Arrhythmogenic Right Ventricular Research Articles

Cardiac MRI findings to differentiate athlete's heart from hypertrophic (HCM), arrhythmogenic right ventricular (ARVC) and dilated (DCM) cardiomyopathy

To provide clinically relevant criteria for differentiation between the athlete’s heart and similar appearing hypertrophic (HCM), dilated (DCM), and arrhythmogenic right-ventricular cardiomyopathy (ARVC) in MRI. 40 top-level athletes were prospectively examined with cardiac MR (CMR) in two university centres and compared to retrospectively recruited patients diagnosed with HCM (n = 14), ARVC (n = 18), and DCM (n = 48). Analysed MR imaging parameters in the whole study cohort included morphology, functional parameters and late gadolinium enhancement (LGE). Mean left-ventricular enddiastolic volume index (LVEDVI) was high in athletes (105 ml/m2) but significantly lower compared to DCM (132 ml/m2; p = 0.001). Mean LV ejection fraction (EF) was 61% in athletes, below normal in 7 (18%) athletes vs. EF 29% in DCM, below normal in 46 (96%) patients (p < 0.0001). Mean RV-EF was 54% in athletes vs. 60% in HCM, 46% in ARVC, and 41% in DCM (p < 0.0001). Mean interventricular myocardial thickness was 10 mm in athletes vs. 12 mm in HCM (p = 0.0005), 9 mm in ARVC, and 9 mm in DCM. LGE was present in 1 (5%) athlete, 8 (57%) HCM, 10 (56%) ARVC, and 21 (44%) DCM patients (p < 0.0001). Healthy athletes’ hearts are characterized by both hypertrophy and dilation, low EF of both ventricles at rest, and increased interventricular septal thickness with a low prevalence of LGE. Differentiation of athlete’s heart from other non-ischemic cardiomyopathies in MRI can be challenging due to a significant overlap of characteristics also seen in HCM, ARVC, and DCM.

Real-life clinical practice of genetic counselling and testing in adult patients with cardiomyopathies: Insight from the ESC EORP Cardiomyopathy Registry

Cardiomyopathies comprise a heterogeneous group of diseases, often of genetic origin. Genetic counselling and testing are recommended but the practices in real-life have not been evaluated. We assessed the current practice of genetic counselling and testing in the prospective ESC EORP cardiomyopathy registry. 3208 adult patients from sixty-nine centres in 18 countries were enrolled. Genetic counselling was performed in 60.8% of all patients (75.4% in hypertrophic (HCM), 39.2% in dilated (DCM), 70.8% in arrhythmogenic right ventricular (ARVC) and 49.2% in restrictive cardiomyopathy (RCM), P < 0.001). Comparing European geographical areas, genetic counselling was performed from 42.4% to 83.3% ( P < 0.001). It was provided by a cardiologist (85.3%), geneticist (15.1%), genetic counsellor (11.3%), or a nurse (7.5%), ( P < 0.001). Genetic testing was performed in 37.3% of all patients (48.8% in HCM, 18.6% in DCM, 55.6% % in ARVC and 43.6% in RCM, P < 0.001). Index patients with genetic testing were younger at diagnosis, had more familial disease, family history of sudden cardiac death or implanted cardioverter defibrillators but less comorbidities than those not tested ( P < 0.001 for each comparison). At least 1 disease-causing variant was found in 41.7% of index patients with genetic testing (43.3% in HCM, 33.3% in DCM, 51.4% in ARVC and 42.9% in RCM, P = 0.13). This is the first detailed report on the real-life practice of genetic counselling and testing in cardiomyopathies in Europe. Genetic counselling and testing were performed in a substantial proportion of patients but less often than recommended by European guidelines, and much less in DCM than in HCM and ARVC, despite evidence for genetic background.

