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Misclassification of Pulmonary Hypertension in Adults with Sickle Hemoglobinopathies Using Doppler Echocardiography

Meshann Fitzgerald, MD, MPH, Karen Fagan, MD, Donald E. Herbert, PhD, Mohammad Al-Ali, MD, Majid Mugal, MD, Johnson Haynes Jr, MD
Volume: 105 Issue: 6 June, 2012

Abstract:

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.

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