Author + information
- Claire Zhang,
- Ilan Goldenberg,
- Valentina Kutyifa,
- Scott McNitt,
- Bronislava Polonsky,
- Christine Tompkins,
- Wojciech Zareba and
- Arthur Moss
To date most risk stratification studies in long QT syndrome (LQTS) have focused on identifying high-risk subjects. Current data on the long-term clinical course of low-risk adult LQTS patients are limited.
Patients in this study were from the Rochester-based LQTS Registry. We hypothesized that long-term survival of LQT1-2 patients with QTc <500ms and no cardiac symptoms before age 20 (n=523) would be similar to that of their unaffected genotype-negative family members (n=1134). Kaplan-Meier and Cox proportional hazards regression analyses were the statistical techniques utilized.
The low-risk LQTS study group comprised 27% of genetically confirmed LQTS Registry patients alive at age 20. The cumulative probability of all-cause mortality to age 65 was similar between the low-risk LQTS group and the genotype-negative control group (4.3% and 4.4%, respectively at age 65; p=0.49 for overall difference [Figure 1]). Multivariate analysis showed no significant difference in the risk of all-cause mortality between the two groups (HR = 0.94; 95% CI 0.52-1.69, p=0.82). Similar results were seen in females, males, LQT1, and LQT2 subjects. LQTS females had a lower mortality risk than males (HR = 0.59; 95% CI 0.38-0.93, p=0.02), particularly after age 50.
We identified low-risk, adult LQTS patients with a 45-year survival similar to unaffected family members. These low-risk LQTS patients should qualify for normal, age-related life insurance.
Poster Sessions, Expo North
Sunday, March 10, 2013, 3:45 p.m.-4:30 p.m.
Session Title: Congenital Cardiology Solutions: Congenital Electrophysiology and Genetics
Abstract Category: 12. Congenital Cardiology Solutions: Adult
Presentation Number: 1247-124
- 2013 American College of Cardiology Foundation