Faculty Collaboration Database

Increased prevalence of potential right-to-left shunting in children with sickle cell anaemia and stroke. Br J Haematol 2017 Jan;176(2):300-308

Date

10/22/2016

Pubmed ID

27766637

Pubmed Central ID

PMC5239723

DOI

10.1111/bjh.14391

Scopus ID

2-s2.0-84995483170 (requires institutional sign-in at Scopus site)   30 Citations

Abstract

'Paradoxical' embolization via intracardiac or intrapulmonary right-to-left shunts (RLS) is an established cause of stroke. Hypercoagulable states and increased right heart pressure, which both occur in sickle cell anaemia (SCA), predispose to paradoxical embolization. We hypothesized that children with SCA and overt stroke (SCA + stroke) have an increased prevalence of potential RLS. We performed contrasted transthoracic echocardiograms on 147 children (aged 2-19 years) with SCA + stroke) mean age 12·7 ± 4·8 years, 54·4% male) and a control group without SCA or stroke (n = 123; mean age 12·1 ± 4·9 years, 53·3% male). RLS was defined as any potential RLS detected by any method, including intrapulmonary shunting. Echocardiograms were masked and adjudicated centrally. The prevalence of potential RLS was significantly higher in the SCA+stroke group than controls (45·6% vs. 23·6%, P < 0·001). The odds ratio for potential RLS in the SCA + stroke group was 2·7 (95% confidence interval: 1·6-4·6) vs controls. In post hoc analyses, the SCA + stroke group had a higher prevalence of intrapulmonary (23·8% vs. 5·7%, P < 0·001) but not intracardiac shunting (21·8% vs. 18·7%, P = 0·533). SCA patients with potential RLS were more likely to report headache at stroke onset than those without. Intrapulmonary and intracardiac shunting may be an overlooked, independent and potentially modifiable risk factor for stroke in SCA.

Author List

Dowling MM, Quinn CT, Ramaciotti C, Kanter J, Osunkwo I, Inusa B, Iyer R, Kwiatkowski JL, Johnson C, Rhodes M, Owen W, Strouse JJ, Panepinto JA, Neumayr L, Sarnaik S, Plumb PA, Dlamini N, Kirkham F, Hynan LS, PFAST Investigators

MESH terms used to index this publication - Major topics in bold

Adolescent
Anemia, Sickle Cell
Child
Child, Preschool
Echocardiography
Embolism, Paradoxical
Female
Headache
Heart Septal Defects
Humans
Male
Prevalence
Risk Factors
Stroke
Young Adult