Fontan procedure

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The Fontan procedure is a repair surgical strategy for congenital cardiac anomalies. It is not usually used in isolation, but in combination with other repair procedures in a staged manner in an attempt to correct the underlying cardiac pathology.

Rationale

The procedure attempts to bypass the right heart. Systemic circulation is redirected into the pulmonary arteries.

It can be used in multiple situations, including:

tricuspid atresia
hypoplastic left heart (part of the overall repair strategy)
other types of single ventricle physiology

Procedure

The original procedure involved:

closure of atrial septal defect (ASD) and tricuspid valve
creating an anastomosis between the right atrial appendage and the main pulmonary artery

To improve flow dynamics, a "lateral tunnel" was introduced. The patient essentially received a bidirectional Glenn procedure:

the superior vena cava (SVC) is divided
the proximal and distal ends are anastomosed to the right pulmonary artery
an atrial "baffle" is put in the right atrium to direct blood toward the right pulmonary anastomosis and away from the tricuspid valve

Currently, an extracardiac conduit directs blood flow from the inferior vena cava (IVC) at the inferior cavoatrial junction to the right pulmonary artery, bypassing the right atrium. Systemic flow from the SVC is directed into the right pulmonary artery with a Glenn procedure.

Treatment and prognosis

Although a palliative procedure, patients treated with the Fontan procedure can reach adulthood. The maximum duration of the procedure is not well-defined.

A patient with a failing Fontan repair can receive an extracardiac Fontan conduit, if they do not already have one. The definitive procedure is cardiac transplantation.

Intracardiac and central venous thromboses are major causes of morbidity and mortality in patients after Fontan procedure. It can occur in 1%-33% of patients who underwent the procedure ⁴.

History and etymology

The procedure was originally suggested in 1971 by F Fontan and E Baudet as a correction for tricuspid atresia.

~~-<li>the <a title="Superior vena cava" href="/articles/superior-vena-cava">superior vena cava</a> (SVC) is divided</li>~~
+<li>the <a href="/articles/superior-vena-cava">superior vena cava</a> (SVC) is divided</li>
-</ul><p>Currently, an extracardiac conduit directs blood flow from the <a title="Inferior vena cava" href="/articles/inferior-vena-cava-1">inferior vena cava</a> (IVC) at the inferior cavoatrial junction to the right pulmonary artery, bypassing the right atrium. Systemic flow from the SVC is directed into the right pulmonary artery with a <a href="/articles/glenn-procedure">Glenn procedure</a>.</p><h4>Treatment and prognosis</h4><p>Although a palliative procedure, patients treated with the Fontan procedure can reach adulthood. The maximum duration of the procedure is not well-defined.</p><p>A patient with a failing Fontan repair can receive an extracardiac Fontan conduit, if they do not already have one. The definitive procedure is <a href="/articles/cardiac-transplantation">cardiac transplantation</a>.</p><h4>History and etymology</h4><p>The procedure was originally suggested in 1971 by <strong>F Fontan</strong> and <strong>E Baudet</strong> as a correction for tricuspid atresia.</p>
+</ul><p>Currently, an extracardiac conduit directs blood flow from the <a href="/articles/inferior-vena-cava-1">inferior vena cava</a> (IVC) at the inferior cavoatrial junction to the right pulmonary artery, bypassing the right atrium. Systemic flow from the SVC is directed into the right pulmonary artery with a <a href="/articles/glenn-procedure">Glenn procedure</a>.</p><h4>Treatment and prognosis</h4><p>Although a palliative procedure, patients treated with the Fontan procedure can reach adulthood. The maximum duration of the procedure is not well-defined.</p><p>A patient with a failing Fontan repair can receive an extracardiac Fontan conduit, if they do not already have one. The definitive procedure is <a href="/articles/cardiac-transplantation">cardiac transplantation</a>.</p><p><!--[if gte mso 9]><xml>
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+<![endif]--></p><p>Intracardiac and central venous thromboses are major causes of morbidity and mortality in patients after Fontan procedure. It can occur in 1%-33% of patients who underwent the procedure <sup>4</sup>. </p><h4>
+History and etymology</h4><p>The procedure was originally suggested in 1971 by <strong>F Fontan</strong> and <strong>E Baudet</strong> as a correction for tricuspid atresia.</p>

References changed:

4. Ghadimi Mahani M, Agarwal P, Rigsby C et al. CT for Assessment of Thrombosis and Pulmonary Embolism in Multiple Stages of Single-Ventricle Palliation: Challenges and Suggested Protocols. Radiographics. 2016;36(5):1273-84. <a href="https://doi.org/10.1148/rg.2016150233">doi:10.1148/rg.2016150233</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/27618316">Pubmed</a>

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