scholarly journals Arrhythmogenic Right Ventricular Cardiomyopathy

2021 ◽  
Author(s):  
Sukanya Ghosh
Keyword(s):  
Right Ventricular ◽  
Task Force ◽  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
Disease Process ◽  
Ventricular Cardiomyopathy ◽  
Molecular Genetic Study ◽  
Icd Implantation ◽  
Fatty Replacement ◽  
Genes Encoding ◽  
Long Term Prognosis

Arrythmogenic right ventricular cardiomyopathy (ARVC) is a genetic form of cardiomyopathy causing fibro-fatty replacement of the myocardium. Although usually transmission is autosomal dominant, 12 genes encoding cardiac desmosomes have been found to be closely linked to this disease process shifting the congenital disease theory to a genetic one. The categorisation of ARVC as a myocyte adhesion disorder was first suggested by a molecular genetic study involving patients with Naxos disease. Misnomeric to only affect the right ventricle, ARVC also affects the left ventricle - culminating into biventricular failure as a long term prognosis. Epidemiology is well established with a male to female preponderance. It is currently the second most common cause of sudden cardiac death (SCD) in population < 35 yrs. Pathological basis of the varied clinical presentation is explained at the molecular level with myocardial atrophy, fibro-fatty replacement and chamber dilatation. Diagnosing the condition by ruling out the pitfall differentials is an enormous challenge due to the broad phenotypic spectrum including syncope on one end and SCD on the other. Task Force Criteria combines electrocardiography (ECG), echocardiography (ECHO), cardiac magnetic resonance imaging (CMRI), myocardial biopsy for diagnosis; early detection, family screening and risk stratification being the cornerstones. Therapeutic options although limited due to the progressive nature of the disease is based on preventing life threatening arrhythmias encompassing primary and secondary prevention - Implantable cardioverter -defibrillator (ICD) implantation, radiofrequency ablation and heart transplantation are the main ones.

scholarly journals Characteristics of Patients With Arrhythmogenic Left Ventricular Cardiomyopathy

2020 ◽  
Vol 13 (12) ◽  
Author(s):  
Michela Casella ◽  
Alessio Gasperetti ◽  
Rita Sicuso ◽  
Edoardo Conte ◽  
Valentina Catto ◽  
...  
Keyword(s):  
Late Gadolinium Enhancement ◽  
Right Ventricular ◽  
Genetic Variants ◽  
Task Force ◽  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
Left Ventricular ◽  
Ventricular Cardiomyopathy ◽  
Gadolinium Enhancement ◽  
Voltage Mapping ◽  
Fatty Replacement

Background: Arrhythmogenic left ventricular cardiomyopathy (ALVC) is an under-characterized phenotype of arrhythmogenic cardiomyopathy involving the LV ab initio. ALVC was not included in the 2010 International Task Force Criteria for arrhythmogenic right ventricular cardiomyopathy diagnosis and data regarding this phenotype are scarce. Methods: Clinical characteristics were reported from all consecutive patients diagnosed with ALVC, defined as a LV isolated late gadolinium enhancement and fibro-fatty replacement at cardiac magnetic resonance plus genetic variants associated with arrhythmogenic right ventricular cardiomyopathy and of an endomyocardial biopsy showing fibro-fatty replacement complying with the 2010 International Task Force Criteria in the LV. Results: Twenty-five patients ALVC (53 [48–59] years, 60% male) were enrolled. T wave inversion in infero-lateral and left precordial leads were the most common ECG abnormalities. Overall arrhythmic burden at study inclusion was 56%. Cardiac magnetic resonance showed LV late gadolinium enhancement in the LV lateral and posterior basal segments in all patients. In 72% of the patients an invasive evaluation was performed, in which electroanatomical voltage mapping and electroanatomical voltage mapping-guided endomyocardial biopsy showed low endocardial voltages and fibro-fatty replacement in areas of late gadolinium enhancement presence. Genetic variants in desmosomal genes (desmoplakin and desmoglein-2) were identified in 12/25 of the cohort presenting pathogenic/likely pathogenic variants. A definite/borderline 2010 International Task Force Criteria arrhythmogenic right ventricular cardiomyopathy diagnosis was reached only in 11/25 patients. Conclusions: ALVC presents with a preferential involvement of the lateral and postero-lateral basal LV and is associated mostly with variants in desmoplakin and desmoglein-2 genes. An amendment to the current International Task Force Criteria is reasonable to better diagnose patients with ALVC.

