Ablation strategies for arrhythmogenic right ventricular cardiomyopathy: a systematic review and meta-analysis

Background Catheter ablation for ventricular tachycardia(VT) in patients with arrhythmogenic right ventricular cardiomyopathy(ARVC) has significantly evolved over the past decade. However, different ablation strategies showed inconsistency in acute and long-term outcomes. Methods We searched the databases of Medline, Embase and Cochrane Library through October 17, 2019 for studies describing the clinical outcomes of VT ablation in ARVC. Data including VT recurrence, all-cause mortality, acute procedural efficacy and major procedural complications were extracted. A meta-analysis with trial sequential analysis was further performed in comparative studies of endo-epicardial versus endocardial-only ablation. Results A total of 24 studies with 717 participants were enrolled. The literatures of epicardial ablation were mainly published after 2010 with total ICD implantation of 73.7%, acute efficacy of 89.8%, major complication of 5.2%, follow-up of 28.9 months, VT freedom of 75.3%, all-cause mortality of 1.1% and heart transplantation of 0.6%. Meta-analysis of 10 comparative studies revealed that compared with endocardial-only approach, epicardial ablation significantly decreased VT recurrence(OR: 0.50;95% CI: 0.30–0.85;P = 0.010), but somehow increased major procedural complications(OR: 4.64;95% CI: 1.28–16.92;P = 0.02), with not evident improvement of acute efficacy(OR: 2.74;95% CI: 0.98–7.65;P = 0.051) or all-cause mortality(OR: 0.87;95% CI: 0.09–8.31;P = 0.90). Conclusion Catheter ablation for VT in ARVC is feasible and effective. Epicardial ablation is associated with better long-term VT freedom, but with more major complications and unremarkable survival or acute efficacy benefit.

Arrhythmogenic right ventricular cardiomyopathy: From genetics to diagnostic and therapeutic challenges

Arrhythmogenic right ventricular cardiomyopathy(ARVC) is a genetic disease characterized by myocyte loss and fibro-fatty tissue replacement. Diagnosis of ARVC remains a clinical challenge mainly at its early stages and in patients with minimal echocardiographic right ventricular(RV) abnormalities. ARVC shares some common features with other cardiac diseases, such as RV outflow ventricular tachycardia, Brugada syndrome, and myocarditis, due to arrhythmic expressivity and biventricular involvement. The identification of ARVC can be often challenging, because of the heterogeneous clinical presentation, highly variable intra- and inter-family expressivity and incomplete penetrance. This genotypephenotype "plasticity" is largely unexplained. A familial history of ARVC is present in 30% to 50% of cases, and the disease is considered a genetic cardiomyopathy, usually inherited in an autosomal dominant pattern with variable penetrance and expressivity; in addition, autosomal recessive forms have been reported(Naxos disease and Carvajal syndrome). Diagnosis of ARVC relays on a scoring system, with major or minorcriteria on the Revised Task Force Criteria. Implantable cardioverter defibrillators(ICDs) are increasingly utilized in patients with ARVC who have survived sudden death(SD)(secondary prevention). However, there are few data available to help identifying ARVC patients in whom the prophylactic implantation of an ICD is truly warranted. Prevention of SD is the primary goal of management. Pharmacologic treatment of arrhythmias, catheter ablation of ventricular tachycardia, and ICD are the mainstay of treatment of ARVC.

Arrhythmogenic right ventricular cardiomyopathy characterized by recurrent syncope during exercise:A case report

BACKGROUND Arrhythmogenic right ventricular(RV)cardiomyopathy is a rare and currently underrecognized cardiomyopathy characterized by the replacement of RV myocardium by fibrofatty tissue.It may be asymptomatic or symptomatic(palpitations or syncope)and may induce sudden cardiac death,especially during exercise.To prevent adverse events such as sudden cardiac death and heart failure,early diagnosis and treatment of arrhythmogenic RV cardiomyopathy(ARVC)are crucial.We report a patient with ARVC characterized by recurrent syncope during exercise who was successfully treated with combined endocardial and epicardial catheter ablation.CASE SUMMARY A 43-year-old man was referred for an episode of syncope during exercise.Previously,the patient experienced two episodes of syncope without a firm etiological diagnosis.An electrocardiogram obtained at admission indicated ventricular tachycardia originating from the inferior wall of the right ventricle.The ventricular tachycardia was terminated with intravenous propafenone.A repeat electrocardiogram showed a regular sinus rhythm with negative T waves and a delayed S-wave upstroke from leads V1 to V4.Cardiac magnetic resonance imaging showed RV free wall thinning,regional RV akinesia,RV dilatation and fibrofatty infiltration(RV ejection fraction of 38%).An electrophysiological study showed multiple inducible ventricular tachycardia as of a focal mechanism from the right ventricle.Endocardial and epicardial voltage mapping demonstrated scar tissue in the anterior wall,free wall and posterior wall of the right ventricle.Late potentials were also recorded.The patient was diagnosed with ARVC and treated with combined endocardial and epicardial catheter ablation with a very satisfactory follow-up result.CONCLUSION Clinicians should be aware of ARVC,and further workup,including imaging with multiple modalities,should be pursued.The combination of epicardial and endocardial catheter ablation can lead to a good outcome.

