Longitudinal Arrhythmic Risk Assessment Based on Ejection Fraction in Patients with Recent-Onset Nonischemic Dilated Cardiomyopathy

Angelis, Giulia De; Merlo, Marco; Barbati, Giulia; Bertolo, Silvia; Luca, Antonio De; Ramani, Federica; Adamo, Luigi; Sinagra, Gianfranco

doi:10.1016/j.echo.2022.03.019

Cited by 3 publications

(3 citation statements)

References 22 publications

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“…Patients with IGCM should be initiated on standard GDMT, as indicated for heart failure, and up-titrated as tolerated. Patients presenting with ventricular tachyarrhythmias and those with an ejection fraction <35% despite 3 to 6 months of GDMT, should be considered for ICD implantation, as was the case of our patient [ 22 ]. Immunosuppressive therapy plays an integral role in the management of patients with IGCM.…”

Section: Discussionmentioning

confidence: 99%

“…Activated leukocytes, especially macrophages, are known to express high levels of glucose transporters, which result in rapid accumulation of 18F-FDG at the site of inflammation [ 21 ]. Additionally, FDG-PET can be helpful in identifying lymph nodes as a target site for biopsy to rule out cardiac sarcoidosis, with which there can be substantial clinical overlap [ 22 ].…”

Section: Discussionmentioning

confidence: 99%

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Unraveling the Uncommon: A Case Report of Giant Cell Myocarditis and Examination of Existing Literature

Gonzalez Moret,

Jarrett,

Ahktar

et al. 2024

Am J Case Rep

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Patient: Female, 65-year-old Final Diagnosis: Giant cell myocarditis Symptoms: Palpitation • shortness of breath Clinical Procedure: — Specialty: Cardiology Objective: Unusual clinical course Background: Idiopathic giant cell myocarditis (IGCM) is an uncommon and frequently fatal type of myocarditis. It primarily affects young individuals and has the potential to result in heart failure and life-threatening arrhythmias. IGCM seems to be dependent on activation of CD4-positive T lymphocytes and can show improvement with treatment aimed at reducing T-cell function. We present a case of a 65-year-old patient who presented with features of acute heart failure refractory to guideline-directed medical therapy (GDMT), due to IGCM. A review of the natural history and treatment of IGCM is also presented. Case Report: A 65-year-old woman with multiple comorbidities was admitted to our hospital for ventricular tachycardia in the setting of progressive non-ischemic heart failure, unresponsive to GDMT. This led to further investigation, including an endomyocardial biopsy, which revealed inflammatory infiltration, with multinucleated giant cells and lymphocytes in the absence of granuloma formation, prompting a diagnosis of IGCM. An implantable cardioverter-defibrillator (ICD) was placed for secondary prevention of sudden cardiac death and the patient was initiated on combined immunosuppressive therapy. Owing to numerous comorbidities, she was determined to be unsuitable for a heart transplant. Unfortunately, she eventually died from complications secondary to the disease. Conclusions: IGCM remains a challenging clinical diagnosis with a poor long-term outcome without heart transplantation. This case highlights the importance of considering atypical causes of heart failure in patients who do not respond to conventional therapies. Early recognition and appropriate management, involving medical and interventional approaches, are crucial in improving outcomes for patients with IGCM.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Unraveling the Uncommon: A Case Report of Giant Cell Myocarditis and Examination of Existing Literature

Gonzalez Moret,

Jarrett,

Ahktar

et al. 2024

Am J Case Rep

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“…The search for feasible and reliable tools able to identify AR‐DCM patients at high arrhythmic risk and with a higher probability of carrying a malignant arrhythmogenic genotype represents an unmet clinical need. Notably, in DCM, left ventricular ejection fraction (LVEF) and New York Heart Association (NYHA) functional class are insufficient to characterize overall arrhythmogenic risk 8–10 . To address this knowledge gap, we investigated the role of specific early and easily accessible clinical and Holter electrocardiogram (ECG) arrhythmic markers in predicting adverse arrhythmic outcomes and identifying high‐risk pathogenic/likely pathogenic (P/LP) variants among a large, well‐clinically and genetically characterized DCM population.…”

