Table 2.

Suggested approach to screening, diagnosis and management of pulmonary hypertension in SCD

Screening for pulmonary hypertension by echocardiography Perform echocardiography every 1 to 3 years OR Perform echocardiography based on one or more findings consistent with increased risk - Dyspnea on exertion - Limited exercise capability as determined by the 6-minute walk test (<350 m) - History of thromboembolism - Pulse oximetry <95% at rest - Previous echocardiogram with TRV of >2.5 m/sec - Physical exam finding of right sided heart failure; lower extremity edema, hepatomegaly or jugular venous distension - Elevated serum N- Terminal proBNP concentration - Elevated serum creatinine concentration (>1.0 mg/dL for severe sickling phenotype and >1.4 mg/dL for mild sickling phenotype) - LDH >475 U/L or reticulocyte count >300,000 - Serum ferritin >1000 ng/ml Actions to be taken based on the results of the echocardiography TRV <2.5 m/sec: Ensure that patient is being managed according to NHLBI guidelines TRV 2.5–2.9 m/sec; normal right ventricle by echocardiography, no findings suggestive of pulmonary hypertension: Ensure that patient is being managed according to NHLBI guidelines and repeat echocardiogram yearly TRV 2.5–2.9 m/sec; right ventricular hypertrophy by echocardiography and/or other findings consistent with increased pulmonary hypertension risk: Ensure that patient is being managed according to NHLBI guidelines; refer to pulmonary hypertension expert for right heart catheterization TRV >3 m/sec: Ensure that patient is being managed according to NHLBI guidelines and refer to pulmonary hypertension expert for right heart catheterization Actions to be taken based on the results of the right heart catheterization mPAP <25 mm Hg: Pulmonary hypertension not present. Ensure that patient is being managed according to NHLBI guidelines and repeat echocardiogram yearly mPAP >25 mm Hg and PCWP <15 mm Hg: precapillary pulmonary hypertension - Perform ventilation/perfusion scan of the lung and consider long-term anticoagulation if segmental defect found - Refer for overnight polysomnography to evaluate for sleep disordered breathing include obstructive sleep apnea; provide appropriate intervention if present - Chronic low-flow oxygen by nasal cannula if pulse ox <90% - Refer to pulmonary hypertension expert for follow-up and consideration whether endothelin receptor blocker or prostacyclin agent should be attempted - Consider initiating therapy with hydroxyurea if patient is not receiving this medication or with exchange blood transfusion program if patient does not tolerate or respond to hydroxyurea - Consider allogenic hematopoietic stem cell transplantation with low intensity conditioning regimen if patient has HLA matched sibling. mPAP >25 mm Hg and PCWP >15 mm Hg: postcapillary pulmonary hypertension - Refer to cardiologist to evaluate for left ventricular systolic or diastolic dysfunction; manage according to established guidelines for left ventricular failure - Consider initiating therapy with hydroxyurea if patient not receiving this medication or with exchange blood transfusion program if patient does not tolerate or respond to hydroxyurea - Consider allogenic hematopoietic stem cell transplantation with low intensity conditioning regimen if patient has HLA matched sibling

^[82]Adapted from Gordeuk et al. Blood 2016; 127(7): 820–828