Skip to main content Accessibility help

Pulmonary artery wall thickness in children with Fontan physiology: an optical coherence tomography case control study

  • Eimear McGovern (a1), Christine Voss (a1), Nathan W. Brunner (a2), Stephanie Duncombe (a1), Kevin C. Harris (a1) and Martin H. Hosking (a1)...



Failure of the Fontan circulation is not a well-understood clinical phenomena.For some patients, a gradual increase in pulmonary vascular resistance (PVR) and structural changes in the pulmonary artery may be an important causative factor. To further investigate this issue, we employed optical coherence tomography (OCT) to evaluate structural changes within the pulmonary arteries of Fontan patients and compared to those with a normal pulmonary circulation.

Materials and Methods:

Pulmonary artery OCT was performed, without complications, in 12 Fontan and 11 control patients. Wall thickness and wall:vessel cross-sectional area (CSA) ratio were calculated after image acquisition, using digital planimetry.


There was no difference in wall thickness between both groups. Median wall thickness for Fontan patients was 0.12 mm (IQR, 0.10–0.14) and for controls was 0.11 mm (IQR, 0.10–0.12; p = 0.62). Wall:vessel CSA ratio for Fontan patients was 0.13 (IQR, 0.12–0.16) and for controls was 0.13 (IQR, 0.11–0.15) (p = 0.73). There was no association between wall thickness and ventricle morphology, age at catheterisation, age at Fontan, years since Fontan completion, pulmonary artery pressure, and PVR. The vessel media was more readily visualised in control patients.


OCT of the pulmonary arteries in Fontan patients is safe and feasible. Our OCT findings suggest that during childhood, pulmonary artery wall dimensions are normal in Fontan children with reassuring hemodynamics. Further evaluation of Fontan patients with abnormal hemodynamics and serial evaluation into adulthood are required to conclude on the utility of OCT for identifying early pulmonary artery structural changes.


Corresponding author

Author for correspondence: Eimear McGovern, MB, BCh, BAO, Division of Cardiology, Department of Pediatrics, Children’s Heart Centre, British Columbia Children’s Hospital, 1F3 - 4480 Oak Street, Vancouver, BC, V6H 3V4, Canada. Tel: +1 604 875 2345; Fax number +1 604 875 3463; E-mail:


Hide All
1. 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: S32–8.
2. Gewillig, M, Goldberg, DJ Failure of the Fontan circulation. Heart Fail Clin 2014; 10: 105–16.
3. Khambadkone, S, Li, J, de Leval, MR, Cullen, S, Deanfield, JE, Redington, AN. Basal pulmonary vascular resistance and nitric oxide responsiveness late after Fontan-type operation. Circulation 2003; 107: 3204–8.
4. Zongtao, Y, Huishan, W, Zengwei, W, et al. Experimental study of nonpulsatile flow perfusion and structural remodeling of pulmonary microcirculation vessels. Thorac Cardiovasc Surg 2010; 58; 468–72.
5. Gewillig, MH, Lundstrom, UR, Deanfield, JE, et al. Impact of Fontan operation on left ventricular size and contractility in tricuspid atresia. Circulation 1990; 81: 118–27.
6. Harris, KC, Manouzi, A, Fung, AY, et al. Feasibility of optical coherence tomography in children with Kawasaki disease and pediatric heart transplant recipients. Circ Cardiovasc Imaging 2014; 7: 671–8.
7. Ulrich, SM, Lehner, A, Birnbaum, A, et al. Safety of optical coherence tomography in pediatric heart transplant patients. Int J Cardiol 2017; 228: 205208.
8. Karanasos, A, Ligthart, A, Witberg, J, van Soest, G, Bruining, N, Regar, E. Optical coherence tomography: potential clinical applications. Curr Cardiovasc Imaging Rep 2012; 5: 206220.
9. Tearney, GJ, Regar, E, Akasaka, T, et al. Consensus standards for acquisition, measurement, and reporting of intravascular optical coherence tomography studies: a report from the International Working Group for Intravascular Optical Coherence Tomography Standardization and Validation. J Am Coll Cardiol 2012; 59: 10581072.
10. Jorge, E, Baptista, R, Calisto, J, et al. Pulmonary vascular remodeling in mitral valve disease: An optical coherence tomography study. Int J Cardiol 2016; 203: 576578.
11. Li, N, Zhang, S, Hou, J, Jang, IKK, Yu, B. Assessment of pulmonary artery morphology by optical coherence tomography. Heart Lung Circ 2012; 21: 778781.
12. Hayabuchi, Y, Sakata, M, Kagami, S. Optical coherence tomography can visualize the pulmonary artery in Williams-Beuren syndrome. Eur Heart J Cardiovasc Imaging 2015; 16: 967.
13. Homma, Y, Hayabuchi, Y, Ono, A, Kagami, S. Pulmonary artery wall thickness assessed by optical coherence tomography correlates with pulmonary hemodynamics in children with congenital heart disease. Circ J 2018; 82: 23502357.
14. Kurotobi, S, Sano, T, Kogaki, S, et al. Bidirectional cavopulmonary shunt with right ventricular outflow patency: the impact of pulsatility on pulmonary endothelial function. J Thorac Cardiovasc Surg 2001; 121: 11611168.
15. Lévy, M, Danel, C, Tamisier, D, Vouhé, P, Leca, F. Histomorphometric analysis of pulmonary vessels in single ventricle for better selection of patients for the Fontan operation. J Thorac Cardiovasc Surg 2002; 123: 263270.
16. Adachi, I, Ueno, T, Hori, Y, Sawa, Y. Alterations in the medial layer of the main pulmonary artery in a patient with longstanding Fontan circulation. Interact Cardiovasc Thorac Surg 2010; 11: 682683.
17. Maeda, K, Yanaki, S, Kado, H, Asou, T, Murakami, A, Takamoto, S. Reevaluation of histomorphometric analysis of lung tissue in decision making for better patient selection for Fontan-type operations. Ann Thorac Surg 2004; 78: 13711381.
18. Ridderbos, FJ, Wolf, D, Timmer, A, et al. Adverse pulmonary vascular remodeling in the Fontan circulation. J Heart Lung Transplant 2015; 34: 404413.
19. Ishida, H, Kogaki, S, Ichimori, H, et al. Overexpression of endothelin-1 and endothelin receptors in the pulmonary arteries of failed Fontan patients. Int J Cardiol 2012; 159: 3439.
20. Jorge, E, Baptista, R, Calisto, J, et al. Optical coherence tomography of the pulmonary arteries: a systematic review. J Cardiol 2016; 67: 614.
21. Dai, Z, Fukumoto, Y, Tatebe, S, et al. OCT imaging for the management of pulmonary hypertension. JACC Cardiovasc Imaging 2014; 7: 843845.
22. Domingo, E, Grignola, JC, Aguilar, R, et al. In vivo assessment of pulmonary arterial wall fibrosis by intravascular optical coherence tomography in pulmonary arterial hypertension: a new prognostic marker of adverse clinical follow-up. Open Respir Med J 2013; 7: 2632.
23. Brunner, NW, Zamanian, RT, Ikeno, F, et al. Optical coherence tomography of pulmonary arterial walls in humans and pigs (Sus scrofa domesticus). Comp Med 2015; 65: 217224.



Full text views

Total number of HTML views: 0
Total number of PDF views: 0 *
Loading metrics...

Abstract views

Total abstract views: 0 *
Loading metrics...

* Views captured on Cambridge Core between <date>. This data will be updated every 24 hours.

Usage data cannot currently be displayed