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Echocardiographic Evaluation of Patients With Sickle Cell Disease FREE TO VIEW

Sikandar Ansari, MD; Muhammad Usman, MD; Bhavin Dalal, MD; Balaji Karthik, MD; Littsey Lisabette, NP; Paul Swerdlow, MD; Kamal Mubarak, MD; Ghulam Saydain, MD
Chest. 2011;140(4_MeetingAbstracts):729A. doi:10.1378/chest.1118585
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PURPOSE: Pulmonary hypertension(PH) is defined as right ventricular systolic pressure (RVSP) > 40 mmHg by echocardiography (ECHO). However, mortality in sickle cell disease (SCD) has been linked to tricuspid regurgitant velocity (TRV) > 2.5m/sec. The purpose of the study was to find an association of RVSP and TRV with mortality in SCD.

METHODS: We reviewed the medical records and ECHO data of SCD patients seen in the SCD and/or PH clinic at Detroit Medical Center, Wayne State University.

RESULTS: Out of 293 patients screened, 250 [median age, 35 {range 17-78} years, 56 % women) who had ECHO performed between June 2004 and April 2011, were included. Over all mortality during this period was 14%. Dilatation of right atrium (RA) (OR 7.42 CI 3.47-15.87, p<0.001) or right ventricle (RV) (OR 6.71 CI 2.94 -15.34 p< 0.001) or left atrium (LA) (OR 2.31, CI 1.13- 4.74, p= 0.02) or left ventricle (LV) ( OR 2.57 CI 1.03-6.36, p=0.041) were associated with mortality. Sensitivity and specificity of predicting mortality for chamber dilatation were 58 & 84% for RA, 39 & 91% for RV, 50 and 69% for LA, and 22 & 90% for LV. RVSP (n=190) (OR 1.064, CI 1.03-1.09, P < 0.001) and TRV (n=92) (OR 1.02, CI 1.008-1.03 p=0.001) were associated with mortality. Mean RVSP and TRV for survivors and non-survivors were (38.4±13.2 vs 56.5±19.8 mmHg p < 0.001) & (2.62±0.59 vs 3.25±1.29 m/sec p <0.001) respectively. Sensitivity and specificity for predicting mortality for RVSP were 96 and 28% for >30, 79 and 71% for >40, 59 and 82% for >45 mmHg. For TRV sensitivity and specificity were 100 and 45% for >2.50, 83 and 71% for >2.80 and 75 and 78% for >3 m/sec respectively. Mortality amongst patients with RVSP>40 or TRV>2.8m/sec vs those patients with RVSP<40 or TRV<2.8m/sec was 29.1vs 3.8 % (p <0.001) and 30.3vs 3.4 %, (p <0.001) respectively.

CONCLUSIONS: PH in SCD is associated with higher mortality. Dilatation of either RA or RV is associated with mortality but lacks sensitivity and is generally a late manifestation. A cut off point at 2.5 m/sec for TRV is sensitive but lacks specificity. RVSP > 40 mmHg or TRV > 2.8m/sec is associated with higher mortality with acceptable sensitivity and specificity.

CLINICAL IMPLICATIONS: In SCD, PH defined as RVSP > 40 mmHg or TRV >2.8m/sec is likely to identify patients at risk for high mortality who may benefit from therapy.

DISCLOSURE: The following authors have nothing to disclose: Sikandar Ansari, Muhammad Usman, Bhavin Dalal, Balaji Karthik, Littsey Lisabette, Paul Swerdlow, Kamal Mubarak, Ghulam Saydain

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