amined the impact of an elevated TR jet on mortality in 215 inpatients or outpatients with SCD who were followed in an urban community-based practice. In multivariate analyses, we found that a mildly elevated ePASP was highly predictive for mortality in this unselected SCD cohort. Although our data could not discriminate whether an elevated ePASP was causal for mortality or epiphenomenological to severe underlying SCD, additional evidence for vasculopathy, i.e. depressed renal function, was also associated with an increased risk for mortality. The Clinical Looking Glass (CLG) is a searchable electronic database developed at Montefiore Medical Center (Bronx, N.Y., USA) that retains clinical and follow-up information on annual inpatient and outpatient visits. All subjects preliminarily identified as having SCD (including homozygous HbSS or compound heterozygous HbS 0-thalassemia and HbSC) were confirmed by review of clinically obtained hemoglobin electrophoreses. Charts for subjects with SCD who had undergone echogardiographic examinations between 2000 and 2008 (identified via ICD9 diagnostic codes) were reviewed for comorbid conditions or complications. Use of hydroxyurea, angiotensin-converting enzyme inhibitors, angiotensin receptor blockers,  -blockers, and calcium chanSickle cell disease (SCD) has a complex clinical phenotype that belies the simple underlying molecular defect, in which a single mutation in the  -globin chain causes abnormal hemoglobin polymerization. The pleiotropic clinical features of SCD include cardiac, cerebral, and renal vasculopathy, osteonecrosis, and recurrent painful vaso-occlusive crises. An elevated estimated pulmonary arterial systolic pressure (ePASP) has emerged as a major predictor of morbidity and mortality in adults with SCD [1–3] at levels, estimated by tricuspid regurgitant (TR) jet velocity, that are not clinically significant in nonanemic patients. A TR jet velocity of 6 2.5 m/s is present at steady state in approximately 1/3 of SCD outpatients [2] , and is prospectively associated with increased mortality. In addition, mortality during long-term follow-up is increased in SCD patients who have short-term elevations in TR jetestimated PASP during an episode of acute chest syndrome [4] ; transient elevations in TR jet velocity have also been described during vaso-occlusive crises [5] , although the impact of these on long-term mortality is not known. We reasoned that an elevated TR jet, whenever measured (whether during disease activity or during clinical steady state), would be associated with a long-term risk of excess mortality in patients with SCD. Therefore, we exReceived: June 30, 2010 Accepted after revision: December 9, 2010 Published online: February 1, 2011

Abstract 1539 Poster Board I-562 Background Pulmonary arterial hypertension (PAH) has recently been shown to be an important predictor of prognosis in sickle cell disease (SCD). We studied this association in a large population of adults with SCD. Methods and Results 215 patients with sickle cell syndromes in whom echoes had been performed, 113 as outpatients and 102 as inpatients, were identified from our database. Clinical characteristics were analyzed, and survival data were extracted from a national mortality registry. At the time of examination, subjects were 39 ± 14 years old, with hematocrits (Hcts) of 23.6 ± 4.4. 78 out of 213 patients who had undergone echoes had PAH (PASP ≥ 40 mmHg) on echocardiogram. Those patients were older (41 ± 15 vs 37 ± 13 years, p=0.03), had higher creatinine values (1.3 vs 0.9 mg/dL, p=0.01) higher serum alkaline phosphatase levels (172.2 ± 23.2 vs 104.5 ± 5.5 U/L, p=.0058), and were more often taking anti-hypertensive medications (38 vs 17%, p<0.001). During a median follow-up of 7.8 years [interquartile range (IR); 3.9, 10.0] there were 30 deaths. In a multivariate Cox proportional hazards model, PAH (hazard ratio (HR) 11.3; 95% CI 2.7, 47.9), chronic kidney disease (CKD) (HR 17.0; 95% CI 3.7, 79.1, defined as an estimated glomerular filtration rate, eGFR, of <60 cc/min/1.73 m2) and hyperbilirubinemia (HR 13.1; 95% CI 2.0, 83.9) independently predicted mortality after adjustment for inpatient/outpatient status, age, sex, blood counts, measurement of chamber size and ventricular function on echocardiogram, oxygen saturation, history of transfusion and background medical therapy. The association of PAH and mortality was not modified by inpatient/outpatient status (p=0.6). Conclusion In a community-based academic center, we found that mild elevation in PASP on echocardiogram, whenever obtained (see figure), depressions in eGFR (see figure), and hyperbilirubinemia were highly predictive for mortality in sickle cell disease. It is unclear whether these relationships are causal or an epiphenomenon of severe underlying disease. Hemolysis (lower Hcts, higher Lactate dehydrogenase levels), implicated in other studies, was not prominent in the PAH cohort. ![][1] Disclosures No relevant conflicts of interest to declare. [1]: pending:yes