Fenestrated Fontan for Hypoplastic Left Heart Syndrome Thomas L. Spray  Operative Techniques in Cardiac and Thoracic Surgery  Volume 2, Issue 3, Pages 239-252 (August 1997) DOI: 10.1016/S1085-5637(07)70063-3 Copyright © 1997 Elsevier Inc. Terms and Conditions

1 Anatomy and incisions for the hemi-Fontan after stage I palliation for hypoplastic left heart syndrome. The neoaorta is cannulated and a cardioplegia needle inserted in the proximal neoaorta for cardioplegia injection and for aspiration of air following release of the clamp. After mobilization of the cardiac structures and dissection of the pulmonary bifurcation behind the neoaorta as completely as can be accomplished before initiation of bypass, the right modified Blalock Taussig shunt is encircled with a braided polyester ligature. When the patient is placed on cardiopulmonary bypass and cooled to a nasopharyngeal temperature of 18°C, the shunt is ligated in situ. After cooling is completed, circulatory arrest is established, the aorta cross-clamped, and cardioplegia administered. The venous cannula is removed and the shunt divided at the pulmonary arterial end and the residual stump of shunt on the pulmonary artery excised. (SVC, superior vena cava; r. atrium, right atrium; pulmonary a, pulmonary artery) Operative Techniques in Cardiac and Thoracic Surgery 1997 2, 239-252DOI: (10.1016/S1085-5637(07)70063-3) Copyright © 1997 Elsevier Inc. Terms and Conditions

2 Hemi-Fontan procedure. An incision is made in the pulmonary artery from hilum to hilum to allow the widest possible opening of the pulmonary bifurcation. An incision is also made in the medial aspect of the superior vena cava across the cavoatrial junction onto the right atrial appendage as delineated in this figure. The azygous vein is ligated with a clip if it is readily accessible; however, this is not necessary in all patients. Operative Techniques in Cardiac and Thoracic Surgery 1997 2, 239-252DOI: (10.1016/S1085-5637(07)70063-3) Copyright © 1997 Elsevier Inc. Terms and Conditions

3 Hemi-Fontan procedure. With the pulmonary artery widely opened and the incision in the superior vena cava right atrial junction, suturing is initiated connecting the posterior aspect of the superior vena cava and right atrium to the right pulmonary artery using a running 6–0 polypropylene suture. In this fashion, a wide connection between the superior vena cava and pulmonary artery can be ensured. Operative Techniques in Cardiac and Thoracic Surgery 1997 2, 239-252DOI: (10.1016/S1085-5637(07)70063-3) Copyright © 1997 Elsevier Inc. Terms and Conditions

4 Herni-Fontan procedure. After the posterior suture line is created, a large triangular shaped patch of pulmonary homograft is utilized to augment the pulmonary artery bifurcation. As the suture line along the inferior aspect of the pulmonary bifurcation is created, the suture line is carried into the cavoatrial junction, using the pulmonary homograft to create a dam between the superior vena cava and right atrium. This suture line is brought completely across the cavo-atrial junction to the anterior incision across the cavoatrial junction. Next, the homograft is folded down to create the dam at the level of the atrium and secured with a 5–0 polypropylene suture. Care must be taken with this suture line to completely incorporate the doubled flap of pulmonary homograft along the right lateral aspect of the suture line to prevent any baffle leaks entering the right atrium following the repair. Operative Techniques in Cardiac and Thoracic Surgery 1997 2, 239-252DOI: (10.1016/S1085-5637(07)70063-3) Copyright © 1997 Elsevier Inc. Terms and Conditions

5 Completion of the hemi-Fontan operation. When the baffle is completed, a billowing connection between the superior vena cava and pulmonary bifurcation is created, separating the superior caval flow from the remainder of the atrium and inferior vena cava. We have found it useful to monitor left atrial and pulmonary artery pressures in the immediate postoperative period, and it is simple to place transthoracic lines directly through the suture lines after the hemi-Fontan operation. Placement of a catheter in the medial aspect of the baffle suture line must enter the left atrial side of the baffle because there is no opening into the pulmonary artery at this site. Placement of a catheter in the right lateral aspect of the suture line must enter the pulmonary artery because there is no direct connection to the left atrium in this area. Operative Techniques in Cardiac and Thoracic Surgery 1997 2, 239-252DOI: (10.1016/S1085-5637(07)70063-3) Copyright © 1997 Elsevier Inc. Terms and Conditions

