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- Performance of Task Force diagnostic criteria for identification of symptomatic patients with Arrhythmogenic Right Ventricular Cardiomyopathy in the Nordic ARVC Registry
- American Heart Association Scientific Sessions 2012
- PG Platonov; AG Holst; KH Haugaa; T Edvardsen; T Gilljam; C Lundin; O Eschen; J Hansen; H Bundgaard; JH Svendsen
- Background: Revision of arrhythmogenic right ventricular cardiomyopathy (ARVC) Task Force diagnostic criteria in 2010 (TF2010) increased the sensitivity for detection of patients at early stages of the disease. Whether this is associated with increased detection of patients at risk of severe manifestations of the disease, has not been fully clarified. Our aim was to assess the relation between baseline diagnostic criteria and severe debut of the disease in patients enrolled in the Nordic ARVC Registry.
Methods: Patients with definite ARVC by TF2010 enrolled in the registry in Denmark, Norway and Sweden were included in the analysis: n=139 (102 families), age 48±15 years, 57% male. Patients were defined as symptomatic based on the occurrence of syncope, documented ventricular tachycardia (VT) or aborted cardiac arrest (ACA) by enrolment. Using this definition, the performance of TF2010 criteria was tested for prediction of symptoms.
Results: The study population comprised 102 probands and 37 family members, of whom 24 were identified via family screening (17%). Initial disease manifestations were VT (n=43, 31%), syncope (n=16, 12%) or ACA (n=12, 9%), while 68 (49%) patients had not experienced any of these symptoms at baseline. Median age at first symptom was 40 [range 17-75] years. ACA occurred earlier (28 [range 18-50] years) than syncope (41[18-75] years) or VT (42[17-75] years) as the first symptom (p=0.013). Syncope, VT or ACA as an initial manifestation of ARVC were independently associated with the presence of major depolarisation (OR=2.54 95%CI 1.03-6.31, p=0.044), repolarisation (OR=2.34 95%CI 1.11-4.93, p=0.025) or imaging (OR=5.45 95%CI 2.32-12.78, p<0.001) criteria. The family history of sudden death or ARVC in a 1st degree relative did not predict symptoms. The freedom from any imaging criteria was strongly associated with freedom from symptoms (OR=0.18 95%CI 0.08-0.43, p<0.001).
Conclusion: In patients with definite ARVC enrolled in the Nordic ARVC Registry the presence of major imaging, depolarisation or repolarisation diagnostic criteria were independently associated with syncope, VT or ACA as an initial manifestation of the disease. A family history of sudden death or ARVC, however, was not associated with a severe disease debut.
- Publication date
- Risk factors for sudden cardiac death: Results from the Nordic arrhythmogenic right ventricular cardiomyopathy registry.
- European Society of Cardiology Congress 2012
- Anders Gaarsdal Holst; Kristina H Haugaa; Pyotr G Platonov; Thor Edvardsen; Catarina Lundin; Thomas Gilljam; Ole Eschen; Jim Hansen; Henning Bundgaard; Jesper Hastrup Svendsen
Risk factors for sudden cardiac death (SCD) in arrhythmogenic right ventricular cardiomyopathy (ARVC) are not clear. We aimed to study this in a registry study of ARVC patients.
The study was based on a newly started Nordic ARVC registry including patients from centers in Denmark, Sweden and Norway. It was performed as a retrospective cross sectional case control study. The outcome was defined as a composite of SCD, aborted SCD, electrical storms or appropriate implantable cardioverter-defibrillator (ICD) shocks. The inclusion criterion was a diagnosis of definite ARVC according to the 2010 task force criteria (TF2010).
The following factors were studied for their association with the outcome: age, gender, history (Hx) of syncope, Hx of atrial fibrillation, inverted T waves in ECG leads V1 to V3, epsilon wave in the ECG, right bundle branch block, presence of a pathogenic mutation, family Hx of sudden death, inducibility during electrophysiological study, > 500 ventricular premature complex / 24H during Holter monitoring, positive ventricular late potential on (TF2010), Hx of ventricular tachycardia (VT), being an competitive athlete, right ventricular dilation (TF2010), left ventricular ejection fraction < 50%. All factors were primarily analyzed univariately using logistic regression, if they reached a univariate P-value < 0.05 they were secondarily studied in a multivariable logistic regression model.
