Imperial College London

Dr Nicky Whiffin

Faculty of MedicineNational Heart & Lung Institute

Research Fellow







Hammersmith HospitalHammersmith Campus






BibTex format

author = {Corden, B and Jarman, J and Whiffin, N and Tayal, U and Buchan, R and Sehmi, J and Harper, A and Midwinter, W and Lascelles, K and Markides, V and Mason, M and Pennell, DJ and Barton, PJ and Prasad, SK and Wong, T and Cook, SA and Ware, JS},
pages = {E96--E96},
publisher = {American Heart Association},
title = {Titin Truncating Variants Predict Life-threatening Arrhythmias in Patients With Dilated Cardiomyopathy},
url = {},
year = {2017}

RIS format (EndNote, RefMan)

AB - Introduction: There is an urgent need for better arrhythmic risk stratification in non-ischaemic dilated cardiomyopathy (DCM), where the benefit of ICD implantation is unclear. Titin truncating variants (TTNtv) are the commonest genetic cause of DCM and are associated with early onset non-sustained ventricular tachycardia (NSVT) and atrial fibrillation (AF) in these patients.Hypothesis: We hypothesize that TTNtv status can predict potentially life threatening ventricular tachycardia (VT) or fibrillation (VF) and development of new persistent AF in DCM patients with CRT-D or ICD devices.Methods: We studied 117 DCM patients with an ICD or CRT-D and documented device-recorded arrhythmia over a median period of 4.2 years. Patients were stratified by TTN genotype (28 positive for a TTNtv, 89 negative). The primary outcome was time to first device-treated VT >200bpm or VF. Secondary outcome measures included time to first development of persistent AF.Results: TTNtv predicted the risk of receiving an appropriate ICD therapy for VT/VF (hazard ratio [HR] = 4.9, 95% confidence interval [CI]=2.3-10.7, P<0.0001). This association was independent of all covariates, including replacement fibrosis measured by late-gadolinium enhancement (LGE), (adjusted HR = 8.2, 95% CI 1.9-36.5, P=0.005). Individuals with both a TTNtv and fibrosis had a markedly greater risk for appropriate device therapy than those with neither (HR = 16.6, CI 3.5-79.3, P<0.0001). TTNtv were also a risk factor for developing new persistent AF (HR = 4.4, 95% CI = 1.45-13.1, P=0.006).Conclusion: TTNtv status is an important risk factor for clinically significant arrhythmia in patients with DCM and CRT-D or ICD devices. TTNtv status alone, or more powerfully in combination with fibrosis imaging by MRI, may provide an effective approach for risk stratifying the need for ICD therapy in DCM patients.
AU - Corden,B
AU - Jarman,J
AU - Whiffin,N
AU - Tayal,U
AU - Buchan,R
AU - Sehmi,J
AU - Harper,A
AU - Midwinter,W
AU - Lascelles,K
AU - Markides,V
AU - Mason,M
AU - Pennell,DJ
AU - Barton,PJ
AU - Prasad,SK
AU - Wong,T
AU - Cook,SA
AU - Ware,JS
EP - 96
PB - American Heart Association
PY - 2017///
SN - 0009-7322
SP - 96
TI - Titin Truncating Variants Predict Life-threatening Arrhythmias in Patients With Dilated Cardiomyopathy
UR -
UR -
ER -