Retrospective Review of the Natural History of Pulmonary Hypertension in Sickle Cell Disease Demonstrates That Progressive Enlargement of the Left Atrium Is a Strong Predictor of Death.

Abstract 1529 Poster Board I-552 Pulmonary hypertension (PAH) is an independent risk factor for death in sickle cell disease (SCD). We performed a retrospective chart review to determine the natural history of PAH in our adult SCD population. We hypothesized that increased pulmonary artery pressures seen during hospitalizations for painful crisis or acute chest syndrome would be reflected in a faster progression of PAH measured during steady state. We reviewed charts and echocardiograms of 362 patients seen at Duke University from January 1980 to March 2009. A total of 878 2D echocardiograms were reviewed, with 196 patients having 2 or more echocardiographic procedures. Fifth-three of the 81 patients who died during this period had had at least 2 or more echocardiograms. Studies were considered done at steady state if they were performed as an outpatient procedure. Out of 878 total echocardiograms, 460 had either no measurable tricuspid regurgitation jet (TR) or failed to report it and were excluded from further analysis, leaving 418 echocardiograms in 252 patients (167 at steady state and 251 as inpatient). Among patients with multiple echocardiograms and measurable TR jet performed at steady state, we used a linear mixed model to identify a mean yearly rate of progression of 0.04 m/s (p Disclosures No relevant conflicts of interest to declare.