scholarly journals 406 A 50 years old man with a late presentation of arrhythmogenic cardiomyopathy

2021 ◽  
Vol 23 (Supplement_G) ◽  
Author(s):  
Carlo Maria Dellino ◽  
Riccardo Bariani ◽  
Alberto Cipriani ◽  
Martina Perazzolo Marra ◽  
Kalliopi Pilichou ◽  
...  

Abstract In January 2014 a 50 years old man without previous medical history experienced a syncopal episode. During evaluation at Emergency department (ED) a sustained ventricular tachycardia (VT) with haemodynamic compromise was found and successfully treated with DC shock. The patient was admitted to the Coronary Care Unit (CCU) where the ECG showed diffuse low QRS voltages and flattened T waves. Coronary angiography showed normal coronary arteries. 2-D echocardiogram documented the presence of a mildly dilated left ventricle (LV) with a mildly decreased systolic function (EF : 41%); the right ventricle (RV) was severely dilated (REDV : 41 cmq/mq) with a severe systolic dysfunction (fractional area change: 21%) with diffuse hypokinesia and akinesia of subtricuspid region. Cardiac magnetic resonance (CMR) confirmed ventricular dimensional and kinetic abnormalities and tissue characterization sequences demonstrated the presence of fatty infiltration of the epicardial segments of LV lateral wall and of RV free wall. After gadolinium injection, late gadolinium enhancement (LGE) presented the same distribution of the fatty infiltration. A diagnosis of arrhythmogenic cardiomyopathy (AC) was made and ICD in secondary prevention was implanted. The patient was treated with Sotalol (240 mg/daily) and remained asymptomatic and free from sustained ventricular arrhythmias for five years. In January 2019 he started to complain asthenia, dyspnoea (NYHA II) and anorexia and he was admitted to ED where a persistent slow VT was detected. Echocardiogram showed a severely dilated LV with severe systolic dysfunction (EF: 30%) with substantially unchanged RV features. One year later he experienced an heart failure (HF) episode with further reduction of LV systolic function (EF: 21%). Cardiopulmonary test documented a severe ventilation/perfusion mismatch (VE/VCO2 slope 50.6) and severe reduction of the exercise tolerance (VO2 peak 9.2 ml/kg/min). In March 2021 the patient started heart transplantation check list. Three weeks after the discharge he was transplanted. In conclusion, this clinical case highlights an infrequent late presentation of AC, with an initial high arrhythmic burden and a following rapid progression to refractory HF requiring heart transplantation.

2011 ◽  
Vol 51 (2) ◽  
pp. 79
Author(s):  
Erlina Masniari Napitupulu ◽  
Fera Wahyuni ◽  
Tina Christina L. Tobing ◽  
Muhammad Ali ◽  
Bidasari Lubis

Background Chronic severe anemia is a connnon disease. Cardiac output may increase when the hemoglobin (Hb) level decreases to < 7 g/dL for 3 months or more. Alteration of left ventricular (LV) function occurs frequently in children 'With chronic severe anemia, in the {onn of concentric LV hypertrophy, LV dilatation with or v.ithout LV hypertrophy, or systolic dysfunction. Objective To examine the correlation between Hb level and alteration of LV systolic function in children with chronic severe anemia. Methods We conducted a cross-sectional study in Adam Malik Hospital from October to December 2009. Subjects were chronic severely anemic children. Left ventricular systolic function (ejection fraction/EF, fractional shortening/FS) and dimensions (left ventricular end diastolic diameter/LVEDD and left ventricular end systolic diameter/LVESD) were measured using Hitachi EUB 5500 echocardiography unit. Univariate analysis  and Pearson correlation were performed.Results Thirty children were enrolled in the study. The mean of age was 113.5 months (SD 53.24). Hb values ranged from 2.1 to 6.9 g/dL with mean value of 4.6 g/dL (SD 1.44). Mean duration of anemia was 3.9 months (SD 0.70). Chronic severe anemia was not associated \\lith decreased LV systolic function [EF 62.2% (SD 9.16), r =0.296, P=0.112; FS 33.8% (SD 7.26), r =0.115, P=0.545], nor LV dimension changes [LVEDD 40.2 mm (SD 6.85), r = -0.192, P=0.308; LVESD 26.2 mm (SD 4.98), r=-0.266, P=0.156]. Conclusion There was no correlation between Hb level in chronically anemic children and changes in LV systolic function or dimension.


