PURPOSE: Pulmonary hypertension has been shown to increase mortality in sickle cell disease. However most studies have utilized non-invasive echocardiogram and hemodynamic confirmation has been elusive. Recent reports of occlusion pressure misclassifying patients as pulmonary artery hypertension have further magnified the need for hemodynamic data validation. We hypothesized that the prevalence of true PAH by hemodynamic criteria would be significantly less than the traditional echocardiogram based diagnosis. We also hypothesized that measuring left ventricular end diastolic pressure would help avoid over diagnosis. We also looked at factors which are associated with PAH in sickle cell disease(SSD)
METHODS: We reviewed hemodynamic data on 22 patients with sickle cell disease who underwent gold standard right heart catheterization for evaluation of pulmonary hypertension
RESULTS: Out of 22 patients with echocardiogram diagnosed pulmonary hypertension 6 patients (27%) had PAH based on right heart catheterization (RHC) criteria utilizing pulmonary artery occlusion pressure. However when Left ventricular end diastolic pressure criteria (LVEDP)was used only 2/22 (9%) patients had PAH. False positive rate using occlusion pressure was 67% (95% CI 0.22-0.96). Occlusion pressure consistently underestimated LVEDP (11.74 vs 16.20 (95% CI 10.1-13.3 Vs 14.1-17.9) with p value of 0.0002. This difference persisted even in patients with RHC proven PAH. Examining several independent factors, only low albumin was associated with PAH
CONCLUSIONS: These findings suggest the true prevalence of PAH in sickle cell disease may be much lower than originally believed. Pulmonary artery occlusion pressure consistently underestimated LVEDP and resulted in 66% of cases being misclassified.
CLINICAL IMPLICATIONS: This data suggests that LVEDP may be a better tool to diagnose true PAH in SSD patients and better guide therapy.
DISCLOSURE: The following authors have nothing to disclose: Sunil Sharma, Renuka Kadali, Ramesh Daggubati, Jimmy Efird, Hadi Chohan, Mark Mazer, Charles Knupp, Peter Boettger, Darla Liles
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