scholarly journals Long term CMR follow up of patients with right ventricular abnormality and clinically suspected arrhythmogenic right ventricular cardiomyopathy (ARVC)

2019 ◽  
Vol 21 (1) ◽  
Author(s):  
Giuseppe Femia ◽  
Christopher Semsarian ◽  
Mark McGuire ◽  
Raymond W. Sy ◽  
Rajesh Puranik
Keyword(s):  
Family History ◽  
Ejection Fraction ◽  
Right Ventricular ◽  
Task Force ◽  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
Premature Death ◽  
Ventricular Cardiomyopathy ◽  
Degree Relative ◽  
History Of

Abstract Background The Task Force Criteria (TFC) for arrhythmogenic right ventricular cardiomyopathy (ARVC) was updated in 2010 to improve specificity. There was concern however that the revised cardiovascular magnetic resonance (CMR) criteria was too restrictive and not sensitive enough to detect early forms of the condition. We previously described patients with clinically suspected ARVC who satisfied criteria from non-imaging TFC categories and fulfilled parameters from the original but not the revised CMR criteria; as a result, these patients were not confirmed as definite ARVC but may represent an early phenotype. Methods Patients scanned between 2008 and 2015 who had either right ventricular (RV) dilatation or regional dyskinesia satisfying at least minor imaging parameters from the original criteria and without contra-indication underwent serial CMR scanning using a 1.5 T scanner. The aims were to assess the risk of progressive RV abnormalities, evaluate the accuracy of the revised CMR criteria and the need for guideline directed CMR surveillance in at-risk individuals. Results Overall, 48 patients were re-scanned; 24 had a first-degree relative diagnosed with ARVC using the revised TFC or a first-degree relative with premature sudden death from suspected ARVC and 24 patients had either left bundle branch morphology ventricular tachycardia or > 500 ventricular extra-systoles in 24-h. Mean follow up was 69+/− 25 months. The indexed RV end-diastolic, end-systolic volumes and ejection fraction were calculated for both scans. There was significant reduction in RV volumes and improvement in RV ejection fraction (EF) irrespective of changes to body surface area; − 11.7+/− 15.2 mls/m2, − 6.4+/− 10.5 mls/m2 and + 3.3 +/− 7.9% (p = 0.01, 0.01 and 0.04). Applying the RV parameters to the revised CMR criteria, two patients from the family history group (one with confirmed ARVC and one with a premature death) had progressive RV abnormalities satisfying major criteria. The remaining patients (n = 46) did not satisfy the criteria and either had normal RV parameters with regression of structural abnormalities (27,56.3%) or stable abnormalities (19,43.7%). Conclusion The revised CMR criteria represents a robust tool in the evaluation of patients with clinical suspicion of ARVC, especially for those with ventricular arrhythmias without a family history for ARVC. For patients with RV abnormalities that do not fulfill the revised criteria but have a family history of ARVC or an ARVC associated gene mutation, a surveillance CMR scan should be considered as part of the clinical follow up protocol.

scholarly journals Clinical Presentation, Cardiac Magnetic Resonance Findings, and Prognosis of Patients with Arrhythmogenic Right Ventricular Cardiomyopathy – An Experience from Pakistan

2020 ◽  
Vol 10 ◽  
pp. 48
Author(s):  
Intisar Ahmed ◽  
Fateh Ali Tipoo
Keyword(s):  
Cardiac Magnetic Resonance ◽  
Family History ◽  
Magnetic Resonance ◽  
Right Ventricular ◽  
Clinical Presentation ◽  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
Left Ventricular ◽  
Ventricular Cardiomyopathy ◽  
History Of

Objectives: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited heart-muscle disease, characterized by fibro-fatty replacement and ventricular arrhythmias, that primarily affects the right ventricle (RV). We aimed to look at the clinical presentation, cardiac magnetic resonance (CMR) imaging findings and prognosis of patients with ARVC in Pakistan. Material and Methods: It is a retrospective observational study, 17 consecutive patients with CMR and other findings consistent with ARVC, were enrolled from 2010 to 2019 at a single center. Results: Out of 17 patients, 12 (70.6%) were male with a mean age of 33.5 ± 17.5 years. Family history of sudden cardiac death was present in 3 (17.7%) patients while one (5.9%) patient had family history of ARVC. Syncope was the first presenting symptom in eight (47.1%) patients. On 12 leads ECG, T wave inversion in precordial leads was found in 6 (35.4%) patients, and epsilon wave was present in only 3 (17.7%) patients. On echocardiogram, 13 (76.5%) patients had dilated RV with reduced systolic function. On CMR, majority of patients (n = 14, 82.4%) were found to have RV dilatation with regional dyskinesia and fatty infiltration, 9 (52.9%) of them had left ventricular involvement also. Follow-up was available for 14 patients (82.4%) with a mean follow-up period of 35.5 ± 19.7 months. Three (21.4%) of them died and 10 (71.4%) got admissions for heart failure during follow-up period. Conclusion: Arrhythmia related events are the main presenting symptoms of ARVC in this region, and left ventricular involvement in ARVC is not rare in this population. The mortality is relatively high, probably due to advanced disease at the time of presentation and less medical facilities available.