2018 ◽  
Vol 2018 ◽  
pp. 1-3
Author(s):  
C. Henriquez ◽  
R. Landau ◽  
N. Sabharwal ◽  
D. Rodriguez ◽  
V. Virparia ◽  
...  

A 73-year-old female with multiple comorbidities including coronary artery disease was admitted for an elective PCI of a lesion detected in the RCA. On the day of the planned PCI, shortly after right femoral artery cannulation, the patient developed a sudden complete heart block requiring the administration atropine and insertion of a temporary pacemaker. Concomitantly, the patient developed acute pulmonary edema, hypotension, and hypoxia requiring intubation for mechanical ventilation. Vasopressors were administered. A coronary angiogram showed patent left and right coronary arteries, unchanged when compared to the previous angiogram. An echocardiogram performed in the cardiac catheterization lab revealed global hypokinesis of the left and right ventricles, with severe LV systolic dysfunction (EF<20%). Following an insertion of an intra-aortic balloon pump, the patient was transferred to the CICU. A repeat echocardiogram in the CICU two hours later revealed a classical echocardiographic presentation of Takotsubo syndrome, apical hypokinesis. By the next morning the patient’s hemodynamic status significantly improved, the balloon pump was removed, and vasopressors were discontinued. Another echocardiogram was performed 24 hours after the event occurred and revealed a marked improvement in LV systolic function (EF 60%), with complete resolution of apical and septal wall motion abnormalities. Three days after the event, the patient was successfully discharged and asymptomatic at two-month follow-up. This case illustrates an atypical presentation of Takotsubo syndrome that was witnessed from onset to its complete resolution during the patient’s hospital stay.


2019 ◽  
Vol 23 (1) ◽  
pp. 17
Author(s):  
Yu. S. Sinelnikov ◽  
E. N. Orekhova ◽  
T. V. Matanovskaya

<p><strong>Aim.</strong> To evaluate left ventricular systolic function in the early postoperative period after surgical perimembranous ventricular septal defect (VSD) closure in children aged &lt;1 year using longitudinal mechanics.</p><p><strong>Methods.</strong> Between 2014 and 2017, we examined 65 children aged 2–11 months with perimembranous VSD (mean diameter = 8.6 ± 1.7 mm) and a pulmonary-to-systemic blood flow ratio of &gt;1.5/1. The longitudinal mechanics of the left ventricle (LV) were assessed using vector velocity imaging. All the children underwent surgical perimembranous VSD closure. Echocardiographic studies were performed preoperatively, immediately postoperatively, and 1 and 20 days postoperatively.</p><p><strong>Results.</strong> In all the children, significant decreases in the LV end-systolic volume index, end-diastolic volume index, stroke volume index (SVI), and ejection fraction (EF) were identified immediately postoperatively when compared with the preoperative findings. One day postoperatively, the LV volume data normalized; however, in 10.8% of the children, the SVI and EF remained impaired. The percentage EF reduction showed weak correlations with the childrens’ weights (Rs = 0.3; p = 0.039), aortic cross-clamping durations (Rs = 0.36; p = 0.03), and VSD sizes (Rs = 0.33; p = 0.006). Twenty days postoperatively, the LV volumetric parameters normalized; however, the EF did not reach the initial value. The longitudinal strain and strain rate were reduced preoperatively when compared with normal values. For the prediction of a postoperatively significant EF reduction (≤35%), the preoperative LV strain had a sensitivity of 88%, specificity of 98.2%, and cutoff value of –9.2%; the LV strain rate had a sensitivity of 87.8%, specificity of 94.7%, and cutoff value of –0.47 s<sup>−</sup><sup>1</sup>.</p><p><strong>Conclusion.</strong> Children aged &lt;1 year with perimembranous VSD showed reductions in the longitudinal mechanics of the LV, reflecting subclinical systolic dysfunction. One day after surgical correction, a transient decrease in LV systolic function was noted in all the children; however, a significantly impaired LV systolic function was noted in 10.8% of the children. Our findings indicate that the longitudinal mechanics of the LV can predict LV systolic dysfunction in the early postoperative period with high sensitivity and specificity.</p><p>Received 17 January 2019. Revised 18 April 2019. Accepted 25 April 2019.</p><p><strong>Funding:</strong> The study did not have sponsorship.</p><p><strong>Conflict of interest:</strong> Authors declare no conflict of interest.</p>


