Misclassification of pulmonary hypertension in adults with sickle hemoglobinopathies using Doppler echocardiography.

Objective: To compare the diagnostic utility of Doppler echocardiography-derived tricuspid regurgitant jet velocity (TRV) >= 2.5 m/s to right heart catheterization (RHC) in defining pulmonary hypertension (PH) in adult patients with sickle cell disease (SCD). Methods: This is a retrospective chart review of adults with SCD who had a TRV >= 2.5 m/s and RHC. A TRV >= 2.5 m/s is suggestive of PH. Pulmonary arterial hypertension (PAH) was defined as a mean pulmonary artery pressure (mPAP) >= 25 mm Hg and pulmonary capillary wedge pressure <= 15 mm Hg. Pulmonary venous hypertension was defined as an mPAP >= 25 mm Hg and pulmonary capillary wedge pressure >15 mm Hg. Results: Twenty-five patients with SCD met the inclusion criteria. Nine of the 25 (36%) patients had an mPAP >= 25 mm Hg. Of these 9, 3 (33%) had PAH and 6 (66%) had pulmonary venous hypertension. Patients with PH did not have a higher TRV (3.1 +/- 0.68 vs 2.70 +/- 0.16 m/s; P = 0.12), but they did have higher cardiac outputs (10.4 +/- 2.7 vs 7.81 +/- 1.85 L/min; P = 0.012. The specificity of TRV equal to 2.51 m/s in diagnosing PH was 18.8%. At a TRV of 2.88 m/s, the specificity increased to 81%. Conclusions: In adults with SCD, a TRV of 2.5 m/s lacks specificity for use as a screening tool in the diagnosis of PH. Using a TRV of >= 2.88 m/s allows the TRV to be used as a screening tool and reduces the false-positive rate and need for unnecessary RHC.