Arrhythmic risk stratification in non-ischaemic dilated cardiomyopathy beyond ejection fraction

Heart ◽  
2020 ◽  
Vol 106 (9) ◽  
pp. 656-664 ◽  
Author(s):  
Antonio Cannatà ◽  
Giulia De Angelis ◽  
Andrea Boscutti ◽  
Camilla Normand ◽  
Jessica Artico ◽  
...  
Keyword(s):  
Ejection Fraction ◽  
Risk Stratification ◽  
Dilated Cardiomyopathy ◽  
Left Ventricular ◽  
Left Ventricular Ejection ◽  
Ventricular Ejection Fraction ◽  
Novel Approach ◽  
Magnetic Resonance Parameters ◽  
Reverse Remodelling ◽  
Life Threatening

Sudden cardiac death and arrhythmia-related events in patients with non-ischaemic dilated cardiomyopathy (NICM) have been significantly reduced over the last couple of decades as a result of evidence-based pharmacological and non-pharmacological therapeutic strategies. Nevertheless, the arrhythmic stratification in patients with NICM remains extremely challenging, and the simple indication based on left ventricular ejection fraction appears to be insufficient. Therefore, clinicians need to go beyond the current criteria for implantable cardioverter-defibrillator implantation in the direction of a multiparametric evaluation of arrhythmic risk. Several parameters for arrhythmic risk stratification, ranging from electrocardiographic, echocardiographic, imaging-derived and genetic markers, are crucial for proper arrhythmic risk stratification and a multiparametric evaluation of risk in patients with NICM. In particular, integration of cardiac magnetic resonance parameters (mostly late gadolinium enhancement) and specific genetic information (ie, presence of LMNA, PLN, FLNC mutations) appears fundamental for proper implementation of the current arrhythmic risk stratification. Finally, a novel approach focused on both arrhythmic risk and prediction of left ventricular reverse remodelling during follow-up might be useful for effective multiparametric and dynamic arrhythmic risk stratification in NICM. In the future, a complete and integrated evaluation might be mandatory to implement arrhythmic risk prediction in patients with NICM and to discriminate the competing risk between heart failure-related events and life-threatening arrhythmias.

scholarly journals P52 Two pathologies one heart: life-threatening lupus myocarditis in a patient with pre-existing dilated cardiomyopathy

Rheumatology ◽  
2020 ◽  
Vol 59 (Supplement_2) ◽  
Author(s):  
Harold Wilson-Morkeh ◽  
Taryn Youngstein ◽  
Shabnam Shabbir ◽  
Jyoti Bakshi ◽  
Tomi Pihlajavaara ◽  
...  
Keyword(s):  
Ejection Fraction ◽  
Dilated Cardiomyopathy ◽  
Immunosuppressive Treatment ◽  
Left Ventricular ◽  
Delayed Enhancement ◽  
Left Ventricular Ejection ◽  
Ventricular Ejection Fraction ◽  
Life Threatening ◽  
Lupus Myocarditis ◽  
Cmr Imaging

