Limits...
Clinical characterisation and risk stratification of patients with arrhythmogenic right ventricular dysplasia/cardiomyopathy ≥ 50   years of age

View Article: PubMed Central - PubMed

ABSTRACT

Purpose: With the increased use of genetic testing for arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C), this disease is being increasingly recognised among elderly patients. However, elderly ARVD/C patients were underrepresented in prior cohorts. We aimed to describe the phenotypical characteristics and outcomes among ARVD/C patients surviving ≥50 years.

Methods: We assessed detailed phenotypical data of 29 patients who (1) presented at ≥50 years of age; and (2) fulfilled 2010 Task Force Criteria (TFC) for ARVD/C by last follow-up. Primary outcome was the occurrence of a major ventricular arrhythmia (sudden cardiac death, resuscitated sudden cardiac arrest or sustained ventricular tachycardia).

Results: The majority (55 %) of elderly ARVD/C subjects were male, with a mean age of 59.0 ± 5.8 years at presentation. Study participants fulfilled a median of six (IQR 5–8) TFC criteria by last follow-up, of which arrhythmia criteria were most frequent (97 %), followed by structural criteria (83 %), depolarisation criteria (72 %) and repolarisation criteria (69 %). By last follow-up, 15 (52 %) patients had experienced major ventricular arrhythmias. Most patients (n = 12) presented with this arrhythmia, while three experienced the event during 5.4 ± 3.2 years of follow-up. Compared with patients without an arrhythmic event, patients with major arrhythmias were more likely to be proband (p < 0.001) and male (p = 0.042). Likewise, survival free from sustained ventricular arrhythmia was lower among probands and males.

Conclusion: Phenotypic characteristics of elderly ARVD/C patients are characterised by depolarisation abnormalities and structural cardiac changes. Ventricular arrhythmias in this elderly cohort are associated with male gender and proband status.

No MeSH data available.


Related in: MedlinePlus

Survival free from major ventricular arrhythmias among the study population. Kaplan-Meier survival analyses demonstrating time to first major ventricular arrhythmia during follow-up among the overall cohort
© Copyright Policy - OpenAccess
Related In: Results  -  Collection

License
getmorefigures.php?uid=PMC5120007&req=5

Fig3: Survival free from major ventricular arrhythmias among the study population. Kaplan-Meier survival analyses demonstrating time to first major ventricular arrhythmia during follow-up among the overall cohort

Mentions: Fig. 3 shows cumulative survival free from major ventricular arrhythmia in 17 patients who did not present with an arrhythmia. Over a mean follow-up of 5.4 ± 3.2 years, 3/17 (18 %) patients experienced a sustained monomorphic VT. Median cycle length of the VT was 240 ms (range 231–429). VT episodes showed LBBB morphology: two with superior, one with inferior axis. As shown in Fig. 3, median time to first VT was 4 months after first cardiological evaluation. Cumulative survival free from major ventricular arrhythmia after 6, 12 and 24 months was 88 % (95 % CI 72.3–100.0), 88 % (95 % CI 72.3–100.0), and 80 % (95 % CI 60.4–99.6), respectively.Fig. 3


Clinical characterisation and risk stratification of patients with arrhythmogenic right ventricular dysplasia/cardiomyopathy ≥ 50   years of age
Survival free from major ventricular arrhythmias among the study population. Kaplan-Meier survival analyses demonstrating time to first major ventricular arrhythmia during follow-up among the overall cohort
© Copyright Policy - OpenAccess
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC5120007&req=5

Fig3: Survival free from major ventricular arrhythmias among the study population. Kaplan-Meier survival analyses demonstrating time to first major ventricular arrhythmia during follow-up among the overall cohort
Mentions: Fig. 3 shows cumulative survival free from major ventricular arrhythmia in 17 patients who did not present with an arrhythmia. Over a mean follow-up of 5.4 ± 3.2 years, 3/17 (18 %) patients experienced a sustained monomorphic VT. Median cycle length of the VT was 240 ms (range 231–429). VT episodes showed LBBB morphology: two with superior, one with inferior axis. As shown in Fig. 3, median time to first VT was 4 months after first cardiological evaluation. Cumulative survival free from major ventricular arrhythmia after 6, 12 and 24 months was 88 % (95 % CI 72.3–100.0), 88 % (95 % CI 72.3–100.0), and 80 % (95 % CI 60.4–99.6), respectively.Fig. 3

View Article: PubMed Central - PubMed

ABSTRACT

Purpose: With the increased use of genetic testing for arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C), this disease is being increasingly recognised among elderly patients. However, elderly ARVD/C patients were underrepresented in prior cohorts. We aimed to describe the phenotypical characteristics and outcomes among ARVD/C patients surviving ≥50 years.

Methods: We assessed detailed phenotypical data of 29 patients who (1) presented at ≥50 years of age; and (2) fulfilled 2010 Task Force Criteria (TFC) for ARVD/C by last follow-up. Primary outcome was the occurrence of a major ventricular arrhythmia (sudden cardiac death, resuscitated sudden cardiac arrest or sustained ventricular tachycardia).

Results: The majority (55 %) of elderly ARVD/C subjects were male, with a mean age of 59.0 ± 5.8 years at presentation. Study participants fulfilled a median of six (IQR 5–8) TFC criteria by last follow-up, of which arrhythmia criteria were most frequent (97 %), followed by structural criteria (83 %), depolarisation criteria (72 %) and repolarisation criteria (69 %). By last follow-up, 15 (52 %) patients had experienced major ventricular arrhythmias. Most patients (n = 12) presented with this arrhythmia, while three experienced the event during 5.4 ± 3.2 years of follow-up. Compared with patients without an arrhythmic event, patients with major arrhythmias were more likely to be proband (p < 0.001) and male (p = 0.042). Likewise, survival free from sustained ventricular arrhythmia was lower among probands and males.

Conclusion: Phenotypic characteristics of elderly ARVD/C patients are characterised by depolarisation abnormalities and structural cardiac changes. Ventricular arrhythmias in this elderly cohort are associated with male gender and proband status.

No MeSH data available.


Related in: MedlinePlus