Truncating mutations in FLNC caused an overlapping phenotype of dilated and left-dominant arrhythmogenic cardiomyopathies complicated by frequent premature sudden death. Prompt implantation of a cardiac defibrillator should be considered in affected patients harboring truncating mutations in FLNC.

Aims: To compare the performance of the CRUSADE, ACUITY-HORIZONS, and ACTION risk models in the STsegment elevation myocardial infarction (STEMI) undergoing primary percutaneous coronary intervention (PPCI). Methods: We studied all consecutive patients with STEMI who underwent PPCI at our institution between 2006 and 2010 (n=1391). The CRUSADE, ACUITY-HORIZONS, and ACTION risk scores were calculated based on the patients' clinical characteristics. The occurrence of in-hospital major bleeding (defined as the composite of intracranial or intraocular bleeding, access site haemorrhage requiring intervention, reduction in haemoglobin ≥4 g/dl without or ≥3g/dl with overt bleeding source, reoperation for bleeding, or blood transfusion) reached 9.8%. Calibration and discrimination of the three risk models were evaluated by the Hosmer−Lemeshow test and the C-statistic, respectively. We compared the predictive accuracy of the risk scores by the DeLong non-parametric test. Results: Calibration of the three risk scores was adequate, given the non-significant results of Hosmer−Lemeshow test for the three risk models. Discrimination of CRUSADE, ACUITY-HORIZONS, and ACTION models was good (C-statistic 0.77, 0.70, and 0.78, respectively). The CRUSADE and ACTION risk scores had a greater predictive accuracy than the respectively). There was no significant difference between the CRUSADE and ACTION models (z=0.63, p=0.531). Conclusions: The CRUSADE, ACUITY-HORIZONS, and ACTION scores are useful tools for the risk stratification of bleeding in STEMI treated by PPCI. Our findings favour the CRUSADE and ACTION risk models over the ACUITY-HORIZONS risk score.

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