume index at 12-month follow-up was found to be an independent predictor of outcome in DCM.

Asunto(s)

Cardiomiopatía Dilatada , Progresión de la Enfermedad , Fibrosis , Imagen por Resonancia Cinemagnética , Humanos , Cardiomiopatía Dilatada/diagnóstico por imagen , Cardiomiopatía Dilatada/patología , Masculino , Femenino , Estudios Prospectivos , Persona de Mediana Edad , Adulto , Imagen por Resonancia Cinemagnética/métodos , Estudios de Seguimiento , Manejo de la Enfermedad , Factores de Tiempo , Miocardio/patología

RESUMEN

BACKGROUND. MRI-based prognostic evaluation in patients with dilated cardiomyopathy (DCM) has historically used markers of late gadolinium enhancement (LGE) and feature tracking (FT)-derived left ventricular global longitudinal strain (LVGLS). Early data indicate that FT-derived left atrial strain (LAS) parameters, including reservoir, conduit, and booster, may also have prognostic roles in such patients. OBJECTIVE. The purpose of our study was to evaluate the prognostic utility of LAS parameters, derived from MRI FT, in patients with ischemic or nonischemic DCM, including in comparison with the traditional parameters of LGE and LVGLS. METHODS. This retrospective study included 811 patients with ischemic or nonischemic DCM (median age, 60 years; 640 men, 171 women) who underwent cardiac MRI at any of five centers. FT-derived LAS parameters and LVGLS were measured using two- and four-chamber cine images. LGE percentage was quantified. Patients were assessed for a composite outcome of all-cause mortality or heart failure hospitalization. Multivariable Cox regression analyses including demographic characteristics, cardiovascular risk factors, medications used, and a wide range of cardiac MRI parameters were performed. Kaplan-Meier analyses with log-rank tests were also performed. RESULTS. A total of 419 patients experienced the composite outcome. Patients who did, versus those who did not, experience the composite outcome had larger LVGLS (-6.7% vs -8.3%, respectively; p < .001) as well as a smaller LAS reservoir (13.3% vs 19.3%, p < .001), LAS conduit (4.7% vs 8.0%, p < .001), and LAS booster (8.1% vs 10.3%, p < .001) but no significant difference in LGE (10.1% vs 11.3%, p = .51). In multivariable Cox regression analyses, significant independent predictors of the composite outcome included LAS reservoir (HR = 0.96, p < .001) and LAS conduit (HR = 0.91, p < .001). LAS booster and LGE were not significant independent predictors in the models. LVGLS was a significant independent predictor only in a model that initially included LAS booster but not the other LAS parameters. In Kaplan-Meier analysis, all three LAS parameters were significantly associated with the composite outcome (p < .001). CONCLUSION. In this multicenter study, LAS reservoir and LAS conduit were significant independent prognostic markers in patients with ischemic or nonischemic DCM, showing greater prognostic utility than the currently applied markers of LVGLS and LGE. CLINICAL IMPACT. FT-derived LAS analysis provides incremental prognostic information in patients with DCM.

Asunto(s)

Cardiomiopatía Dilatada , Imagen por Resonancia Cinemagnética , Humanos , Femenino , Masculino , Cardiomiopatía Dilatada/diagnóstico por imagen , Persona de Mediana Edad , Pronóstico , Estudios Retrospectivos , Imagen por Resonancia Cinemagnética/métodos , Atrios Cardíacos/diagnóstico por imagen , Atrios Cardíacos/fisiopatología , Anciano , Isquemia Miocárdica/diagnóstico por imagen , Medios de Contraste , Imagen por Resonancia Magnética/métodos

Asunto(s)

Cardiomiopatía Dilatada , Humanos , Cardiomiopatía Dilatada/diagnóstico por imagen , Cardiomiopatía Dilatada/fisiopatología , Pronóstico , Atrios Cardíacos/diagnóstico por imagen , Atrios Cardíacos/fisiopatología