PURPOSE: Limited data are available regarding the hemodynamic features of patients with sickle cell disease (SCD) related pulmonary hypertension (PH). The purpose of this study was to describe the findings on right heart catheterization (RHC) of SCD patients and any correlation of hemodynamic data (HD) with mortality.
METHODS: We reviewed the medical records and HD of consecutive SCD patients who underwent RHC between April 2003 and March 2010. We defined PH as mean pulmonary artery pressure (MPAP) >25 mmHg. Pulmonary vascular resistance (PVR) was calculated.
RESULTS: Of the 42 patients (mean age 43±11 years, 18 men) included in the study, 32 had SS genotype. Many patients had one or more co-morbid conditions and major conditions included chronic kidney disease (CKD) 17 (40%), hypertension 15 (36%), cardiovascular disease 13 (30%), non-vascular pulmonary disease 11 (26%), stroke 11 (26%), and liver disease 10 (24%). Twenty-two (52%) patients had PH. Of these pulmonary artery occlusion pressure (PAOP) was <15mmHg in 7 and >15mmHg in 15 patient; 9 patients had PVR >240 dyn°s°cm−5. PH tended to be more frequent with CKD (71vs41%,p=0.05) or liver disease(67vs49%,p=0.2). MPAP tended to be higher with liver disease (33.2±13.9 vs26.7±10.0 mmHg p=0.2) and CKD (30.1±12.1vs 26.8±10.4 mmHg, p=0.3). Overall mortality during the study period was 29%. PH was more common among non-survivors (75vs43%, p=0.06) who compared to survivors tended to have higher MPAP (32.6±14.1vs26.3±9.3 mmHg, p=0.1), right atrial pressure (11.3±8.3vs9.2±4.4 mmHg, p=0.4) transpulmonary gradient (16.8±10.5vs12.6±6.1mmHg, p=0.2) and PVR (253±207vs188±102 dyn°s°cm−5, p=0.3), while as PAOP (15.9±8.0vs15.3±7.3mmHg, p= 0.8), cardiac output (5.87±1.98 vs5.86±2.09L/min, p=0.9) and cardiac index (3.16±1.07vs3.27±1.07 L/min/m2, p=0.7) were similar.
CONCLUSIONS: PH in SCD may be associated with CKD or liver disease. Many patients with PH had low PVR. More than half of the patients with PH had left ventricular dysfunction (LVD). PH appears to be more common among non-survivors who tend to have higher pressures. Larger hemodynamic studies are needed to characterize the relationship of hemodynamics with outcome.
CLINICAL IMPLICATIONS: SCD patients with CKD or liver disease need to be monitored more closely for development of PH. PH in SCD patients is often associated with LVD; therapy of underlying cause like hypertension and optimization of left ventricular function is likely to improve pulmonary pressures.
DISCLOSURE: The following authors have nothing to disclose: Sikandar Ansari, Muhammad Usman, Ashis Bharghava, Bhavin Dalal, Littsey Lisabette, Paul Swerdlow, Kamal Mubarak, Ghulam Saydain
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