Diagnosis
Figure 1. Proposed Algorithm for Evaluation of
Pulmonary Hypertension Related to Sickle Cell Disease
TRV ≤2.5 m/sec
Consider increased frequency
of screening and escalation of
SCD specific therapies
Screening Echocardiography
a
TRV 2.5-2.9 m/sec TRV≥3.0 m/sec
Symptoms of PH,
Decreased 6MWD,
Elevated NT-pro-BNP
Continue routine
screening
Right heart
catheterization
mPAP ≥25 mm Hg
PAWP ≤15 mm Hg
PVR ≥160 dynes
˙
sec/cm
5
Pre-capillary
PH
mPAP ≥25 mm Hg
PAWP >15 mm Hg
PVR <160 dynes
˙
sec/cm
5
Post-
capillary PH
mPAP <25 mm Hg
No PH
Increased frequency
of screening
ANA, HIV, LFTs, CXR, EKG
Pulmonary Function Testing
Ventilation Perfusion Scan
Sleep Study
Referral to PH/SCD Center
Consider PAH
erapy
b
a
Note: e use of the term "screening" refers to mortality risk assessment. Echocardiography should be
performed while patients are clinically stable. Patients with an mPAP between 20 and 25 mm Hg need
further study as they may be at increased mortality risk.
b
Note: PAH therapy is to be considered on the basis of a weak recommendation and very low-quality
evidence.