Prospective follow-up in various subtypes of cardiomyopathies: Insights from the EORP Cardiomyopathy Registry of the ESC

The EORP Cardiomyopathy Registry is a prospective, observational, multinational registry of consecutive patients with cardiomyopathies. The objective of this report is to describe the outcomes at one year of follow-up of adult patients (> 18 years old) enrolled in the registry. A total of 3208 patients [median age: 55.0 (43.0;64.0) years, males: 65.1%] were recruited at baseline. Follow-up data at 1 year were obtained in 2,713 patients (84.6%), including 1420 with hypertrophic (HCM), 1,105 dilated (DCM), 128 arrhythmogenic right ventricular (ARVC) and 60 restrictive cardiomyopathy (RCM) ( Fig. 1 ). Improvement of symptoms (NYHA, chest pain, syncope) was globally observed over time ( P < 0.001 for each). Additional invasive therapeutics were performed during follow-up: implantation of ICD (primary prevention) ( N = 109 patients, 5.2%), pacemaker ( N = 28, 1.2%), heart transplant ( N = 30, 1,1%), ablation for atrial or ventricular arrhythmia (0.5% & 0.1%). The proportion of patients with history of AF increased from baseline to FU in 3.6% (from 28.2% to 31.8%). ICD therapy at 1 year was delivered more frequently in ARVC then in DCM, HCM and RCM (11.4%, 9.0%; 8.1%, 0% respectively for primary prevention). Major cardiovascular events (MACE) occurred in 29.3% of RCM, 10.5% of DCM, 7.9% of ARVC and 5.3% of HCM. MACE were globally higher in index patients compared to relatives (10.8% vs. 4.4%, P < 0.001). When considering geographical areas, MACE were higher in East Europe (13.1%) and lower in South Europe (5.3%) (univariate); heart transplant was higher in West Europe (2.40%) and lower in South Europe (0.25%) (univariate). Despite symptomatic improvement in most cases, there is still a significant burden of arrhythmic and heart failure events in patients with cardiomyopathies. Outcomes were different not only according to cardiomyopathy subtypes but also in relatives versus index patients.

Genetic counselling and testing of adult patients with cardiomyopathies: insight from the EORP cardiomyopathy and myocarditis registry of the European Society of Cardiology

Abstract Introduction Cardiomyopathies comprise a heterogeneous group of diseases, often of genetic origin. Purpose We assessed the current practice of genetic counselling and testing of adult cardiomyopathy patients in the prospective ESC EORP cardiomyopathy registry. Methods 3 208 adult patients from sixty-nine centres in 18 countries were enrolled. Clinical data on genetic counselling and testing and on the presentation of cardiomyopathies were gathered. Results Genetic counselling was performed in 60.8% of all patients (75.4% in hypertrophic (HCM), 39.2% in dilated (DCM), 70.8% in arrhythmogenic right ventricular (ARVC) and 49.2% in restrictive cardiomyopathy (RCM), p&amp;lt;0.001). Comparing European geographical areas, genetic counselling was performed from 42.4% to 83.3% (p&amp;lt;0.001). It was provided by a cardiologist (85.3%), geneticist (15.1%), genetic counsellor (11.3%), or a nurse (7.5%), (p&amp;lt;0.001). Genetic testing was performed in 37.3% of all patients (48.8% in HCM, 18.6% in DCM, 55.6% % in ARVC and 43.6% in RCM, p&amp;lt;0.001). Index patients with genetic testing were younger at diagnosis, had more familial disease, family history of sudden cardiac death or implanted cardioverter defibrillators but less comorbidities than those not tested (p&amp;lt;0.001 for each comparison). At least 1 disease causing variant was found in 41.7% of index patients with genetic testing (43.3% in HCM, 33.3% in DCM, 51.4% in ARVC and 42.9% in RCM, p=0.13). Conclusion We report on the practice of genetic counselling and testing in cardiomyopathies in Europe. Genetic counselling and testing were performed in a substantial proportion of patients but less often than recommended by European guidelines, and much less in DCM than in HCM and ARVC, despite evidence for genetic background. Funding Acknowledgement Type of funding source: None

Prospective follow-up in various subtypes of cardiomyopathies: insights from the ESC EORP Cardiomyopathy Registry.

