Prevalence of pulmonary hypertension in hereditary spherocytosis.

Abstract:

:Vascular complications, including pulmonary hypertension (PH), have been reported to occur following splenectomy for various disorders,including hereditary spherocytosis (HS). We performed a prospective cross-sectional study of 36 adults with HS (78% with prior splenectomy)utilizing echocardiography to estimate tricuspid regurgitant jet velocity (TRV) as well as measurement of N-terminal pro-B-type natriuretic peptide (NT-proBNP) to screen for PH. No participant with HS hada significantly elevated TRV or NT-proBNP level, despite a median 25-year interval since splenectomy (95% confidence interval for point prevalence 0, 0.097). Although our study was limited by a small sample size, it appears that persons with HS, following splenectomy, appear unlikely to be at significantly increased risk of developing PH to the degree reported for thalassemia and sickle cell disease

journal_name

Am J Hematol

authors

Crary SE,Ramaciotti C,Buchanan GR

doi

10.1002/ajh.22182

subject

Has Abstract

pub_date

2011-12-01 00:00:00

pages

E73-6

issue

12

eissn

0361-8609

issn

1096-8652

journal_volume

86

pub_type

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