What are the ASH guidelines on pulmonary hypertenstion (PH) screening and diagnosis in patients with sickle cell disease (SCD)?

Updated: Oct 25, 2022
  • Author: Joseph E Maakaron, MD; Chief Editor: Emmanuel C Besa, MD  more...
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Given the risk for cardiopulmonary disease in individuals with SCD, it is good practice to routinely take a targeted history for signs and symptoms that might indicate a need for further evaluation, including consideration for a diagnostic echocardiogram. [109] In asymptomatic children and adults with SCD, the ASH guideline panel suggests against performing a routine screening echocardiogram to identify pulmonary hypertension (PH). However, the following findings may warrant a consultation with a PH expert or a diagnostic echocardiogram to evaluate for PH:

  • Dyspnea, hypoxemia, or chest pain, at rest or with exertion, that is out of proportion to known condition, increased compared with baseline, or unexplained
  • Increase in exercise limitation compared with baseline that is unexplained by other factors
  • History of recurrent hypoxemia at rest or with exertion
  • Evidence for sleep-disordered breathing with or without hypoxemia
  • History of syncope or presyncope
  • Loud P2 component of second heart sound or unexpected or new murmur on examination
  • Signs of heart failure and/or fluid overload on examination
  • History of pulmonary embolism

In addition, a diagnostic echocardiogram should be considered for patients with SCD who also have comorbid conditions (eg, connective tissue disease) or disease complications (eg, leg ulcers, priapism) known to be associated with PH, when signs or symptoms of PH are present.

The ASH panel considers it good practice to obtain echocardiograms at steady state and not during acute illness, such as hospitalization for pain or acute chest syndrome, when the results will be used as the basis for decisions about the need for right-heart catheterization.

Recommendations for patients with an abnormal echocardiogram are as follows:

  • For asymptomatic children and adults with SCD and an isolated peak tricuspid regurgitant jet velocity (TRJV) of 2.5 to 2.9 m/sec, the ASH guideline panel suggests against right-heart catheterization.
  • For patients with peak TRJV of ≥2.5 m/sec who are asymptomatic, measurement of N-terminal pro-B-type natriuretic peptide (NT-BNP) levels and 6-minute walk distance (6MWD) may help to improve the diagnostic accuracy for PH. Cutoff values for abnormal NT-BNP and 6MWD have not been firmly determined for patients with SCD. However, NT-BNP values of ≥160 pg/mL and 6MWD values of < 333 m represent reasonable thresholds for adults with SCD, based on published studies in this population. Referrals for right-heart catheterization should also account for clinical judgment and discussion with a PH expert.
  • For children and adults with SCD and a peak TRJV of ≥2.5 m/sec who also have a reduced 6MWD and/or elevated NT-BNP, the ASH guideline panel suggests right-heart catheterization.
  • Patients whose echocardiograms demonstrate elevated peak TRJV should undergo repeat echocardiography prior to referral for right-heart catheterization under the guidance of a PH expert, because reproducibility of TRJV measurements may vary due to technical factors, severity of anemia, or increased cardiac output.
  • For patients with peak TRJV of ≥2.5 m/sec who have normal 6MWD and NT-BNP, serial noninvasive monitoring with echocardiograms should be considered if clinically indicated.
  • Consultation with a PH expert regarding the need for a right-heart catheterization should be considered for patients with TRJV of >2.9 m/sec who have normal 6MWD and NT-BNP or other echocardiographic findings, in addition to elevated peak TRJV, that could suggest significant PH (eg, right atrial enlargement, pericardial effusion, right ventricular failure, or septal flattening).
  • PH is defined hemodynamically by right-heart catheterization using a mean pulmonary artery pressure threshold of > 20 mm Hg (recently reduced from ≥25 mm Hg). However, the mean pulmonary artery pressure alone does not distinguish pulmonary artery hypertension (PAH) from other forms of PH. Additional criteria for the diagnosis of PAH include a pulmonary artery wedge pressure of ≤15 mm Hg and a pulmonary vascular resistance of ≥3 Wood units (240 dyn × seconds × cm −5).

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