The population was comprised of 129 patients of which 57% were male and 71% probands. The median age was 49 (IQR 38-59) years and 73% had an ICD implanted. Median retrospective follow up was 7 (IQR 4-12) years and during follow up there were 2 patients suffering SCD, 12 suffering aborted SCD, 6 patients suffering an electric storm and 25 patients experiencing appropriate ICD shocks.
Of the tested factors, epsilon waves on the ECG and HX of VT were found to be univariately associated to the outcome. The other factors were not significantly associated. The odds ratios (OR) from the multivariable model were 3.4 (95%CI 1.2-9.7) for epsilon waves on the ECG and 8.4 (95%CI 1.8-38.6) for HX VT. They differed only insignificantly from the ones found univariately.
In this registry study of risk factors for sudden cardiac death in ARVC we found that a presence of an epsilon wave on the ECG and a history of ventricular tachycardia were associated with the composite outcome of sudden cardiac death, aborted sudden cardiac death, electrical storms and appropriate ICD shocks.
- Publication date
- Performance of task force diagnostic criteria for identification of symptomatic patients in the nordic arrhythmogenic right ventricular cardiomyopathy registry.
- European Society of Cardiology Congress 2012
- PG Platonov; AG Holst; K Haugaa; T Edvardsen; T Gilljam; C Lundin; O Eschen; J Hansen; H Bundgaard; JH Svendsen
- Purpose: Revision of arrhythmogenic right ventricular cardiomyopathy (ARVC) Task Force diagnostic criteria in 2010 (TF2010) increased their sensitivity for detection of patients at early stages of the disease. The association between TF2010 and symptoms, however, has not been fully clarified. Our aim was to review baseline clinical and demographic characteristics of patients enrolled in the Nordic ARVC Registry and assess their relation to the early manifestations of the disease
Methods: Patients with definite ARVC according to TF2010 enrolled in the registry at 7 sites in Denmark, Norway and Sweden were included in the analysis: n=127 (103 families), age 48±16 years, 57% male. Patients were defined as symptomatic based on the occurrence of syncope, documented ventricular tachycardia (VT) or aborted cardiac arrest (ACA) by enrolment in the registry. The performance of TF2010 and TF1994 diagnostic criteria was tested for prediction of symptoms. Minor criteria were assigned 1 point and major criteria 2 points when calculating the total diagnostic score.
Results: 1). Study population comprised 95 probands and 32 family members, of whom 25 were identified via family screening (20%). Mean diagnostic scores were 5.8±1.8 (TF2010) and 3.6±1.7 (TF1994). Initial disease manifestations were VT (n=55, 43%), syncope (n=20, 16%) or aborted cardiac arrest (n=13, 10%) while 39 patients did not have any of those at baseline (30%). Mean age at first symptom was earlier for syncope than for VT or ACA (34±16 vs 43±15 years, p=0.028) as the first symptom. 2). Neither age, gender, imaging or depolarisation criteria were predictive of symptom occurrence, however patients with documented VT or ACA were older than those without (51±15 vs 42±15, p=0.018). The presence of inverted T-waves in leads V1-V3 was associated with symptom occurrence (OR=2.90 95%CI 1.26-6.66, p=0.012). Neither the history of sudden death nor the presence of ARVC in 1st degree relative predicted symptom occurrence. TF1994 score >3 demonstrated association with symptoms (OR=2.4, 95%CI 1.06-5.23, p=0.035 for any symptom and OR=2.7 95%CI 1.29-5.51, p=0.008 for VT or ACA only) while TF2010 score did not.
Conclusion: In patients with definite ARVC enrolled in the Nordic ARVC Registry, abnormal repolarisation is associated with history of syncope, VT or ACA while none of other diagnostic criteria has shown any significant association with the symptoms. We found no relationship between TF2010 score and symptoms as baseline.
- Publication date