Primary Fontan Procedure in an Adult Patient with Apicocaval Juxtaposition

The Fontan procedure is predominantly performed in children with a functionally univentricular heart. It remains unclear as to whether the Fontan procedure can be applied for adult patients. Furthermore, the optimal conduit route remains unknown for adults with apicocaval juxtaposition. Adult Fontan candidates are likely to have dilated ventricles with poor cardiac function caused by long-term hypoxia and ventricular volume overload. Consequently, an ideal course from the inferior vena cava to the pulmonary artery should be created with caution to avoid the formation of kink or collapse of the conduit. A 48-year-old man with a double outlet right ventricle, single right ventricle, along with valvular and supravalvular pulmonary stenosis, presented with exertional dyspnea and palpitation. The patient showed situs inversus with the cardiac apex and inferior vena cava on the left side. Preoperative catheterization data showed low pulmonary vascular resistance and pulmonary artery pressure because of valvular, supravalvular, and bilateral pulmonary artery stenosis. Primary fenestrated total cavopulmonary connection (TCPC), concomitant with pulmonary artery plasty, was performed. The conduit was constructed via an intra-atrial route to avoid being compressed posteriorly by the dilated ventricle. The postoperative course was uneventful, and the patient was discharged 32 days after surgery. His exercise tolerance improved gradually thereafter. He returned to his previous job 3 months after surgery. Our case demonstrates that adult Fontan candidates whose preoperative conditions satisfy appropriate criteria are capable of undergoing primary TCPC. However, surgical indications for adult TCPC should be carefully determined. In our present case, the intra-atrial conduit route was useful for an adult patient with apicocaval juxtaposition.

[1]  A. Capderou,et al.  The modified Fontan procedure with use of extracardiac conduit in adults: analysis of 32 consecutive patients. , 2014, The Annals of thoracic surgery.

[2]  N. Goel,et al.  Comparison of fenestrated and nonfenestrated patients undergoing extracardiac Fontan. , 2014, The Annals of thoracic surgery.

[3]  S. Vaziri,et al.  Outcomes of adolescents and adults undergoing primary Fontan procedure. , 2013, The American journal of cardiology.

[4]  Prahlad G. Menon,et al.  Total cavopulmonary connection in patients with apicocaval juxtaposition: optimal conduit route using preoperative angiogram and flow simulation. , 2013, European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery.

[5]  R. Hénaine,et al.  Early to midterm results of total cavopulmonary connection in adult patients. , 2013, The Annals of thoracic surgery.

[6]  H. Kato,et al.  Appropriate route selection for extracardiac total cavopulmonary connection in apicocaval juxtaposition. , 2012, The Annals of thoracic surgery.

[7]  R. Abella,et al.  Fontan Operation for Patients With Complex Anatomy: The Intra-Atrial Conduit Technique , 2012, World journal for pediatric & congenital heart surgery.

[8]  T. Travison,et al.  Late status of Fontan patients with persistent surgical fenestration. , 2011, Journal of the American College of Cardiology.

[9]  V. Podzolkov,et al.  Immediate results of bidirectional cavopulmonary anastomosis and Fontan operations in adults. , 2011, Interactive cardiovascular and thoracic surgery.

[10]  Y. Ueda,et al.  The impact of extracardiac conduit-total cavopulmonary connection on apicocaval juxtaposition. , 2010, European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery.

[11]  S. Sano,et al.  Midterm to long-term outcome of total cavopulmonary connection in high-risk adult candidates. , 2009, The Annals of thoracic surgery.

[12]  Hartzell V Schaff,et al.  The modified Fontan procedure: early and late results in 132 adult patients. , 2003, The Journal of thoracic and cardiovascular surgery.

[13]  S. Dymarkowski,et al.  The ratio of flow in the superior and inferior caval veins after construction of a bidirectional cavopulmonary anastomosis in children , 2003, Cardiology in the Young.

[14]  W. Williams,et al.  The Fontan procedure in adults , 2001, Heart.

[15]  J. Somerville,et al.  Experience with the Glenn anastomosis in the adult with cyanotic congenital heart disease , 1999, Cardiology in the Young.

[16]  K. Arheart,et al.  Contribution of superior vena caval flow to total cardiac output in children. A Doppler echocardiographic study. , 1995, Circulation.

[17]  A. Choussat Selection criteria for Fontan's procedure , 1978 .

[18]  F. Fontan,et al.  Surgical repair of tricuspid atresia , 1971, Thorax.