Prognostic Significance of Myocardial Fibrosis in Hypertrophic Cardiomyopathy

O’Hanlon, Rory; Grasso, A; Roughton, Michael; Moon, James; Clark, Susan; Wage, Ricardo; Webb, Jessica; Kulkarni, Meghana; Dawson, Dana; Sulaibeekh, Leena; Chandrasekaran, Badri; Bucciarelli-Ducci, Chiara; Pasquale, Ferdinando; Cowie, Martin R.; McKenna, William J.; Sheppard, Mary N.; Elliott, Perry; Pennell, Dudley J.; Prasad, Sanjay

doi:10.1016/j.jacc.2010.05.010

Cited by 734 publications

(538 citation statements)

References 34 publications

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“…As in previous studies, 11,15 it remains to be proved whether LGE is an independent prognostic marker. Maron et al 12 reported a high prevalence, although not reaching statistical significance, of cardiovascular events during follow-up of 202 patients with HCM and LGE.…”

Section: Discussionmentioning

confidence: 83%

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Phenotypic expression in hypertrophic cardiomyopathy and late gadolinium enhancement on cardiac magnetic resonance

Caetano

Botelho

Trigo

et al. 2014

Revista Portuguesa de Cardiologia (English Edition)

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Introduction and Aim:The prognostic value of late gadolinium enhancement (LGE) for risk stratification of hypertrophic cardiomyopathy (HCM) patients is the subject of disagreement. We set out to examine the association between clinical and morphological variables, risk factors for sudden cardiac death and LGE in HCM patients. Methods: From a population of 78 patients with HCM, we studied 53 who underwent cardiac magnetic resonance. They were divided into two groups according to the presence or absence of LGE. Ventricular arrhythmias and morbidity and mortality during follow-up were analyzed. Results: Patients with LGE were younger at the time of diagnosis (p=0.046) and more often had a family history of sudden death (p=0.008) and known coronary artery disease (p=0.086). On echocardiography they had greater maximum wall thickness (p=0.007) and left atrial area (p=0.037) and volume (p=0.035), and more often presented a restrictive pattern of diastolic dysfunction (p=0.011) with a higher E/E ratio (p=0.003) and left ventricular systolic dysfunction (p=0.038). Cardiac magnetic resonance supported the association between LGE and previous echocardiographic findings: greater left atrial area (p=0.029) and maximum wall thickness (p<0.001) and lower left ventricular ejection fraction (p=0.056). Patients with LGE more often had an implantable cardioverter-defibrillator (ICD) (p=0.015). At follow-up, no differences were found in the frequency of ventricular arrhythmias, appropriate ICD therapies or mortality. Conclusions:The presence of LGE emerges as a risk marker, associated with the classical predictors of sudden cardiac death in this population. However, larger studies are required to confirm its independent association with clinical events. PALAVRAS-CHAVE

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Section: Discussionmentioning

confidence: 83%

“…The mean age of our population (56.4±17.0 years) was older than in most studies in the literature 5,11,12 and included few patients in the age-group most associated with sudden death (<35 years). Most were asymptomatic or mildly symptomatic, as in other published series.…”

Section: Discussionmentioning

confidence: 83%

Phenotypic expression in hypertrophic cardiomyopathy and late gadolinium enhancement on cardiac magnetic resonance

Caetano

Botelho

Trigo

et al. 2014

Revista Portuguesa de Cardiologia (English Edition)

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“…The DE is associated with the record of nonsustained ventricular tachycardia in Holter in multivariate analyses 75,78 , as well as the left ventricular reshaping and evolution of terminal dilated forms 74 . The association with the obstruction was not found in a study in which the prevalence of obstructive forms was similar between those with and without DE 76 . Longitudinal analyses showed relation between DE, sudden death and increased mortality, but it is still not proved [76][77][78] .…”

Section: Magnetic Resonance Imagingmentioning

confidence: 74%

“…MRI with gadolinium detects and quantifies fibrosis areas with delayed enhancement (DE) of focal or diffuse character in 60-80% of patients with HCM, representing 0-40% of the LV myocardial mass [74][75][76][77][78] (Figure 4). The DE is associated with the record of nonsustained ventricular tachycardia in Holter in multivariate analyses 75,78 , as well as the left ventricular reshaping and evolution of terminal dilated forms 74 .…”

Section: Magnetic Resonance Imagingmentioning

confidence: 99%

O diagnóstico da obstrução da via de saída do ventrículo esquerdo na cardiomiopatia hipertrófica

Mattos¹,

Torres²,

Rebelatto³

et al. 2012

Arq. Bras. Cardiol.

