Echocardiographic phenotypes of patients with sickle cell disease. An unsupervised analysis based on etendard cohort

Abstract Funding Acknowledgements Type of funding sources: Public grant(s) – National budget only. Main source(s): PHRC Backgroung Echocardiography is the cornerstone in diagnosis cardiopulmonary involvement sickle cell disease (SCD). However, given unique pathophysiology SCD associating high cardiac output, and various degrees peripheral vasculopathy, differentiate pathological from physiological using echocardiography can be particularly challenging. Purpose This study sought to link phenotypes homozygous patients with clinical profiles outcomes cluster analysis. Methods We analyzed data 379 a sufficient echographic dataset included French Etendard Cohort, prospective cohort initially designed assess prevalence pulmonary hypertension. A analysis was performed on echocardiographic variables, association between clusters assessed. Results Three were identified. Cluster 1 (N = 122) had lowest only mild left cavities remodeling, diastolic dysfunction, tricuspid regurgitation velocity (TRV). They predominantly female, as old 2, displayed most severe functional limitation. 2 103) highest ventricular mass severely dilated atrium. Diastolic function TRV similar 1. These higher blood pressure hemolytic anemia. 3 154) best TRV. younger hemoglobin markers. Right heart catheterization 94 patients. gathered majority precapillary PH while postcapillary no found 3. After follow-up 9.9 years (IQR: 9.3 10.5 years) death occurred 38 (10%). Clusters worst prognosis 18% mortality rate vs. 12% 5% (P log-rank 0,02). are summarized central illustration. Conclusions variables identified among patients, each associated different features outcome. findings underlines necessity rethink evaluation an integrative approach based simultaneous along remodeling parameters. Figure.