Abstract Background Acute myocarditis is a life-threatening complication of systemic lupus erythematosus (SLE) often indicative of severe multisystem disease. Treatment of lupus myocarditis (LM) is well described in the literature with high-dose corticosteroids and cyclophosphamide providing the backbone of induction therapy. Less commonly reported are outcomes of patients with SLE and pre-existing structural heart disease. Methods We present a case of acute LM occurring in the context of established non-ischaemic dilated cardiomyopathy (DCM) presenting a significant diagnostic challenge. Results A 45-year-old woman from Afghanistan with known SLE presented with acute chest pain and dyspnoea. She had a previous mild SLE disease course limited to her skin and joints and was historically non-compliant with immunosuppressive treatment. She also had non-ischaemic DCM diagnosed six years prior to presentation with non-progressive imaging over this time. Upon acute assessment at the referring centre a widespread livedoid rash and cool peripheries were noted. Initial investigations revealed an elevated Troponin-I of 111ng/L and Brain Natriuretic Peptide (BNP) of 4497ng/L. An electrocardiogram revealed no acute ischaemic changes whilst her chest radiograph revealed features in keeping with pulmonary oedema. Transthoracic echocardiogram (TTE) demonstrated a dilated left ventricle with severe global systolic impairment. Her left ventricular ejection fraction (LVEF) was visually estimated at 10-15%, significantly reduced from her last recorded LVEF of 52% on a cardiovascular magnetic resonance (CMR) scan 18-months previously. Intravenous (iv) methylprednisolone, iv diuresis and B-cell depletion therapy were commenced prior to transfer to our department. Once transferred, a CMR scan confirmed an ejection fraction of only 12%. Septal mid-wall delayed enhancement was seen consistent with a non-ischaemic DCM, however, subepicardial delayed enhancement was visualised inferolaterally on native T1-mapping in keeping with perimyocarditis. Additional results on admission were supportive of a multisystem SLE flare. She was lymphopaenic (1.0x109/L), thrombocytopaenic (66x109/L), hypocomplimentaemic (C3 0.33g/L, C4 <0.03g/L), and had elevated dsDNA antibodies (83unit/mL). Serum creatinine had risen from a baseline of 47µmol/L to 110µmol/L and her urine protein: creatinine ratio (414mg/mmol) raised the suspicion of lupus nephritis (LN). Renal biopsy was not performed due to thrombocytopaenia. An MDT discussion with the cardiology, CMR imaging and renal teams took place with CMR imaging proving pivotal in establishing new LM on a background of established DCM. The decision to treat with intravenous (iv) cyclophosphamide was made and repeat TTE at 14 days revealed a significant improvement in LVEF to 25-30%. Conclusion CMR imaging was crucial in delineating acute and potentially reversible myocarditis on a background of chronic cardiomyopathy. This finding supported the escalation in immunosuppressive treatment to a cyclophosphamide-based regimen and led to the rapid improvement in ejection fraction in this young patient. She received 6 fortnightly doses of iv cyclophosphamide and is now maintained on hydroxychloroquine, mycophenolate mofetil and rituximab. Disclosures H. Wilson-Morkeh: None. T. Youngstein: None. S. Shabbir: None. J. Bakshi: None. T. Pihlajavaara: None. M. Bellamy: None. T. Cairns: None.

scholarly journals Familial dilated cardiomyopathy with RBM20 mutation in an Indian patient: a case report

2021 ◽  
Vol 73 (1) ◽  
Author(s):  
Soumi Das ◽  
Sandeep Seth
Keyword(s):  
Ejection Fraction ◽  
Dilated Cardiomyopathy ◽  
Age Of Onset ◽  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
Left Ventricular ◽  
Left Ventricular Ejection ◽  
Early Age ◽  
Indian Patient ◽  
Ventricular Ejection Fraction ◽  
First Case

Abstract Background Dilated cardiomyopathy (DCM) is a disease of the heart muscle characterized by ventricular dilation and a left ventricular ejection fraction of less than 40%. Unlike hypertrophic cardiomyopathy (HCM) and arrhythmogenic right ventricular cardiomyopathy (ARVC), DCM-causing mutations are present in a large number of genes. In the present study, we report a case of the early age of onset of DCM associated with a pathogenic variant in the RBM20 gene in a patient from India. Case presentation A 19-year-old Indian male diagnosed with DCM was suggested for heart transplantation. His ECG showed LBBB and echocardiography showed an ejection fraction of 14%. He had a sudden cardiac death. A detailed family history revealed it to be a case of familial DCM. Genetic screening identified the c.1900C>T variant in the RBM20 gene which led to a missense variant of amino acid 634 (p.Arg634Trp). Conclusion To the best of our knowledge, the variant p.Arg634Trp has been earlier reported in the Western population, but this is the first case of p.Arg634Trp in an Indian patient. The variant has been reported to be pathogenic at an early age of onset; therefore, close clinical follow-up should be done for the family members caring for the variant.

