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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: [email protected]
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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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References

d’Udekem, Y, Iyengar, AJ, Galati, JC, et al.Redefining expectations of long-term survival after the fontan procedure: twenty-five years of follow-up from the entire population of Australia and New Zealand. Circulation 2014; 130: S32–S38.CrossRefGoogle ScholarPubMed
d’Udekem, Y, Iyengar, AJ, Cochrane, AD, et al.The Fontan procedure: contemporary techniques have improved long-term outcomes. Circulation 2007; 116: I157–I164.Google ScholarPubMed
Dennis, M, Zannino, D, du Plessis, K, et al.Clinical outcomes in adolescents and adults after the Fontan procedure. J Am Coll Cardiol 2018; 71: 1009–1017.CrossRefGoogle ScholarPubMed
Alsaied, T, Bokma, JP, Engel, ME, et al.Predicting long-term mortality after Fontan procedures: a risk score based on 6707 patients from 28 studies. Congenit Heart Dis 2017; 12: 393–398.CrossRefGoogle ScholarPubMed
Rychik, J, Atz, AM, Celermajer, DS, et al. Evaluation and management of the child and adult with Fontan circulation: a scientific statement from the American Heart Association. Circulation 2019. doi:10.1161/CIR.0000000000000696.CrossRefGoogle Scholar
Alsaied, T, Bokma, JP, Engel, ME, et al.Factors associated with long-term mortality after Fontan procedures: a systematic review. Heart 2017; 103: 104–110.CrossRefGoogle ScholarPubMed
James, H, Witte, MH, Bernas, M, Barber, B.Proposal for prevention or alleviation of Protein/Lymph-Losing Enteropathy (PLE/LLE) after Fontan circulation treatment of univentricular hearts: restoration of Lymph balance with a “Lymphatic Right-to-Left Shunt”. Lymphology 2016; 49: 114–127.Google Scholar
Udink Ten Cate, FEA, Tjwa, E.Imaging the lymphatic system in Fontan patients. Circ Cardiovasc Imaging 2019; 12: e008972.CrossRefGoogle ScholarPubMed
Cheung, YF, Tsang, HY, Kwok, JS.Immunologic profile of patients with protein-losing enteropathy complicating congenital heart disease. Pediatr Cardiol 2002; 23: 587–593.CrossRefGoogle ScholarPubMed
Mattes, M, Connor, J, Kelly, SS, Schwartz, MC.Lymphopenia in Patients with Single-ventricle Heart Disease after the Fontan Operation. Congenit Heart Dis 2016; 11: 270–275.CrossRefGoogle ScholarPubMed
Elder, RW, McCabe, NM, Hebson, C, et al.Features of portal hypertension are associated with major adverse events in Fontan patients: the VAST study. Int J Cardiol 2013; 168: 3764–3769.CrossRefGoogle ScholarPubMed
Udink Ten Cate, FE, Hannes, T, Germund, I, et al.Towards a proposal for a universal diagnostic definition of protein-losing enteropathy in Fontan patients: a systematic review. Heart 2016; 102: 1115–1119.CrossRefGoogle ScholarPubMed
Alsaied, T, Possner, M, Lubert, AM, et al.Relation of magnetic resonance elastography to Fontan failure and portal hypertension. Am J Cardiol 2019; 124: 1454–1459.CrossRefGoogle ScholarPubMed
Bossers, SS, Helbing, WA, Duppen, N, et al.Exercise capacity in children after total cavopulmonary connection: lateral tunnel versus extracardiac conduit technique. J Thorac Cardiovasc Surg 2014; 148: 1490–1497.CrossRefGoogle ScholarPubMed
Balady, GJ, Arena, R, Sietsema, K, et al.Clinician’s Guide to cardiopulmonary exercise testing in adults: a scientific statement from the American Heart Association. Circulation 2010; 122: 191–225.CrossRefGoogle ScholarPubMed
Arena, R, Myers, J, Abella, J, et al.Determining the preferred percent-predicted equation for peak oxygen consumption in patients with heart failure. Circ Heart Fail 2009; 2: 113–120.CrossRefGoogle ScholarPubMed
Alsaied, T, Sleeper, LA, Masci, M, et al.Maldistribution of pulmonary blood flow in patients after the Fontan operation is associated with worse exercise capacity. J Cardiovasc Magn Resonance 2018; 20: 85.CrossRefGoogle ScholarPubMed
Morsheimer, MM, Rychik, J, Forbes, L, et al.Risk factors and clinical significance of Lymphopenia in survivors of the Fontan procedure for single-ventricle congenital cardiac disease. J Allergy Clin Immunol Pract 2016; 4: 491–496.CrossRefGoogle ScholarPubMed
Eysteinsdottir, JH, Freysdottir, J, Haraldsson, A, et al.The influence of partial or total thymectomy during open heart surgery in infants on the immune function later in life. Clin Exp Immunol 2004; 136: 349–355.CrossRefGoogle ScholarPubMed
Stosio, M, Ruszkowski, J, Mikosik-Roczynska, A, Haponiuk, I, Witkowski, JM.The significance of neonatal thymectomy for shaping the immune system in children with congenital heart defects. Kardiochir Torakochirurgia Pol 2017; 14: 258–262.Google ScholarPubMed
Albillos, A, Lario, M, Alvarez-Mon, M.Cirrhosis-associated immune dysfunction: distinctive features and clinical relevance. J Hepatol 2014; 61: 1385–1396.CrossRefGoogle ScholarPubMed
Ohuchi, H.Adult patients with Fontan circulation: what we know and how to manage adults with Fontan circulation? J Cardiol 2016; 68: 181–189.CrossRefGoogle ScholarPubMed