2021 ◽  
Vol 23 (Supplement_D) ◽  
Author(s):  
Mohamed ElGendi ◽  
Mohamed Ayman ◽  
Mohamed Sadaka ◽  
Gehan Magdi

Abstract Aim The aim was to evaluate left ventricular (LV) systolic function in patients with isolated mitral stenosis (MS) using 2D speckle tracking echocardiography. Methods 24 patients (39.50 ± 5.55 years, 17 females) with isolated MS (MVA: 1.35 ± 0.16 cm2) with preserved LV systolic function and sinus rhythm were compared to 12 matched healthy control subjects (36.42 ± 5.99 years, 8 females). Conventional echocardiography was performed to both groups. Longitudinal strain and Circumferential strain echocardiography were obtained. Peak systolic strain was measured from the mean strain profile for a total of 17 segments of the LV for the longitudinal strain and 16 segments for the circumferential strain. Global longitudinal (G.L.) and circumferential strain (G.C.) were calculated separately as the average of the sum of the studied segments. Results The global longitudinal strain of the cases group ranged from -11 – -17% with a mean value of -14.67 ± -1.69% and that of the control group ranged from -15 – -20% with a mean value of -17.83 ± -1.53% with a statistically significant difference between the two groups. In our study, there was a negative but non-significant correlation between LV GLSS and LA diameter (r = -0.054, p = 0.802), Echo score (r = -0.018, p = 0.933) and PASP (r = 0.021, p = 0.922) in patients group. Also, the correlation was negative but non-significant between LV GCSS and LA diameter (r = -0.142, p = 0.507), Echo score (r = -0.200, p = 0.349) and PASP (r = -0.155, p = 0.471) in patients group. Conclusion • 2D speckle tracking echocardiography can detect subclinical LV systolic dysfunction which cannot be recognized by 2D conventional echocardiography. • Isolated rheumatic MS may be associated with subclinical LV systolic dysfunction.


2021 ◽  
Vol 5 (6) ◽  
Author(s):  
Nicole Lao ◽  
Zenab Laiq ◽  
Jeffrey Courson ◽  
Adeeb Al-Quthami

Abstract Background Desmosomes are specialized intercellular adhesive junctions of cardiac and epithelial cells that provide intercellular mechanical coupling through glycoproteins, one of which is desmoglein (DSG). DSG-2 mutations are frequently associated with biventricular arrhythmogenic cardiomyopathy (ACM). We report a case of left-dominant ACM in a patient who initially was misclassified as dilated cardiomyopathy (DCM). Case summary A 28-year-old-woman was found to have a moderately reduced left ventricular (LV) systolic function and frequent premature ventricular contractions (PVCs). Targeted genetic testing revealed a heterozygous likely pathogenic variant associated with ACM in exon 15 of the DSG-2 gene (c.3059_3062del; p.Glu1020Alafs*18). Subsequent cardiac magnetic resonance (CMR) imaging showed epicardial and mid-myocardial fatty infiltration involving multiple LV wall segments, multiple areas of mid-myocardial fibrosis/scar, regional dyskinesis involving both ventricles, and an overall reduced left ventricular ejection fraction. The patient’s right ventricular (RV) cavity size and overall RV systolic function were normal. Based on the patient’s frequent PVCs, family history, fibrofatty myocardial replacement in multiple LV segments, and dyskinetic motion of multiple ventricular wall segments (predominantly affecting the LV), the patient was diagnosed with left-dominant ACM. Discussion Identifying a likely pathogenic mutation associated with ACM in a patient with ventricular arrhythmias and a family history of sudden cardiac death increased the possibility of ACM. Subsequent CMR imaging confirmed the diagnosis of left-dominant ACM by demonstrating regional biventricular dyskinesia and a characteristic pattern of fibrofatty myocardial replacement. Our case highlights the importance of targeted genetic testing and advanced cardiac imaging in distinguishing left-dominant ACM from DCM.


Circulation ◽  
2018 ◽  
Vol 138 (Suppl_1) ◽  
Author(s):  
Carlos G SantosGallego ◽  
Juan Antonio Requena-Ibanez ◽  
Rodolfo San Antonio ◽  
Kiyotake Ishikawa ◽  
Belén Picatoste ◽  
...  

