Prognostic Relevance of a Score for Identifying Diastolic Dysfunction according to the 2016 American Society of Echocardiography/European Association of Cardiovascular Imaging Recommendations in Patients with Hypertrophic Cardiomyopathy

Saito, Chihiro; Minami, Yuichiro; Haruki, Shintaro; Arai, Kotaro; Ashihara, Kaoru; Hagiwara, Nobuhisa

doi:10.1016/j.echo.2021.12.006

Cited by 7 publications

(4 citation statements)

References 20 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…2 In many other diseases, higher e9, E/e9, and LAVI values are strongly associated with premature mortality and increased occurrence of clinical cardiac end points. [33][34][35][36] The associations indicate that lysoGb3 does not only have a relationship with cardiac morphology but also with cardiac function and the risk of developing HFpEF. Finally, we found that higher lysoGb3 levels were associated with a faster progression of white matter lesions in the brain on MRI.…”

Section: Discussionmentioning

confidence: 98%

Early Risk Stratification for Natural Disease Course in Fabry Patients Using Plasma Globotriaosylsphingosine Levels

van der Veen,

Sayed,

Hollak

et al. 2023

CJASN

View full text Add to dashboard Cite

Background: Fabry disease is a very heterogeneous X-linked lysosomal storage disease. Disease manifestations in the kidneys, heart and brain vary greatly, even between patients of the same sex and with the same disease classification (classical or non-classical). A biomarker with a strong association with the development of disease manifestations is needed to determine the need for Fabry-specific treatment and appropriate frequency of follow-up, since clinical manifestations of the disorder may take decennia to develop. Methods: We investigated the levels of plasma lysoGb3 levels over time and its association with disease manifestations and disease course in 237 untreated Fabry patients (median age 42, 38% male) using linear mixed effect models. Results: LysoGb3 levels are stable over time in plasma of untreated Fabry patients. Higher levels of lysoGb3 were associated with steeper decline in estimated glomerular filtration rate (eGFR, p=0.05) and a faster increase in albuminuria (measured as the urinary albumin to creatinine ratio, UACR, p<0.001), left ventricular mass (measured on echocardiography, p<0.001), left atrial volume index (LAVI, p=0.003) and Fazekas score (p=0.003). Additionally, regardless of age, higher lysoGb3 levels were associated with higher relative wall thickness (p<0.001) and unfavorable functional markers on echocardiography, including septal mitral annular early diastolic velocity (e’, p<0.001) and the ratio of early trans mitral velocity (E) to e’ (E/e’, p=0.001). Conclusions: In an individual Fabry disease patient, the plasma lysoGb3 level reached a specific level in early childhood which, in the absence of Fabry specific treatment, remained stable throughout life. The level of lysoGb3 in untreated patients was associated with nearly all Fabry specific disease manifestations, regardless of the sex of the patient.

show abstract

Section: Discussionmentioning

confidence: 98%

Early Risk Stratification for Natural Disease Course in Fabry Patients Using Plasma Globotriaosylsphingosine Levels

van der Veen,

Sayed,

Hollak

et al. 2023

CJASN

View full text Add to dashboard Cite

show abstract

“…For the assessment of LV diastolic function in HCM patients, current guidelines recommend a comprehensive approach including the E/e' ratio (>14), LAVI (>34 mL/m 2 ), pulmonary vein atrial reversal velocity (Ar-A duration ≥ 30 ms; A, mitral end-diastolic inflow; Ar, pulmonary vein reversal flow), and peak velocity of TR jet by CW Doppler (>2.8 m/s), which can be applied with or without the presence of LVOT obstruction [41]. A recent study of 290 patients with HCM showed that if more than three of the four variables are presented in the guidelines, the risk of HCM-related adverse outcomes increases [42]. During AF, the transmitral A and pulmonary Ar velocities are absent because atrial contraction is lost.…”

Section: Assessmentmentioning

confidence: 99%

Multimodality Imaging in Patients with Hypertrophic Cardiomyopathy and Atrial Fibrillation

