Cardiomyopathies

Author(s):  
Patrizio Lancellotti ◽  
Bernard Cosyns

This chapter focuses on the role of echocardiography in dilated cardiomyopathy, showing diagnostic and associated findings along with the prognostic role of echocardiography. Primary myocardial disease is inadequate hypertrophy, independent of loading conditions and often other affected structures such as mitral valve apparatus, small coronary arteries, and cardiac interstitium. Arrhythmogenic RV cardiomyopathy is fatty or fibro-fatty infiltration of the RV with apoptosis and hypertrophied trabeculae of the RV. This chapter also details diagnostic findings and progression of this condition alongside relevant echocardiographic findings. Previously known as ‘spongy heart syndrome’, left ventricular non compaction is characterized by the absence of involution of LV trabeculae during the embryogenic process. This chapter demonstrates the diagnostic findings of this condition, and looks at the diagnostic findings and complications of Takotsubo cardiomyopathy, illustrating typical, RV apical and variant views. It also shows diagnostic findings in myocarditis in both the acute phase and follow-up.

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
A Chaloupka ◽  
J Krejci ◽  
H Poloczkova ◽  
P Hude ◽  
E Ozabalova ◽  
...  

Abstract Background The aetiology of recent-onset dilated cardiomyopathy (RODCM) includes inflammatory, genetic, toxic and metabolic causes. Delineating the role of inflammation on the genetic background could improve risk stratification. Purpose We aimed to ascertain the role of inflammation evaluated by serum CRP immunohistochemical and PCR analysis of endomyocardial biopsy (EMB) in conjunction with genetic testing in left ventricular reverse remodelling (LVRR) in 12-month follow-up. Methods 83 RODCM patients enrolled in this prospective observational study underwent 12-month echocardiographic follow up whole-exome sequencing, and EMB. Presence of cardiotropic viruses was determined by PCR analysis of the EMB samples. Inflammation was defined according to TIMIC immunohistochemical criteria as the presence of >7 CD3+ lymphocytes/mm2 and/or >14 infiltrating leukocytes (LCA+ cells/mm2). LVRR was defined as an absolute increase in LV ejection fraction > +10% and a relative decrease of LV end-diastolic diameter >−10% at 12 months. Results LVRR occurred in 28 (34%) of all cases. PCR analysis uncovered cardiotropic viruses in 55 (66%) patients, with highest prevalence of parvovirus B19 (47%). (Figure 1) EMB analysis detected inflammation in 28 (34%) cases and inflammation significantly positively predicted LVRR (P=0.019). Sequencing identified disease-related gene variants (ACMG class 3–5) in 45 (54%) patients. Carriers of non-titin gene variants showed a lowest probability of 12-month LVRR (19%) P=0.041. Combination of genetic findings and inflammation did not improve the prediction of LVRR in 12 months. (Table 1) Conclusion Both myocardial inflammation and disease-causing variants can be identified in a large proportion of RODCM cases. Prognostic value of CRP and virus detection is low. Non-titin disease-related variants carriers of are less likely to reach LVRR. In contrast, myocardial inflammation detected by EMB predicts favourable remodelling in 12 months. Figure 1 Funding Acknowledgement Type of funding source: None


2020 ◽  
Vol 93 (1115) ◽  
pp. 20200514
Author(s):  
Vineeta Ojha ◽  
Rishabh Khurana ◽  
Kartik P Ganga ◽  
Sanjeev Kumar

Takotsubo cardiomyopathy (TC) is a reversible condition in which there is transient left ventricular (LV) dysfunction characterised most commonly by basal hyperkinesis and mid-apical LV ballooning and hypokinesia. It is said to be triggered by stress and mimics, such as acute coronary syndrome (ACS) clinically. Diagnosis is usually suspected on echocardiography due to the characteristic contraction pattern in a patient with symptoms and signs of ACS but normal coronary arteries on catheter angiography. Cardiac magnetic resonance (CMR), with its latest advancements, is the diagnostic modality of choice for diagnosis, prognosis and follow-up of patients. The advances in CMR (including T1, T2, ECV mapping and threshold-based late gadolinium enhancement (LGE) measurements have revolutionised the role of CMR in tissue characterisation and prognostication in patients with TC. In this review, we highlight the current role of CMR in management of TC and enumerate the CMR findings in TC as well the current advances in the field of CMR, which could help in prognosticating these patients.


2021 ◽  
Vol 23 (Supplement_G) ◽  
Author(s):  
Rocco Antonio Montone ◽  
Riccardo Rinaldi ◽  
Filippo Gurgoglione ◽  
Marco Del Buono ◽  
Michele Russo ◽  
...  

