scholarly journals Utility of 4-Dimensional echocardiography and left ventricular systolic strain measured by 2-dimensional speckle-tracking for Danon Disease- a case series

2021 ◽  
Author(s):  
Ma Changsheng ◽  
Fan Jiali ◽  
Zhou Bingyuan ◽  
Zhou Jiawei ◽  
Wang Li ◽  
...  
Keyword(s):  
Cardiac Fibrosis ◽  
Case Series ◽  
Left Ventricular ◽  
Left Ventricular Ejection ◽  
Functional Evaluation ◽  
Systolic Strain ◽  
Ventricular Ejection Fraction ◽  
Danon Disease ◽  
Specific Strain ◽  
Dimensional Echocardiography

Abstract Background Danon disease is an X-linked multisystemic disorder characterised by skeletal myopathy, cardiomyopathy and intellectual disability. Summary of cases Herein we describe two patients affected by Danon disease from the same family, a father (patient 1) and his daughter (patient 2). In patient 1 a short PR interval with pre-excitation was evident. In patient 2, over a 24-hour period 2369 atrial premature beats and rare isolated ventricular ectopics were detected. Both patients exhibited left ventricular hypertrophy with non-compaction myocardium, and the left ventricular ejection fraction was impaired in patient 1 and normal in patient 2. In patient 2, the total left ventricular strain value was reduced, and layer-specific strain revealed that subepicardial strain impaired more than in other layers. Late gadolinium enhancement was detected both in left and right ventricles in patient 2, and cardiac fibrosis was more apparent in the subepicardium of left ventricular free wall. Four-dimensional echocardiography revealed that left atrial reservoir strain and left ventricular total longitudinal strain were induced. Discussion Novel four-dimensional echocardiography and left ventricular systolic strain may play important role in diagnosis and myocardial functional evaluation in Danon disease.

scholarly journals Rationale and design of the PHOspholamban RElated CArdiomyopathy intervention STudy (i-PHORECAST)

2021 ◽  
Author(s):  
W. P. te Rijdt ◽  
E. T. Hoorntje ◽  
R. de Brouwer ◽  
A. Oomen ◽  
A. Amin ◽  
...  
Keyword(s):  
Disease Progression ◽  
Cardiac Fibrosis ◽  
Intervention Study ◽  
Left Ventricular ◽  
Left Ventricular Ejection ◽  
Probe Design ◽  
Mineralocorticoid Receptor Antagonist ◽  
Ventricular Ejection Fraction ◽  
Increased Risk ◽  
Overt Disease

Abstract Background The p.Arg14del (c.40_42delAGA) phospholamban (PLN) pathogenic variant is a founder mutation that causes dilated cardiomyopathy (DCM) and arrhythmogenic cardiomyopathy (ACM). Carriers are at increased risk of malignant ventricular arrhythmias and heart failure, which has been ascribed to cardiac fibrosis. Importantly, cardiac fibrosis appears to be an early feature of the disease, occurring in many presymptomatic carriers before the onset of overt disease. As with most monogenic cardiomyopathies, no evidence-based treatment is available for presymptomatic carriers. Aims The PHOspholamban RElated CArdiomyopathy intervention STudy (iPHORECAST) is designed to demonstrate that pre-emptive treatment of presymptomatic PLN p.Arg14del carriers using eplerenone, a mineralocorticoid receptor antagonist with established antifibrotic effects, can reduce disease progression and postpone the onset of overt disease. Methods iPHORECAST has a multicentre, prospective, randomised, open-label, blinded endpoint (PROBE) design. Presymptomatic PLN p.Arg14del carriers are randomised to receive either 50 mg eplerenone once daily or no treatment. The primary endpoint of the study is a multiparametric assessment of disease progression including cardiac magnetic resonance parameters (left and right ventricular volumes, systolic function and fibrosis), electrocardiographic parameters (QRS voltage, ventricular ectopy), signs and/or symptoms related to DCM and ACM, and cardiovascular death. The follow-up duration is set at 3 years. Baseline results A total of 84 presymptomatic PLN p.Arg14del carriers (n = 42 per group) were included. By design, at baseline, all participants were in New York Heart Association (NHYA) class I and had a left ventricular ejection fraction > 45% and < 2500 ventricular premature contractions during 24-hour Holter monitoring. There were no statistically significant differences between the two groups in any of the baseline characteristics. The study is currently well underway, with the last participants expected to finish in 2021. Conclusion iPHORECAST is a multicentre, prospective randomised controlled trial designed to address whether pre-emptive treatment of PLN p.Arg14del carriers with eplerenone can prevent or delay the onset of cardiomyopathy. iPHORECAST has been registered in the clinicaltrials.gov-register (number: NCT01857856).