Abstract 3003: Programmed Ventricular Stimulation Does Not Predict the Outcome of Patients with Arrhythmogenic Right Ventricular Cardiomyopathy (from DARVIN study)

Circulation ◽  
2007 ◽  
Vol 116 (suppl_16) ◽  
Author(s):  
Domenico Corrado ◽  
Loira Leoni ◽  
Mark S Link ◽  
Hugh Calkins ◽  
Thomas Wichter ◽  
...  
Keyword(s):  
Sudden Death ◽  
Right Ventricular ◽  
Predictive Value ◽  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
Ventricular Cardiomyopathy ◽  
Icd Therapy ◽  
Programmed Ventricular Stimulation ◽  
Ventricular Stimulation ◽  
History Of

Background: The Defibrillator in Arrhythmogenic Right Ventricular Cardiomyopathy International (DARVIN) study was a multicenter investigation that enrolled patients with arrhythmogenic right ventricular cardiomyopathy (ARVC) who received an implantable defibrillator (ICD) for either secondary or primary prevention of sudden death. Methods: In this DARVIN substudy, we examined whether programmed ventricular stimulation (PVS) is able to predict the arrhythmic risk in a large cohort of 201 ARVC patients (133 males, 68 females, aged 36 ± 12 years) who received an ICD. Implant indications were a history of cardiac arrest in 13 (6%) patients; sustained ventricular tachycardia (VT) in 82 (41%); syncope in 42 (21%); asymptomatic nonsustained VT in 40 (20%); and a family history of sudden death in 24 (12%). PVS prior to ICD implantation was carried out in 143 of 201 patients (71%). All antiarrhythmic drugs were discontinued ≥ 5 half-lives (≥ 6 weeks for amiodarone) before the study. PVS included a minimum of 2 drive cycles length and up to 3 ventricular extrastimuli while pacing from two right ventricular sites. Results: One hundred-nine patients (76%) were inducible to either sustained VT (patients 70; 64%), with a mean cycle length of 287 ± 66ms (range 220 to 410 ms), or ventricular fibrillation/flutter (VF) (patients 39; 36%). Of 109 patients who were inducible at PVS, 56 (52%) did not experience ICD therapy during a mean follow-up of 47 ± 22 months, whereas 11 of 34 (33%) noninducible patients had appropriate ICD interventions. Overall, the positive predictive value of PVS was 48%, the negative predictive value 67%, and the test accuracy 53%. The incidence of ICD discharges on VF, which in all likelihood would have been fatal in the absence of ICD therapy, did not differ between patients who were and were not inducible at PVS (26 of 109, 24% vs 7 of 34, 21%; p=0.87), regardless of clinical presentation. The type of ventricular arrhythmia inducible at PVS did not predict VF during the follow-up. Conclusions: The presence (or absence) of an inducible arrhythmia on PVS did not correlate with subsequent appropriate ICD interventions, suggesting a limited role for PVS in arrhythmic risk stratification of ARVC patient population. A negative PVS may not indicate better prognosis.

scholarly journals Clinical Findings and Diagnostic Yield of Arrhythmogenic Cardiomyopathy Through Genomic Screening of Pathogenic or Likely Pathogenic Desmosome Gene Variants

2021 ◽  
Vol 14 (2) ◽  
Author(s):  
Eric D. Carruth ◽  
Dominik Beer ◽  
Amro Alsaid ◽  
Marci L.B. Schwartz ◽  
Megan McMinn ◽  
...  
Keyword(s):  
Family History ◽  
Right Ventricular ◽  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
Diagnostic Testing ◽  
Great Promise ◽  
Arrhythmogenic Cardiomyopathy ◽  
Ventricular Cardiomyopathy ◽  
Patient Reported ◽  
Genomic Screening

