Clinical variants of respiratory dysfunction in children with Fontan hemodynamic

Cover Page

Cite item

Full Text

Open Access Open Access
Restricted Access Access granted
Restricted Access Subscription or Fee Access

Abstract

Children who born with a functional single ventricle undergo a series of surgical interventions at an early age, leading to the formation of Fontan circulation. Fontan circulation can lead to various disorders of pulmonary blood flow, depending on the specific heart defect, the volume of surgical interventions, the development of complications in the postoperative period, etc. Fontan circulation during the first years of life can lead to significant changes in pulmonary blood flow, which can affect long-term lung function. Also, functional changes in the lungs can also cause persistent circulatory disorders. Understanding the cardiopulmonary interactions along with improved recognition and treatment of pulmonary abnormalities may improve the long-term outcomes in this patient population.

Full Text

Restricted Access

About the authors

A. R. Shudueva

N.I. Pirogov Russian National Research Medical University, Ministry of Health of Russia

Author for correspondence.
Email: yulmiz@mail.ru
ORCID iD: 0000-0002-6956-1418
Russian Federation, Moscow

I. A. Kovalev

A.N. Bakulev National Medical Research Center for Cardiovascular Surgery, Ministry of Health of Russia

Email: yulmiz@mail.ru
ORCID iD: 0000-0001-8195-5682
SPIN-code: 5024-6323

Professor, MD

Russian Federation, Moscow

Yu. L. Mizernitskiy

N.I. Pirogov Russian National Research Medical University, Ministry of Health of Russia