Predicting mortality for patients with heart failure beyond oxygen consumption: a prognostic risk score

2021 ◽  
Author(s):  
Hanaa Shafiek ◽  
Andres Grau ◽  
Jaume Pons ◽  
Pere Pericas ◽  
Xavier Rossello ◽  
...  
Keyword(s):  
Heart Failure ◽  
Chronic Heart Failure ◽  
Risk Score ◽  
Cardiac Transplantation ◽  
Left Ventricular ◽  
Left Ventricular Ejection ◽  
Functional Evaluation ◽  
Cardiac Events ◽  
Ventricular Ejection Fraction

Background: Cardiopulmonary exercise test (CPET) is a crucial tool for the functional evaluation of cardiac patients. We hypothesized that VO2 max and VE/VCO2 slope are not the only parameters of CPET able to predict major cardiac events (mortality or cardiac transplantation urgently or elective). Objectives: We aimed to identify the best CPET predictors of major cardiac events in patients with severe chronic heart failure and to propose an integrated score that could be applied for their prognostic evaluation. Methods: We evaluated 140 patients with chronic heart failure who underwent CPET between 2011 and 2019. Major cardiac events were evaluated during follow-up. Univariate and multivariate logistic regression analysis were applied to study the predictive value of different clinical, echocardiographic and CPET parameters in relation to the major cardiac events. A score was generated and c-statistic was used for the comparisons. Results: Thirty-nine patients (27.9%) died or underwent cardiac transplantation over a median follow-up of 48 months. Five parameters (maximal workload, breathing reserve, left ventricular ejection fraction, diastolic dysfunction and non-idiopathic cardiomyopathy) were used to generate a risk score that had better risk discrimination than NYHA dyspnea scale, VO2 max, VE/VCO2 slope > 35 alone, and combined VO2 max and VE/VCO2 slope (p= 0.009, 0.004, < 0.001 and 0.005 respectively) in predicting major cardiac events. Conclusions: A composite score of CPET and clinical/echocardiographic data is more reliable than the single use of VO2max or combined with VE/VCO2 slope to predict major cardiac events.

Intra-Cardiac Thrombus in COVID-19 pandemic – Case Series and Review

2020 ◽  
Author(s):  
Kartik Pandurang Jadhav ◽  
Pankaj Jariwala
Keyword(s):  
Myocardial Infarction ◽  
Thrombus Formation ◽  
Case Series ◽  
Left Ventricular ◽  
Left Ventricular Ejection ◽  
Ventricular Ejection Fraction ◽  
Systemic Thrombolysis ◽  
Cardiac Thrombus ◽  
Exact Etiology ◽  
St Elevation

ABSTRACT Various publications have increasingly reported the development of the prothrombotic state and its consequences associated with coronavirus disease 2019 (COVID-19). Although the exact etiology is uncertain, various factors collectively increase the risk of thrombus formation in COVID-19 patients. We present a case series of four patients with left ventricular (LV) thrombus formation along with simultaneous COVID-19 infection. All these patients had acute myocardial infarction with left ventricular ejection fraction (LVEF) between 35-45%. Among the series, two patients had favourable outcomes with complete resolution of LV thrombus, whereas the other two suffered cerebral embolization followed by mortality. This study looks in depth at all cases of intracardiac thrombi formation in patients with COVID-19 published worldwide. n addition to the increased predisposition for venous/ arterial thrombosis, even a few cases of intra- cardiac thrombus have been reported. Systemic thrombolysis is an initial treatment of choice for the management of right cardiac thrombi with pulmonary thromboembolism (PTE) and ST-elevation myocardial infarction (STEMI) in COVID-19. Right cardiac thrombi have better outcomes when compared to left cardiac thrombi.

scholarly journals Who ′Nose′, is it the ARNi?: A Case Series of Persistent Nasal Pruritus in Heart Failure Patients receiving Sacubitril/Valsartan

2021 ◽  
Author(s):  
Jaclyn Gan ◽  
Haunnah Rheault ◽  
Yee Weng Wong
Keyword(s):  
Heart Failure ◽  
Adverse Effect ◽  
Product Information ◽  
Case Series ◽  
Left Ventricular ◽  
Left Ventricular Ejection ◽  
Ventricular Ejection Fraction ◽  
Mortality And Morbidity ◽  
Case Summary

Abstract Background Sacubitril/valsartan is approved for the treatment of chronic heart failure with reduced left ventricular ejection fraction (HFrEF) of less than or equal to 40% to decrease mortality and morbidity. Nasal pruritus is not a recognised adverse effect in the product information. In this case series, we encountered three patients presented with nasal pruritus that improved after discontinuation of sacubitril/valsartan. Case Summary Three patients aged 58-73 years-old presented with pruritus at the nasal septum post-initiation of sacubitril/valsartan. The pruritus did not subside despite the use of anti-histamines. Within 3-6 months, all individuals discontinued sacubitril/valsartan with complete resolution of their nasal pruritus. Discussion Many physicians may not aware of this unusual but reversible adverse effect of sacubitril/valsartan. Despite the positive prognostic value of sacubitril/valsartan, the constant nasal pruritus had impacted the quality of life of our patients, leading them to discontinue sacubitril/valsartan permanently.

