Young patients with hypertrophic cardiomyopathy, but not subjects at risk, show decreased myocardial perfusion reserve quantified with CMR

Gyllenhammar, Tom; Fernlund, Eva; Jablonowski, Robert; Jögi, Jonas; Engblom, Henrik; Liuba, Petru; Arheden, Håkan; Carlsson, Marcus

doi:10.1093/ehjci/jeu137

Cited by 20 publications

(18 citation statements)

References 35 publications

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“…The global myocardial perfusion data are overlapping with the results previously reported [31]. MP was decreased in the HCM group versus controls ( p = 0.02), even when using Z score > 2.5 SD for HCM diagnosis, but no significant changes in MP were found in the HCM-risk group vs. controls (Fig.…”

Section: Resultssupporting

confidence: 88%

“…However, when using mitral E/e´ > 8 as a cut-off for LV diastolic dysfunction, we found higher levels of ICTP ( p = 0.02) in the HCM group, a finding that could suggest alterations in ECM prior to development of fibrosis. As recently reported [31], the degree of myocardial fibrosis was assessed using CMR -LGE, and the association between regional hypoperfusion and fibrosis [39] in young patients with HCM. In line with previous studies showing a certain correlation between the magnitude of fibrosis and the degree of hypertrophy [2, 7–9], fibrosis was predominantly found in myocardial segments with advanced hypertrophy [39] and also indicating a pathology beyond LVH and LGE by showing greater extent of areas with impaired myocardial perfusion even in non-fibrotic myocardium during adenosine hyperemia indicating regional hypoxemia, suggesting an important relationship of myocardial hypoperfusion with fibrosis occurrence in HCM [39].…”

Section: Discussionmentioning

confidence: 99%

“…Myocardial perfusion (MP) was measured using flow quantification of the coronary sinus during adenosine-induced hyperemia and at rest. The flow was divided by LVM from cine images as earlier described [31, 32] and validated [33]. MP was expressed in ml/min/g and the MP ratio (MPR) was calculated by dividing MP during adenosine with MP at rest.…”

Section: Methodsmentioning

confidence: 99%

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Serum Biomarkers of Myocardial Remodeling and Coronary Dysfunction in Early Stages of Hypertrophic Cardiomyopathy in the Young

et al. 2017

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Hypertrophic cardiomyopathy (HCM) remains the leading cause of sudden cardiac death in the young. Early markers for HCM are important to identify individuals at risk. The aim of this study was to investigate novel serum biomarkers reflecting myocardial remodeling, microfibrosis, and vascular endotheliopathy in the early stages of familial HCM in young patients. Twenty-three HCM patients, 16 HCM-risk individuals, and 66 controls (median 15 years) underwent echocardiography and serum analysis for cathepsin S, endostatin, myostatin, type I collagen degradation marker (ICTP), matrix metalloproteinase (MMP)-9, vascular endothelial growth factor receptor (VEGFR)-1, and vascular and intercellular adhesion molecules (VCAM, ICAM). In a subset of the population, global myocardial perfusion was performed by magnetic resonance imaging. Cathepsin S (p = 0.0009), endostatin (p < 0.0001), MMP-9 (p = 0.008), and VCAM (p = 0.04) were increased in the HCM group and correlated to left ventricular mass index and mitral E/e′ (p < 0.01). In the HCM-risk group, myostatin was decreased (p = 0.004), whereas ICAM was increased (p = 0.002). Global perfusion was decreased in the HCM group (p < 0.05) versus controls. Endostatin and mitral E/e′ correlated inversely to myocardial perfusion (p ≤ 0.05). This is the first study demonstrating adverse changes in biomarkers reflecting myocardial matrix remodeling, microfibrosis, and vascular endotheliopathy in early stage of hypertrophic cardiomyopathy in the young.

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Section: Resultssupporting

confidence: 88%

Section: Discussionmentioning

confidence: 99%

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Serum Biomarkers of Myocardial Remodeling and Coronary Dysfunction in Early Stages of Hypertrophic Cardiomyopathy in the Young

et al. 2017

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“…The prognostic value of LGE in HCM remains elusive [ 11 , 18 ], and ischemia has been proposed as an earlier marker of disease [ 4 , 29 , 32 ]. We have earlier showed that global perfusion is decreased in the same cohort of young patients with HCM investigated in the current study [ 12 ]. However, the relationship between regional perfusion, hypertrophy and fibrosis has not been studied in young HCM patients and HCM-risk subjects.…”

