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

Publication Date: March 5, 2014

Key Points

Key Points

Pulmonary hypertension (PH) and right heart failure are well-established risk factors for mortality in sickle cell disease (SCD).

Observational studies have consistently shown that increased tricuspid regurgitant jet velocity (TRV) measured by Doppler echocardiography, an increased serum N-terminal pro–brain natriuretic peptide (NT-pro-BNP) level, and pulmonary hypertension measured by right heart catheterization are all independent risk factors for mortality in adults.

To reduce the variability and to improve the quality of care that patients with SCD receive, the ATS developed clinical practice guidelines to advise hematologists, pulmonologists, cardiologists, pediatricians, and internists about how to identify and manage patients with SCD who are at increased risk for mortality.

Diagnosis

...agnosis...

...atification guides clinical decision making in S...


...risk for mortality is defined as a TRV ≥2.5...


...ple Questions for Evaluation of Dyspnea Do you g...


...ed Algorithm for Evaluation of Pulmo...


...Echocardiogram of a Patient with...


Treatment

Treatment

...ients with SCD who have an increased ri...


...th SCD who have an increased risk...


...ith SCD who have RHC-confirmed PH, venous...


...patients with SCD who have elevated TRV a...


...t patients with SCD who have RHC-confirmed PH...


...r select patients with SCD who have RHC-conf...


...tients with SCD who have RHC-confirmed marked el...


...ble 2. MedicationsHaving trouble v...