Increased prevalence of potential right-to-left shunting in children with sickle cell anaemia and stroke

Michael M. Dowling, Charles T. Quinn, Claudio Ramaciotti, Julie Kanter, Ifeyinwa Osunkwo, Baba Inusa, Rathi Iyer, Janet L. Kwiatkowski, Clarissa Johnson, Melissa Rhodes, William Owen, John J. Strouse, Julie A. Panepinto, Lynne Neumayr, Sharada Sarnaik, Patricia A. Plumb, Nomazulu Dlamini, Fenella Kirkham, Linda S. Hynan

Research output: Contribution to journalArticle

9 Citations (Scopus)

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.

Original languageEnglish (US)
JournalBritish Journal of Haematology
DOIs
StateAccepted/In press - 2016

Fingerprint

Sickle Cell Anemia
Stroke
Control Groups
Headache
Odds Ratio
Confidence Intervals
Pressure

Keywords

  • Cardiology
  • Clinical research
  • Sickle cell anaemia
  • Stroke

ASJC Scopus subject areas

  • Hematology

Cite this

Increased prevalence of potential right-to-left shunting in children with sickle cell anaemia and stroke. / Dowling, Michael M.; Quinn, Charles T.; Ramaciotti, Claudio; Kanter, Julie; Osunkwo, Ifeyinwa; Inusa, Baba; Iyer, Rathi; Kwiatkowski, Janet L.; Johnson, Clarissa; Rhodes, Melissa; Owen, William; Strouse, John J.; Panepinto, Julie A.; Neumayr, Lynne; Sarnaik, Sharada; Plumb, Patricia A.; Dlamini, Nomazulu; Kirkham, Fenella; Hynan, Linda S.

In: British Journal of Haematology, 2016.

Research output: Contribution to journalArticle

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 2016, 'Increased prevalence of potential right-to-left shunting in children with sickle cell anaemia and stroke', British Journal of Haematology. https://doi.org/10.1111/bjh.14391
Dowling, Michael M. ; Quinn, Charles T. ; Ramaciotti, Claudio ; Kanter, Julie ; Osunkwo, Ifeyinwa ; Inusa, Baba ; Iyer, Rathi ; Kwiatkowski, Janet L. ; Johnson, Clarissa ; Rhodes, Melissa ; Owen, William ; Strouse, John J. ; Panepinto, Julie A. ; Neumayr, Lynne ; Sarnaik, Sharada ; Plumb, Patricia A. ; Dlamini, Nomazulu ; Kirkham, Fenella ; Hynan, Linda S. / Increased prevalence of potential right-to-left shunting in children with sickle cell anaemia and stroke. In: British Journal of Haematology. 2016.
@article{e6b4bbfc1abf4edc94df9b2a261c5564,
title = "Increased prevalence of potential right-to-left shunting in children with sickle cell anaemia and stroke",
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.",
keywords = "Cardiology, Clinical research, Sickle cell anaemia, Stroke",
author = "Dowling, {Michael M.} and Quinn, {Charles T.} and Claudio Ramaciotti and Julie Kanter and Ifeyinwa Osunkwo and Baba Inusa and Rathi Iyer and Kwiatkowski, {Janet L.} and Clarissa Johnson and Melissa Rhodes and William Owen and Strouse, {John J.} and Panepinto, {Julie A.} and Lynne Neumayr and Sharada Sarnaik and Plumb, {Patricia A.} and Nomazulu Dlamini and Fenella Kirkham and Hynan, {Linda S.}",
year = "2016",
doi = "10.1111/bjh.14391",
language = "English (US)",
journal = "British Journal of Haematology",
issn = "0007-1048",
publisher = "Wiley-Blackwell",

}

TY - JOUR

T1 - Increased prevalence of potential right-to-left shunting in children with sickle cell anaemia and stroke

AU - Dowling, Michael M.

AU - Quinn, Charles T.

AU - Ramaciotti, Claudio

AU - Kanter, Julie

AU - Osunkwo, Ifeyinwa

AU - Inusa, Baba

AU - Iyer, Rathi

AU - Kwiatkowski, Janet L.

AU - Johnson, Clarissa

AU - Rhodes, Melissa

AU - Owen, William

AU - Strouse, John J.

AU - Panepinto, Julie A.

AU - Neumayr, Lynne

AU - Sarnaik, Sharada

AU - Plumb, Patricia A.

AU - Dlamini, Nomazulu

AU - Kirkham, Fenella

AU - Hynan, Linda S.

PY - 2016

Y1 - 2016

N2 - '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.

AB - '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.

KW - Cardiology

KW - Clinical research

KW - Sickle cell anaemia

KW - Stroke

UR - http://www.scopus.com/inward/record.url?scp=84995483170&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=84995483170&partnerID=8YFLogxK

U2 - 10.1111/bjh.14391

DO - 10.1111/bjh.14391

M3 - Article

JO - British Journal of Haematology

JF - British Journal of Haematology

SN - 0007-1048

ER -