Section: Introductionmentioning

confidence: 60%

Regional Stress-Induced Ischemia in Non-fibrotic Hypertrophied Myocardium in Young HCM Patients

et al. 2015

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The relationship between hypertrophy, perfusion abnormalities and fibrosis is unknown in young patients with hypertrophic cardiomyopathy (HCM). Since mounting evidence suggests causal relationship between myocardial ischemia and major adverse cardiac events, we sought to investigate whether (1) regional myocardial perfusion is decreased in young HCM patients and in individuals at risk of HCM, and (2) hypoperfused areas are larger than areas with fibrosis. HCM patients (n = 12), HCM-risk subjects (n = 15) and controls (n = 9) were imaged on a 1.5 T MRI scanner. Myocardial hypertrophy was assessed on cine images. Perfusion images were acquired during adenosine hyperemia and at rest. Maximum upslope ratios of perfusion (stress/rest) were used for semiquantitative analysis. Fibrosis was assessed by late gadolinium enhancement (LGE). Results are presented as median and range. Perfusion in HCM-risk subjects and in non-hypertrophied segments in HCM patients showed no difference compared to controls (P = ns). Hypertrophic segments in HCM patients without LGE showed decreased perfusion compared to segments without hypertrophy [1.5 (1.1–2.3) vs. 2.0 (1.8–2.6), P < 0.001], and hypertrophic segments with LGE showed even lower perfusion using a segmental analysis [0.9 (0.6–1.8), P < 0.05]. The extent of hypoperfused myocardium in HCM patients during adenosine exceeded the extent of fibrosis on LGE [20 (0–48) vs. 4 (0–7) % slice area, P < 0.05] and hypoperfused areas at rest (P < 0.001). Regional perfusion is decreased in hypertrophied compared to non-hypertrophied myocardium and is lowest in fibrotic myocardium in young HCM patients but does not discriminate HCM-risk subjects from controls. The stress-induced hypoperfused regions exceed regions with LGE, indicating that hypoperfusion precedes fibrosis and may be a more sensitive marker of diseased myocardium in HCM.

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“…92 Assessment of coronary microvascular dysfunction is challenging and stress perfusion CMR imaging could have a future role in risk stratification. [94][95][96] Exercise capacity has also been proposed to help risk stratification in different studies. [97][98][99] It has been demonstrated that peak VO2 is associated with an increased risk of major events during short-term follow-up.…”

Section: Other Potential Risk Factors and Arbitrators Not Included Inmentioning

confidence: 99%

Risk Stratification in Hypertrophic Cardiomyopathy

Steriotis

Sharma

2015

European Cardiology Review

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Hypertrophic cardiomyopathy (HCM) is a hereditary primary myocardial disease that is most commonly due to mutations within genes encoding sarcomeric contractile proteins and is characterised by left ventricular hypertrophy in the absence of a cardiac or systemic cause. Although the overall prognosis is relatively good with an annual mortality rate <1 %, the propensity to potentially fatal ventricular arrhythmias is the most feared complication. The identification of patients at risk of arrhythmogenic sudden cardiac death (SCD) is an essential component in disease management. Aborted SCD and malignant ventricular arrhythmias are the most powerful risk factors for SCD and ICD implantation is recommended in such circumstances. The selection of patients who may benefit from ICD therapy for primary prevention purposes is more challenging. The heterogeneous nature of the disease and the variation in trigger factors provides an adequate explanation for the low predictive accuracy of most conventional risk factors in isolation. A new risk model for risk stratification proposed by the European Society of Cardiology HCM outcome group shows promise but requires validation in different cohorts. The ICD is the only effective therapy in preventing SCD for the disease with a relatively low adverse event rate, but most deaths occur in relatively young patients. However, it is also difficult to ignore the complications with the ICD, therefore, the strive to perfect risk stratification in HCM should continue to ensure that only the most high-risk patients receive an ICD.

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Young patients with hypertrophic cardiomyopathy, but not subjects at risk, show decreased myocardial perfusion reserve quantified with CMR

Cited by 20 publications

References 35 publications

Serum Biomarkers of Myocardial Remodeling and Coronary Dysfunction in Early Stages of Hypertrophic Cardiomyopathy in the Young

Serum Biomarkers of Myocardial Remodeling and Coronary Dysfunction in Early Stages of Hypertrophic Cardiomyopathy in the Young

Regional Stress-Induced Ischemia in Non-fibrotic Hypertrophied Myocardium in Young HCM Patients

Risk Stratification in Hypertrophic Cardiomyopathy

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