scholarly journals Histopathological specificity of hypertrophic obstructive cardiomyopathy. Myocardial fibre disarray and myocardial fibrosis.

Heart ◽  
1980 ◽  
Vol 44 (4) ◽  
pp. 433-443 ◽  
Author(s):  
M G St John Sutton ◽  
J T Lie ◽  
K R Anderson ◽  
P C O'Brien ◽  
R L Frye
Keyword(s):  
Myocardial Fibrosis ◽  
Hypertrophic Obstructive Cardiomyopathy ◽  
Myocardial Fibre

ID: 6: HYPERTROPHIC OBSTRUCTIVE CARDIOMYOPATHY IN THE SETTING OF SYSTEMIC SCLERODERMA

2016 ◽  
Vol 64 (4) ◽  
pp. 919.1-919
Author(s):  
R Sogomonian ◽  
H Alkhawam ◽  
S Lee ◽  
D Chang ◽  
EA Moradoghli Haftevani
Keyword(s):  
Systemic Sclerosis ◽  
Myocardial Fibrosis ◽  
Hospice Care ◽  
Vital Signs ◽  
Restrictive Cardiomyopathy ◽  
Hypertrophic Obstructive Cardiomyopathy ◽  
Left Ventricular ◽  
Diffuse Fibrosis ◽  
Septal Hypertrophy ◽  
Hypertrophied Ventricle

Restrictive cardiomyopathy has been a common variant seen in systemic sclerosis (SS) with myocardial fibrosis. The association of SS with restrictive cardiomyopathy has well been established, but that with HOCM is not clearly understood. Herein, we report a case of a patient with SS, identified to have both HOCM and myocardial fibrosis.A 54-year-old woman with systemic sclerosis, idiopathic lung disease with moderate pulmonary hypertension, presented with fatigue, decreased appetite and shortness of breath. Vital signs were significant for oxygen saturation of 86% on room air, tachycardia of 117 bpm, and blood pressure of 110/53 mm Hg. Physical examination revealed diffuse rhonchi in all lung fields, malar rash and skin excoriation in bilateral lower extremities without edema. Laboratory studies were significant for elevated brain natriuretic peptide (BNP) of 858 pg/mL. Transthoracic echocardiography revealed left ventricular hypertrophy (LVH) with ejection fraction of 78%. Electrocardiography illustrated LVH. Cardiac magnetic resonance imaging (cMRI) was significant for severe left ventricular cardiac asymmetric septal hypertrophy with outflow obstruction caused by anterior motion of the mitral valve. Cardiac biopsy revealed evidence of diffuse fibrosis, but did not show iron, glycogen, or amyloid depositions.Patient was maintained on mycophenolate mofetil, low dose of methylprednisolone, morphine, clonazepam and transferred to hospice care.Hypertrophic obstructive cardiomyopathy (HOCM) is the most common genetic cardiac disorder with an autosomal dominant transmission. It is characterized by asymmetric LVH out of proportion of systemic after load. The most common cardiac involvement in SS is myocardial fibrosis in a restrictive pattern, while HOCM is rarely seen in SS.Abstract ID: 6 Figure 1Cardiac MRI demonstrating hypertrophied ventricle with fibrosis. This image demonstrates the features of both hypertrophic and restrictive cardiomyopathy.

MiRNAs as Biomarkers in Hypertrophic Cardiomyopathy: Current State of the Art

2021 ◽  
Vol 28 ◽  
Author(s):  
Andreas Angelopoulos ◽  
Evangelos Oikonomou ◽  
Georgia Vogiatzi ◽  
Alexios Antonopoulos ◽  
Sotirios Tsalamandris ◽  
...  
Keyword(s):  
Hypertrophic Cardiomyopathy ◽  
Myocardial Fibrosis ◽  
Hypertrophic Obstructive Cardiomyopathy ◽  
Serum Markers ◽  
Current State ◽  
Inherited Cardiomyopathy ◽  
Asymptomatic Phase ◽  
End Stage ◽  
Additional Role

