Cardiovascular Magnetic Resonance in Heritable Cardiomyopathies

Hammersley, Daniel; Jones, Richard E.; Mach, Lukáš; Halliday, Brian P; Prasad, Sanjay K.

doi:10.1016/j.hfc.2020.08.004

Cited by 4 publications

(6 citation statements)

References 96 publications

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“… 26 , 27 The standard imaging modality for evaluation of the cardiac morphology and function is cine-CMR imaging, which provides quantification of cardiac parameters such as atrial and ventricular volumes, myocardial mass, wall motion, and ejection fraction. 28 , 29 Cine sequences, based on steady-state free-precession sequences, are not subject to acoustic window limitations of the heart as they can offer multiplanar imaging with complete coverage of the entire myocardium. 20 , 28 The high blood-myocardial contrast resolution of cine-CMR allows for precise delineation of the endocardial border, so myocardial wall thickness can be assessed and a differential diagnosis of non-compaction can be excluded ( Figure 1A-C ).…”

Section: Cmr Imaging Techniquesmentioning

confidence: 99%

“…LGE image acquisition following gadolinium administration therefore allows for precise identification of the location and extent of the myocardial fibrotic tissue. 5 , 29 , 33 , 34 Parametric mapping is an alternative myocardial tissue characterisation technique, based on changes in the (myocardial) T1 relaxation times without the need of a contrast agent. 32 T1 mapping sequences can be used for the detection of diffuse and interstitial myocardial fibrosis, as native T1 (pre-contrast) values of the myocardium are increased in these regions compared to healthy myocardium.…”

Section: Cmr Imaging Techniquesmentioning

confidence: 99%

“…The most common presentation of HCM is characterised by LVH at the basal anterior septum in continuity with the anterior free wall. 29 , 42 , 43 CMR allows identification of the location and extent of these regional areas of LVH which are not easily identified by echocardiography, particularly at the level of the lateral or basal anterior wall of the LV and the apex. 23 , 27 Therefore, CMR contributes to obtaining correct and differential diagnosis when assessing the location and extent of hypertrophy in patients, using its multiplanar imaging of the entire myocardium.…”

Section: Diagnostic Value Of Cmr In Childhood Hcmmentioning

confidence: 99%

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Cardiac MRI in diagnosis, prognosis, and follow-up of hypertrophic cardiomyopathy in children: current perspectives

Spaapen,

Bohte,

Slieker

et al. 2024

British Journal of Radiology

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Hypertrophic Cardiomyopathy (HCM) is an inherited myocardial disease characterised by left ventricular hypertrophy, which carries an increased risk of life-threatening arrhythmias and sudden cardiac death. The age of presentation and the underlying aetiology have a significant impact on the prognosis and quality of life of children with HCM, as childhood-onset HCM is associated with high mortality risk and poor long-term outcomes. Accurate cardiac assessment and identification of the HCM phenotype are therefore crucial to determine the diagnosis, prognostic stratification, and follow-up. Cardiac magnetic resonance (CMR) is a comprehensive evaluation tool capable of providing information on cardiac morphology and function, flow, perfusion, and tissue characterisation. CMR allows to detect subtle abnormalities in the myocardial composition and characterise the heterogeneous phenotypic expression of HCM. In particular, the detection of the degree and extent of myocardial fibrosis, using late-gadolinium enhanced sequences or parametric mapping, is unique for CMR and is of additional value in the clinical assessment and prognostic stratification of paediatric HCM patients. Additionally, childhood HCM can be progressive over time. The rate, timing, and degree of disease progression vary from one patient to the other, so close cardiac monitoring and serial follow-up throughout the life of the diagnosed patients is of paramount importance. In this review, an update of the use of CMR in childhood HCM is provided, focussing on its clinical role in diagnosis, prognosis, and serial follow-up.