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Hypertrophic cardiomyopathy is a prevalent genetic disease characterized by left ventricular hypertrophy, presenting dynamic obstruction of outflow tract with subaortic gradient happening at rest in 30% of the cases. It is attributed to the intricate interaction between the anterior mitral leaflet, the interventricular septum and altered flow vectors generated in left ventricle along with changes in outflow tract geometry. Mitral regurgitation in varying degrees is found with or without association with structural deformities of the valve apparatus. The exercise echocardiogram evidences latent obstruction easily induced by exercise in 60 to 75% of nonobstructive forms. The determination of the gradient under this condition must be considered in routine investigation of patients with mild or no obstruction at rest. The evaluation of hypertrophic cardiomyopathy incorporates methods based on the ultrasound image, which, along with MRI, allow recognizing ventricular obstruction generating mechanisms, thus facilitating the diagnosis and management of obstructive and latent obstructive forms.

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“…Assessment of late gadolinium enhancement (LGE) in the left or right ventricle was interpreted by blinded observers. When present, the amount of LGE was quantified in a 16-segment model based on the "full width at half maximum" technique by customized analysis software (MRI-MASS, Medis, Leiden, the Netherlands) [11]. …”

Section: Methodsmentioning

confidence: 99%

Right ventricular dysfunction is a predictor of non-response and clinical outcome following cardiac resynchronization therapy

Alpendurada

Guha

Sharma

et al. 2011

Journal of Cardiovascular Magnetic Resonance

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BackgroundCardiac resynchronization therapy (CRT) is an established treatment in advanced heart failure (HF). However, an important subset does not derive a significant benefit. Despite an established predictive role in HF, the significance of right ventricular (RV) dysfunction in predicting clinical benefit from CRT remains unclear. We investigated the role of RV function, assessed by cardiovascular magnetic resonance (CMR), in predicting response to and major adverse clinical events in HF patients undergoing CRT.MethodsSixty consecutive patients were evaluated with CMR prior to CRT implantation in a tertiary cardiac centre. The primary end-point was a composite of death from any cause or unplanned hospitalization for a major cardiovascular event. The secondary end-point was response to therapy, defined as improvement in left ventricular ejection fraction ≥ 5% on echocardiography at one year.ResultsEighteen patients (30%) met the primary end-point over a median follow-up period of 26 months, and 27 out of 56 patients (48%) were considered responders to CRT. On time-to-event analysis, only atrial fibrillation (HR 2.6, 95% CI 1.02-6.84, p = 0.047) and RV dysfunction, either by a reduced right ventricular ejection fraction-RVEF (HR 0.96, 95% CI 0.94-0.99, p = 0.006) or tricuspid annular plane systolic excursion-TAPSE (HR 0.88, 95% CI, 0.80-0.96, p = 0.006), were significant predictors of adverse events. On logistic regression analysis, preserved RVEF (OR 1.05, 95% CI 1.01-1.09, p = 0.01) and myocardial scar burden (OR 0.90, 95% CI 0.83-0.96, p = 0.004) were the sole independent predictors of response to CRT. Patients with marked RV dysfunction (RVEF < 30%) had a particularly low response rate (18.2%) to CRT.ConclusionsRight ventricular function is an important predictor of both response to CRT and long-term clinical outcome. Routine assessment of the right ventricle should be considered in the evaluation of patients for CRT.

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Prognostic Significance of Myocardial Fibrosis in Hypertrophic Cardiomyopathy

Abstract: In patients with HCM, myocardial fibrosis as measured by late gadolinium enhancement cardiovascular magnetic resonance is an independent predictor of adverse outcome. (The Prognostic Significance of Fibrosis Detection in Cardiomyopathy; NCT00930735).

Cited by 734 publications

References 34 publications

Phenotypic expression in hypertrophic cardiomyopathy and late gadolinium enhancement on cardiac magnetic resonance

Phenotypic expression in hypertrophic cardiomyopathy and late gadolinium enhancement on cardiac magnetic resonance

O diagnóstico da obstrução da via de saída do ventrículo esquerdo na cardiomiopatia hipertrófica

Right ventricular dysfunction is a predictor of non-response and clinical outcome following cardiac resynchronization therapy

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