Section: Introductionmentioning

confidence: 99%

Role of arrhythmic phenotype in prognostic stratification and management of dilated cardiomyopathy

Setti,

Merlo,

Gigli

et al. 2024

European J of Heart Fail

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AimsDilated cardiomyopathy (DCM) with arrhythmic phenotype combines phenotypical aspects of DCM and predisposition to ventricular arrhythmias, typical of arrhythmogenic cardiomyopathy. The definition of DCM with arrhythmic phenotype is not universally accepted, leading to uncertainty in the identification of high‐risk patients. This study aimed to assess the prognostic impact of arrhythmic phenotype in risk stratification and the correlation of arrhythmic markers with high‐risk arrhythmogenic gene variants in DCM patients.Methods and resultsIn this multicentre study, DCM patients with available genetic testing were analysed. The following arrhythmic markers, present at baseline or within 1 year of enrolment, were tested: unexplained syncope, rapid non‐sustained ventricular tachycardia (NSVT), ≥1000 premature ventricular contractions/24 h or ≥50 ventricular couplets/24 h. LMNA, FLNC, RBM20, and desmosomal pathogenic or likely pathogenic gene variants were considered high‐risk arrhythmogenic genes. The study endpoint was a composite of sudden cardiac death and major ventricular arrhythmias (SCD/MVA). We studied 742 DCM patients (45 ± 14 years, 34% female, 410 [55%] with left ventricular ejection fraction [LVEF] <35%). During a median follow‐up of 6 years (interquartile range 1.6–12.1), unexplained syncope and NSVT were the only arrhythmic markers associated with SCD/MVA, and the combination of the two markers carried a significant additive risk of SCD/MVA, incremental to LVEF and New York Heart Association class. The probability of identifying an arrhythmogenic genotype rose from 8% to 30% if both early syncope and NSVT were present.ConclusionIn DCM patients, the combination of early detected NSVT and unexplained syncope increases the risk of life‐threatening arrhythmic outcomes and can aid the identification of carriers of malignant arrhythmogenic genotypes.

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Arrhythmic risk stratification in non-ischaemic dilated cardiomyopathy

Mestre¹,

Gava²,

Paldino³

et al. 2023

European Heart Journal Supplements

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Dilated cardiomyopathy is a primary disease of the heart muscle, which affects relatively young patients with a low comorbidity profile. It is characterized by structural and/or functional abnormalities leading to systolic dysfunction of the left ventricle or of both ventricles, often associated with dilatation, in the absence of an ischaemic, valvular, or pressure overload cause sufficient to explain the phenotype. Although the prognosis of the disease has greatly improved over the last few decades, prognostic stratification remains a fundamental objective, especially about the prediction of potentially life-threatening arrhythmic events. An accurate diagnostic work-up and an appropriate aetiopathogenetic characterization affect the patients’ outcome and represent the essential basis of an adequate prognostic stratification. It is necessary to adopt a multiparametric approach, especially when the aim is the prediction of arrhythmic risk; it includes an integration of medical history and physical examination with cardiac imaging and genetic testing, in order to obtain a personalized diagnosis and therapeutic strategies. Furthermore, the evaluation should be repeated at every clinical check-up, considering the dynamic trend of the pathology and the arrhythmic risk changes over time. This article aims to illustrate how, starting from an exhaustive aetiological and clinical–instrumental characterization, including all diagnostic methods available at present time, it is possible to obtain a tailored diagnostic evaluation and stratification of the arrhythmic risk as accurate as possible.

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Longitudinal Arrhythmic Risk Assessment Based on Ejection Fraction in Patients with Recent-Onset Nonischemic Dilated Cardiomyopathy

Cited by 3 publications

References 22 publications

Unraveling the Uncommon: A Case Report of Giant Cell Myocarditis and Examination of Existing Literature

Unraveling the Uncommon: A Case Report of Giant Cell Myocarditis and Examination of Existing Literature

Role of arrhythmic phenotype in prognostic stratification and management of dilated cardiomyopathy

Arrhythmic risk stratification in non-ischaemic dilated cardiomyopathy

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