Imaging in Fontan Procedure: Normal Anatomy and Common Complications

Congress:

ECR 2019

Poster Number:

C-3259

Type:

Scientific Exhibit

Keywords:

Congenital, Cirrhosis, Arteriovenous malformations, Statistics, Diagnostic procedure, Complications, Ultrasound, MR, CT, Pulmonary vessels, Cardiac, Abdomen

Authors:

N. Pereira da Silva, A. Antunes, A. Marinho da Silva, F. Cruz, P. Donato; Coimbra/PT

DOI:

10.26044/ecr2019/C-3259

DOI-Link:

https://dx.doi.org/10.26044/ecr2019/C-3259

Fig. 1: Schematic representation of the Fontan procedure. On the left the anatomy of...

Fig. 2: Total cavopulmonary connection with intraarterial connection. On the right, a...

Fig. 5: Parasagittal, axial and VR images of cardiac CT shows “classic” Fontan...

Fig. 9: Arterial phase contrast-enhanced abdominal CT show situs inversus liver with...

Aims and objectives

Section: Aims and objectives The aim of this presentation is to review and describe the normal anatomy of Fontan circulation and the imaging spectrum of its cardiac and extracardiac complications.

Methods and materials

A review of our institution’s database over the last 30 years retrieved data from 37 patients (19 female, 18 male) that underwent Fontan procedures from January of 1987 to October 2014. Of these, 18 patients were excluded due to incomplete imaging records. Imaging studies (including “B-mode” US, CT, MRI and conventional angiography) from the remaining 19 patients (8 female, 11 male; age range: 2-39 years old at the date of the last imagological study, median age: 19 years old) were reviewed in order to determine...

Results

Normal Fontan Anatomy The Fontan procedure refers to any surgical technique aimed at the complete separation of pulmonary and systemic blood flows, redirecting the normal venous blood flow to the lungs and bypassing the right ventricle. It is used in cases of complex congenital heart defects such as pulmonary valve atresia with intact ventricular septum, tricuspid valve atresia, mitral valve atresia, hypoplastic left heart syndrome and double-inlet ventricle. Since its first description by Fontan and Baudet in 1971, the procedure has undergone numerous modifications. Originally,...

Conclusion

The Fontan procedure is often the only available treatment option for patients with complex congenital cardiac conditions. It is of paramount importance that radiologists are familiar with the post-surgical anatomy and physiology, as well as with the most common imaging findings of cardiac and extracardiac complications. Active surveillance and detection of these abnormalities can greatly improve both quality of life and life expectancy of these patients.

Personal information

References

Monagle P, Karl TR. Thromboembolic problems after the Fontan operation. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2002;5:36–47 Daniels CJ, Bradley EA, Landzberg MJ, Aboulhosn J, Beekman RH, Book W, et al. Fontan-Associated Liver Disease: Proceedings from the American College of Cardiology Stakeholders Meeting, October 1 to 2, 2015, Washington DC. J Am Coll Cardiol. 2017 Dec 18;70(25):3173–94 Fredenburg, T. B., Johnson, T. R., & Cohen, M. D. (2011). The Fontan Procedure: Anatomy, Complications, and Manifestations of Failure. RadioGraphics, 31(2), 453–463.https://doi.org/10.1148/rg.312105027 V. Navarro-Aguilar ,...