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Diagnosis, Risk Stratification, And Management Of Pulmonary Hypertension Of Sickle Cell Disease

Publication Date: March 5, 2014

Last Updated: September 2, 2022

Diagnosis

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).

Treatment

For patients with SCD who have an increased risk for mortality, the ATS recommends hydroxyurea. (S, M)

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Overview

Title

Diagnosis, Risk Stratification, And Management Of Pulmonary Hypertension Of Sickle Cell Disease.

Authoring Organization