Background: Genomic screening holds great promise for presymptomatic identification of hidden disease, and prevention of dramatic events, including sudden cardiac death associated with arrhythmogenic cardiomyopathy (ACM). Herein, we present findings from clinical follow-up of carriers of ACM-associated pathogenic/likely pathogenic desmosome variants ascertained through genomic screening. Methods: Of 64 548 eligible participants in Geisinger MyCode Genomic Screening and Counseling program (2015–present), 92 individuals (0.14%) identified with pathogenic/likely pathogenic desmosome variants by clinical laboratory testing were referred for evaluation. We reviewed preresult medical history, patient-reported family history, and diagnostic testing results to assess both arrhythmogenic right ventricular cardiomyopathy and left-dominant ACM. Results: One carrier had a prior diagnosis of dilated cardiomyopathy with arrhythmia; no other related diagnoses or diagnostic family history criteria were reported. Fifty-nine carriers (64%) had diagnostic testing in follow-up. Excluding the variant, 21/59 carriers satisfied at least one arrhythmogenic right ventricular cardiomyopathy task force criterion, 11 (52%) of whom harbored DSP variants, but only 5 exhibited multiple criteria. Six (10%) carriers demonstrated evidence of left-dominant ACM, including high rates of atypical late gadolinium enhancement by magnetic resonance imaging and nonsustained ventricular tachycardia. Two individuals received new cardiomyopathy diagnoses and received defibrillators for primary prevention. Conclusions: Genomic screening for pathogenic/likely pathogenic variants in desmosome genes can uncover both left- and right-dominant ACM. Findings of overt cardiomyopathy were limited but were most common in DSP -variant carriers and notably absent in PKP2 -variant carriers. Consideration of the pathogenic/likely pathogenic variant as a major criterion for diagnosis is inappropriate in the setting of genomic screening.

P310Differentiation between arrhythmogenic right ventricular cardiomyopathy and athlete's heart using cardiac magnetic resonance based derived parameters and strain analysis

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
C Czimbalmos ◽  
I Csecs ◽  
Z Dohy ◽  
A Toth ◽  
F I Suhai ◽  
...  
Keyword(s):  
Ejection Fraction ◽  
Diagnostic Accuracy ◽  
Right Ventricular ◽  
Task Force ◽  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
Strain Analysis ◽  
Volume Index ◽  
Ventricular Cardiomyopathy ◽  
End Diastolic Volume ◽  
Regional Strain

Abstract Arrhythmogenic right ventricular cardiomyopathy (ARVC) is a major cause of sudden cardiac death in young athletes. However diagnosing ARVC in highly trained athletes may be complicated because of overlapping features such as elevated right ventricular (RV) end-diastolic volume index or T-wave inversion in precordial leads. The revised Task Force criteria contain no specific cut-off value for professional athletes. Additional CMR parameters and CMR deformation imaging may have an added diagnostic value in this special patient population. Our goal was to determine novel CMR parameters which can help to distinguish between ARVC and athlete's heart. CMR examination of ARVC patients with definite diagnosis based on the revised Task Force criteria (n=34; 41±13 y, 22 male) and healthy professional athletes (members of the Hungarian national water polo, canoing or rowing team performing minimum of 15 hours of training per week, n=34, 32±6 y, 22 male) was performed. We evaluated left and right ventricular end-systolic, end-diastolic (EDVi) and stroke volume index, ejection fraction (EF) and mass. We established derived parameters such as ejection fraction ratio (LVEF/RVEF) and end-diastolic volume ratio (LVEDV/RVEDV). Global and regional strain analysis for the right ventricle was performed using feature tracking technique. Area under the ROC curves (AUC) of conventional and derived CMR parameters and CMR based strain values were analysed. There was no significant difference between RVEDVi of ARVC patients and athletes (124±17 vs 142±47), RVEF was lower in ARVC patients compared to athletes (56±5 vs 41±14%; p<0.001). Significant differences were found between athletes and ARVC patients in LVEDV/RVEDV (0.96±0.08 vs 0.82±0.23), LVEF/RVEF (1.04±0.06 vs 1.41±0.56), global circumferential strain (−34.8±5.9 vs −25.2±12.2) and regional strain values such as midventricular RV strain (−31.5±10.2 vs −20.0±13.4) or midventricular RV strain rate (−1.37±0.56 vs −1.04±0.68), respectively. RVEF and LVEF/RVEF showed excellent (AUC of 0.9–1.0), RV global strain and RV midventricular strain values showed good diagnostic accuracy (AUC of 0.8–0.9), while RVEDVi showed poor diagnostic accuracy (AUC of 0.59). Consequently, in highly trained healthy athletes RVEDVi is in the range of major Task Force criteria, while CMR based derived parameters such as LVEDV/RVEDV or LVEF/RVEF and both global and regional RV strain parameters can be useful parameters in the differential diagnosis. Acknowledgement/Funding National Research, Development and Innovation Office (NKFIH) of Hungary (K 120277), ÚNKP-18-3-IV New National Excellence Program of Human Capacities.