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Section: Cmr Imaging Techniquesmentioning

confidence: 99%

Section: Cmr Imaging Techniquesmentioning

confidence: 99%

Section: Diagnostic Value Of Cmr In Childhood Hcmmentioning

confidence: 99%

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Cardiac MRI in diagnosis, prognosis, and follow-up of hypertrophic cardiomyopathy in children: current perspectives

Spaapen,

Bohte,

Slieker

et al. 2024

British Journal of Radiology

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“…Distribution and extent of replacement fibrosis in DCM can be documented and quantified with late gadolinium enhancement (LGE), which is present in ∼30% of patients with DCM, usually with the midwall (in patients with LMNA mutations), ring-like (in patients with DSP and FLNC mutations) or subepicardial distribution (in post-myocarditis cardiomyopathy). 35,36 Extracellular matrix expansion due to interstitial fibrosis can be detected with native and contrast-enhanced T1 mapping. 37 Multiple studies have documented a strong association between the presence of LGE on cardiac magnetic resonance (CMR) and the risk of SCD, whereas the association with death due to progressive HF was less compelling.…”

Section: Myocardial Fibrosismentioning

confidence: 99%

Incidence, risk assessment and prevention of sudden cardiac death in cardiomyopathies

Polovina,

Tschöpe,

Rosano

et al. 2023

European J of Heart Fail

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Cardiomyopathies are a significant contributor to cardiovascular morbidity and mortality, mainly due to the development of heart failure and increased risk of sudden cardiac death (SCD). Despite improvement in survival with contemporary treatment, SCD remains an important cause of mortality in cardiomyopathies. It occurs at a rate ranging between 0.15% and 0.7% per year (depending on the cardiomyopathy), which significantly surpasses SCD incidence in the age‐ and sex‐matched general population. The risk of SCD is affected by multiple factors including the aetiology, genetic basis, age, sex, physical exertion, the extent of myocardial disease severity, conduction system abnormalities, and electrical instability, as measured by various metrics. Over the past decades, the knowledge on the mechanisms and risk factors for SCD has substantially improved, allowing for a better‐informed risk stratification. However, unresolved issues still challenge the guidance of SCD prevention in patients with cardiomyopathies. In this review, we aim to provide an in‐depth discussion of the contemporary concepts pertinent to understanding the burden, risk assessment and prevention of SCD in cardiomyopathies (dilated, non‐dilated left ventricular, hypertrophic, arrhythmogenic right ventricular, and restrictive). The review first focuses on SCD incidence in cardiomyopathies and then summarizes established and emerging risk factors for life‐threatening arrhythmias/SCD. Finally, it discusses validated approaches to the risk assessment and evidence‐based measures for SCD prevention in cardiomyopathies, pointing to the gaps in evidence and areas of uncertainties that merit future clarification.

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“…Clasificación de las miocardiopatías secundarias cantidad de información valiosa que brinda la cual es sumamente útil para hacer la diferenciación de los diversos tipos de cardiomiopatías: la resonancia magnética cardíaca. (6) Esta revisión se basará en hacer un recorrido por la definición de diferentes tipos de cardiomiopatías así como la utilidad de la resonancia magnética cardíaca en su diagnóstico así como también pronóstico de la enfermedad.…”

Section: Tablaunclassified

Utilidad de la resonancia magnética cardíaca en el diagnóstico de cardiomiopatías

Flores¹

2022

Interciencia méd.

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Las cardiomiopatías son un grupo de enfermedades de etiología primaria o secundaria, utilizadas por alteraciones en el músculo cardiaco (dilatación, hipertrofia, disfunción sistólica y/o diastólica), las cuales han cobrado notoriedad en los últimos años debido al desarrollo de nuevas técnicas de imagen. La resonancia magnética cardíaca es una técnica robusta, gold-standard para la evaluación de la morfología cardíaca y de la caracterización tisular, con muy poca variabilidad intraobservador e interobservador que brinda información muy útil permitiendo la identificación de la causa subyacente lo que permite brindar información no sólo pronóstica y de diagnóstico diferencial sino también para guiar el tratamiento y evaluar la respuesta del mismo.

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Cardiovascular Magnetic Resonance in Heritable Cardiomyopathies

Cited by 4 publications

References 96 publications

Cardiac MRI in diagnosis, prognosis, and follow-up of hypertrophic cardiomyopathy in children: current perspectives

Cardiac MRI in diagnosis, prognosis, and follow-up of hypertrophic cardiomyopathy in children: current perspectives

Incidence, risk assessment and prevention of sudden cardiac death in cardiomyopathies

Utilidad de la resonancia magnética cardíaca en el diagnóstico de cardiomiopatías

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