Chung,

Choi

2023

Diagnostics

View full text Add to dashboard Cite

Ventricular hypertrophy is associated with diastolic dysfunction, resulting in increased left atrial (LA) pressure, enlargement, fibrosis, and decreased LA function. Hypertrophic cardiomyopathy (HCM) is characterized by myocyte disarray, myocardial fibrosis, and hypertrophy. Notably, a thickened and noncompliant LV results in the impairment of diastolic function. These conditions promote LA remodeling and enlargement, which contribute to developing and maintaining atrial fibrillation (AF). AF is an atrial arrhythmia that occurs frequently in HCM, and evaluating the morphology and physiology of the atrium and ventricle is important for treatment and prognosis determination in HCM patients with AF. In addition, it provides a clue that can predict the possibility of new AF, even in patients not previously diagnosed with AF. Cardiac magnetic resonance (CMR), which can overcome the limitations of transthoracic echocardiography (TTE), has been widely used traditionally and even enables tissue characterization; moreover, it has emerged as an essential imaging modality for patients with HCM. Here, we review the role of multimodal imaging in patients with HCM and AF.

show abstract

“…LVOTO and SAM cause both high intracavitary pressures, further begetting LVH, and MR from the loss of leaflet coaptation [ 5 ]. LVH causes diastolic dysfunction through decreased chamber compliance, resulting in high intracavitary filling pressures [ 5 , 16 ]. Myocardial ischemia in HCM can be present even without epicardial coronary atherosclerotic obstructive disease and is mainly attributable to myocardial oxygen supply-demand mismatch secondary to myocardial hypertrophy, microvascular dysfunction, and impaired coronary flow reserve, all of which are worsened by the increased wall stress from high intracavitary pressures [ 5 , 17 ].…”

Section: Phenotypes Genotypes and Clinical Pathophysiologymentioning

confidence: 99%

“…Diastolic dysfunction in HCM is a major contributor to symptoms in those with and without LVOT of other cavitary obstruction and results from a combination of impaired LV compliance and relaxation and associated elevated filling pressures, as well as atrial myopathy and further impairment of LV diastolic filling [ 5 , 6 , 16 , 35 ]. The 2020 ACC/AHA and 2022 ASE/ASNC/SCMR/SCCT guidelines recommend that the echocardiographic evaluation of the HCM includes a comprehensive assessment of diastolic function, which includes mitral inflow velocities, early diastolic velocity by tissue Doppler, peak tricuspid regurgitation (TR) velocity (TR Vmax), biplane LA volume with LA volume indexing (LAVI) to body surface area, and pulmonary vein flow [ 5 , 6 ].…”

Section: Echocardiographymentioning

confidence: 99%

Cardiac Multimodality Imaging in Hypertrophic Cardiomyopathy: What to Look for and When to Image

Wengrofsky

Akivis

Bukharovich

2023

CCR

View full text Add to dashboard Cite

Hypertrophic cardiomyopathy (HCM), now recognized as a common cardiomyopathy of complex genomics and pathophysiology, is defined by the presence of left ventricular hypertrophy of various morphologies and severity, significant hemodynamic consequences, and diverse phenotypic, both structural and clinical, profiles. Advancements in cardiac multimodality imaging, including echocardiography, cardiac magnetic resonance imaging, and cardiac computed tomography, with and without angiography have greatly improved the diagnosis of HCM, and enable precise measurements of cardiac mass, volume, wall thickness, function, and physiology. Multimodality imaging provides comprehensive and complementary information and hasemerged as the bedrock for the diagnosis, clinical assessment, serial monitoring, and sudden cardiac death risk stratification of patients with HCM. This review highlights the role of cardiac multimodality imaging in the modern diagnosis and management of HCM.

show abstract

Prognostic Relevance of a Score for Identifying Diastolic Dysfunction according to the 2016 American Society of Echocardiography/European Association of Cardiovascular Imaging Recommendations in Patients with Hypertrophic Cardiomyopathy

Cited by 7 publications

References 20 publications

Early Risk Stratification for Natural Disease Course in Fabry Patients Using Plasma Globotriaosylsphingosine Levels

Early Risk Stratification for Natural Disease Course in Fabry Patients Using Plasma Globotriaosylsphingosine Levels

Multimodality Imaging in Patients with Hypertrophic Cardiomyopathy and Atrial Fibrillation

Cardiac Multimodality Imaging in Hypertrophic Cardiomyopathy: What to Look for and When to Image

Contact Info

Product

Resources

About