Rapid right ventricular pacing for balloon valvuloplasty in congenital aortic stenosis:A systematic review

BACKGROUND Balloon aortic valvuloplasty(BAV)is a well-established treatment modality for congenital aortic valve stenosis.AIM To evaluate the role of rapid right ventricular pacing(RRVP)in balloon stabilization during BAV on aortic regurgitation(AR)in pediatric patients.METHODS A systematic review of the MEDLINE,Cochrane Library,and Scopus databases was conducted according to the PRISMA guidelines(end-of-search date:July 8,2020).The National Heart,Lung,and Blood Institute and Newcastle-Ottawa scales was utilized for quality assessment.RESULTS Five studies reporting on 72 patients were included.The studies investigated the use of RRVP-assisted BAV in infants(>1 mo)and older children,but not in neonates.Ten(13.9%)patients had a history of some type of aortic valve surgical or catheterization procedure.Before BAV,58(84.0%),7(10.1%),4(5.9%)patients had AR grade 0(none),1(trivial),2(mild),respectively.After BAV,34(49.3%),6(8.7%),26(37.7%),3(4.3%),patients had AR grade 0,1,2,and 3(moderate),respectively.No patient developed severe AR after RRVP.One(1.4%)developed ventricular fibrillation and was defibrillated successfully.No additional arrhythmias or complications occurred during RRVP.CONCLUSION RRVP can be safely used to achieve balloon stability during pediatric BAV,which could potentially decrease AR rates.

Arrhythmogenic Right Ventricular Disease on a Young Peaseant

Abstract

Prognostic value of the echocardiographic right/left ventricular end-diastolic diameter ratio in patients with idiopathic pulmonary arterial hypertension

Background Previous studies have shown that an echocardiographic right/left ventricular end-diastolic diameter ratio(RV/LV ratio)≥0.9 is an independent predictor of poor prognosis in patients with acute pulmonary embolism. The prognostic value of the RV/LV ratio in patients with idiopathic pulmonary arterial hypertension(IPAH) is still unknown. Methods We retrospectively enrolled 95 consecutive patients with newly diagnosed IPAH and 16 of them were reevaluated by echocardiography at 3-12 months following targeted therapy.Follow-up data were obtained by telephone interviews and review of the patients’ records.Results The RV/LV ratio was in parallel with the severity of World Health Orgnization(WHO) functional class and mean right atrial pressure.The RV/LV ratio was positively correlated with total pulmonary resistance(P P P 2 saturation(P P = 0.001),weight and absence of targeted therapy were independent predictors of death.No significant changes in the RV/LV ratio before and after targeted therapy were observed. A baseline RV/LV ratio≥0.84 or a further increase in the RV/LV ratio during targeted therapy indicated a poor prognosis. Conclusions The RV/LV ratio helps to assess the severity of IPAH and serves as an independent predictor of prognosis in patients with IPAH.

Lesson Eighty four Ventricular arrhythmias originating from papillary muscles in the right ventricle

Patientcharacteristics Patients in this study consisted of eight consecutive patients with frequent premature ventricular complexes(PVCs) or both PVCs and ventricular tachycardia(VT)who had been referred for catheter ablation and whose arrhythmia was mapped to one of the right ventricular(RV)papillary muscles(PAPs).The control group consisted of 10 consecutive patients who were referred for ablation of symptomatic idiopathic

Arrhythmogenic ventricular cardiomyopathy:A paradigm shift from right to biventricular disease

