Abstract 9710: Prevalence of Ekg Criteria Suggestive of Fabry’S Disease in Heart Failure Patients

Abstract

Introduction: Cardiac disease is the most important cause of morbidity and mortality in Fabry disease, an X-linked genetic condition caused by a defect in alpha-galactosidase A. Diagnosis of Fabry’s disease requires high index of suspicion and includes EKG assessment. Prevalence of EKG changes consistent with Fabry disease in patients admitted with heart failure has not been well investigated. Methods: Clinical, EKG and echocardiographic data were collected in 460 non-V paced consecutive CHF patients enrolled over 2 years in the Get with The Guidelines Heart failure (GWG-HF) registry at a single academic center. Fabry disease EKG features including shortened PR intervals, AV block, LVH criteria, QRS prolongation, left atrial enlargement (LAE), atrial fibrillation (AFIB), and bradycardia were recorded. Results: Of the studied criteria, shortened PR was noted in only 1.85% (6/324) of patients in sinus rhythm. Of the 460 non-V-paced rhythms patients, LVH was noted in 19% (87/460), QRS>120 ms in 20% (96/460), LAE in 21% (98/460), AFIB in 21% (100/460), and HR <60 bpm in 8% (37/460). Overall, 15% (70/460) of patients with non-V-paced rhythms had a combination of LVH/QRS and LAE/AFIB; this combination of ECG findings is potentially suggestive of Fabry disease. There were no significant differences in gender, age, BMI, BNP, GFR, or Hgb levels, degree LV dysfunction, histories of HTN, CAD, diabetes in these 70 patients vs. the rest of the cohort. However, patients with these criteria had significantly increased prevalence of HFrEF (55.7%, 39/70, vs. 42.8% in the rest of the cohort, 167/390, p=0.031). There was no difference in re-admission and/or mortality rates during the 70.7+/-25.2 month follow-up according to these ECG criteria. Conclusions: In our study of unselected patients with ascertained CHF diagnosis, many manifested with multiple EKG features associated with Fabry’s disease. While these patients had increased prevalence of HFrEF, there were no clinical or demographic characteristics separating these individuals from the rest of the cohort. Given poor prognosis and treatable nature of Fabry’s disease, these patients may benefit from additional clinical and laboratory evaluation directed at Fabry’s disease diagnosis