Pulmonary hypertension is present in 10-30% of adult patients with sickle cell disease

Pulmonary hypertension (PH) is a serious complication of sickle cell disease (SCO). We sought to determine its frequency and which patients are at higher risk. We reviewed the records of 414 adult patients with SCO seen at an urban academic medical center during a 9-year period. 152 patients had at least one echocardiogram. PH was judged to be present if there was evidence of right ventricle pressure overload (i.e., dilation of the right ventricle with paradoxical motion of the ventricular septum) and peak tricuspid valve régurgitant jet velocity 2,5 m/s. Fifty (32.9%) patients with SCO undergoing echocardiography had evidence of PH. Mortality was significantly greater among patients with PH than those without (22 of 50 [44%] vs. 17 of 102 [17%]; P = 0.0003). In addition, hemoglobin was lower (7.6g/dL vs. 8.3g/dL P=0.02) and pulse-ox O; saturation was lower (74.4% vs. 90.4%; P=0.07). Over 90% of our patients with PH had sickle cell anemia (SS). There were no statistically significant differences between the two groups (pulmonary hypertension vs not) in age (mean 37+10 years vs. 34±11 years), gender (male/female: 19/ 31 vs. 45/57), or left ventricle dysfunction (5 of 50 vs. 10 of 102). Left ventricle dilation (16 of 50 vs. 21 of 102) had a tantalizing but not statistically significant p value of p=0.12. 44% of patients with PH had died by the time the records were reviewed. For those that died, median survival from the time of diagnosis of PH was 2 months (range: 1 to 46 months), The short survival time is partly due to echocardiograms that were performed for the first time in patients who died in the hospital from PH. A life table analysis for all patents with PH is in progress starting with the date of the first echocardiogram. In this population, PH was found in over 30% of patients who had an echocardiogram. While there may be selection bias in patients selected for echocardiogram testing, PH was found in over 10% of the total population even though only 37% were screened. The true frequency is therefore likely between 10 and 30%. Patients with lower Hg and oxygen saturations may be at particular risk. Given the high incidence we have begun screening our entire patient population. Early detection for pulmonary hypertension may be warranted for all patients with sickle cell disease.