The European Society of Cardiology (ESC) European Observational Research Programme (EORP) Cardiomyopathy Registry is a prospective multinational registry of consecutive patients with cardiomyopathies. The objective of this report is to describe the short-term outcomes of adult patients (≥18 years old). Out of 3208 patients recruited, follow-up data at 1 year were obtained in 2713 patients (84.6%) [1420 with hypertrophic (HCM); 1105 dilated (DCM); 128 arrhythmogenic right ventricular (ARVC); and 60 restrictive (RCM) cardiomyopathies]. Improvement of symptoms (dyspnoea, chest pain, and palpitations) was globally observed over time (P < 0.05 for each). Additional invasive procedures were performed: prophylactic implantation of implantable cardioverter-defibrillator (ICD) (5.2%), pacemaker (1.2%), heart transplant (1.1%), ablation for atrial or ventricular arrhythmia (0.5% and 0.1%). Patients with atrial fibrillation increased from 28.7% to 32.2% of the cohort. Ventricular arrhythmias (VF/ventricular tachycardias) in ICD carriers (primary prevention) at 1 year were more frequent in ARVC, then in DCM, HCM, and RCM (10.3%, 8.2%, 7.5%, and 0%, respectively). Major cardiovascular events (MACE) occurred in 29.3% of RCM, 10.5% of DCM, 5.3% of HCM, and 3.9% of ARVC (P < 0.001). MACE were more frequent in index patients compared to relatives (10.8% vs. 4.4%, P < 0.001), more frequent in East Europe centres (13.1%) and least common in South Europe (5.3%) (P < 0.001). Subtype of cardiomyopathy, geographical region, and proband were predictors of MACE on multivariable analysis. Despite symptomatic improvement, patients with cardiomyopathies remain prone to major clinical events in the short term. Outcomes were different not only according to cardiomyopathy subtypes but also in relatives vs. index patients, and according to European regions.

P334Prospective FU in various subtypes of cardiomyopathies: insights from the EORP Cardiomyopathy Registry of the ESC

Abstract Background The EORP Cardiomyopathy Registry is a prospective, observational, multinational registry of consecutive patients with cardiomyopathies. The objective of this report is to describe the outcomes at one year of follow-up of adult patients (&gt;18 years old) enrolled in the registry. Methods A total of 3,208 patients (median age: 55.0 (43.0; 64.0) years, males: 65.1%) were recruited at baseline. Follow-up data at 1 year were obtained in 2,713 patients (84.6%), including 1,420 with hypertrophic (HCM), 1,105 dilated (DCM), 128 arrhythmogenic right ventricular (ARVC) and 60 restrictive cardiomyopathy (RCM). Results Improvement of symptoms (NYHA, chest pain, syncope) was globally observed over time (p&lt;0.001 for each). Additional invasive therapeutics were performed during follow-up: implantation of ICD (primary prevention) (N=109 patients, 5.2%), pacemaker (N=28, 1.2%), heart transplant (N=30, 1,1%), ablation for atrial or ventricular arrhythmia (0.5% &amp; 0.1%). The proportion of patients with history of AF increased from baseline to FU in 3.6% (from 28.2% to 31.8%). ICD therapy at 1 year was delivered more frequently in ARVC then in DCM, HCM and RCM (11.4%, 9.0%; 8.1%, 0% respectively for primary prevention). Major cardiovascular events (MACE) occurred in 29.3% of RCM, 10.5% of DCM, 7.9% of ARVC and 5.3% of HCM. MACE were globally higher in index patients compared to relatives (10.8% vs 4.4%, p&lt;0.001). When considering geographical areas, MACE were higher in East Europe (13.1%) and lower in South Europe (5.3%) (univariate); heart transplant was higher in West Europe (2.40%) and lower in South Europe (0.25%) (univariate). Conclusions Despite symptomatic improvement in most cases, there is still a significant burden of arrhythmic and heart failure events in patients with cardiomyopathies. Outcomes were different not only according to cardiomyopathy subtypes but also in relatives versus index patients. Acknowledgement/Funding None