scholarly journals ACUTE MYOCARDIAL INFARCTION

2018 ◽  
Vol 25 (08) ◽  
pp. 223-1228
Author(s):  
Gul Hassan Brohi ◽  
Shahzeb Rasool Memon ◽  
Muhammad Yaqoob Shahani ◽  
Samreen Memon ◽  
Umbreen Bano
Keyword(s):  
Case Series ◽  
Left Ventricular ◽  
University Hospital ◽  
Left Ventricular Ejection ◽  
Control Group ◽  
Aspiration Thrombectomy ◽  
Ventricular Ejection Fraction ◽  
Subtle Change ◽  
Case Series Study ◽  
Primary Stenting

Objectives: To evaluate the effects of aspiration thrombectomy (AT) using anAspiration Catheter (INVATEC SPA DIVER C.E. MAX, Italy) in patients with acute myocardialinfarction (AMI). Study Design: Cross sectional and Case series study. Setting: Departmentof Cardiology, Liaquat University Hospital, Hyderabad. Period: January 2012 to December2013. Methodology and Results: Measurement of left ventricular ejection fraction (LVEF) byechocardiography was obtained before (acute LVEF) percutaneous coronary intervention (PCI)and 1 week after (late LVEF) the procedure. Most of the patients with full restoration of STsegment elevation following PCI were higher in the aspiration Catheter control group (90 vs68%, P < 0.01), (86 vs 62%, P < 0.01) and (90 vs 64%, P < 0.01) respectively. Minimal changein LVEF was observed in patients between the Aspiration Catheter group and control group at1 week post procedure. Conclusion: Combination of AT using Aspiration Catheter (INVATECSPA DIVER C.E. MAX) with primary stenting can better outcome, while left ventricle (LV) functionhad subtle change in AMI when compared to primary stenting after balloon predilation withoutthrombectomy.

scholarly journals Del-Nido Cardioplegia in cardiac surgery for elderly patients: a propensity score-matched analysis

2021 ◽  
Author(s):  
Wenda Gu ◽  
Hongkun Qing ◽  
Xiang Luo ◽  
Xin Zang ◽  
Kan Zhou ◽  
...  
Keyword(s):  
Propensity Score ◽  
Elderly Patients ◽  
Artery Bypass ◽  
Case Series ◽  
Left Ventricular ◽  
Valve Surgery ◽  
Left Ventricular Ejection ◽  
Ventricular Ejection Fraction ◽  
Safety And Efficacy ◽  
Case Series Study

OBJECTIVES To compare the safety and efficacy of del-Nido cardioplegia (DNC) with traditional 4:1 cold blood cardioplegia (CBC) in coronary artery bypass grafting and/or valve surgeries in elderly patients. METHODS The present study is a retrospective case-series study that included 302 consecutive patients aged 70 years and over who underwent on-pump valve surgery and/or CABG. DNC was administered to 90 patients and CBC to 212 patients. After propensity-score matching, 89 pairs were compared. The safety and efficacy were analysed between the two groups. RESULTS Compared with the CBC group (2185.8±582.9ml, P<0.001), the volume of infused cardioplegia was less in the DNC group (1423.6±259.5ml). The DNC group had a lower incidence of postoperative intra-aortic balloon pump (IABP) implantation (1.1% vs 9.0%, RR=0.791, P=0.034) and higher left ventricular ejection fraction (LVEF) at discharge (58.5±9.1% vs 55.7±8.6%, P=0.007). The estimated glomerular filtration rate (eGFR) in the DNC group was higher when the patient was transferred to the intensive care unit (78.2±25.1 ml/min/1.73m vs 69.7±20.2 ml/min/1.73m , P=0.021), but no significant differences were identified after 24 hours. The serum lactate values of the DNC group were significantly lower than those of the CBC group (0hrs: 2.8±1.5 vs 3.6±2.0, P=0.001; 3hrs: 3.6±2.7 vs 4.9±2.6, P<0.001; 6hrs: 4.0±3.0 vs 6.1±3.3, P<0.001; 9hrs: 4.4±3.3 vs 5.9±3.6, P=0.004). There were no differences between the two groups in respect of lactate levels at 12 hours and thereafter. Postoperative creatinine kinase-MB concentrations were similar between the two groups. CONCLUSIONS Del-Nido cardioplegia is safe and effective in elderly patients undergoing CABG and/or valve surgery.

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