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.
University of South Alabama Medical Center, Mobile, AL 36617, USA. fitzgerald@usouthal.edu
Meshann
M
Fitzgerald
Karen
K
Fagan
Donald E
DE
Herbert
Mohammad
M
Al-Ali
Majid
M
Mugal
Johnson
J
Haynes
D000328:Adult; D000755:Anemia, Sickle Cell / Q000000981:diagnostic imaging*; D006328:Cardiac Catheterization; D003951:Diagnostic Errors; D015150:Echocardiography, Doppler; D005260:Female; D006453:Hemoglobinopathies / Q000000981:diagnostic imaging*; D006801:Humans; D006976:Hypertension, Pulmonary / Q000000981:diagnostic imaging*; D016014:Linear Models; D008297:Male; D011651:Pulmonary Artery / Q000000981:diagnostic imaging*; D011669:Pulmonary Wedge Pressure; D012189:Retrospective Studies; D012680:Sensitivity and Specificity; D014262:Tricuspid Valve Insufficiency / Q000000981:diagnostic imaging*