5207Bilateral cardiac sympathetic denervation in structural heart disease

Abstract Background Left cardiac sympathetic denervation (LCSD) is an established therapy for refractory ventricular arrhythmias (VAs) in channelopathies. A multicentric American and Indian case series suggested a greater efficacy of bilateral denervation (BCSD) in patients with structural heart disease (SHD). Purpose To describe our single-center experience with BCSD in SHD. Methods Nine patients (78% male, mean 55±18 yrs, mean LVEF 31±14%) with SHD and refractory VAs underwent BCSD. All had a Video-Assisted Thoracoscopic Surgery (VATS), in 2 cases associated with the robotic technique. The underlying cardiomyopathy (CMP) was non-ischemic (NICMP) in most cases (n=5, 55%), ischemic in 2 cases, arrhythmogenic right ventricular (ARVC) in one and related to lamin A/C deficiency in one. All patients had an ICD, 44% (n=4) a CRT-D. NYHA functional class I was present in 4 patients, the rest were in NYHA class II (n=3) or III (n=2). Three patients were candidates to heart transplant/LV assistance device. The arrhythmic burden pre BCSD included in 7 pts (78%) a history of electrical storm (ES); the median number of shocks/patient in the 12 months before BCSD was 5 (IQ range 3–18). Except for 2 patients with previous thyrotoxicosis, the remaining were either on amiodarone (n=6) or on sotalol (n=1) before BCSD. Main BCSD indications were represented by drug refractory fast VT in 7 pts (cycle &lt;250 msec) and by recurrent monomorphic VT episodes (mean cycle 351 msec) after endocardial VT ablation in 2 patients. Results No major complication occurred. One patient (NICMP, NYHA II), has an uneventful follow up (FU) of less than 1 month and was excluded from the efficacy analysis. The median FU in the remaining 8 patients is 10 months (IQ range 6–19), during which the median number of shocks/patients was 0.5 (IQ range 0–3). Overall, 4 patients (50%) had ICD shock recurrences. Two cases (mean LVEF 17.5%, NYHA class III) had an ES during severe hemodynamic instability and subsequently died because of cardiogenic shock respectively 1 and 7 months after BCSD. One case had three, not consecutive ICD shocks 20 months after BCSD in the setting of severe amiodarone-induced thyrotoxicosis. Finally, one patient received a single intra-hospital ICD shock 5 days after BCSD before reintroduction of full-dose beta-blockers. The figure summarizes ICD shocks burden in the 6 months before and after BCSD. Among the 5 patients with NICMP/ARVC (4 in NYHA class I), only 1 had a single ICD shock recurrence. ICD shocks pre versus post BCSD, n=8 Conclusions Our case series, although numerically small, has a good follow-up and is the first reported in Europe. The results are in agreement with the suggested remarkable efficacy of BCSD in patients with good functional capacity and fast VAs. Therefore, cardiac sympathetic denervation should always be considered in patients with SHD and refractory ventricular tachyarrhythmias, especially in case VT ablation is either not indicated or fails.

Sex differences in cardiomyopathies

Cardiomyopathies are a heterogeneous group of heart muscle diseases with a variety of specific phenotypes. According to the contemporary European Society of Cardiology classification, they are classified into hypertrophic (HCM), dilated (DCM), arrhythmogenic right ventricular (ARVC), restrictive (RCM), and unclassified cardiomyopathies. Each class is aetiologically further categorized into inherited (familial) and non-inherited (non-familial) forms. There is substantial evidence that biological sex is a strong modulator of the clinical manifestation of these cardiomyopathies, and sex-specific characteristics are detectable in all classes. For the clinician, it is important to know the sex-specific aspects of clinical disease expression and the potential modes of inheritance or the hereditary influences underlying the development of cardiomyopathies, since these may aid in diagnosing such diseases in both sexes.

СARDIOMYOPATHY IN CHILDREN (literature review)

Cardiomyopathy is a chronic and progressive disease of the heart muscle (myocardium). These diseases have a variety of causes, symptoms, and manifestations. According to the World Health Organization, there are four main types of nonischemic cardiomyopathy: dilated (DCM), hypertrophic (HCM), restrictive (RCM) and arrhythmogenic right ventricular (ARVC). Each form is determined by the nature of muscle damage. According to the current literature, the prognosis on pediatric cardiomyopathy remains unfavourable with very high mortality rates. Although, at present, there is no cure for cardiomyopathy, some symptoms and complications can be managed and controlled with medication and implantable devices.

