Learning objectives
Learning objectives
1.
Indications for Fontan procedure and types of surgeries
2.
To review the haemodynamics of Fontan circulation in an adult patient
3.
Imaging protocols
4.
Case-based review of complications on MRI and CT in adults: ventricular failure,
cavity dilatation,
Fontan pathway stenosis,
shunt formation,
specific sites and appearances of thrombus formation,
pulmonary arteriovenous malformations,
failure of thoracic lymphatic drainage and Fontan hepatopathy.
Background
Fontan procedure (Dr François Fontan,
1929-2018) reflects a spectrum of cardiothoracic surgeries when systemic venous return is diverted directly to the pulmonary arteries,
eliminating the ventricular component of the circulation chain.
Since the original surgery in 1971 (1),
the procedure has been modified several times and has resulted in prolonged survival of an important group in the population of congenital heart disease.
These surgeries have been performed in cases of functionally single ventricle: either only one anatomical ventricle or two that cannot be effectively separated....
Imaging findings OR Procedure details
1.
Fontan anatomy
Fontan anatomy differs,
depending on the performed procedures.
See the following case examples ofseveral variants.
Fig. 5shows two different types of Fontan anatomy: TCPC and atriopulmonary circulation.
Fig. 6 is a good exampleof Glenn procedure with formation of venous collaterals through the azygos and hemiazygos systems.
SeeFig. 7showing variants of the intraatrial tunnel and aFontan pathway stenosis.
2.
Complications
Fontan circulation is unique,
resulting in a specific spectrum of complications in end-organ systems (see Table 3).
Cardiovascular complications are more known and...
Conclusion
Conclusion
Fontan procedure is a group of palliative surgical techniques used to recreate right side of the circulatory chain in cases of congenital functionally single ventricle.
These surgeries result in significantly prolonged survival and quality of life for an important group of now adult patient population,
who get referred also to general healthcare institutions.
Knowledge of the specific haemodynamic features,
wide spectrum of possible complications,
imaging protocols and typical imaging appearances is important for both cardiothoracic and general radiologists.
References
1.
Fontan F,
Baudet E.
Surgical repair of tricuspid atresia.
Thorax.
1971 May 1;26(3):240–8.
2.de Leval MR,
Kilner P,
Gewillig M,
Bull C.
Total cavopulmonary connection: a logical alternative to atriopulmonary connection for complex Fontan operations.
Experimental studies and early clinical experience.
J Thorac Cardiovasc Surg.
1988 Nov;96(5):682–95.
3.Bossers SSM,
Cibis M,
Kapusta L,
Potters WV,
Snoeren MM,
Wentzel JJ,
et al.
Long-Term Serial Follow-Up of Pulmonary Artery Size and Wall Shear Stress in Fontan Patients.
Pediatr Cardiol.
2016;37:637–45.
4.Baumgartner H,
Bonhoeffer P,
Groot D,...