Associations of Prolonged QTc in Sickle Cell DiseaseReportar como inadecuado

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Sudden death is a leading cause of mortality in sickle cell disease, implicating ventricular tachyarrhythmias. Prolonged QTc on an electrocardiogram ECG, commonly seen with myocardial ischemia, is a known risk for polymorphic ventricular tachycardia VT. We hypothesized that prolonged QTc is associated with mortality in sickle cell disease. ECG were analyzed from a cohort of 224 sickle patients University of Illinois at Chicago, UIC along with available laboratory, and echocardiographic findings, and from another cohort of 38 patients University of Chicago, UC for which cardiac MRI and free heme values were also measured. In the UIC cohort, QTc was potentially related to mortality with a hazard ratio HR of 1.22 per 10ms, P = 0.015, and a HR = 3.19 P = 0.045 for a QTc>480ms. In multivariate analyses, QTc remained significantly associated with survival after adjusting for inpatient ECG status HR 1.26 per 10ms interval, P = 0.010 and genotype status HR 1.21 per 10ms interval, P = 0.037. QTc trended toward association with mortality after adjusting for both LDH and hydroxyurea use HR 1.21 per 10ms interval, P = 0.062 but was not significant after adjusting for TRV. In univariate analyses, QTc was related to markers of hemolysis including AST P = 0.031, hemoglobin P = 0.014, TR velocity P = 0.036, higher in inpatients P<0.001 and those with an SS compared to SC genotype P<0.001 in the UIC cohort as well as to free heme in the UC cohort P = 0.002. These findings support a relationship of prolonged QTc with hemolysis and potentially mortality in sickle cell disease.

Autor: Julia H. Indik, Vineet Nair, Ruslan Rafikov, Iwan S. Nyotowidjojo, Jaskanwal Bisla, Mayank Kansal, Devang S. Parikh, Melissa Robi



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