ORAL PRESENTATION

ORAL PRESENTATION

Sudden killer

Sudden killer

Classification of Cardiomyopathies and Indication for Endomyocardial Biopsy Revisited

The first classifications of cardiomyopathies from 1980 and 1996 described them as heart muscle diseases, with dilated (DCM), hypertrophic (HCM), restrictive (RCM), arrhythmogenic right ventricular (ARVC), and nonclassifiable cardiomyopathies. Furthermore, the World Health Organization/International Society and Federation of Cardiology (WHO/ISFC) classification from 1996 listed among the specific cardiomyopathies inflammatory cardiomyopathy as a new and distinct entity, which was defined histologically as myocarditis in association with cardiac dysfunction. Infectious and autoimmune forms of inflammatory cardiomyopathy were recognized. Viral cardiomyopathy was defined as viral persistence in a dilated heart without ongoing inflammation. If it was accompanied by myocardial inflammation, it was termed inflammatory viral cardiomyopathy (or viral myocarditis with cardiomegaly). This entity was further elucidated in a World Heart Federation consensus meeting in 1999 by quantitative immunohistological criteria (< 14 infiltrating cells/mm(2)) and the etiology by molecular biological methods, e.g., polymerase chain reaction, as viral, bacterial, or autoimmune (= nonmicrobial). The development of molecular genetics, with the discovery of a genetic background in several forms of cardiomyopathies previously alluded to as "of unknown origin", was the origin of a debate on a new classification based on genomics. A genomic/postgenomic classification was postulated taking the underlying gene mutations and the cellular level of expression of encoded proteins into account, thus distinguishing cytoskeleton (cytoskeletalopathies, e.g., DCM or ARVC), sarcomeric (sarcomyopathies as in HCM and RCM) and ion channel (channelopathies, e.g., long or short QT syndrome and Brugada's syndrome) cardiomyopathies. Such a classification of cardiomyopathies was proposed in 2006 by the American Heart Association (AHA), which took the rapid evolution of molecular genetics in cardiology into account. It also introduced several recently described diseases, and is unique in that it incorporated ion channelopathies even without hemodynamic dysfunction as a "primary" cardiomyopathy. The ESC (European Society of Cardiology) Working Group on Myocardial and Pericardial Diseases has deliberately taken a different approach based on a clinically oriented classification in which heart muscle disorders were grouped according to morphology and function. This obviously remains the clinically most useful approach for the diagnosis and management of patients and families with heart muscle disease. In the ESC position statement published in 2008, cardiomyopathies were defined as myocardial disorders in which the heart muscle is structurally and functionally abnormal, and in which coronary artery disease, hypertension, valvular and congenital heart disease are absent or do not sufficiently explain the observed myocardial abnormality. The aim was to help clinicians look beyond generic diagnostic labels in order to reach more specific diagnoses. In parallel, a scientific statement on the role of endomyocardial biopsy in the management of cardiovascular disease was published at the end of 2007 making useful recommendations for clinical practice and providing an understanding for the use of endomyocardial biopsy in an individual patient. Taking the classification of cardiomyopathies and the statement on the role of endomyocardial biopsies in different clinical scenarios together, the clinician is now able to identify genetic, autoimmune and viral causative factors by using a thorough and logical approach to reach a diagnosis in patients with familial and nonfamilial forms of the underlying structural heart muscle diseases.

