Diagnosis, Risk Stratification, And Management Of Pulmonary Hypertension Of Sickle Cell Disease
Publication Date: March 5, 2014
Diagnosis
Risk stratification guides clinical decision making in SCD:
- Mortality risk can be accurately determined by noninvasive measurement of the TRV via Doppler echocardiography.
- 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).
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.
may be consistent with pre- or postcapillary PH or have features of both.
Treatment
For patients with SCD who have an increased risk for mortality, the ATS recommends hydroxyurea. (S, M)
620
For patients with SCD who have an increased risk for mortality and who either are not responsive to or not candidates for hydroxyurea, the ATS suggests chronic transfusion therapy. (, L)
weak recommendation
620
For patients with SCD who have RHC-confirmed PH, venous thromboembolism, and no additional risk factors for hemorrhage, the ATS suggests indefinite anticoagulant therapy rather than a limited duration of therapy. (, L)
weak recommendation
620
For all patients with SCD who have elevated TRV alone or elevated NT-pro- BNP alone, the ATS recommends against targeted PAH therapy. (S, M)
Targeted PAH therapy currently includes prostacyclin agonist, endothelin receptor antagonist, and phosphodiesterase-5 inhibitor therapy.
620
For most patients with SCD who have RHC-confirmed PH, the ATS recommends against targeted PAH therapy. (S, M)
620
For select patients with SCD who have RHC-confirmed marked elevation of their pulmonary vascular resistance, normal pulmonary artery wedge pressure, and related symptoms, the ATS suggests a trial of either a prostacyclin agonist or an endothelin receptor antagonist (, VL)
weak recommendation
620
For patients with SCD who have RHC-confirmed marked elevation of their pulmonary vascular resistance, normal pulmonary artery wedge pressure, and related symptoms the ATS recommends against phosphodiesterase-5 inhibitor therapy as a first-line agent (S, M)
620
Recommendation Grading
Disclaimer
Overview
Title
Diagnosis, Risk Stratification, And Management Of Pulmonary Hypertension Of Sickle Cell Disease.
Authoring Organization
American Thoracic Society
Publication Month/Year
March 5, 2014
Supplemental Implementation Tools
Document Type
Guideline
External Publication Status
Published
Country of Publication
US
Target Provider Population
Hematologists, pulmonologists, cardiologists, pediatricians, and internists
Inclusion Criteria
Male, Female, Adolescent, Adult, Child, Older adult
Health Care Settings
Ambulatory
Intended Users
Nurse, nurse practitioner, physician, physician assistant
Scope
Diagnosis, Treatment
Diseases/Conditions (MeSH)
D006976 - Hypertension, Pulmonary, D000755 - Anemia, Sickle Cell
Keywords
sickle cell disease, pulmonary hypertension, SCD, PAH