Fontan circulation

Case contributed by Tom Foster
Diagnosis almost certain

Presentation

Chest pain and dizziness. High risk for aortic dissection (previous cardiac operation and aortic mechanical valve replacement).

Patient Data

Age: 40 years
Gender: Male
ct

Non-gated acquisition.

Previous Fontan procedure - Glenn shunt to the right main pulmonary artery and extracardiac conduit from IVC to the presumed main pulmonary artery.
Previous mechanical aortic valve replacement.
Previous sternotomy, presumed redundant epicardial pacing wires in situ.

Situs solitus, levocardia.
Double inlet left ventricle - small hypoplastic right ventricle anterior and to the left of the left ventricle - with VSD communicating.
Transposition of the great arteries, with aorta arising anterior and to the left of the residual pulmonary artery stump.
Nondilated atria, with ASD.
Single coronary artery arising from the right - the circumflex artery loops anterior to the aorta.

No aortic dissection or other acute aortic syndrome.
Aortic root dilatation (~48 mm) and dilatation of ascending aorta (~47 mm), similar to the previous examination.
Left vertebral artery arises directly from the aortic arch. Otherwise conventional arch anatomy.
Normal descending thoracic aorta and partially imaged upper abdominal aorta.

Case Discussion

An example of a patient with complex background congenital heart disease and subsequent correction with a Fontan circulation. There was no obvious cause for acute presentation.

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