Arrhythmogenic ventricular cardiomyopathy(AVC) isgenerally referred to as arrhythmogenic right ventricu-lar(RV) cardiomyopathy/dysplasia and constitutesan inherited cardiomyopathy.Affected patients maysuccumb to sudden cardiac death(SCD),ventriculartachyarrhythmias(VTA) and heart failure.Geneticstudies have identified causative mutations in genesencoding proteins of the intercalated disk that lead toreduced myocardial electro-mechanical stability.Theterm arrhythmogenic RV cardiomyopathy is somewhatmisleading as biventricular involvement or isolated leftventricular(LV) involvement may be present and thus abroader term such as AVC should be preferred.The di-agnosis is established on a point score basis accordingto the revised 2010 task force criteria utilizing imagingmodalities,demonstrating fibrous replacement throughbiopsy,electrocardiographic abnormalities,ventricu-lar arrhythmias and a positive family history includingidentification of genetic mutations.Although severarisk factors for SCD such as previous cardiac arrest,syncope,documented VTA,severe RV/LV dysfunctionand young age at manifestation have been identified,risk stratification still needs improvement,especially inasymptomatic family members.Particularly,the roleof genetic testing and environmental factors has to befurther elucidated.Therapeutic interventions include re-striction from physical exercise,beta-blockers,sotalol,amiodarone,implantable cardioverter-defibrillators andcatheter ablation.Life-long follow-up is warranted insymptomatic patients,but also asymptomatic carriersof pathogenic mutations.

Evaluation of Right Ventricular Volume and Systolic Function by Real-time Three-dimensional Echocardiography

The optimal plane for measurement of the right ventricular (RV) volumes by real-time three-dimensional echocardiography (RT3DE) was determined and the feasibility and accuracy of RT3DE in studying RV systolic function was assessed. RV “Full volume” images were acquired by RT3DE in 22 healthy subjects. RV end-diastolic volumes (RVEDV) and end-systolic volumes (RVESV) were outlined using apical biplane, 4-plane, 8-plane, 16-plane offline separately. RVSV and RVEF were calculated. Meanwhile tricuspid annual systolic excursion (TASE) was measured by M-mode echo. LVSV was outlined by 2-D echo according to the biplane Simpson's rule. The results showed: (1) There was a good correlation between RVSV measured from series planes and LVSV from 2-D echo (r=0.73; r=0.69; r=0.63; r=0.66, P<0.25—0.0025); (2) There were significant differences between RVEDV in biplane and those in 4-, 8-, 16-plane (P<0.001). There was also difference between RV volume in 4-plane and that in 8-plane (P<0.05), but there was no significant difference between RV volume in 8-plane and that in 16-plane (P>0.05); (3) Inter-observers and intro-observers variability analysis showed that there were close agreements and relations for RV volumes (r=0.986, P<0.001; r=0.93, P<0.001); (4) There was a significantly positive correlation of TASE to RVSV and RVEF from RT3DE (r=0.83; r=0.90). So RV volume measures with RT3DE are rapid, accurate and reproducible. In view of RV's complex shape, apical 8-plane method is better in clinical use. It may allow early detection of RV systolic function.

The importance of avoiding unnecessary right ventricular pacing in clinical practice

Symptomatic bradycardia is effectively treated with the implantation of a cardiac pacemaker. Although a highly successful therapy, during recent years there has been a focus on the negative effects associated with longterm pacing of the apex of the right ventricle(RV). It has been shown in both experimental and clinical studies that RV pacing leads to ventricular dyssynchrony, similar to that of left bundle branch block, with subsequent detrimental effects on cardiac structure and function, and in some cases adverse clinical outcomes such as atrial fibrillation, heart failure and death. There is substantial evidence that patients with reduced left ventricular function(LVEF) are at particular high risk of suffering the detrimental clinical effects of long-term RV pacing. The evidence is, however, incomplete, coming largely from subanalyses of pacemaker and implantable cardiac defibrillator studies. In this group of patients with reduced LVEF and an expected high amount of RV pacing, biventricular pacing(cardiac resynchronization therapy) devices can prevent the negative effects of RV pacing and reduce ventricular dyssynchrony. Therefore, cardiac resynchronization therapy has emerged as an attractive option with promising results and more clinical studies are underway. Furthermore, specific pacemaker algorithms, which minimize RV pacing, can also reduce the negative effects of RV stimulation on cardiac function and may prevent clinical deterioration.