Beyond ejection fraction: Novel clinical approaches towards sudden cardiac death risk stratification in patients with dilated cardiomyopathy

2021 ◽  
Vol 17 ◽  
Author(s):  
Issa Pour-Ghaz ◽  
Mark Heckle ◽  
Ikechukwu Ifedili ◽  
Sharif Kayali ◽  
Christopher Nance ◽  
...  
Keyword(s):  
Sudden Cardiac Death ◽  
Ejection Fraction ◽  
Risk Stratification ◽  
Cardiac Death ◽  
Ventricular Tachyarrhythmia ◽  
Total Mortality ◽  
Left Ventricular ◽  
Left Ventricular Ejection ◽  
Ventricular Ejection Fraction ◽  
Icd Therapy

: Implantable cardioverter-defibrillator (ICD) therapy is indicated for patients at risk for sudden cardiac death due to ventricular tachyarrhythmia. The most commonly used risk stratification algorithms use left ventricular ejection fraction (LVEF) to determine which patients qualify for ICD therapy, even though LVEF is a better marker of total mortality than ventricular tachyarrhythmias mortality. This review evaluates imaging tools and novel biomarkers proposed for better risk stratifying arrhythmic substrate, thereby identifying optimal ICD therapy candidates.

Abstract 16457: Markers of Diabetes and Renal Function Extend Sudden Death Risk Assessment Beyond the Ejection Fraction

Circulation ◽  
2015 ◽  
Vol 132 (suppl_3) ◽  
Author(s):  
Kumar Narayanan ◽  
Audrey Uy-Evanado ◽  
Carmen Teodorescu ◽  
Kyndaron Reinier ◽  
Karen Gunson ◽  
...  
Keyword(s):  
Ejection Fraction ◽  
Risk Stratification ◽  
Serum Creatinine ◽  
Glycosylated Hemoglobin ◽  
Left Ventricular ◽  
Metropolitan Region ◽  
Left Ventricular Ejection ◽  
Ventricular Ejection Fraction ◽  
Catchment Population ◽  
Risk Stratification Model

Introduction: Although diabetes and renal dysfunction are known to be associated with SCD risk, the cumulative risk of objective laboratory markers in combination with left ventricular ejection fraction (LVEF) has not been previously evaluated. Hypothesis: Addition of glycosylated hemoglobin (HbA1C) and serum creatinine levels to LVEF can improve the SCD risk stratification model. Methods: As part of a large, prospective, ongoing study of SCD in a Northwestern US metropolitan region (catchment population about 1 million), SCD cases were compared with controls with coronary artery disease (CAD) and no SCD from the same geographic location. HbA1C, serum creatinine levels and LVEF (all prior and unrelated to the SCD event for cases) were obtained for all subjects. Odds ratios for SCD associated with abnormal HbA1C (≥ 7%) and creatinine (≥ 1.5 mg/dL) levels was calculated. Cumulative odds and improvement in model performance on addition of elevated lab markers to low LVEF (≤ 35%) was assessed. Results: 243 SCD cases (68.7 ± 13.2 yrs; 62.1% male) and 159 CAD controls (66.2 ± 9.9 yrs; 65.6% male) with appropriate lab values and LVEF information were evaluated. The mean HbA1C (7.3 ± 2.3 vs. 6.6 ± 1.5%) and creatinine (1.8 ± 1.7 vs. 1.2 ± 0.7 mg/dL) levels were significantly higher in cases. Cases were significantly more likely to have HbA1C ≥ 7% (49.4 vs. 27.7%; p<0.0001) or creatinine ≥ 1.5 mg/dL (39.1% vs. 13.8%) (all p<0.0001). After adjustment for age, sex and low LVEF, high HbA1C (OR 2.3, 95% CI 1.4-3.6; p=0.001) and high creatinine (OR 3.3, 95% CI 1.9-5.7; p<0.0001) were independently associated with SCD; LVEF ≤ 35% was associated as well (OR 1.8, 95% CI 1.1-3.2; p=0.05). As compared to neither lab marker being high, elevation of one marker (OR 2.4, 95% CI 1.5-3.9) or both markers (OR 7.9, 95% CI 3.5-17.6) was associated with progressive increase in SCD odds. Addition of lab markers to a risk stratification model with only LVEF improved model discrimination significantly (AUC 0.613 vs. 0.709; p=0.01). Conclusions: HbA1C and serum creatinine improved the SCD risk stratification model when added to LVEF. Further investigation is warranted before clinical use, including consideration of competing risks that influence overall mortality.

