The Fontan Procedure: Anatomy, Complications, and Manifestations of Failure

Abstract

The Fontan procedure refers to any operation that results in the flow of systemic venous blood to the lungs without passing through a ventricle. It is performed to treat several complex congenital heart abnormalities including tricuspid atresia, pulmonary atresia with intact ventricular septum, hypoplastic left heart syndrome, and double-inlet ventricle. The original Fontan procedure included direct anastomosis of the right atrium to the main pulmonary artery; however, multiple modifications have been employed. Creation of Fontan circulation is palliative in nature, with good results in patients with ideal hemodynamics and substantial morbidity and mortality in those with poor hemodynamics. Complications of Fontan circulation include exercise intolerance, ventricular failure, right atrium dilatation and arrhythmia, systemic and hepatic venous hypertension, portal hypertension, coagulopathy, pulmonary arteriovenous malformation, venovenous shunts, and lymphatic dysfunction (eg, ascites, edema, effusion, protein-losing enteropathy, and plastic bronchitis). Magnetic resonance imaging is best for postoperative evaluation of patients who underwent the Fontan procedure, and cardiac transplantation remains the only definitive treatment for those with failing Fontan circulation.

Immunophenotyping and Protein Profiling of Fontan-associated Plastic Bronchitis Airway Casts

“…In addition, our clinical observation has been that airway cast production and expectoration is sporadic, suggesting an inflammatory trigger (9). Although there is no evidence of an infectious etiology (7,8), infection or inflammation seems to precipitate disease exacerbations (9). Collectively, these findings and observations suggest that cast pathogenesis may be more complex than presently appreciated and may involve a fibrin-inflammatory process in the airways.…”

“…It is characterized by the formation of obstructive airway plugs or casts reported to be composed of mucin and/or fibrin (3)(4)(5)(6). Because the Fontan circuit often results in abovenormal central venous pressure, it has been postulated that PB casts form because of lymph leak into the airways or are secondary to lymphatic dysfunction or lymphatic trauma during surgery (7,8). Abnormal hemodynamics inherent to Fontan physiology may also contribute to injury of the alveolar-capillary barrier (1).…”

Recurrent thrombosis despite a therapeutic international normalised ratio

“…Amy Carroll, 1 medical student; Linda Griffiths, 2 specialist nurse in adult congenital heart disease service; Vaikom Mahadevan, 2 consultant cardiologist in adult congenital heart disease service; Jaspal Dua, 2 consultant cardiologist in adult congenital heart disease service; Jecko Thachil, 3 …”

“…2,3 Intracardiac thrombi can manifest clinically as pulmonary embolism if formed on the right side of the heart, or ischaemic stroke if on the left side. 2 Massive pulmonary embolism accounts for the majority of sudden out-of-hospital death in patients with a Fontan circuit.…”

The Adult With a Fontan: A Panacea Without a Cure?

“…The PAs may often be abnormal (ie, small, discontinuous, or stenosed) and it is important to identify restricted blood flow to and from the lungs. 23 It is well known that pulmonary over circulation leads to increased PVR and over time will lead to PA hypertension atrial mixing via an atrial septectomy. The first cavopulmonary connection is created in the first year of life via the bidirectional Glenn shunt (Figure 1).…”