Background: Hypertrophic Cardiomyopathy (HCM) is the most common inherited Cardiomyopathy. The hallmark of HCM is myocardial fibrosis that contributes to heart failure, arrhythmias and sudden cardiac death. Objective: Currently there are no reliable serum biomarkers for detection of myocardial fibrosis, while cardiac magnetic resonance (CMR) is an imaging technique to detect myocardial fibrosis. MicroRNAs (miRNAs) have been increasingly suggested as biomarkers in cardiovascular diseases. However, in HCM there is as yet no identified and verified specific circulating miRNA signature. Methods: We conducted a review of literature to identify the studies that indicate the possible roles of miRNAs in HCM. Results: From studies in transgenic mice with HCM, miR-1, -133 may identify HCM in the early asymptomatic phase. Human miR-29a could be used as a circulating biomarker for detection of both myocardial hypertrophy and fibrosis in HCM, while it could also have a possible additional role in discrimination of hypertrophic obstructive cardiomyopathy from non-obstructive HCM. Additionally, miR-29a-3p is associated with diffuse myocardial fibrosis in HCM while miR-1-3p could discriminate end-stage HCM from dilated cardiomyopathy and left ventricle dilation. Another role of miRNAs could also be the contribution in differential diagnosis between HCM and phenocopies. Moreover, miRNA-targeted therapy (miR-133 mimics) is promising in inhibiting cardiac hypertrophy but this is still in the early stages. Conclusion: A more reliable and specific signature of miRNAs is expected with forthcoming studies in samples from HCM patients and correlation of miRNAs with CMR and serum markers of fibrosis may implicate novel diagnostic and therapeutic pathways.

Microvascular Rarefaction and Myocardial Fibrosis in Hypertrophic Obstructive Cardiomyopathy

Cardiology ◽  
2018 ◽  
Vol 141 (4) ◽  
pp. 202-211 ◽  
Author(s):  
Hongwei Tian ◽  
Chengzhi Yang ◽  
Yunhu Song ◽  
Hongyue Wang ◽  
Jiansong Yuan ◽  
...  
Keyword(s):  
Late Gadolinium Enhancement ◽  
Myocardial Fibrosis ◽  
Critical Role ◽  
Significant Negative Correlation ◽  
Hypertrophic Obstructive Cardiomyopathy ◽  
Left Ventricular ◽  
Gadolinium Enhancement ◽  
Septal Wall ◽  
Microvascular Rarefaction ◽  
Severe Fibrosis

Background: Hypertrophic obstructive cardiomyopathy (HOCM) is a myocardial disease characterized by fibrosis and microvascular ischemia. Microvessels play a critical role in myocardial fibrosis in HOCM. However, it remains unclear whether or not myocardial fibrosis is associated with microvascular density (MVD) changes. Objective: The aim of the present study was to investigate whether a reduction in MVD is related to myocardial fibrosis in HOCM cardiac samples. Methods: We analyzed MVD and fibrosis in myectomy left ventricular (LV) septal wall specimens from 53 HOCM patients. Control myocardium from the LV septal wall was collected at autopsy of 9 individuals who died of noncardiac causes. Results: The fibrosis ratio (% area) in HOCM was higher and the MVD was lower than that in control subjects (i.e., 12.7 ± 10.0 vs. 4.0 ± 1.4%, p = 0.012, and 480.9 ± 206.7 vs. 1,425 ± 221/mm2, p < 0.001). Patients with mild fibrosis had a higher MVD than patients with moderate fibrosis (i.e., 568.2 ± 214.8 vs. 403.2 ± 167.8/mm2, p = 0.006) and patients with severe fibrosis (i.e., 568.2 ± 214.8 vs. 378.6 ± 154.0/mm2, p = 0.024). Furthermore, a significant negative correlation was found between myocardial fibrosis and MVD in HOCM patients (r = –0.40, p = 0.003), which was also found in mild fibrosis (r = –0.40, p = 0.043), moderate fibrosis (r = –0.50, p = 0.024), and severe fibrosis (r = –0.24, p = 0.61), although no significant differences were observed in severe fibrosis. Additionally, we demonstrated that late gadolinium enhancement was negatively correlated with MVD (r = –0.37, p = 0.03) and positively correlated with fibrosis (r = 0.44, p = 0.01). Conclusion: HOCM patients had a higher myocardial fibrosis ratio and a lower MVD. The severity of myocardial fibrosis was negatively correlated with MVD in HOCM. These findings showed that a reduced MVD may contribute to myocardial fibrosis in HOCM.

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