P5694Outcomes of patients with arrhythmogenic right ventricular cardiomyopathy after ventricular tachycardia ablation without an implantable cardioverter-defibrillator: a multicenter international study

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
E Gandjbakhch ◽  
M Laredo ◽  
A Berruezo ◽  
J B Gourraud ◽  
R Martins ◽  
...  
Keyword(s):  
Ventricular Tachycardia ◽  
Left Ventricular Ejection Fraction ◽  
Ejection Fraction ◽  
Right Ventricular ◽  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
Left Ventricular ◽  
Left Ventricular Ejection ◽  
Ventricular Cardiomyopathy ◽  
Ventricular Ejection Fraction

Abstract Background In arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D), implantable cardioverter-defibrillators (ICD) after an episode of sustained monomorphic ventricular tachycardia (MVT) are currently recommended in most situations. However, radiofrequency catheter ablation (RCA) is effective in reducing recurrent VT and whether MVT is a surrogate of sudden cardiac death is debated when other risk factors are lacking. Purpose To report the outcomes of patients with ARVC/D who underwent RCA of well-tolerated MVT without a back-up ICD. Methods Patients with a definite ARVC/D diagnosis according to the 2010 Task Force revised criteria who underwent RCA of well-tolerated MVT at 9 tertiary centers across 5 countries, without an ICD prior to RCA and in the 3 following months were retrospectively included. Patients presenting with syncope or electrical storm, and patients with left ventricular ejection fraction <50% were excluded. Similar patients implanted with an ICD prior or without RCA in the same period served as controls. Results Sixty-five patients [median age 46.1 years, range (19.5–73.8), 75% males] underwent RCA of MVT between 2003 and 2016. Familial history of ARVC/D was found in 11% of patients. Epsilon-waves were present in 19% and T-waves inversion beyond V2 in 43%. A right ventricular (RV) ejection fraction ≤40% or fractional area change ≤33% was found in 14 (25%) patients. Median left ventricular ejection fraction was 61% (50–70). Clinical presentation was palpitations in 81% of patients and near-syncope in 14%. Prior to RCA, patients were on beta-blockers alone in 18%, class I drugs in 37% and amiodarone in 9%, while 15% of patients were free any antiarrhythmic medication. Only 1 patient (2%) had >1 clinical VT morphology. Median VT rate was 180 (110–270). An epicardial approach was used in 31% patients. The clinical VT was inducible in 84% of patients. The median number of targeted RV site was 1 (1–3) (RV outflow tract in 72%). Full acute success defined inability to induce any VT was achieved in 72% of patients. During a median follow-up time of 49 month (1.4–162), there was no death or aborted cardiac arrest. Survival without VT recurrence was estimated at 82%, 71% and 60%, 12-, 36- and 60-months after RCA. No VT recurrence was observed among patient who had undergone an epicardial ablation. Among patients with VT recurrence, 6 (35%) did not receive an ICD, and 14 (70%) underwent redo RCA. An ICD was implanted in 10 patients, including 5 for VT recurrence. Fifty-eight patients constituted the control group, and 64% had appropriate ICD interventions during follow-up. Conclusions Despite a significant rate of VT recurrence, selected patients with ARVC/D who underwent RCA for stable MVT without an ICD did not experience any arrhythmic death. Further prospective studies are mandatory to precise the respective places of ICD and RCA in the management of ARVC/D patients with well-tolerated MVT. Acknowledgement/Funding None

scholarly journals Diagnosing arrhythmogenic right ventricular cardiomyopathy by 2010 Task Force Criteria: clinical performance and simplified practical implementation

EP Europace ◽  
2020 ◽  
Vol 22 (5) ◽  
pp. 787-796 ◽  
Author(s):  
Laurens P Bosman ◽  
Julia Cadrin-Tourigny ◽  
Mimount Bourfiss ◽  
Mounes Aliyari Ghasabeh ◽  
Apurva Sharma ◽  
...  
Keyword(s):  
Family History ◽  
Right Ventricular ◽  
Task Force ◽  
Clinical Performance ◽  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
False Negative ◽  
Screening Strategy ◽  
Practical Implementation ◽  
Ventricular Cardiomyopathy ◽  
Net Reclassification Improvement

