Key Points
Î Pulmonary hypertension (PH) and right heart failure are well-established
risk factors for mortality in sickle cell disease (SCD).
Î Observational studies have consistently shown that increased tricuspid
regurgitant jet velocity (TRV) measured by Doppler echocardiography, an
increased serum N-terminal pro–brain natriuretic peptide (NT-pro-BNP)
level, and pulmonary hypertension measured by right heart catheterization
are all independent risk factors for mortality in adults.
Î To reduce the variability and to improve the quality of care that patients
with SCD receive, the ATS developed clinical practice guidelines to advise
hematologists, pulmonologists, cardiologists, pediatricians, and internists
about how to identify and manage patients with SCD who are at increased
risk for mortality.
Diagnosis
Î Risk stratification guides clinical decision making in SCD:
• Mortality risk can be accurately determined by noninvasive measurement of the TRV via
Doppler echocardiography. (Note: In children ≥8 yr old the TRV determines morbidity
risk, rather than mortality risk, and provides a baseline for future comparisons.)
• Serum NT-pro-BNP measurement is a reasonable noninvasive alternative when Doppler
echocardiography is either unavailable or cannot obtain adequate images.
Note: Measurements may be misleading in patients with renal insufficiency.
• Mortality risk can also be determined invasively by direct hemodynamic measurements
via right heart catheterization (RHC).
Î An increased risk for mortality is defined as a TRV ≥2.5 m/second, an
NT-pro-BNP level ≥160 pg/ml, or RHC-confirmed PH (a resting mean
pulmonary arterial pressure ≥25 mm Hg). Hemodynamics in PH of SCD
may be consistent with pre- or postcapillary PH or have features of both.
