Hemodynamic Predictors of Mortality in Adults with Sickle Cell Disease

• Published in: American journal of respiratory and critical care medicine Vol. 187; no. 8; pp. 840 - 847
• Main Authors: MEHARI, Alem, ALAM, Shoaib, MINNITI, Caterina P, SACHDEV, Vandana, TAYLOR, James G, KATO, Gregory J, MACHADO, Roberto F, XIN TIAN, CUTTICA, Michael J, BARNETT, Christopher F, MILES, George, DIHUA XU, SEAMON, Catherine, ADAMS-GRAVES, Patricia, CASTRO, Oswaldo L
• Format: Journal Article
• Language: English
• Published: New York, NY American Thoracic Society 15.04.2013
• Subjects: Adult; Anemia; Anemia, Sickle Cell - complications; Anemia, Sickle Cell - mortality; Anemias. Hemoglobinopathies; Anesthesia. Intensive care medicine. Transfusions. Cell therapy and gene therapy; Autopsies; Biological and medical sciences; Cardiac Catheterization; Clinical Trials as Topic; Diseases of red blood cells; Echocardiography, Doppler; Female; Follow-Up Studies; Hematologic and hematopoietic diseases; Hemodynamics; Hemoglobin; Humans; Hypertension, Pulmonary - diagnosis; Hypertension, Pulmonary - etiology; Hypertension, Pulmonary - mortality; Intensive care medicine; Intubation; Kaplan-Meier Estimate; Logistic Models; Male; Medical prognosis; Medical sciences; Mortality; Mortality, Premature; Physiology; Pneumology; Proportional Hazards Models; Pulmonary arteries; Pulmonary hypertension; Pulmonary hypertension. Acute cor pulmonale. Pulmonary embolism. Pulmonary vascular diseases; Pulmonary Wedge Pressure - physiology; Risk Assessment; Risk factors; Sickle cell disease; Survival Rate; Veins & arteries; Human; Hemoglobinopathy; Intensive care; Sickle cell anemia; Prognosis; Respiratory disease; Mortality; Prediction; Cardiovascular disease; Hemopathy; Epidemiology; Pulmonary hypertension; Genetic disease; Hemolytic anemia; Autopsy; sickle cell; Adult; Hemodynamics; Predictive factor; Resuscitation
• ISSN: 1073-449X; 1535-4970
• DOI: 10.1164/rccm.201207-1222OC

Pulmonary hypertension (PH) in adults with sickle cell disease (SCD) is associated with early mortality, but no prior studies have evaluated quantitative relationships of mortality to physiological measures of pre- and postcapillary PH. To identify risk factors associated with mortality and to estimate the expected survival in a cohort of patients with SCD with PH documented by right heart catheterization. Nine-year follow-up data (median, 4.7 yr) from the National Institutes of Health SCD PH screening study are reported. A total of 529 adults with SCD were screened by echocardiography between 2001 and 2010 with no exclusion criteria. Hemodynamic data were collected from 84 patients. PH was defined as mean pulmonary artery pressure (PAP) ≥ 25 mm Hg. Survival rates were estimated by the Kaplan-Meier method, and mortality risk factors were analyzed by the Cox proportional hazards regression. Specific hemodynamic variables were independently related to mortality: mean PAP (hazard ratio [HR], 1.61; 95% confidence interval [CI], 1.05-2.45 per 10 mm Hg increase; P = 0.027), diastolic PAP (HR, 1.83; 95% CI, 1.09-3.08 per 10 mm Hg increase; P = 0.022), diastolic PAP - pulmonary capillary wedge pressure (HR, 2.19; 95% CI, 1.23-3.89 per 10 mm Hg increase; P = 0.008), transpulmonary gradient (HR, 1.78; 95% CI, 1.14-2.79 per 10 mm Hg increase; P = 0.011), and pulmonary vascular resistance (HR, 1.44; 95% CI, 1.09-1.89 per Wood unit increase; P = 0.009) as risk factors for mortality. Mortality in adults with SCD and PH is proportional to the physiological severity of precapillary PH, demonstrating its prognostic and clinical relevance despite anemia-induced high cardiac output and less severely elevated pulmonary vascular resistance.