Pulmonary hypertension in sickle cell disease: cardiac catheterization results and survival

Abstract

Few results on cardiac catheterization have been published for patients with sickle cell disease (SCD) with pulmonary hypertension (PHTN). Their survival once this complication develops is unknown. We analyzed hemodynamic data in 34 adult patients with SCD at right-sided cardiac catheterization and determined the relationship of PHTN to patient survival. In 20 patients with PHTN the average systolic, diastolic, and mean pulmonary artery pressures were 54.3, 25.2, and 36.0 mm Hg, respectively. For 14 patients with SCD without PHTN these values were 30.3, 11.7, and 17.8 mm Hg, respectively. The mean pulmonary capillary wedge pressure in patients with PHTN was higher than that in patients without PHTN (16.0 versus 10.6 mm Hg; P ‫؍‬ .0091) even though echocardiography showed normal left ventricular systolic function. Cardiac output was high (8.6 L/min) for both groups of patients. The median postcatheterization follow-up was 23 months for patients with PHTN and 45 months for those without PHTN. Eleven patients (55%) with PHTN died compared to 3 (21%) patients without PHTN ( 2 ‫؍‬ 3.83; P ‫؍‬ .0503). The mean pulmonary artery pressure had a significant inverse relationship with survival (Cox proportional hazards modeling). Each increase of 10 mm Hg in mean pulmonary artery pressure was associated with a 1.7-fold increase in the rate (hazards ratio) of death (95% CI ‫؍‬ 1.1-2.7; P ‫؍‬ .028). The median survival for patients with PHTN was 25.6 months, whereas for patients without PHTN the survival was still over 70% at the end of the 119-month observation period (P ‫؍‬ .044, Breslow-Gehan logrank test). Our findings suggest that PHTN in patients with SCD shortened their sur-

Echocardiographic abnormalities in sickle cell disease

“…It is associated with impaired exercise tolerance, progressive heart failure, and a high mortality [3,[11][12][13]. Therapies that are effective in primary pulmonary hypertension should be considered in symptomatic SCD patients with severe pulmonary hypertension [18].…”

Evolution of sickle cell disease from a life‐threatening disease of children to a chronic disease of adults: The last 40 years

“…Over a third of adults with SCD have elevated tricuspid jet velocity on Doppler echocardiography [85]. Individuals with SCD with elevated tricuspid regurgitant jet velocity (!3.0 cm/sec) have a 10-fold higher mortality rate than patients with SCD without elevated tricuspid regurgitant jet velocity [85,[88][89][90]. N-terminal probrain natriuretic peptide (NT-ProBNP) is also associated with catheterization detected pulmonary hypertension, and was identified as an independent predictor of death in the CSSCD and MSH cohorts [91,92].…”

Uric Acid as a Potential Biomarker of Pulmonary Arterial Hypertension in Patients with Sickle Cell Disease

“…Pulmonary complications, acute chest syndrome (ACS) and PAH account for a large proportion of deaths in adults with SCD [3,4]. One retrospective study reported a median survival of 25.6 months for SCD patients with PAH confirmed by right heart catheterization [5]. Autopsy studies revealed that up to 75% of SCD patients have histological evidence of PAH [6].…”