Issue Highlights

HomeCirculationVol. 113, No. 13Issue Highlights Free AccessIn BriefPDF/EPUBAboutView PDFView EPUBSections ToolsAdd to favoritesDownload citationsTrack citationsPermissions ShareShare onFacebookTwitterLinked InMendeleyReddit Jump toFree AccessIn BriefPDF/EPUBIssue Highlights Originally published4 Apr 2006https://doi.org/10.1161/circ.113.13.1633Circulation. 2006;113:1633CLINICAL FEATURES OF ARRHYTHMOGENIC RIGHT VENTRICULAR DYSPLASIA/CARDIOMYOPATHY ASSOCIATED WITH MUTATIONS IN PLAKOPHILIN-2, by Dalal et al.andPLAKOPHILIN-2 MUTATIONS ARE THE MAJOR DETERMINANT OF FAMILIAL ARRHYTHMOGENIC RIGHT VENTRICULAR DYSPLASIA/CARDIOMYOPATHY, by van Tintelen et al.Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) may be challenging to diagnose because of variable expressivity and penetrance. There are multiple gene mutations responsible for the disease, with overlapping clinical phenotypes, analogous to other inherited cardiac diseases with arrhythmia vulnerability. Most of the genes associated with ARVD/C encode for desmosomal proteins involved in cell-to-cell adhesion. In this issue of Circulation, 2 reports by Dalal et al and van Tintelen et al sought to determine the prevalence and clinical expression of mutations in the plakophilin-2 gene (PKP2) in ARVD/C patients. Both groups found a high frequency of PKP2 mutations in patients with familial ARVD/C. Dalal and colleagues found that patients with PKP2 mutations presented earlier in life. However, neither study could identify distinguishing clinical features associated with PKP2 mutations compared with other (non-PKP2) patients with ARVD/C. They conclude that molecular genetic testing for PKP2 mutations may allow presymptomatic identification of ARVD/C and may refine treatment strategies. See pp 1641 and 1650 (and editorial on p 1634).EFFECT OF CLOPIDOGREL PREMEDICATION IN OFF-PUMP CARDIAC SURGERY: ARE WE FORFEITING THE BENEFITS OF REDUCED HEMORRHAGIC SEQUELAE? by Kapetanakis et al.Dual antiplatelet therapy with aspirin and clopidogrel reduces major adverse cardiovascular events after unstable angina, enhances infarct-related artery patency after thrombolytic therapy in ST-elevation myocardial infarction, and decreases post--percutaneous coronary intervention thrombotic complications after both elective and urgent stenting procedures. However, the enhanced efficacy of dual antiplatelet therapy is associated with an increased risk of bleeding that is particularly troublesome for patients requiring surgical revascularization because of the irreversible nature of clopidogrel action. In this issue of Circulation, Kapetanakis and colleagues report on their retrospective study of 1572 patients undergoing isolated off-pump coronary artery bypass surgery (OPCAB) to evaluate the effect of preoperative clopidogrel administration on bleeding. Risk-adjusted logistic regression and matched pair propensity score analyses demonstrated that clopidogrel preadministration increased the risk for hemostatic reoperation and blood transfusion requirements during and after OPCAB surgery. The findings of this nonrandomized, nonblinded retrospective analysis add to the controversy surrounding the optimal timing of clopidogrel administration given the nonsignificant trends for only modest increases in bleeding observed in patients who continued clopidogrel within 5 days before surgery in prospective, double-blind, randomized trials (Clopidogrel in Unstable Angina to Prevent Recurrent Events [CURE] and Clopidogrel as Adjunctive Reperfusion Therapy [CLARITY]-Thrombolysis in Myocardial Infarction [TIMI] 28). See p 1667.MAGNESIUM INTAKE AND INCIDENCE OF METABOLIC SYNDROME AMONG YOUNG ADULTS, by He et al.The metabolic syndrome is defined as a constellation of risk factors that appear to directly promote the development of atherosclerotic cardiovascular disease. Owing to its potential to adversely affect cardiovascular health, much attention is now focused on strategies that may prevent and treat this condition. Prior studies suggest that increased magnesium intake is associated with a more favorable lipid profile and a lower risk of hypertension and diabetes. Accordingly, these data prompt the question of whether there is a relationship between dietary magnesium and the development of metabolic syndrome. In this issue, He and colleagues report the results of their carefully conducted observational study to assess the association of dietary magnesium and the incidence of metabolic syndrome among 4637 young adults, who were followed up for 15 years. Their provocative results suggest a promising role for dietary magnesium intake that can only be verified by the future performance of randomized controlled trials. Whether taking magnesium supplements can provide similar benefits requires further study as well. See p 1675.Visit http://circ.ahajournals.org:Cardiology Patient PageCatheter Ablation of Atrial Fibrillation. See p e666.Images in Cardiovascular MedicineAcute Myocardial Infarction Caused by Extension of a Proximal Aortic Dissection Flap Into the Right Coronary Artery: An Intracoronary Ultrasound Image. See p e669. Download figureDownload PowerPoint“Shock Advised”: Inappropriate Public Access Defibrillation. See p e672.Magnetic Resonance Imaging and Computed Tomography Findings in Arrhythmogenic Right Ventricular Cardiomyopathy. See p e673.CorrespondenceSee p e676. Previous Back to top Next FiguresReferencesRelatedDetailsCited By Joondeph B Federalism, the Rehnquist Court, and the Modern Republican Party, SSRN Electronic Journal, 10.2139/ssrn.1019648 April 4, 2006Vol 113, Issue 13 Advertisement Article InformationMetrics https://doi.org/10.1161/circ.113.13.1633 Originally publishedApril 4, 2006 PDF download Advertisement

