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Eur J Heart Fail. 2015 Aug;17(8):837-45. doi: 10.1002/ejhf.316. Epub 2015 Jul 16.

Prediction of thrombo-embolic risk in patients with hypertrophic cardiomyopathy (HCM Risk-CVA).

Author information

The Inherited Cardiac Diseases Unit, The Heart Hospital/University College London, London, UK.
Department of Statistical Science, University College London, London, UK.
Cardiology Department and Research Unit, A Coruña University Hospital, Galician Health Service, Spain.
Unit of Inherited Cardiovascular Diseases, 1st Department of Cardiology, University of Athens, Athens, Greece.
Institute of Cardiology, Department of Specialised, Experimental and Diagnostic Medicine, University of Bologna, Bologna, Italy.
Cardiac Department, University Hospital Virgen Arrixaca, Murcia-Cartagena s/n, El Palmar, Murcia, Spain.
Monaldi Hospital, Second University of Naples, Naples, Italy.
Heart Failure and Inherited Cardiac Diseases Unit, Hospital Universitario Puerta del Hierro Majadahonda, Madrid, Spain.
Biostatistics Group, University College London Hospitals/University College London Clinical Research Centre, University College London, London, UK.



Atrial fibrillation (AF) and thrombo-embolism (TE) are associated with reduced survival in hypertrophic cardiomyopathy (HCM), but the absolute risk of TE in patients with and without AF is unclear. The primary aim of this study was to derive and validate a model for estimating the risk of TE in HCM. Exploratory analyses were performed to determine predictors of TE, the performance of the CHA2 DS2 -VASc score, and outcome with vitamin K antagonists (VKAs).


A retrospective, longitudinal cohort of seven institutions was used to develop multivariable Cox regression models fitted with pre-selected predictors. Bootstrapping was used for validation. Of 4821 HCM patients recruited between 1986 and 2008, 172 (3.6%) reached the primary endpoint of cerebrovascular accident (CVA), transient ischaemic attack (TIA), or systemic peripheral embolus within 10 years. A total of 27.5% of patients had a CHA2 DS2 -VASc score of 0, of whom 9.8% developed TE during follow-up. Cox regression revealed an association between TE and age, AF, the interaction between age and AF, TE prior to first evaluation, NYHA class, left atrial (LA) diameter, vascular disease, and maximal LV wall thickness. There was a curvilinear relationship between LA size and TE risk. The model predicted TE with a C-index of 0.75 [95% confidence interval (CI) 0.70-0.80] and the D-statistic was 1.30 (95% CI 1.05-1.56). VKA treatment was associated with a 54.8% (95% CI 31-97%, P = 0.037) relative risk reduction in HCM patients with AF.


The study shows that the risk of TE in HCM patients can be identified using a small number of simple clinical features. LA size, in particular, should be monitored closely, and the assessment and treatment of conventional vascular risk factors should be routine practice in older patients. Exploratory analyses show for the first time evidence for a reduction of TE with VKA treatment. The CHA2 DS2 -VASc score does not appear to correlate well with the clinical outcome in patients with HCM and should not be used to assess TE risk in this population.


Atrial fibrillation; Hypertrophic cardiomyopathy; Thrombo-embolism

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