Quantitative fragmented QRS has a good diagnostic value on myocardial fibrosis in hypertrophic obstructive cardiomyopathy based on clinical-pathological study

Abstract The phenotypic expression of non-compaction cardiomyopathy (NCM) ranges from subclinical arrhythmias to sudden cardiac death. Objective Search of identifying predictors of life-threatening ventricular tachyarrhythmias (ltVTA) and analysis arrhythmic risk stratification capacity of ECG (heart rate turbulence – HRT, microvolt T-wave alternans – mTWA, QT interval corrected - QTc, and fragmented QRS - frQRS), Echo and MRI features in pts with NCM was the goal of this study. Methods The study enrolled and followed up 59,7±16,2 months 72 pts (family – 13; sporadic – 59; aged 39.2±14.1 years; 43/59.7% male; LVEF 44.7±13.2%) with morphological signs of non-compaction myocardium, confirmed by any three of the four standart criteria: Echo (Jenni, Stöllberger), MRI (Petersen, Jacquier). For analysis as primary end-points the following ltVTA events were accepted: SCD, successful resuscitation, stVT/VF/syncope, appropriated ICD discharges. Results As a result of the rank correlation Spearman analysis positive correlations (p<0.0001) of an MRI markers of myocardial fibrosis were revealed with ECG parameters; thus, leads (n) with frQRS (frQRSn: r=0.65) and QT interval corrected (QTc: r=0.66) correlated with T1 native relaxation time (ms) and the LGE myocardial segments (n). Significant correlations (p<0.0005) are found between LGE (%) and frQRS(n): r=0.61; QTc: r=0.56; VTA (r=0.48); systolic dysfunction (LVEF: r=−0.59; GLS: r=0.58; TAPSE: r=−0.55; RVEF: r=−0.54) and biventricular non-compaction (r=0.60). Positive correlations of the frQRS(n) with the pathological mTWA test (mTWA mean: r=0.55; p<0.001), VTA (r=0.61; p=0.0008) and negative correlations with systolic/contractile function (LVEF: r=−0.56, p=0.001; TAPSE: r=−0.51, p=0.003; LV GLS “–”%: r=−0.57, p<0.001) were determined. Surprisingly, significant correlations between NC/C ratio (one of the main diagnostic criteria of LVNC) and other phenotypic signs or clinical complications were not identified. As a result of step-by-step multiple regression analysis, a predictive model was constructed (R=0.53; F=8.50; p<0.ehz748.05257) and 3 independent predictors of ltVTA were revealed: HRT slope (TS: β=−0.36; p=0.002), QRS fragmentation (nQRSfr: β=0.32; p=0.001), and myocardial fibrosis (LGE%: β=0.27; p=0.02). As a result of ROC analysis following cut-off value were detected: fibrosis (LGE ≥22.5%; AUC 0.742; 95% CI: 0.594–0.889; sensitivity 77%; specificity 78%), pathological HRT (TS ≤2.5 ms/RR; AUC 0.771; 95% CI: 0.629–0.912; sensitivity 67%, specificity 87%), and fragmented QRS (frQRSn ≥5 leads; AUC 0.721; 95% CI: 0.559–0.883; sensitivity 80%, specificity 87%). Conclusion These markers (frQRSn along with LGE) reflect pathophysiological substrate triggering fatal arrhythmias; association of higher number of leads with frQRS, expressed LGE and pathological TS-HRT may be used as independent predictors of ltVTA for NCM pts

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

Heart ◽

10.1136/hrt.44.4.433 ◽

1980 ◽

Vol 44 (4) ◽

pp. 433-443 ◽

Cited By ~ 116

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

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

Journal of Investigative Medicine ◽

10.1136/jim-2016-000120.17 ◽

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.

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