Abstract Aims Arrhythmogenic right ventricular cardiomyopathy (ARVC) is diagnosed by a complex set of clinical tests as per 2010 Task Force Criteria (TFC). Avoiding misdiagnosis is crucial to prevent sudden cardiac death as well as unnecessary implantable cardioverter-defibrillator implantations. This study aims to validate the overall performance of the TFC in a real-world cohort of patients referred for ARVC evaluation. Methods and results We included patients consecutively referred to our centres for ARVC evaluation. Patients were diagnosed by consensus of three independent clinical experts. Using this as a reference standard, diagnostic performance was measured for each individual criterion as well as the overall TFC classification. Of 407 evaluated patients (age 38 ± 17 years, 51% male), the expert panel diagnosed 66 (16%) with ARVC. The clinically observed TFC was false negative in 7/66 (11%) patients and false positive in 10/69 (14%) patients. Idiopathic outflow tract ventricular tachycardia was the most common alternative diagnosis. While the TFC performed well overall (sensitivity and specificity 92%), signal-averaged electrocardiogram (SAECG, P = 0.43), and several family history criteria (P ≥ 0.17) failed to discriminate. Eliminating these criteria reduced false positives without increasing false negatives (net reclassification improvement 4.3%, P = 0.019). Furthermore, all ARVC patients met at least one electrocardiogram (ECG) or arrhythmia criterion (sensitivity 100%). Conclusion The TFC perform well but are complex and can lead to misdiagnosis. Simplification by eliminating SAECG and several family history criteria improves diagnostic accuracy. Arrhythmogenic right ventricular cardiomyopathy can be ruled out using ECG and arrhythmia criteria alone, hence these tests may serve as a first-line screening strategy among at-risk individuals.

Abstract 12941: Disease Progression in Patients With Arrhythmogenic Right Ventricular Cardiomyopathy and Ventricular Tachycardia: A Longitudinal Study With Unipolar Voltage Mapping

Circulation ◽  
2014 ◽  
Vol 130 (suppl_2) ◽  
Author(s):  
Pasquale Santangeli ◽  
Ioan Liuba ◽  
Cory Tschabrunn ◽  
Michael Riley ◽  
Fermin Garcia ◽  
...  
Keyword(s):  
Ventricular Tachycardia ◽  
Right Ventricular ◽  
Disease Progression ◽  
Surface Area ◽  
Task Force ◽  
Arrhythmogenic Right Ventricular Cardiomyopathy ◽  
Ventricular Cardiomyopathy ◽  
Voltage Mapping

Introduction: Arrhythmogenic right ventricular cardiomyopathy (ARVC) is believed to result in progressive fibrofatty replacement of the RV myocardium with development of multiple ventricular tachycardia (VT) circuits. Endocardial unipolar voltage mapping has been shown to reliably identify epicardial and endocardial (epi-endo) scar in ARVC. Hypothesis: We studied the prevalence and mechanisms of disease progression in patients with ARVC and VT through longitudinal unipolar voltage mapping studies. Methods: Eighteen consecutive patients (age 38±14 years) who fulfilled the revised Task Force criteria for ARVC underwent two detailed sinus rhythm endocardial unipolar voltage maps (mean 348±118 points) performed a median of 36 months apart (interquartile range 21 to 36 months, minimum 9 months) as part of VT ablation procedures. Epi-endo scar was defined using established unipolar voltage cutoff (5.5 mV). The extent of RV scar and total RV volume was measured by a dedicated software. A >5% increase in RV scar area or volume over follow-up was considered significant. Results: At baseline, all patients had evidence of epi-endo RV scar (mean 141±82 cm 2 ; 56±27% of the RV surface area). After a mean follow-up of 49±36 months, no significant progression of unipolar voltage scar was observed (mean 159±83 cm 2 , P=0.14; 63±27% of the RV surface area, P=0.07). Specifically, only 3 (17%) patients presented with progression of the RV scar >5%. The RV volumes increased during follow-up (from 206±74 mL to 258±77 mL, P=0.0003), with the majority of patients (15/18, 83%) having a significant increase in the RV volume (mean increase = 38.9%). Only 3 (17%) patients had no change in both RV scar size and volume over time. Conclusions: In patients with ARVC and VT, progressive RV dilatation is almost uniformly observed, while rapid epi-endo scar progression is rare. These findings suggest that aggressive epi-endocardial substrate ablation should provide long-term VT control, and further research is needed to identify the mechanism(s) for and to prevent ongoing RV dilatation in these patients.

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.

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