Email: yulmiz@mail.ru
ORCID iD: 0000-0002-0740-1718
SPIN-code: 6135-5260

Professor, MD

Russian Federation, Moscow

References

  1. Fontan F., Baudet E. Surgical repair of tricuspid atresia. Thorax. 1971; 26 (3): 240–8. doi: 10.1136/thx.26.3.240
  2. Corno A.F., Owen M.J., Cangiani A. et al. Physiological Fontan Procedure. Front Pediatr. 2019; 7: 196. doi: 10.3389/fped.2019.00196
  3. Mazza G.A., Gribaudo E., Agnoletti G. The pathophysiology and complications of Fontan circulation. Acta Biomed. 2021; 92 (5): e2021260. doi: 10.23750/abm.v92i5.10893
  4. Glenn W.W. Circulatory bypass of the right side of the heart. IV. Shunt between superior vena cava and distal right pulmonary artery; report of clinical application. N Engl J Med. 1958; 259 (3): 117–20. doi: 10.1056/NEJM195807172590304
  5. Björk V.O., Olin C.L., Bjarke B.B. et al. CA. Right atrial-right ventricular anastomosis for correction of tricuspid atresia. J Thorac Cardiovasc Surg. 1979; 77 (3): 452–8.
  6. Ahmad Z., Jin L.H., Penny D.J. et al. Optimal Fenestration of the Fontan Circulation. Front Physiol. 2022; 13: 867995. doi: 10.3389/fphys.2022.867995
  7. Becker K., Uebing A., Hansen J.H. Pulmonary vascular disease in Fontan circulation-is there a rationale for pulmonary vasodilator therapies? Cardiovasc Diagn Ther. 2021; 11 (4): 1111–21. doi: 10.21037/cdt-20-431
  8. Egbe A.C., Reddy Y.N.V., Khan A.R. et al. Venous congestion and pulmonary vascular function in Fontan circulation: Implications for prognosis and treatment. Int J Cardiol. 2018; 271: 312–6. doi: 10.1016/j.ijcard.2018.05.039
  9. Gewillig M. The Fontan circulation. Heart. 2005; 91 (6): 839–46. doi: 10.1136/hrt.2004.051789
  10. Egbe A.C., Connolly H.M., Miranda W.R. et al. Hemodynamics of Fontan Failure: The Role of Pulmonary Vascular Disease. Circ Heart Fail. 2017; 10 (12): e004515. doi: 10.1161/CIRCHEARTFAILURE.117.004515
  11. d'Udekem Y., Iyengar A.J., Galati J.C. 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 (11 Suppl 1): S32–8. doi: 10.1161/CIRCULATIONAHA.113.007764
  12. Laohachai K., Ayer J. Impairments in Pulmonary Function in Fontan Patients: Their Causes and Consequences. Front Pediatr. 2022; 10: 825841. doi: 10.3389/fped.2022.825841
  13. Abdulkarim A., Shaji S., Elfituri M. et al. Pulmonary Complications in Patients With Fontan Circulation: JACC Review Topic of the Week. J Am Coll Cardiol. 2023; 81 (25): 2434–44. doi: 10.1016/j.jacc.2023.04.036
  14. Wei Z., Whitehead K.K., Khiabani R.H. et al. Respiratory Effects on Fontan Circulation During Rest and Exercise Using Real-Time Cardiac Magnetic Resonance Imaging. Ann Thorac Surg. 2016; 101 (5): 1818–25. doi: 10.1016/j.athoracsur.2015.11.011
  15. Callegari A., Neidenbach R., Milanesi O. et al. A restrictive ventilatory pattern is common in patients with univentricular heart after Fontan palliation and associated with a reduced exercise capacity and quality of life. Congenit Heart Dis. 2019; 14 (2): 147–55. doi: 10.1111/chd.12694
  16. Alonso-Gonzalez R., Borgia F., Diller G.P. et al. Abnormal lung function in adults with congenital heart disease: prevalence, relation to cardiac anatomy, and association with survival. Circulation. 2013; 127 (8): 882–90. doi: 10.1161/CIRCULATIONAHA.112.126755
  17. Ravndal M., Idorn L., Nielsen K.G. et al. Lung Function in Fontan Patients Over a Ten-Year Period: Is the Fontan Circulation Impairing Lung Development? Pediatr Cardiol. 2024; 45 (3): 500–12. doi: 10.1007/s00246-023-03389-2
  18. Becker K., Uebing A., Hansen J.H. Pulmonary vascular disease in Fontan circulation-is there a rationale for pulmonary vasodilator therapies? Cardiovasc Diagn Ther. 2021; 11 (4): 1111–21. doi: 10.21037/cdt-20-431
  19. Kovaćević-Kuśmierek K., Mazurek-Kula A., Moszura T. et al. Lung perfusion scintigraphy in the assessment of pulmonary circulation after completion of surgical treatment of a hypoplastic left heart syndrome (HLHS). Nucl Med Rev Cent East Eur. 2019; 22 (2): 81–4. doi: 10.5603/NMR.2019.0018
  20. Gewillig M., Goldberg D.J. Failure of the fontan circulation. Heart Fail Clin. 2014; 10 (1): 105–16. doi: 10.1016/j.hfc.2013.09.010
  21. Feltez G., Coronel C.C., Pellanda L.C. et al. Exercise capacity in children and adolescents with corrected congenital heart disease. Pediatr Cardiol. 2015; 36 (5): 1075–82. doi: 10.1007/s00246-015-1129-1
  22. Шудуева А.Р., Ковалев И.А., Мизерницкий Ю.Л. Состояние дыхательной системы у детей с гемодинамикой Фонтена. Педиатрия им. Г.Н. Сперанского. 2023; 102 (6): 115–22 [Shudueva A.R., Kovalev I.A., Mizernitskiy Yu.L. Respiratory system in children with Fontan circulation from the pulmonologist’s point of view. Pediatria n.a. G.N. Speransky. 2023; 102 (6): 115–22 (in Russ.)]. doi: 10.24110/0031-403X-2023-102-6-115-122
  23. Шудуева А.Р., Мизерницкий Ю.Л., Грознова О.С. и др. Легочная гипертензия при хронической бронхолегочной патологии у детей: взгляд на проблему с точки зрения пульмонолога. Медицинский совет. 2021; 17: 300–9 [Shudueva A.R., Mizernitskiy Yu.L., Groznova O.S. et al. Pulmonary hypertension in children with chronic bronchopulmonary pathology: a pulmonologist’s perspective to the problem. Medical Council. 2021; 17: 300–9 (in Russ.)]. doi: 10.21518/2079-701X-2021-17-300-309

Supplementary files

Supplementary Files
Action
1. JATS XML
Download
2. Fig. 1. Fontan hemodynamics (description in text)

Download (124KB)
Indexing metadata
3. Fig. 2. Perfusion pneumoscintigraphy of girl A. demonstrates a marked impairment of perfusion of the right lung

Download (387KB)
Indexing metadata
4. Fig. 3. Perfusion pneumoscintigraphy of girl S. demonstrates impaired perfusion of the right lung

Download (154KB)
Indexing metadata

Copyright (c) 2025 Russkiy Vrach Publishing House