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Lymphopenia in adults after the Fontan operation: prevalence and associations

Published online by Cambridge University Press:  06 April 2020

Tarek Alsaied Open the ORCID record for Tarek Alsaied [Opens in a new window] ,
Mathias Possner Open the ORCID record for Mathias Possner [Opens in a new window] ,
Nicole Brown ,
Hassan Almeneisi ,
Cassandra Szugye ,
Andrew T. Trout ,
Omar Niss ,
Joseph J. Palermo ,
Faizeen Zafar  and
Jonathan R. Dillman
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Tarek Alsaied*
Affiliation:
Cincinnati Children’s Hospital Heart Institute, Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, OH, USA
Mathias Possner
Affiliation:
Cincinnati Children’s Hospital Heart Institute, Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, OH, USA
Nicole Brown
Affiliation:
Cincinnati Children’s Hospital Heart Institute, Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, OH, USA
Hassan Almeneisi
Affiliation:
Cincinnati Children’s Hospital Heart Institute, Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, OH, USA
Cassandra Szugye
Affiliation:
Cincinnati Children’s Hospital Heart Institute, Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, OH, USA
Andrew T. Trout
Affiliation:
Cincinnati Children’s Hospital Medical Center, Department of Radiology, University of Cincinnati College of Medicine, Cincinnati, OH, USA Department of Radiology, University of Cincinnati College of Medicine, Cincinnati, OH, USA
Omar Niss
Affiliation:
Division of Hematology, Cancer and Blood Diseases Institute, Cincinnati Children’s Hospital Medical Centre, Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, OH, USA
Joseph J. Palermo
Affiliation:
Cincinnati Children’s Hospital Medical Center, Division of Gastroenterology, Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, OH, USA
Faizeen Zafar
Affiliation:
Cincinnati Children’s Hospital Heart Institute, Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, OH, USA
Jonathan R. Dillman
Affiliation:
Cincinnati Children’s Hospital Medical Center, Department of Radiology, University of Cincinnati College of Medicine, Cincinnati, OH, USA Department of Radiology, University of Cincinnati College of Medicine, Cincinnati, OH, USA
Gruschen R. Veldtman
Affiliation:
Cincinnati Children’s Hospital Heart Institute, Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, OH, USA Adult Congenital Heart Disease, Heart Centre, King Faisal Specialist Hospital and Research Centre, Riyadh, Saudi Arabia
Alexander R. Opotowsky
Affiliation:
Cincinnati Children’s Hospital Heart Institute, Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, OH, USA Boston Adult Congenital Heart Service, Boston Children’s Hospital, Brigham and Women’s Hospital, Harvard Medical School, Boston, Massachusetts, USA
Adam M. Lubert
Affiliation:
Cincinnati Children’s Hospital Heart Institute, Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, OH, USA
*
Author for correspondence: Tarek Alsaied, MD, Cincinnati Children’s Hospital Medical Center, University of Cincinnati College of Medicine, Cincinnati, OH, USA. Tel: +513 6521226; Fax: +513 6363952. E-mail: tarek.alsaied@cchmc.org
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Abstract

Lymphopenia is common in adults who have had a Fontan operation although its aetiology and clinical implications remain unknown. Previous work suggests an association between lymphopenia and both liver disease and splenomegaly. The objective of this study was to assess the prevalence of lymphopenia in adults with a Fontan circulation and evaluate its associations with risk factors and clinical outcomes. Using a retrospective cohort study design, we studied 73 adult Fontan patients (age 25.0 ± 8.4 years) who had a complete blood count and abdominal imaging performed. Patients with protein-losing enteropathy were excluded. Clinical data were extracted from hospital records. The mean white blood cell count was 6580 ± 220/ml with a mean lymphocyte count of 1223 ± 508/ml. Lymphopenia, defined as lymphocyte count <1000/ml, was present in 23 (32%) patients. Patients with lymphopenia had a lower total white blood cell count (5556 ± 2517 versus 7136 ± 1924/ml, p = 0.009) and a lower platelet count (162 ± 69 versus 208 ± 69 k/ml, p = 0.008). Lymphopenia was also associated with findings of portal hypertension, including splenomegaly (36 versus 14%, p = 0.04), varices (22 versus 6%, p = 0.04), and ascites (39 versus 14%, p = 0.02). Lymphopenia did not correlate with any cardiac imaging, haemodynamic or exercise testing variables. In conclusion, lymphopenia is common in adult Fontan patients and is associated with markers of portal hypertension. Larger studies are needed to better define the relationship between lymphopenia and clinical outcomes.

Keywords

Fontan operationsingle-ventricle CHDlymphopeniaadult CHDsplenomegalyportal hypertension
Type
Original Article
Information
Cardiology in the Young , Volume 30 , Issue 5 , May 2020 , pp. 641 - 648
Copyright
© The Author(s) 2020. Published by Cambridge University Press

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