Clinical study of transcatheter occlusion in treating ventricular septal defect combined with right coronary cusp bulge

Background: Perimembranous ventricular septal defect combined with right coronary cusp bulge generally should be treated with surgical thoracotomy, owing to the potential aortic regurgitation. However, the minimally invasive method of transcatheter closure has always attracted the attention of cardiologists and patients. The present study aimed to apply transcatheter occlusion in treating ventricular septal defect with right coronary cusp bulge and further evaluate the clinical effect through follow-up. Materials and methods: A total of 40 children diagnosed as having a ventricular septal defect with right coronary cusp bulge, examined using transthoracic echocardiography and cardiovascular angiography, were enrolled in this study. The ventricular septal defects were closed by placing occluders through transcatheter occlusion treatment. During the operation process, the children underwent angiography and transthoracic echocardiography examinations to check the position of the occlude and the extent of aortic regurgitation. The influence of occlusion on the conduction system was evaluated using a surface electrocardiogram. The children were followed up after their procedures. Results: All 40 patients were immediately and successfully occluded. Three patients with filament residual shunts were observed during the operations. No major surgical complications occurred during the perioperative period. During the follow-up period, the positions of all the occluders were good, the residual shunts in the three patients disappeared, and no new or aggravated aortic regurgitation occurred. Electrocardiogram did not reveal any atrioventricular blocks. Only one patient suffered from an incomplete right bundle branch block. Conclusions: Children diagnosed with ventricular septal defect combined with right coronary cusp bulge could be considered for transcatheter occlusion. With appropriate indications and methods, the effect may be favorable.

Quantitative Assessment of Right Ventricular Systolic Function by the Analysis of Right Ventricular Contrast Time-intensity Curve

Summary: To study reliability and reliable indices of quantitative assessment of right ventricular systolic function by time-intensity curve (TIC) with right ventricular contrast, 5 % sonicated human albumin was injected intravenously at a does of 0.08 ml/kg into 10 dogs at baseline status and cardiac insufficiency. Apical four-chamber view was observed for washin and washout of contrast agent from right ventricle. The parameters of TIC were obtained by curve fitting. The differences of parameters were analyzed in different states of cardiac functions. Among the parameters derived from TIC, the time constant (k) was decreased significantly with decline of cardiac function (P<0.001). But half-time of decent of peak intensity (HT) and mean-transit-time (MTT) of washout were increased significantly (P<0.001). The k was strongly related to cardiac output of right ventricle (CO) and ejection fraction (EF) of left ventricle and fractional shortening (FS) of left ventricle. Right ventricular systolic function could be assessed reliably by the parameters derived from TIC with right ventricular contrast echocardiography. The k, HT and MTT are reliable indices for quantitative assessment of right ventricular systolic function.

Evaluation of Right Ventricular Global Longitudinal Function in Patients with Tetralogy of Fallot by Two-dimensional Ultrasound Speckle Tracking Imaging

Quantification of right ventricular(RV)volume and function remains a challenge because of RV complex geometry by conventional echocardiography.The purpose of this study was to assess RV global longitudinal function in patients with tetralogy of Fallot(TOF)by 2-dimensional ultrasound speckle tracking imaging(STI).Thirty-eight patients with TOF were enrolled in this study and divided into child group(n=25)and adult group(n=13)according to age.Thirty-eight age-and sex-matched normal subjects were selected as c...

Effect of arotinolol on right ventricular function in patients with dilated cardiomyopathy

Objective Dilated cardiomyopathy(DCM) is generally considered to be accompanied by both left and right ventricular dysfunction,but most studies only analyze the left ventricular function. In this study,we evaluated the effect of arotinolol on right ventricular function in patients with DCM. Methods Right ventricular ejection fraction(RVEF) and right ventricular diameter(RVD) were measured by two-dimensional echocardiography(2-DE) in 33 DCM patients;RVEF measured by first-pass radionuclide angiography(FPRA) was compared with that by 2-DE. Results The treatment with arotinolol for one year resulted in a reduction in the right ventricular diameter(baseline,23.0 ± 8.3 mm vs after one-year treatment,20.7 ± 5.4 mm;P=0.004 ) and an associated increase in ejection fraction(baseline,36.9 ± 10.3% vs after one-year treatment,45.8 ± 9.6%;P < 0.001 ) ;there is a high correlation between the 2-DE method and radionuclide ventriculographic method. The correlation coefficient is 0.933(P<0.001) . Conclusion Arotinolol therapy could not only improve left ventricular function,but also improve right ventricular function in DCM patients.