scholarly journals Role of ventricular tachycardia ablation in arrhythmogenic right ventricular cardiomyopathy

2017 ◽  
Vol 7 (1) ◽  
Author(s):  
Alberto Cipriani ◽  
Riccardo Bariani ◽  
Manuel De Lazzari ◽  
Federico Migliore ◽  
Carlo Angheben ◽  
...  
Keyword(s):  
Ventricular Tachycardia ◽  
Catheter Ablation ◽  
Right Ventricular ◽  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
Ventricular Cardiomyopathy ◽  
Ventricular Tachycardia Ablation ◽  
Fatty Replacement ◽  
Technical Issues ◽  
Long Term Prognosis

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is characterized by progressive fibro-fatty replacement of the myocardium that represents the substrate for recurrent sustained ventricular tachycardia (VT). These arrhythmias characterize the clinical course of a sizeable proportion of patients and have significant implications for their quality of life and long-term prognosis. Antiarrhythmic drugs are often poorly tolerated and usually provide incomplete control of arrhythmia relapses. Catheter ablation is a potentially effective strategy to treat frequent VT episodes and ICD shocks in ARVC patients. The aims of this review are to discuss the electrophysiological and electroanatomic substrates of ventricular tachycardia in patients with ARVC and to analyze the role of catheter ablation in their management with particular reference to selection of patients, technical issues, potential complications and outcomes.

scholarly journals Outcomes and management of arrhythmogenic right ventricular cardiomyopathy in pregnancy: a case report

2019 ◽  
Vol 3 (4) ◽  
pp. 1-5 ◽  
Author(s):  
Marco Schiavone ◽  
Margherita Calcagnino ◽  
Andrea Mazzanti ◽  
Carla Bonanomi
Keyword(s):  
Right Ventricular ◽  
Task Force ◽  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
Elective Caesarean Section ◽  
Favourable Outcome ◽  
Inherited Disease ◽  
Ventricular Cardiomyopathy ◽  
Icd Implantation ◽  
Asymptomatic Patients ◽  
Gestational Week

Abstract Background Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited disease with an estimated prevalence of up to 1:5000 in the general population. Few cases of ARVC during pregnancy are described in literature. Case summary A 32-year-old primigravida was referred to our clinic during the 32nd gestational week. Arrhythmogenic right ventricular cardiomyopathy diagnosis with biventricular involvement was made according to Task Force criteria. Beta-blocker therapy was started and an elective caesarean section was planned, during the 37th gestational week; no complications occurred. Thirteen months after delivery, the patient was readmitted in our hospital due to an episode of pre-syncope and after team discussion, an implantable cardioverter-defibrillator (ICD) was implanted. Discussion This case suggests that the absence of signs and symptoms of heart failure (HF) at a first evaluation plays a major role to predict maternal and foetal outcome in ARVC. Our experience is consistent with the evidence that indicates a favourable outcome in asymptomatic patients treated with optimal medical therapy during pregnancy. In our case, despite no major HF or arrhythmic complications during pregnancy, delivery, and puerperium, we observed an arrhythmic disease progression more likely independent from pregnancy, leading to ICD implantation.

Abstract 1966: Comparison of Right Ventricular Strain and Strain Rate in Patients with Arrhythmogenic Right Ventricular Cardiomyopathy, Right Ventricular Outflow Tract Tachycardia, and Matched Controls

Circulation ◽  
2007 ◽  
Vol 116 (suppl_16) ◽  
Author(s):  
Omid Salehian ◽  
Andrew P Klug ◽  
Jeffrey Healey ◽  
Ian G Burwash
Keyword(s):  
Right Ventricular ◽  
Strain Rate ◽  
Task Force ◽  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
Myocardial Strain ◽  
Strain Rates ◽  
Systolic Strain ◽  
Ventricular Cardiomyopathy ◽  
Strain And Strain Rate ◽  
Fatty Replacement