Three-Dimensional Electroanatomic Voltage Mapping Increases Accuracy of Diagnosing Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia

Background— Three-dimensional electroanatomic voltage mapping offers the potential to identify low-voltage areas that correspond to regions of right ventricular (RV) myocardial loss and fibrofatty replacement in patients with arrhythmogenic RV cardiomyopathy/dysplasia (ARVC/D). Methods and Results— Thirty-one consecutive patients (22 men and 9 women; mean age, 30.8±7 years) who fulfilled the criteria of the Task Force of the European Society of Cardiology and International Society and Federation of Cardiology (ESC/ISFC) for ARVC/D diagnosis after noninvasive clinical evaluation underwent further invasive study including RV electroanatomic voltage mapping and endomyocardial biopsy (EMB) to validate the diagnosis. Multiple RV endocardial, bipolar electrograms (175±23) were sampled during sinus rhythm. Twenty patients (group A; 65%) had an abnormal RV electroanatomic voltage mapping showing ≥1 area (mean 2.25±0.7) with low-voltage values (bipolar electrogram amplitude <0.5 mV), surrounded by a border zone (0...

Do Patients with Right Ventricular Outflow Tract Ventricular Arrhythmias Have a Normal Right Ventricular Wall Motion?

Background/Aim: Patients with ventricular ectopy from the right ventricular (RV) outflow tract (RVOT) are often referred for RV angiography to exclude disorders such as arrhythmogenic RV cardiomyopathy/dysplasia (ARVC/D). This is usually based on a qualitative assessment of the wall motion. We present a method to quantify the wall motion and to apply this method to compare patients with RVOT ectopy to normal subjects. Methods: RV angiograms were analyzed from 19 normal subjects and 11 subjects with RVOT ventricular arrhythmias (RVOT arrhythmia subjects) who had no other clinical or other evidence for ARVC/D. By a newly developed computer-based method, RV contours were first traced from multiple frames spanning the entire cardiac cycle. The fractional change in area between contours was then calculated as a serial function of time and location to determine both total contour area change and timing of contour movement. Contour area strain, defined as the differential change in area between nearby regions, was also computed. Results: The contour area change was greatest in the tricuspid valve region and least in the RVOT and midanterior regions. The onset of contraction was earliest in the RVOT region and latest in the apical, inferior, inferoapical, and subtricuspid valve regions. The contour strain was largest in superior tricuspid valve and inferior wall and near zero within the lateral tricuspid valve region. There were significant pairwise differences in contraction area, timing, and strain in the various regions. There were no significant differences between normal subjects and RVOT arrhythmia subjects. Conclusions: The RV wall motion is nonuniform in contour area change, strain, and timing of motion. Patients with RVOT ventricular ectopy demonstrate wall motion parameters similar to those of normal subjects. This technique should be applicable in analyzing RV wall motion in patients suspected of having ARVC/D.

