A desmoplakin variant associated with isolated arrhythmogenic left ventricular cardiomyopathy with rapid monomorphic ventricular tachycardia at first presentation

Guan, Fu; Wolber, Thomas; Saguner, Ardan M.; Medeiros, Argelia; Müggler, Oliver; Berger, Felix; Gass, Matthias; Molitor, Nadine; Ruschitzka, Frank; Brunckhorst, Corinna; Duru, Fırat

doi:10.1016/j.hrcr.2023.03.017

Cited by 1 publication

(1 citation statement)

References 14 publications

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“…Chest pain is often accompanied by transient ischemic ST-segment changes on 12-lead ECG on a background of abnormal QRS voltages with fractionation and negative T waves in the right ventricular or lateral leads, although up to a third of patients have a normal ECG at presentation. Hemodynamic instability at presentation is rare, but episodes of sustained ventricular arrhythmias and SCD at presentation have been described [ 104 ]. NSVT and increased burden PVCs can be commonly seen on in-hospital telemetry monitoring.…”

Section: Arrhythmogenic Right Ventricular Cardiomyopathy With “Hot Ph...mentioning

confidence: 99%

Myocarditis and Chronic Inflammatory Cardiomyopathy, from Acute Inflammation to Chronic Inflammatory Damage: An Update on Pathophysiology and Diagnosis

Uccello,

Bonacchi,

Rossi

et al. 2023

JCM

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Acute myocarditis covers a wide spectrum of clinical presentations, from uncomplicated myocarditis to severe forms complicated by hemodynamic instability and ventricular arrhythmias; however, all these forms are characterized by acute myocardial inflammation. The term “chronic inflammatory cardiomyopathy” describes a persistent/chronic inflammatory condition with a clinical phenotype of dilated and/or hypokinetic cardiomyopathy associated with symptoms of heart failure and increased risk for arrhythmias. A continuum can be identified between these two conditions. The importance of early diagnosis has grown markedly in the contemporary era with various diagnostic tools available. While cardiac magnetic resonance (CMR) is valid for diagnosis and follow-up, endomyocardial biopsy (EMB) should be considered as a first-line diagnostic modality in all unexplained acute cardiomyopathies complicated by hemodynamic instability and ventricular arrhythmias, considering the local expertise. Genetic counseling should be recommended in those cases where a genotype–phenotype association is suspected, as this has significant implications for patients’ and their family members’ prognoses. Recognition of the pathophysiological pathway and clinical “red flags” and an early diagnosis may help us understand mechanisms of progression, tailor long-term preventive and therapeutic strategies for this complex disease, and ultimately improve clinical outcomes.

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Section: Arrhythmogenic Right Ventricular Cardiomyopathy With “Hot Ph...mentioning

confidence: 99%

Myocarditis and Chronic Inflammatory Cardiomyopathy, from Acute Inflammation to Chronic Inflammatory Damage: An Update on Pathophysiology and Diagnosis

Uccello,

Bonacchi,

Rossi

et al. 2023

JCM

View full text Add to dashboard Cite

show abstract

A desmoplakin variant associated with isolated arrhythmogenic left ventricular cardiomyopathy with rapid monomorphic ventricular tachycardia at first presentation

Cited by 1 publication

References 14 publications

Myocarditis and Chronic Inflammatory Cardiomyopathy, from Acute Inflammation to Chronic Inflammatory Damage: An Update on Pathophysiology and Diagnosis

Myocarditis and Chronic Inflammatory Cardiomyopathy, from Acute Inflammation to Chronic Inflammatory Damage: An Update on Pathophysiology and Diagnosis

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