Introduction: In vitro, ketone bodies (KB) are the most energetically efficient fuel for myocardium. Ex vivo, KB infusion in the perfusion medium of working rat hearts increases the heat of combustion (produced energy) by 31%. However, there is no report about the in vivo effects of KB on LV function. We hypothesized that KB infusion in HFREF would improve energy production and thus LV systolic function. Methods: HFREF was induced in 15 pigs by 2-hour balloon occlusion of LAD: proximal LAD (n=8, severe HREF) and mid LAD (n=7, moderate HFREF). At 2 months, LV systolic function was evaluated during saline infusion and during infusion of the KB β-hydroxybutyrate. Severe HFREF animals underwent cardiac MRI for baseline LVEF, feature tracking strains, and contractile reserve (ΔLVEF under dobutamine 5μg/kg/min). Moderate HFREF pigs underwent invasive hemodynamic assessment (dP/dt) and 3D-echocadiography (3D-LVEF and 3D-strains). Simultaneous sampling from coronary artery and coronary sinus was performed to measure myocardial fuel consumption. Results: Proximal and mid LAD occlusion resulted in severe and moderate LV systolic dysfunction, respectively. In the severe HFREF, ketone infusion improved baseline LVEF, feature-tracking strains (both longitudinal and circumferencial strain), and contractile reserve. In the moderate HFREF, ketone infusion improved 3D-LVEF, 3D-strains and dP/dt (Table). Ketone infusion switched myocardial metabolism from glucose to ketone consumption. Conclusions: Continuous infusion of the KB hydroxybutyrate improves LV systolic function independent of LV systolic dysfunction severity via a shift in myocardial fuel metabolism away from glucose oxidation (energy inefficient) toward a more energy-efficient fuel like KB. This effect can explain the mechanism of action of the benefits of SGLT2 inhibitors in heart failure, as empagliflozin-induced mild kyperketonemia may increase LV systolic function and thus improve patient outcomes.


Circulation ◽  
2015 ◽  
Vol 132 (suppl_3) ◽  
Author(s):  
Abdulla Damluji ◽  
Yahya E Alansari ◽  
Michael Dyal ◽  
Magdalena Murman ◽  
Mohamad Kabach ◽  
...  

Introduction: We sought to describe changes in left ventricular ejection fraction (LVEF) after TAVR procedures. Methods: This is an observational study from 04/2008 to 06/2015 of all consecutive adults who received TAVR for severe symptomatic AS with Edwards Sapien or Medtronic CoreValve at two tertiary academic centers in USA and France. Results: Of 765 patients who received TAVR, 716 (94%) had follow-up echocardiography. Of those, 513 (72%), 143 (20%), 60 (8%) had a baseline EF>50%, EF 30-49%, and EF<30, respectively. Patients with EF < 30% were more likely to be Hispanic males. There were no differences in age, CVD risk factors, or history of multivessel coronary disease among groups. Patients with EF<30% were more likely to have AICD implantation and paced rhythm. All groups had similar rates of IABP insertion for hemodynamic support (EF≥50%: 6%, EF<30-49%: 9%, EF<30: 5%, p=0.544), procedural success (EF≥50%: 94%, EF<30-49%: 97%, EF<30: 98%, p=0.180), in-hospital mortality, procedural complications, and complete heart block. However, one-year all-cause-mortality was higher if baseline LV systolic function was abnormal (EF≥50%: 6%, EF<30-49%: 14%, EF<30: 9%, p=0.036). On 30-day follow-up echocardiography, absolute improvement in LVEF was highest among patients with EF<30% (Figure 1). If baseline LVEF was reduced, unchanged or improved mitral regurgitation were associated with improved LV function on follow-up (Figure 2). Conclusion: Transcatheter treatment of severe symptomatic AS is safe and feasible, even in patients with LVEF<30%. Most patients with LVEF<50% had increased EF after TAVR procedures.


