Summary

ASH 2019 guidelines on cardiopulmonary and kidney disease in sickle cell disease (SCD) address screening and management of pulmonary hypertension, sleep-disordered breathing, abnormal pulmonary function, venous thromboembolism, albuminuria, and chronic kidney disease. The panel generally recommends against routine screening of asymptomatic patients with echocardiography, polysomnography, or pulmonary function testing, favoring targeted evaluation based on signs and symptoms. Recommendations also address anticoagulation duration for VTE, ACEi/ARB use for albuminuria, renal transplant referral, and combined hydroxyurea plus erythropoiesis-stimulating agent therapy for CKD-related anemia.

Key Recommendations

Do not perform routine echocardiogram to screen for pulmonary hypertension in asymptomatic children and adults with SCD.
Obtain diagnostic ECHO or PH expert consultation in SCD patients with unexplained dyspnea, hypoxemia, chest pain, syncope, exercise limitation, signs of heart failure, or history of pulmonary embolism.
Use a multidisciplinary approach (hematology, PH specialist, pulmonary, or cardiology) and right-heart catheterization before initiating PAH-specific therapy.
Do not screen asymptomatic SCD patients with routine polysomnography; obtain sleep study based on symptoms such as snoring, witnessed apneas, nocturnal enuresis ≥10 years, recurrent priapism, or unexplained desaturation.
Do not perform routine pulmonary function testing in asymptomatic SCD patients; reserve PFT (full spirometry, diffusion capacity, lung volumes) for symptomatic patients or those with recurrent acute chest syndrome or PE.
Anticoagulate indefinitely for first unprovoked VTE or recurrent provoked VTE in SCD; treat first provoked VTE for a defined 3–6 month course.
Consider bleeding risk (including concurrent NSAID use) and renal impairment when selecting anticoagulant in SCD patients.
Use ACEi or ARB for albuminuria in SCD, starting at lower dose if GFR <45 mL/min/1.73m², with potassium and GFR checked within 1 week of initiation or dose escalation.
Temporarily suspend ACEi/ARB during intercurrent illness, IV contrast administration, bowel prep, or before major surgery.
Refer SCD patients with advanced CKD or ESRD for renal transplant, using judicious post-transplant steroids and adhering to perioperative transfusion guidelines.
Treat worsening CKD-related anemia in SCD with combined hydroxyurea and erythropoiesis-stimulating agents, particularly when hemoglobin and absolute reticulocyte count both fall.
Do not exceed a hemoglobin threshold of 10 g/dL (Hct 30%) when using erythropoiesis-stimulating agents to reduce vaso-occlusion, stroke, and VTE risk.

Thresholds & Doses

Hemoglobin ceiling on ESA therapy: 10 g/dL (Hct 30%) — hold or decrease dose above this.
ACEi/ARB initiation: start at lower dose if GFR <45 mL/min/1.73m².
Monitoring after ACEi/ARB start or dose escalation: check GFR and serum potassium within 1 week.
First provoked VTE: anticoagulate for defined period of 3–6 months.
First unprovoked VTE or recurrent provoked VTE: indefinite anticoagulation.
Nocturnal enuresis warranting sleep study: age ≥10 years.

Citations

Pulmonary Hypertension: Screening — recommendation against routine ECHO in asymptomatic SCD patients.
PAH Treatment / Figure 2 — multidisciplinary approach and WHO PH group classification (SCD = Group 5).
Sleep-Disordered Breathing: Screening — against routine polysomnography; symptom-based indications.
Pulmonary Function Screening — against routine PFT; comprehensive PFT includes spirometry, DLCO, lung volumes.
VTE Management table — anticoagulation durations for provoked vs unprovoked, first vs recurrent VTE.
Albuminuria Management — ACEi/ARB dosing, monitoring, and suspension recommendations.
Renal Transplant for ESRD — referral recommendation with perioperative transfusion and steroid caution.
Hydroxyurea and Erythropoiesis-Stimulating Agents — combination therapy with Hb ceiling of 10 g/dL.