scholarly journals Analysis of the incidence and baseline predictors of the left ventricular ejection fraction returning to normal after dilated cardiomyopathy in postmenopausal women: a retrospective, observational study

2020 ◽  
Vol 48 (5) ◽  
pp. 030006052092247
Author(s):  
Xiaopin Yuan ◽  
Shuai Mao ◽  
Qizhu Tang
Keyword(s):  
Left Ventricular Ejection Fraction ◽  
Ejection Fraction ◽  
Dilated Cardiomyopathy ◽  
Postmenopausal Women ◽  
Systolic Pressure ◽  
Left Ventricular ◽  
Ventricular Ejection ◽  
Left Ventricular Ejection ◽  
Ventricular Ejection Fraction ◽  
History Of

Objective To analyse the incidence and baseline predictors of the left ventricular ejection fraction (LVEF) returning to normal after dilated cardiomyopathy (DCM) following intervention with standard anti-heart failure (HF) medication in postmenopausal women. Methods Data from consecutive postmenopausal women who were first diagnosed with DCM and received anti-HF treatment during 2011 to 2018 were prospectively retrieved. The study population was divided into the LVEF recovery (LVR) group and the LVEF unrecovered (LVU) group according to whether LVEF was > 50%. The primary endpoint was baseline predictors of LVEF returning to normal. Results LVEF returned to normal in 49.3% (210/426) of patients with DCM. LVEF was significantly higher in the LVR group than in the LVU group (57.4% ± 6.9% vs 44.2% ± 5.3%; hazard ratio 1.312, 95% confidence interval 1.015–1.726) at the final follow-up. High systolic pressure, a short history of HF, a short QRS interval, a small left ventricular end-diastolic diameter (LVEDd), and high LVEF at admission were independent predictors of LVEF returning to normal. Conclusions LVEF returning to normal in postmenopausal women with DCM who receive standard anti-HF treatment is associated with systolic pressure, a history of HF, QRS interval, LVEDd, LVEF at admission, and favourable outcome.

P370Added value changes in signal sonr in patients with = <30% left ventricular ejection fraction

EP Europace ◽  
2020 ◽  
Vol 22 (Supplement_1) ◽  
Author(s):  
J J Garcia Guerrero ◽  
J Fernandez De La Concha Castaneda ◽  
A Chacon Pinero ◽  
J Garcia Fernandez ◽  
I Fernandez Lozano ◽  
...  
Keyword(s):  
Ejection Fraction ◽  
Hospital Admission ◽  
Dilated Cardiomyopathy ◽  
Interim Analysis ◽  
Cardiac Contractility ◽  
Inverse Correlation ◽  
Left Ventricular ◽  
Left Ventricular Ejection ◽  
Ventricular Ejection Fraction ◽  
Lv Ejection Fraction