Author(s):  
David Sidebotham ◽  
Alan Merry ◽  
Malcolm Legget ◽  
Gavin Wright

Chapter 6 is subdivided into three sections: global LV systolic function, regional LV systolic function, and cardiomyopathies. In Section 1, commonly used indices of global systolic function, such as fractional area change and ejection fraction, are reviewed, along with their limitations related to oesophageal imaging. The relationship between stroke volume and ejection fraction is explored. Newer techniques such as quantitative 3D imaging and strain-rate imaging are described. In Section 2, the causes of regional systolic dysfunction are reviewed, along with the different aetiologies of real and apparent segmental wall motion abnormalities. Complications of myocardial infarction such as mitral regurgitation, true and false LV aneurysms, and ventricular septal rupture are also dealt with in this section. Section 3 provides an overview of the echocardiographic findings associated with various cardiomyopathies: dilated, hypertrophic, restrictive, acute myocarditis, LV non-compaction, and Takotsubo.


2020 ◽  
Vol 7 ◽  
Author(s):  
Jin-Tao Wu ◽  
Junaid A. B. Zaman ◽  
H. Yakup Yakupoglu ◽  
Boyalla Vennela ◽  
Cantor Emily ◽  
...  

Background: The efficacy of catheter ablation for atrial fibrillation (AF) in patients with functional mitral regurgitation (MR) and left ventricular (LV) systolic dysfunction (LVSD) is not known. The aim of the study is to determine the efficacy of catheter ablation for AF in patients with functional MR and LVSD, and to validate its effects on the severity of MR and cardiac reverse remodeling.Methods: We performed a retrospective study of 54 patients with functional MR who underwent AF ablation, including 21 (38.9%) with LVSD and 33 (61.1%) with normal LV systolic function (LVF). The primary outcomes evaluated were freedom from recurrent atrial tachyarrhythmia (ATa), severity of MR, and left atrial (LA) and LV remodeling.Results: During a mean follow-up of 20.7 ± 16.8 months, freedom from recurrent ATa was not significantly different between patients with LVSD and those with normal LVF after the first ablation (P = 0.301) and after multiple ablations (P = 0.728). Multivariable predictors of recurrent ATa were AF duration [hazard ratio (HR) 1.12, 95% confidence interval (CI) 1.01–1.25; P = 0.039), previous stroke (HR 5.28, 95% CI 1.46–19.14; P = 0.011), and estimated glomerular filtration rate (HR 0.97, 95% CI 0.95–0.99; P = 0.012). Compared with baseline, there was a significant reduction in severity of MR (P = 0.007), LA size (P &lt; 0.001) and LV end-systolic dimension (P = 0.008), and improvement in the LV ejection fraction (P = 0.001) after restoring sinus rhythm in patients with LVSD.Conclusion: Catheter ablation is a valid option for the treatment of AF in patients with functional MR and LVSD, even though multiple procedures may be required.


2021 ◽  
Vol 8 ◽  
Author(s):  
Yuman Li ◽  
Lingyun Fang ◽  
Shuangshuang Zhu ◽  
Yuji Xie ◽  
Bin Wang ◽  
...  

Background: The cardiac manifestations of coronavirus disease 2019 (COVID-19) patients with cardiovascular disease (CVD) remain unclear. We aimed to investigate the prognostic value of echocardiographic parameters in patients with COVID-19 infection and underlying CVD.Methods: One hundred fifty-seven consecutive hospitalized COVID-19 patients were enrolled. The left ventricular (LV) and right ventricular (RV) structure and function were assessed using bedside echocardiography.Results: Eighty-nine of the 157 patients (56.7%) had underlying CVD. Compared with patients without CVD, those with CVD had a higher mortality (22.5 vs. 4.4%, p = 0.002) and experienced more clinical events including acute respiratory distress syndrome, acute heart injury, or deep vein thrombosis. CVD patients presented with poorer LV diastolic and RV systolic function compared to those without CVD. RV dysfunction (30.3%) was the most frequent, followed by LV diastolic dysfunction (9.0%) and LV systolic dysfunction (5.6%) in CVD patients. CVD patients with high-sensitivity troponin I (hs-TNI) elevation or requiring mechanical ventilation therapy demonstrated worsening RV function compared with those with normal hs-TNI or non-intubated patients, whereas LV systolic or diastolic function was similar. Impaired RV function was associated with elevated hs-TNI level. RV function and elevated hs-TNI level were independent predictors of higher mortality in COVID-19 patients with CVD.Conclusions: Patients with COVID-19 infection and underlying CVD displayed impaired LV diastolic and RV function, whereas LV systolic function was normal in most patients. Importantly, RV function parameters are predictive of higher mortality.


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