Abstract Aims Coronary provocative test with acetylcholine (ACh) is of utmost importance and increasingly used in patients with myocardial ischaemia and non-obstructive coronary arteries. However, data on safety, predictors, and prognostic role of complications during intracoronary provocative testing are scarce. We aimed at assessing the safety of ACh provocative test in patients with myocardial ischaemia and non-obstructive coronary arteries. Moreover, we evaluated the predictors and the prognostic implications of complications occurring during the provocative test. Methods and results We prospectively enrolled consecutive patients undergoing intracoronary ACh provocative test for suspected myocardial ischaemia with angiographic evidence of non-obstructive coronary arteries. Complications during the ACh test were collected. Occurrence of major adverse cardiac events (MACE), arrhythmic events at 24-h ECG dynamic Holter monitoring and angina status were assessed at follow-up. We enrolled 310 patients [mean age 60.6 ± 11.9; 169 (54.5%) chronic coronary syndromes (CCS) and 141 (45.5%) with myocardial infarction and non-obstructive coronary arteries (MINOCA)]. The overall incidence of complications was low (9%) with a similar incidence in MINOCA and CCS [10 (7.1%) vs. 18 (10.7%), P = 0.276, respectively]. At multivariate logistic regression analysis, a previous history of paroxysmal atrial fibrillation [odds ratio (OR): 12.324, confidence interval (CI): 95% (4.641–32.722), P = 0.015] and moderate/severe diastolic dysfunction [OR: 3.827, 95% CI (1.296–11.304), P = 0.015] were independent predictors for occurrence of complications. The occurrence of complications was not associated with a worse clinical outcome at follow-up (median follow-up 22 months) in terms of both MACE, arrhythmic events and angina burden. Conclusions Intracoronary provocative testing with ACh test is safe in patients with myocardial ischaemia and non-obstructive coronary arteries (including MINOCA patients). History of paroxysmal atrial fibrillation and moderate/severe diastolic dysfunction predicted the occurrence of complications during ACh test. However, occurrence of complications did not portend a worse prognosis at follow-up in terms of MACE, arrhythmic events, and angina burden.


2021 ◽  
Vol 22 (Supplement_2) ◽  
Author(s):  
G Casas ◽  
J Limeres ◽  
R Barriales-Villa ◽  
P Garcia-Pavia ◽  
E Zorio ◽  
...  

Abstract Funding Acknowledgements Type of funding sources: None. Background Left ventricular noncompaction (LVNC) is a heterogeneous entity with a wide phenotypic expression. Risk factors have not been well established and prognostic stratification remains challenging. Purpose Describe prognostic role of CMR on long term outcomes of LVNC patients.  Methods   Retrospective multicentric longitudinal cohort study of consecutive patients fulfilling imaging diagnostic criteria for LVNC (Jenni echo criteria and Petersen and Jacquier CMR criteria). Demographic, ECG, genetic, family and treatment variables were recorded. Baseline CMR was used for the analysis. LV ejection fraction (LVEF) was categorized according to heart failure (HF) guidelines and late gadolinium enhancement (LGE) was visually assessed in a binary way. End points were HF, ventricular arrhythmias (VA), systemic embolisms (SE) and all-cause death. Major adverse cardiovascular events (MACE) were the combination of the four previous end points. In patients with initially preserved LVEF (≥ 50%), LV adverse remodelling (LVAR) was defined as an LVEF < 50% and/or absolute decrease of ≥10% in LVEF at last follow-up. Results 585 patients from 12 referral centres were included from 2000 to 2018. Age at diagnosis was 45 ± 20 years, 334 (57%) were male, baseline LVEF was 48 ± 17% and 18% presented LGE. During a median follow-up of 5.1 years (IQR 2.3-8.1), 110 (19%) patients presented HF, 87 (15%) VA, 18 (3%) SE and 34 (6%) died. MACE occurred in 223 (38%) patients. LVEF was independently associated with HF, VA, SE and MACE: HR were 1.08, 1.02, 1.04 and 1.02 respectively (all p < 0.05). LGE was more frequent in patients with reduced LVEF (39 Vs 53%, p < 0.001) and was associated with higher HF and VA risk in patients with an LVEF > 35% (HR 2.69 and 2.48 respectively, p < 0.05) (Figure 1). No MACE (0%) occurred during long-term follow-up in patients with preserved LVEF, no LGE as well as no ECG abnormalities and no family aggregation. 305 (52%) patients presented with initially preserved LVEF, and 230 (75%) of those had LVEF available at last follow-up. LVAR occurred in 50 (22%) patients: 22 (10%) had an LVEF < 50% and 41 (18%) an absolute ≥ 10% decrease in LVEF. LGE was independently associated with LVAR (HR 3.51, p = 0.045) (Figure 2).  Conclusions Cardiac magnetic resonance has an important prognostic role in LVNC. LVEF is the most powerful predictor of events. Myocardial fibrosis is associated with worse outcomes in patients without severe systolic dysfunction, as well as with left ventricular adverse remodelling in those with initially preserved LVEF. Besides, CMR may identify a low-risk subgroup of LVNC patients. Therefore, CMR should be used in risk stratification in LVNC.


2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
G Mattesi ◽  
E Baldi ◽  
F Guerra ◽  
M Toniolo ◽  
A Cipriani ◽  
...  