Background: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is characterized by fibro-fatty replacement of RV myocardium leading to ventricular tachycardia and sudden death in otherwise healthy individuals. Diagnosis is challenging as there are no diagnostic tests with adequate accuracy. Current diagnosis of ARVC is made by the task force criteria. One of the main diagnostic dilemmas continues to be distinguishing ARVC from a relatively benign entity of right ventricular out flow tract tachycardia (RVOT VT). Assessment of myocardial strain and strain rate has been used to study patients with ischemic heart disease and has shown utility in identifying subtle myocardial dysfunction not detected by routine wall motion assessment. The goal of this study was to compare myocardial strain and strain rates in patients with ARVC, RVOT VT and matched controls. Methods: We prospectively enrolled 12 patients with ARVC (based on task force criteria), 10 with RVOT VT, and 22 age and gender matched controls. Echo studies (including tissue Doppler derived myocardial strain echocardiography) were performed with commercially available systems (GE, Vivid 7). Peak systolic RV myocardial strain and strain rate were measured at base, mid, and apical RV free wall and compared. Results: ARVC patients had significantly lower peak systolic RV strain compared to both RVOT VT patients and matched controls (Table ). There was also significantly lower peak systolic strain rate at the apical RV myocardium in patients with ARVC compared to both those with RVOT VT (p=0.02) and controls (p=0.01). Conclusions: Patients with ARVC have abnormally low RV systolic strain and strain rates when compared to matched controls as well as those with RVOT VT. This modality can be routinely used in assessing patients suspected of ARVC as it might assist in earlier diagnosis as well as help in distinguishing patients with ARVC from those with RVOT VT. P<0.05 compared to controls, † P<0.05 compared to RVOT VT group

Evidence-based management of arrhythmogenic right ventricular cardiomyopathy in pregnancy

2020 ◽  
Author(s):  
Jagjit Khosla ◽  
Reshma Golamari ◽  
Alice Cai ◽  
Jamal Benson ◽  
Wilbert S Aronow ◽  
...  
Keyword(s):  
Heart Failure ◽  
Right Ventricular ◽  
Retrospective Studies ◽  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
Genetic Disorder ◽  
Ventricular Myocardium ◽  
Evidence Based ◽  
Ventricular Cardiomyopathy ◽  
Genes Encoding ◽  
In Pregnancy

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a genetic disorder resulting in fibrofatty replacement of the myocardium. Genetic mutations in genes encoding for desmosome proteins result in a ventricular myocardium prone to arrhythmias and heart failure. Although ARVC is known for a few decades, most of the outcomes in pregnancy are reported recently. Pregnancy leads to significant physiological changes with excess mechanical stress on the myocardium. All the retrospective studies suggest that pregnancy is well tolerated in these patients despite the high risk of arrhythmias and heart failure. Our review focuses on the most up-to-date evidence on the management of ARVC patients during the antepartum and postpartum period.

Abstract 15314: Left Ventricular Dysfunction in Children and Adolescents With Arrhythmogenic Right Ventricular Cardiomyopathy

Circulation ◽  
2015 ◽  
Vol 132 (suppl_3) ◽  
Author(s):  
Paweena Chungsomprasong ◽  
Robert Hamilton ◽  
Wietske Luining ◽  
Shi-Joon Yoo ◽  
Meena Fatah ◽  
...  
Keyword(s):  
Right Ventricular ◽  
Task Force ◽  
Myocardial Dysfunction ◽  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
Myocardial Strain ◽  
Young Patients ◽  
Left Ventricular ◽  
Lv Function ◽  
Ventricular Cardiomyopathy ◽  
End Diastolic Volume

