Hostname: page-component-586b7cd67f-2brh9 Total loading time: 0 Render date: 2024-11-22T13:33:33.613Z Has data issue: false hasContentIssue false

Thrombocytopaenia in cyanotic CHD

Published online by Cambridge University Press:  02 December 2020

Simona Danioth
Affiliation:
Department of Cardiology, University Heart Center, University Hospital Zurich, Zurich, Switzerland
Urs Schanz
Affiliation:
Clinic for Medical Oncology and Hematology, University Hospital Zurich, Zurich, Switzerland
Matthias Greutmann*
Affiliation:
Department of Cardiology, University Heart Center, University Hospital Zurich, Zurich, Switzerland
*
Author for correspondence: M. Greutmann, MD, Department of Cardiology, University Heart Centre, University Hospital Zurich, Raemistrasse 100, 8091Zurich, Switzerland. Tel: +41 44 255 3883; Fax: +41 44 255 8701. E-mail: [email protected]

Abstract

Introduction:

Thrombocytopaenia is common in adults with cyanotic heart disease. Our aim was to explore potential mechanisms for thrombocytopaenia in these vulnerable patients.

Methods:

Adults with cyanotic heart defects were identified from our clinical database. Haemoglobin levels, platelet counts, and resting oxygen saturations were determined at baseline and during follow-up. Associations between patient characteristics and cardiac physiology with these parameters at baseline and during follow-up were analysed using regression models. Survival estimates were determined by the Kaplan–Meier method.

Results:

We included 79 patients (mean age 32.2 ± 12.4, 48 (61%) Eisenmenger syndrome, 20 (25%) Down syndrome). Mean oxygen saturation was 84.1 ± 5.9%; 38 (48%) had thrombocytopaenia. There was a strong inverse correlation between platelet count and haemoglobin level (R = −0.655, R2 = 0.429, p < 0.0001) and a weaker but significant positive correlation between platelet count and oxygen saturation (R = 0.345, R2 = 0.119, p = 0.002). There was a significant inverse correlation between decrease in platelet count and increase in haemoglobin level during follow-up (R = −0.401, R2 = 0.161, p = 0.001) but not to changes in oxygen saturation (R = 0.043, R2 = 0.002, p = 0.726). Survival estimates were lower for patients with thrombocytopaenia at baseline (log-rank test p < 0.0001).

Conclusions:

Our findings suggest a direct inverse correlation between platelet counts and haemoglobin levels in adults with cyanotic heart disease. Further studies are required to explore the mechanisms of thrombocytopaenia in cyanotic heart disease and its potential role as an independent marker of risk.

Type
Original Article
Copyright
© The Author(s), 2020. Published by Cambridge University Press

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

Diller, GP, Kempny, A, Alonso-Gonzalez, R, et al. Survival prospects and circumstances of death in contemporary adult congenital heart disease patients under follow-up at a large tertiary centre. Circulation 2015; 132: 21182125.CrossRefGoogle Scholar
Greutmann, M, Tobler, D, Kovacs, AH, et al. Increasing mortality burden among adults with complex congenital heart disease. Congenital Heart Dis 2015; 10: 117127.CrossRefGoogle ScholarPubMed
Kempny, A, Hjortshoj, CS, Gu, H, et al. Predictors of death in contemporary adult patients with Eisenmenger syndrome: a multicenter study. Circulation 2017; 135: 14321440.CrossRefGoogle ScholarPubMed
Chaix, MA, Gatzoulis, MA, Diller, GP, Khairy, P, Oechslin, EN. Eisenmenger syndrome: A multisystem disorder-do not destabilize the balanced but Fragile physiology. Can J Cardiol 2019; 35: 16641674.CrossRefGoogle Scholar
Hjortshoj, CMS, Kempny, A, Jensen, AS, et al. Past and current cause-specific mortality in Eisenmenger syndrome. Eur Heart J 2017; 38: 20602067.CrossRefGoogle ScholarPubMed
Daliento, L, Somerville, J, Presbitero, P, et al. Eisenmenger syndrome. Factors relating to deterioration and death. Eur Heart J 1998; 19: 18451855.CrossRefGoogle ScholarPubMed
Lill, MC, Perloff, JK, Child, JS. Pathogenesis of thrombocytopenia in cyanotic congenital heart disease. Am J Cardiol 2006; 98: 254258.CrossRefGoogle ScholarPubMed
Martin-Garcia, AC, Arachchillage, DR, Kempny, A, et al. Platelet count and mean platelet volume predict outcome in adults with Eisenmenger syndrome. Heart 2018; 104: 4550.CrossRefGoogle ScholarPubMed
Lefrancais, E, Ortiz-Munoz, G, Caudrillier, A, et al. The lung is a site of platelet biogenesis and a reservoir for haematopoietic progenitors. Nature 2017; 544: 105109.CrossRefGoogle Scholar
Saida, S. Predispositions to leukemia in Down syndrome and other hereditary disorders. Curr Treat Options Oncol 2017; 18: 41.CrossRefGoogle Scholar