Primary reperfusion in acute right ventricular infarction: An observational study

AIM: To investigate the impact of primary reperfusion therapy(RT) on early and late mortality in acute right ventricular infarction(RVI). METHODS: RVI patients(n = 679) were prospectivelyclassified as without right ventricular failure(RVF)(class A, n = 425, 64%), with RVF(class B, n = 158, 24%) or with cardiogenic shock(CS)(class C, n = 96, 12%). Of the 679 patients, 148(21.7%) were considered to be eligible for thrombolytic therapy(TT) and 351(51.6%) for primary percutaneous coronary intervention(PPCI). TIMI 3-flow by TT was achieved for A, B and C RVI class in 65%, 64% and 0%, respectively and with PPCI in 93%, 91% and 87%, respectively. RESULTS: For class A without RT, the mortality rate was 7.9%, with TT was reduced to 4.4%(P < 0.01) and with PPCI to 3.2%(P < 0.01). Considering TT vs PPCI, PPCI was superior(P < 0.05). For class B without RT the mortality was 27%, decreased to 13% with TT(P < 0.01) and to 8.3% with PPCI(P < 0.01). In a TT and PPCI comparison, PPCI was superior(P < 0.01). For class C without RT the in-hospital mortality was 80%, with TT was 100% and with PPCI, the rate decreased to 44%(P < 0.01). At 8 years, the mortality rate without RT for class A was 32%, for class B was 48% and for class C was 85%. When PPCI was successful, the longterm mortality was lower than previously reported for the 3 RVI classes(A: 21%, B: 38%, C: 70%; P < 0.001). CONCLUSION: PPCI is superior to TT and reduces short/long-term mortality for all RVI categories. RVI CS patients should be encouraged to undergo PPCI at a specialized center.

Isolated right ventricular noncompaction caused recurrent pulmonary embolism

Clinically, pulmonary embolism （PE） mostly comes from the lower extremity deep vein system, but if such pa- tients have no evidence of deep venous thrombosis （DVT）, other sources of emboli should be considered. It is very rare that embolus comes from the right heart system （in situ thrombosis）, Isolated fight ventricular noncompaction （iRVNC） can cause PE, but it has not been reported so far. Ventricular noncompaction （VNC） is an unusual cause of cardiomyopathy.

Usefulness of myocardial performance index for assessing right ventricular function after percutaneous closure of atrial septal defect

Objective Assessment of right ventricular function in patients with atrial septal defect(ASD)is difficult.The Doppler myocardial performance index(MPI)may provide a method of assessing function in these patients.The purposes of this study were to evaluate the right ventricular function and its changes in patients with ASD after transcatheter closure of ASD.Methods MPI,defined as the sum of isovolumic relaxation time and isovolumic contraction time derived by ejection time,was measured from tricuspid inflow and right ventricular outflow;Doppler velocity profiles recorded during routine echocardiography.Twenty nine patients(13 men,16 women;mean age 25.28±12.69,range 6 to 57 years)were diagnosed to secundum ASD[the stretched diameters of ASD were from 9 To 36(24.91±7.98)mm],and had a successfully placed Amplatzer septal occluder(ASO)(the sizes of ASO were from 11 to 40 mm);there were 81 sex-matched,age-matched healthy people(control group 41men,40 women;mean age 29.02±14.22,range 4 to 45 years).MPI was measured again on 3 days and 1 month after closure of ASD.Change in the study group was assessed and compared to the control subjects with structurally normal hearts.A complete 2-dimensional and Doppler echocardiographic examination was performed in all study groups.Results 1)The isovolumic relaxation and isovolumic contraction times[respectively(77.59±14.39)ms vs(60.93±12.94)ms,P<0.0001;(28.28±10.88)ms vs(23.64±9.01)ms,P=0.027]were prolonged,and ejection time[(260.65±21.86)ms vs(271.85±21.92)ms,P=0.033]was shortened in patients with ASD compared with that in control subjects,resulting in a marked increase in the MPI(0.40±0.07 vs 0.31±0.05,P<0.0001)from normal values;2)by Pearson's correlations,the MPI had no correlation with heart rate and blood pressure in control subjects and patients with ASD,but it correlated positively with age in patients with ASD;3)by Pearson's correlations,the MPI correlated positively with the diameter of ASD and pulmonary artery pressure;4)after transcatheter closure of ASD,the MPI decreased markedly.Conclusions 1)MPI is a conceptually new,simple,and reproducible Doppler index in patients with ASD;2)MPI is free from the effect of age,heart rate and blood pressure;(3)MPI appears to be relatively dependent on changes in the diameter of ASD and pulmonary artery pressure;4)the right ventricular function was improved after transcatheter closure of ASD.