Abstract Abstract/Introduction Decompensated congestive heart failure (CHF) is a main and increasing health problem worldwide, which leads to patients’ bad outcomes and high money expenditure. Direct relationship between Brain Natriuretic Peptides (NT-proBNP) increasing levels and adverse clinical outcomes have been demonstrated in patients with CHF.  SonR signal sensor, a micro-accelerometer embedded in the tip of the atrial lead in patients implanted with devices, picks up cardiac muscle vibration. Its amplitude is a surrogate for cardiac contractility, which is found to be further reduced in patients with decompensated CHF. Purpose We sought to find a significant inverse correlation between SonR signal and NT-proBNP levels, in order to use SonR as a surrogate of NT-proBNP to anticipate worsening CHF leading to hospital admission. Methods AVCs SONR trial is a pilot, prospective, observational, multicentre study, in which patients with dilated cardiomyopathy, any aetiology, LV ejection fraction ≤ 30%, at least one recent (&lt; 1 year) hospital admission due to CHF, and implanted with CRT-D devices (used as dual-chamber, no left ventricular (LV) lead implanted) with SonR sensor feature, were enrolled. During a year, NT-proBNP and SonR values were obtained every month, and both levels compared (Pearson’s test) Results This an interim analysis of our data, 18 months after the first patient was enrolled. Twenty two patients and 116 data pairs were analysed. Most patients were men (91%) and had ischemic dilated cardiomyopathy (59%). Mean age was 61 (range 34-82) and mean LV ejection fraction was 27% (range 15-30). The mean Pearson’s correlation coefficient of the NT-proBNP values and the SonR signal was r = - 0.36 (95% CI -0.51 to -0.19), p &lt; 0.00006 (Figure) Conclusions The interim analysis of this study shows an inverse and very significant relationship between SonR signal and NT-proBNP values. This suggests SonR signal might be used as predictor of worsening CHF. Abstract Figure

Intramyocardial Angiogenic Cell Precursors in Nonischemic Dilated Cardiomyopathy

2009 ◽  
Vol 17 (4) ◽  
pp. 382-388 ◽  
Author(s):  
Kitipan V Arom ◽  
Permyos Ruengsakulrach ◽  
Michael Belkin ◽  
Montip Tiensuwan
Keyword(s):  
Left Ventricular Ejection Fraction ◽  
Ejection Fraction ◽  
Dilated Cardiomyopathy ◽  
Left Ventricular ◽  
Ventricular Ejection ◽  
Left Ventricular Ejection ◽  
Cell Group ◽  
Control Group ◽  
Ventricular Ejection Fraction ◽  
Nonischemic Dilated Cardiomyopathy

To determine the efficacy of intramyocardial injection of angiogenic cell precursors in nonischemic dilated cardiomyopathy, 35 patients with nonischemic dilated cardiomyopathy underwent injections of angiogenic cell precursors into the left ventricle (cell group). Seventeen patients with nonischemic dilated cardiomyopathy were matched from the heart failure database to form a control group that was treated medically. Angiogenic cell precursors were obtained from autologous blood, cultured in vitro, and injected into all free-wall areas of the left ventricle in the cell group. After these injections, New York Heart Association functional class improved significantly by 1.1 ± 0.7 classes at 284.7 ± 136.2 days, and left ventricular ejection fraction improved in 71.4% of patients (25/35); the mean increase in left ventricular ejection fraction was 4.4% ± 10.6% at 192.7 ± 135.1 days. Improved quality of life was demonstrated by better physical function, role-physical, general health, and vitality domains in a short-form health survey at the 3-month follow-up. In the control group, there were no significant improvements in left ventricular ejection fraction or New York Heart Association class which increased by 0.6 ± 0.8 classes. It was concluded that intramyocardial angiogenic cell precursor injection is probably effective in the treatment of nonischemic dilated cardiomyopathy. Disclosures and Freedom of Investigation Professor Michael Belkin is an advisory board member, a minor shareholder, and receives a consulting fee from TheraVitae Co. Ltd. However, the authors had full control of the study, methods used, outcome measurements, data analysis, and production of the written report.

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