Three-Dimensional Electroanatomic Voltage Mapping Increases Accuracy of Diagnosing Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia

Three-dimensional electroanatomic voltage mapping offers the potential to identify low-voltage areas that correspond to regions of right ventricular (RV) myocardial loss and fibrofatty replacement in patients with arrhythmogenic RV cardiomyopathy/dysplasia (ARVC/D). Thirty-one consecutive patients (22 men and 9 women; mean age, 30.8+/-7 years) who fulfilled the criteria of the Task Force of the European Society of Cardiology and International Society and Federation of Cardiology (ESC/ISFC) for ARVC/D diagnosis after noninvasive clinical evaluation underwent further invasive study including RV electroanatomic voltage mapping and endomyocardial biopsy (EMB) to validate the diagnosis. Multiple RV endocardial, bipolar electrograms (175+/-23) were sampled during sinus rhythm. Twenty patients (group A; 65%) had an abnormal RV electroanatomic voltage mapping showing > or =1 area (mean 2.25+/-0.7) with low-voltage values (bipolar electrogram amplitude <0.5 mV), surrounded by a border zone (0.5 to 1.5 mV) that transitioned into normal myocardium (>1.5 mV). Low-voltage electrograms appeared fractionated with significantly prolonged duration and delayed activation. In 11 patients (group B; 35%), electroanatomic voltage mapping was normal, with preserved electrogram voltage (4.4+/-0.7 mV) and duration (37.2+/-0.9 ms) throughout the RV. Low-voltage areas in patients from group A corresponded to echocardiographic/angiographic RV wall motion abnormalities and were significantly associated with myocyte loss and fibrofatty replacement at EMB (P<0.0001) and familial ARVC/D (P<0.0001). Patients from group B had sporadic disease and histopathological evidence of inflammatory cardiomyopathy (P<0.0001). During the time interval from onset of symptoms to the invasive study, 11 patients (55%) with electroanatomic low-voltage regions received an implantable cardioverter/defibrillator because of life-threatening ventricular arrhythmias, whereas all but 1 patient with a normal voltage map remained stable on antiarrhythmic drug therapy (P=0.02). Three-dimensional electroanatomic voltage mapping enhanced accuracy for diagnosing ARVC/D (1) by demonstrating low-voltage areas that were associated with fibrofatty myocardial replacement and (2) by identifying a subset of patients who fulfilled ESC/ISFC Task Force diagnostic criteria but showed a preserved electrogram voltage, an inflammatory cardiomyopathy mimicking ARVC/D, and a better arrhythmic outcome.

Angiographic right and left ventricular function in arrhythmogenic right ventricular dysplasia

We prospectively documented right ventricular (RV) and left ventricular (LV) volumes and ejection fractions in a large series of patients with arrhythmogenic RV dysplasia/cardiomyopathy (ARVD/C). Eighty-five patients with ARVD/C and 11 controls underwent 2 successive orthogonal right and left monoplane x-ray–digitized cineangiographies. Volumes were calculated using the hemielliptical RV and ellipsoidal LV models. All controls and 58 of 85 patients (ARVD/C-I) had a RV ejection fraction ≥35% and 27 patients had a RV ejection fraction <35% (ARVD/C-II). Tricuspid annulus plane systolic excursion (TAPSE) was lower in ARVD/C-II than in ARVD/C-I patients (6 ± 3 vs 14 ± 3 mm) and controls (16 ± 2 mm) (each p <0.001). In patients with ARVD/C, TAPSE was positively related to RV ejection fraction (r = 0.79) and to crista supraventricularis shortening (r = 0.81) (each p <0.001). Sensitivity and specificity of TAPSE <12 mm in identifying patients with RV ejection fraction <35% were 96% and 78%, respectively. LV ejection fraction was ≥50% in 68 patients, 40% to 49% in 10, and <40% in 7. Diffuse RV outflow tract aneurysm was observed in 9 patients, all belonging to ARVD/C-II, and this sign identified patients with LV ejection fraction <40% with 86% sensitivity and 96% specificity. In conclusion, 68% of ARVD/C patients had normal RV ejection fraction and RV volumes, and 80% of ARVD/C patients had normal LV ejection fraction. Decreased TAPSE <12 mm and a diffuse RV outflow tract aneurysm were sensitive and specific indicators of RV ejection fraction <35% and LV ejection fraction <40%, respectively.