Abstract Introduction LV myocardial fibrosis detected as LGE on cardiac magnetic resonance (CMR) is a predictor of arrhythmic risk in primary prevention both in ischaemic and non-ischaemic cardiomyopathy. However, we still do not know the prognostic role of LV-LGE in patients who suffered cardiac arrest (CA). Purpose To evaluate the prognostic role of CMR, and in particular of LV-LGE suggesting myocardial scar, in predicting appropriate ICD interventions in secondary prevention patients. Methods Ninety-seven consecutive patients 1) aged ≥14 years 2) hospitalized for CA because of ventricular arrhythmias from 2009/01/01 3) who underwent/undergoing a CMR within one month from the event 4) who received/receiving an ICD for secondary prevention and 5) with at least 1 year-follow-up, were enrolled for this multicentric study. Results 97 patients (68 males, 70%), mean age 46±16 years, were enrolled. Seventy-six percent of patients received bystander cardiopulmonary resuscitation (CPR) and ventricular fibrillation (VF) was the first rhythm in 86% of cases. ST elevation was present in 18% of cases at the admission; however, angiography was found negative in 80% of patients. Myocardial oedema and LGE were identified in 26% and 64% of patients respectively. A diagnosis was made in 83.5% of cases, while in the remaining 16.5% CA was considered idiopathic. During a four-year-follow-up, 25% of patients had appropriate ICD therapy (10% of which ATP only). A significant correlation between LGE and appropriate ICD intervention was not found (p=0.89). Conclusions One fourth of patients who received ICD for secondary prevention had appropriate ICD therapy during a four-year-follow-up. In this setting, LV-LGE does not provide a prognostic value. Funding Acknowledgement Type of funding source: None


2019 ◽  
Vol 67 (3) ◽  
Author(s):  
Norbert Malli ◽  
Nastasia Wilfinger-Lutz ◽  
Walter Krugluger ◽  
Claudia Stöllberger ◽  
Maria Winkler-Dwora K ◽  
...  

2020 ◽  
Vol 16 (3) ◽  
pp. 241-246
Author(s):  
Dipesh Ludhwani ◽  
Belaal Sheikh ◽  
Vasu K Patel ◽  
Khushali Jhaveri ◽  
Mohammad Kizilbash ◽  
...  

Background: Takotsubo Cardiomyopathy (TTC) is an uncommon cause of acute reversible ventricular systolic dysfunction in the absence of obstructive Coronary Artery Disease (CAD). Typically manifesting as apical wall ballooning, TTC can rarely present atypically with apical wall sparing. Case report: A 62-year-old female presented with complaints of chest pain and features mimicking acute coronary syndrome. Coronary angiogram revealed no obstructive CAD and left ventriculogram showed reduced ejection fraction, normal left ventricular apex and hypokinetic mid-ventricles consistent with atypical TTC. The patient was discharged home on heart failure medications and a follow-up transthoracic echocardiogram demonstrated improved left ventricular function with no wall motion abnormality. Conclusion: This case report provides an insight into the diagnosis and management of TTC in the absence of pathognomic features.


2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
T Benito Gonzalez ◽  
X Freixa ◽  
C Godino ◽  
M Taramasso ◽  
R Estevez-Loureiro ◽  
...  

Abstract Background Limited information has been reported regarding the impact of percutaneous mitral valve repair (PMVR) on ventricular arrhythmic (VA) burden. The aim of this study was to address the incidence of VA and appropriate antitachycardia implantable cardiac defibrillator (ICD) therapies before and after PMVR. Methods We retrospectively analyzed all consecutive patients with heart failure with reduce left ventricular ejection fraction, functional mitral regurgitation grade 3+ or 4+ and an active ICD or cardiac resynchronizer who underwent PMVR in any of the eleven recruiting centers. Only patients with complete available device VA monitoring from one-year before to one year after PMVR were included. Baseline clinical and echocardiographic characteristics were collected before PMVR and at 12-months follow-up. Results 93 patients (68.2±10.9 years old, male 88.2%) were enrolled. PMVR was successfully performed in all patients and device success at discharge was 91.4%. At 12-months follow-up, we observed a significant reduction in mitral regurgitation severity, NT-proBNP and prevalence of severe pulmonary hypertension and severe kidney disease. Patients also referred a significant improvement in NYHA functional class and showed a non-significant trend to reserve left ventricular remodeling. After PMVR a significant decrease in the incidence of non-sustained ventricular tachycardia (VT) (5.0–17.8 vs 2.7–13.5, p=0.002), sustained VT or ventricular fibrillation (0.9–2.5 vs 0.5–2.9, p=0.012) and ICD antitachycardia therapies (2.5–12.0 vs 0.9–5.0, p=0.033) were observed. Conclusion PMVR was related to a reduction in arrhythmic burden and ICD therapies in our cohort. Proportion of patients who presented ven Funding Acknowledgement Type of funding source: None


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