Correlation between ductus venosus spectrum and right ventricular diastolic function in isolated single-umbilical-artery foetus and normal foetus in third trimester

BACKGROUND Single umbilical artery(SUA)is the most common umbilical cord malformation in prenatal diagnosis.The presence of an SUA can cause blood circulation disorder in the foetus and functional changes of the foetal heart,affecting foetal circulation.The right ventricular diastolic functions in foetuses with isolated SUA and in normal foetuses in the third trimester were evaluated using the spectral Doppler of blood flow in the foetal ductus venosus(DV).AIM To evaluate the right ventricular diastolic functions in foetuses with isolated SUA and in normal foetuses in the third trimester.METHODS Colour Doppler was used to measure the spectrum of foetal DV and tricuspid orifice in 34 foetuses with isolated SUA aged 28-39 wk and in age-matched healthy controls.The DV flow velocities and velocity ratios were measured.The early passive/late active(E/A)ratio at the tricuspid orifice and tissue Doppler Tei index of the foetal right ventricular in the two groups were also measured.RESULTS During the third trimester,the isolated SUA group showed a lower‘a’-wave peak velocity in the DV than the control group(P<0.05).The correlations between the velocity ratios and E/A ratio at the tricuspid orifice in the two groups were analysed,and the correlation between the ventricular late diastolic velocity/ventricular diastolic peak flow velocity and E/A ratios was the best(R^2of the isolated SUA group:0.520;R2 of the control group:0.358).The correlations between the velocity ratios and tissue Doppler Tei index of foetal right ventricular in the two groups were analysed,and the correlation between the pulsatility index for veins(PIV)and tissue Doppler Tei index ratios was the best(R2 of the isolated SUA group:0.865;R2 of the control group:0.627).CONCLUSION In the isolated SUA group,the atrial systolic peak velocity‘a’decreased,and this finding might be related to the changes in foetal cardiac functions.The ratio of ventricular late diastolic velocity to ventricular diastolic peak flow velocity was closely related to the E/A ratio at the tricuspid valve and can be used to identify changes in the right ventricular diastolic functions of isolated SUA and healthy foetuses.PIV was closely related to the tissue Doppler Tei index of the foetal right ventricular and can be used to identify the right ventricular overall functions of isolated SUA and healthy foetuses.

Is cardiac MRI an effective test for arrhythmogenic right ventricular cardiomyopathy diagnosis?

AIM:To evaluate the referrals with suspected arrhythmogenic right ventricular cardiomyopathy(ARVC)and compare cardiac MR(cMR)findings against clinical diagnosis.METHODS:A retrospective analysis of 114(age range16 to 83,males 55%and females 45%)patients referred for cMR with a suspected diagnosis of ARVC between May 2006 and February 2010 was performed after obtaining institutional approval for service evaluation.Reasons for referral including clinical symptoms and family history of sudden death,electrocardiogram and echo abnormalities,cMR findings,final clinical diagnosis and information about clinical management were obtained.The results of cMR were classified as major,minor,non-specific or negative depending on both functional and tissue characterisation and the cMR results were compared against the final clinical diagnosis.RESULTS:The most common reasons for referral included arrhythmias(30%)and a family history of sudden death(20%).Of the total cohort of 114 patients:4 patients(4%)had major cMR findings for ARVC,13patients(11%)had minor cMR findings,2 patients had non-specific cMR findings relating to the right ventricle and 95 patients had a negative cMR.Of the 4 patients who had major cMR findings,3(75%)had a positive clinical diagnosis.In contrast,of the 13 patients who had minor cMR findings,only 2(15%)had a positive clinical diagnosis.Out of the 95 negative patients,clinical details were available for 81 patients and none of them had ARVC.Excluding the 14 patients with no clinical data and final diagnosis,the sensitivity of the test was 100%,specificity 87%,positive predictive value29%and the negative predictive value 100%.CONCLUSION:CMR is a useful tool for ARVC evaluation because of the high negative predictive value as the outcome has a significant impact on the clinical decision-making.