Background: Involvement of the left ventricle (LV) is increasingly recognized in adults with arrhythmogenic right ventricular cardiomyopathy (ARVC) but it is unclear whether LV function is compromised in children with this condition. The aim of this study was examine myocardial contractility in pediatric patients with suspected ARVC. Methods: For this retrospective study, patients with a work-up for ARVC were classified into ‘no’, ‘possible’, ‘borderline’ or ‘definite’ ARVC according to the revised Task Force Criteria (rTFC). Ventricular size and function as well as LV myocardial strain and torsion were measured by cardiac magnetic resonance (CMR). Results: A total of 142 patients were enrolled, of whom 58 (41%) had no, 32 (23%) possible, 29 (20%) borderline and 23 (16%) definite ARVC. The groups were similar in age at CMR. With higher rTFC score, z scores (Z) of right ventricular (RV) ejection fraction (EF) were lower (p<0.001) while z-RV end diastolic volume (EDV) and z-LV EDV were larger (p=0.002 and 0.013, respectively). LV EF did not differ between rTFC categories. Global circumferential strain (GCS) of the LV was lower in patients in higher rTFC categories (p=0.018). Z-LVEDV correlated with z-RVEDV (r2 = 0.69, p<0.001) and z- LVEF correlated with z-RVEF (r2 = 0.55, p <0.001). Z-LVEF and z-RVEF correlated with LV GCS (r2 = 0.48, p<0.001 and r2 = 0.46, p<0.001, respectively) and torsion (r2 = 0.21, p=0.032 for both). Forty-two patients had a follow-up CMR, after a median interval of 2.6 years (0.4- 8.4). The rate of deterioration of LV or RV EF or EDV did not differ between rTFC categories. A more rapid increase of z-RVEDV was associated with a faster decline in z-RVEF (r2 = -0.383, p=0.004) and z-LVEF (r2 = -0.45, p=0.001). A decline of z-LVEF over time correlated with that of z-RVEF (r2 = 0.60, p<0.001) and z-LVEDV increase correlated with z-RVEDV increase (r2 = 0.84, p<0.001). Conclusion: LV myocardial dysfunction is present in young patients with suspected or confirmed ARVC. Quantification of myocardial mechanics with CMR may be a useful tool to detect early LV involvement in ARVC. Progressive LV dysfunction and enlargement appear to parallel those of the RV.

P992Incidence, predictors, and success of ventricular tachycardia catheter ablation in arrhythmogenic right ventricular cardiomyopathy (ARVC): A long-term cohort study from the Nordic ARVC registry

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
M K Christiansen ◽  
K Haugaa ◽  
A Svensson ◽  
T Gilljam ◽  
T Madsen ◽  
...  
Keyword(s):  
Ventricular Tachycardia ◽  
Catheter Ablation ◽  
Right Ventricular ◽  
Ventricular Arrhythmias ◽  
Task Force ◽  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
Young Age ◽  
Clinical Indication ◽  
Ventricular Cardiomyopathy ◽  
Vt Ablation

Abstract Background Catheter ablation may reduce ventricular tachycardia (VT) burden in arrhythmogenic right ventricular cardiomyopathy (ARVC) patients. However, little is known about factors predicting need for ablation and various outcomes have been reported. Purpose We sought to investigate predictors and use of VT ablation and to evaluate the post-procedural outcome in ARVC patients. Methods We studied 435 patients from the Nordic ARVC registry including 220 probands with definite ARVC according to the 2010 task force criteria and 215 mutation-carrying relatives identified through cascade screening. Patients were followed until first-time VT ablation, death, heart transplantation, or January 1st 2018. Additionally, patients undergoing VT ablation were further followed from the time of ablation for recurrent ventricular arrhythmias. Results Cumulative use of VT ablation was 4% (95% CI 3%-6%) and 11% (95% CI 8%-15%) after 1 and 10 years. All procedures were performed in probands in whom the cumulative use was 8% (95% CI 5%-12%) and 20% (95% CI 15%-26%). In adjusted analyses restricted to probands, only young age predicted need for ablation. In patients undergoing ablation, risk of recurrent arrhythmias was 59% (95% CI 44%-71%) and 74% (95% CI 59%-84%) 1 and 5 years after the procedure. Despite high recurrence rates, the burden of ventricular arrhythmias was reduced after ablation (p=0.0042). Young age, use of several antiarrhythmic drugs and inducibility to VT immediately after ablation were associated with an unfavorable outcome. Conclusions Twenty percent of ARVC probands developed a clinical indication for VT ablation within 10 years after diagnosis whereas mutation-carrying relatives were without such need. Although the burden of ventricular arrhythmias decreased after ablation, risk of recurrence was substantial.

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