6 Completion Fontan operation after hemi-Fontan procedure. At approximately 1 1/2 to 2 years of age, the completion operation is undertaken. After mobilization of the cardiac structures, which can be limited to mobilization of the neoaorta adequate for cross-clamping and mobilization of the lateral aspect of the right atrium, cannulation of the neoaorta and right atrium is performed and circulatory arrest is utilized. A cardioplegia needle is inserted into the neoaorta as at the hemi-Fontan operation, primarily for cardioplegia injection and for aspiration of air during release of the cross-clamp and early cardiac ejection. With this technique, it is not necessary to completely mobilize the cardiac structures and very limited dissection is required. After circulatory arrest is established, an incision is made along the lateral wall of the right atrium up to and adjacent to the base of the previous hemi-Fontan baffle. Operative Techniques in Cardiac and Thoracic Surgery 1997 2, 239-252DOI: (10.1016/S1085-5637(07)70063-3) Copyright © 1997 Elsevier Inc. Terms and Conditions

7 Completion Fontan. Working through the right atrial incision, the homograft dam between the superior vena cava and right atrium is readily identified and this dam of tissue is excised under direct vision, creating an opening that is adequate in size for baffling of the inferior vena caval flow into the pulmonary arteries. This opening is created in such a way that a rim of tissue can he used for the suture line of the intracardiac lateral tunnel (inset). Operative Techniques in Cardiac and Thoracic Surgery 1997 2, 239-252DOI: (10.1016/S1085-5637(07)70063-3) Copyright © 1997 Elsevier Inc. Terms and Conditions

8 Completion Fontan. A PTFE graft of 10 mm in diameter is opened longitudinally, so that a larger lateral tunnel can be created than the diameter of the graft utilized. Before implantation, an incision is made in the left lower portion of the graft and a 4-mm punch hole made in the graft that can be easily performed before implantation and avoids manipulating the graft after it is sewn in place. The inferior rim of the graft is then trimmed to a gentle curve and sewn into the lateral aspect of the right atrium. Operative Techniques in Cardiac and Thoracic Surgery 1997 2, 239-252DOI: (10.1016/S1085-5637(07)70063-3) Copyright © 1997 Elsevier Inc. Terms and Conditions

9 Completion Fontan. The opened PTFE graft with the previously created fenestration is then secured in the right atrium, excluding the right pulmonary veins behind the baffle with a suture line along the posterior aspect of the right atrium, which is then carried down inferiorly to the eustachian valve, where the graft is sutured along the eustachian valve up to the margin of the incision in the right atrium. Superiorly, the graft is sutured along the wall of the atrial septum up to the level of the excised dam in the cavoatrial junction and then the graft brought to the right and sewn to the margins of the previous excision of the homograft dam up to the level of the incision in the right atrium. The graft at this point can be trimmed if it is excessively wide and then the suture line continued, sandwiching the graft free margin between the two walls of the right atrium to complete the lateral tunnel suture line. After completion of this suture line, the atrium can be filled with saline solution and the venous cannula reinserted. Operative Techniques in Cardiac and Thoracic Surgery 1997 2, 239-252DOI: (10.1016/S1085-5637(07)70063-3) Copyright © 1997 Elsevier Inc. Terms and Conditions

10 Completion Fontan. An advantage of incorporating the wall of the lateral tunnel in the suture line is the ability to easily place lines for postoperative monitoring. Transthoracic atrial lines are positioned on either side of the PTFE baffle, which places one line on the left atrial side of the baffle and the other on the pulmonary arterial side for measurement of pressures postoperatively. In this fashion, no lines need to be placed directly in the internal jugular vein, innominate vein, or superior vena cava, reducing the risk of postoperative caval thrombosis. Operative Techniques in Cardiac and Thoracic Surgery 1997 2, 239-252DOI: (10.1016/S1085-5637(07)70063-3) Copyright © 1997 Elsevier Inc. Terms and Conditions