Scarring/arrhythmogenic cardiomyopathy.
Domenico CorradoAlessandro ZorziAlberto CiprianiBarbara BauceRiccardo BarianiGiulia BrunettiFrancesca GrazianoManuel De LazzariGiulia MattesiFederico MiglioreKalliopi PilichouIlaria RigatoStefania RizzoGaetano ThieneMartina Perazzolo MarraCristina BassoPublished in: European heart journal supplements : journal of the European Society of Cardiology (2023)
The designation of 'arrhythmogenic cardiomyopathy' reflects the evolving concept of a heart muscle disease affecting not only the right ventricle (ARVC) but also the left ventricle (LV), with phenotypic variants characterized by a biventricular (BIV) or predominant LV involvement (ALVC). Herein, we use the term 'scarring/arrhythmogenic cardiomyopathy (S/ACM)' to emphasize that the disease phenotype is distinctively characterized by loss of ventricular myocardium due to myocyte death with subsequent fibrous or fibro-fatty scar tissue replacement. The myocardial scarring predisposes to potentially lethal ventricular arrhythmias and underlies the impairment of systolic ventricular function. S/ACM is an 'umbrella term' which includes a variety of conditions, either genetic or acquired (mostly post-inflammatory), sharing the typical 'scarring' phenotypic features of the disease. Differential diagnoses include 'non-scarring' heart diseases leading to either RV dilatation from left-to-right shunt or LV dilatation/dysfunction from a dilated cardiomyopathy. The development of 2020 upgraded criteria ('Padua criteria') for diagnosis of S/ACM reflected the evolving clinical experience with the expanding spectrum of S/ACM phenotypes and the advances in cardiac magnetic resonance (CMR) imaging. The Padua criteria aimed to improve the diagnosis of S/ACM by incorporation of CMR myocardial tissue characterization findings. Risk stratification of S/ACM patients is mostly based on arrhythmic burden and ventricular dysfunction severity, although other ECG or imaging parameters may have a role. Medical therapy is crucial for treatment of ventricular arrhythmias and heart failure. Implantable cardioverter defibrillator (ICD) is the only proven life-saving treatment, despite its significant morbidity because of device-related complications and inappropriate shocks. Selection of patients who can benefit the most from ICD therapy is one of the most challenging issues in clinical practice.
Keyphrases
- heart failure
- left ventricular
- cardiac resynchronization therapy
- magnetic resonance
- mitral valve
- acute heart failure
- pulmonary artery
- clinical practice
- end stage renal disease
- high resolution
- atrial fibrillation
- congenital heart disease
- ejection fraction
- prognostic factors
- chronic kidney disease
- catheter ablation
- skeletal muscle
- preterm infants
- mycobacterium tuberculosis
- magnetic resonance imaging
- newly diagnosed
- coronary artery
- healthcare
- blood pressure
- gene expression
- genome wide
- dna methylation
- combination therapy
- computed tomography
- meta analyses
- heart rate
- gestational age
- randomized controlled trial