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@article{Tayal:2017:10.1016/j.jacc.2017.08.063,
author = {Tayal, U and Newsome, S and Buchan, R and Whiffin, N and Halliday, B and Lota, A and Roberts, A and Baksi, AJ and Voges, I and Midwinter, W and Wilk, A and Govind, R and Walsh, R and Daubeney, P and Jarman, JWE and Baruah, R and Frenneaux, M and Barton, PJ and Pennell, D and Ware, JS and Prasad, SK and Cook, SA},
doi = {10.1016/j.jacc.2017.08.063},
journal = {Journal of the American College of Cardiology},
pages = {2264--2274},
title = {Phenotype and clinical outcomes of titin cardiomyopathy},
url = {http://dx.doi.org/10.1016/j.jacc.2017.08.063},
volume = {70},
year = {2017}
}

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TY  - JOUR
AB  - Background Improved understanding of dilated cardiomyopathy (DCM) due to titin truncation (TTNtv) may help guide patient stratification.Objectives The purpose of this study was to establish relationships among TTNtv genotype, cardiac phenotype, and outcomes in DCM.Methods In this prospective, observational cohort study, DCM patients underwent clinical evaluation, late gadolinium enhancement cardiovascular magnetic resonance, TTN sequencing, and adjudicated follow-up blinded to genotype for the primary composite endpoint of cardiovascular death, and major arrhythmic and major heart failure events.Results Of 716 subjects recruited (mean age 53.5 ± 14.3 years; 469 men [65.5%]; 577 [80.6%] New York Heart Association function class I/II), 83 (11.6%) had TTNtv. Patients with TTNtv were younger at enrollment (49.0 years vs. 54.1 years; p = 0.002) and had lower indexed left ventricular mass (5.1 g/m2 reduction; padjusted = 0.03) compared with patients without TTNtv. There was no difference in biventricular ejection fraction between TTNtv+/− groups. Overall, 78 of 604 patients (12.9%) met the primary endpoint (median follow-up 3.9 years; interquartile range: 2.0 to 5.8 years), including 9 of 71 patients with TTNtv (12.7%) and 69 of 533 (12.9%) without. There was no difference in the composite primary outcome of cardiovascular death, heart failure, or arrhythmic events, for patients with or without TTNtv (hazard ratio adjusted for primary endpoint: 0.92 [95% confidence interval: 0.45 to 1.87]; p = 0.82).Conclusions In this large, prospective, genotype-phenotype study of ambulatory DCM patients, we show that prognostic factors for all-cause DCM also predict outcome in TTNtv DCM, and that TTNtv DCM does not appear to be associated with worse medium-term prognosis.
AU  - Tayal,U
AU  - Newsome,S
AU  - Buchan,R
AU  - Whiffin,N
AU  - Halliday,B
AU  - Lota,A
AU  - Roberts,A
AU  - Baksi,AJ
AU  - Voges,I
AU  - Midwinter,W
AU  - Wilk,A
AU  - Govind,R
AU  - Walsh,R
AU  - Daubeney,P
AU  - Jarman,JWE
AU  - Baruah,R
AU  - Frenneaux,M
AU  - Barton,PJ
AU  - Pennell,D
AU  - Ware,JS
AU  - Prasad,SK
AU  - Cook,SA
DO  - 10.1016/j.jacc.2017.08.063
EP  - 2274
PY  - 2017///
SN  - 0735-1097
SP  - 2264
TI  - Phenotype and clinical outcomes of titin cardiomyopathy
T2  - Journal of the American College of Cardiology
UR  - http://dx.doi.org/10.1016/j.jacc.2017.08.063
UR  - http://hdl.handle.net/10044/1/50547
VL  - 70
ER  -

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