Last updated September 2, 2022

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

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).

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.

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

Overview

Title

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

Authoring Organization

Publication Month/Year

March 5, 2014

Supplemental Implementation Tools

Quick-Reference Guide

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

Supplemental Methodology Resources

Data Supplement