The Fontan operation was first described for management of univentricular heart disease in 1971. Since it was first described, there have been several modifications to the original operation.1 In 1988, de Laval2 first proposed the concept of total cavopulmonary anastomosis (construction of an intra-atrial lateral tunnel to connect the inferior vena cava to the pulmonary artery together with a bidirectional cavopulmonary shunt) as an alternative to atriopulmonary connection. The extracardiac approach remains popular because it eliminates the need for aortic cross-clamping, ischemic arrest, and avoids extensive atrial incisions.3–5 Because the extracardiac lateral tunnel is constructed utilizing prosthetic material with a fixed diameter, one of its potential drawbacks is the lack of growth, thereby possibly limiting its use in young children. In 1997 Gundry6 first described the use of autologous living pericardium to construct the extracardiac Fontan. Utilizing this technique, an extracardiac tunnel from the inferior vena cava to the right pulmonary artery is created by suturing a pedicled pericardial flap to the free wall of the right atrium. This approach confers all the advantages of the extracardiac approach and also provides potential for growth of the extracardiac lateral tunnel. In Gundry’s initial description of the procedure, none of the patients required fenestration. The paper further postulated that fenestration of this type of connection could be accomplished in the cardiac catheterization laboratory. Review of the literature has not shown a single case of fenestration creation via the transcatheter approach in patients who have undergone APEF. We describe a patient with HLHS who developed PLE following APEF operation with resolution of PLE following transcatheter fenestration creation in the cardiac catheterization laboratory. Case Report. A 3-year-old child previously diagnosed with HLHS initially underwent a Norwood operation with placement of a 4 mm modified right Blalock Taussig shunt at 10 days of age. This was followed by a bidirectional cavopulmonary shunt with patch plasty of both the right and left pulmonary arteries at 9 months of age. Cardiac catheterization prior to APEF demonstrated long segment left pulmonary artery (LPA) stenosis requiring stent implantation. The patient underwent successful APEF at 2 years of age. The postoperative course was significant for prolonged chest tube drainage and persistent bilateral pleural effusion. Cardiac catheterization 6 weeks following APEF demonstrated elevated central venous pressure (CVP)(mean pressure = 20 mmHg) and residual proximal LPA stenosis requiring implantation of a second stent in the proximal LPA. Post-stent implantation, there was an improvement in CVP (mean pressure = 17 mmHg). The child did well and was discharged home. Over the course of the next few months, the patient developed bilateral pleural effusion, abdominal distention, hepatomegaly and periorbital edema. Laboratory tests revealed low serum albumin 1.5 g/dl, low serum total protein 4.3 g/dl, and low serum calcium 7 mg/dl. This was initially managed with diuretic therapy (furosemide and spironolactone) and regularly scheduled albumin infusion. On the recommendation of the gastroenterology service, the patient was also treated with oral steroids and heparin subcutaneously. The patient remained stable for several months until he presented with anasarca. Because of clinical deterioration, the decision was made to proceed with cardiac catheterization with an attempt at Fontan fenestration creation. The risks and benefits of the procedure were discussed at length with the family and consent was obtained. Cardiac catheterization was performed under general anesthesia. Arterial access was obtained and right heart catheterization through a 5 French sheath in the right internal jugular vein (RIJ) was performed (the patient had bilateral femoral vein obstruction). The mean pressure recorded throughout the Fontan circuit was 17 mmHg. The mean pulmonary capillary wedge pressure was 7 mmHg, and right ventricular pressure was 80/0.7 mmHg. Angiography demonstrated an unobstructed Fontan anastomosis. Because of the convex nature of the right atrium (RA), it would have been difficult to perform “transseptal” puncture of the RA from the RIJ approach (the RA forms the medial wall of the extracardiac Fontan baffle in the APEF modification) (Figure 1). For this reason, the decision was made to perform the RA puncture from the transhepatic approach. Technique. The right chest and abdomen were prepared and draped in the usual sterile fashion. Utilizing a commercially available kit (Neff Percutaneous Access Set, Cook Inc., Bloomington, Indiana), transhepatic puncture was performed under fluoroscopic guidance. The chiba needle from the Neff Percutaneous set was introduced into the liver in the right-mid to anterior axillary line at the lower costal margin. The needle was angled superiorly and posteriorly towards the intrahepatic inferior vena cava until it reached the right margin of the spine. The needle was then gently withdrawn and contrast was injected until there was visualization of a hepatic vein. A 0.018 inch floppy-tipped Platinum Plus wire (Boston Scientific/Meditech, Miami Florida) was advanced through the needle into the inferior vena cava. The coaxial dilator from the Neff set was then advanced over the Platinum Plus wire into the Fontan baffle. The 0.018 inch floppy-tipped wire was then exchanged for a 0.025 inch Platinum Plus wire to improve stability within the Fontan baffle. The coaxial dilator was then exchanged for a 6 French Mullins transseptal sheath (TS) (Medtronic AVE, Galway, Ireland), which was positioned within the Fontan baffle against the RA (Figures 2 A and B). The Platinum Plus wire was then exchanged for a transseptal needle. The transseptal needle was then attached to pressure tubing. Transesophageal echocardiography (TEE), together with biplane fluoroscopy, was then utilized to safely gain access into the pulmonary venous atrium. After successfully puncturing the RA, confirmation of proper position within the pulmonary venous atrium was obtained by means of left atrial pressure waveform, hand injection of contrast through the transseptal needle and TEE imaging. With the TS positioned within the pulmonary venous atrium, blade septostomy was performed with a 9.4 Park blade (Cook, Inc., Bloomington, Indiana) (Figures 3 A and B). TEE imaging demonstrated what appeared to be a widely patent fenestration, however after a few minutes, the fenestration appeared to close. Repeat transseptal puncture of the right atrial wall was performed as previously described. Static balloon atrial septostomy was then performed utilizing a Marshal 8 mm x 2 cm balloon dilation catheter (Boston Scientific/Meditech, Natick, Massachusetts). A total of three inflations were performed (Figures 4 A and B). Room air saturation measurement, which had initially been recorded as 90%, decreased to 80%. TEE demonstrated a widely patent fenestration with right-to-left flow. A few minutes later, there was a gradual increase in saturation measurement and TEE demonstrated an almost closed fenestration. An attempt was made to advance an endhole catheter through the TS positioned within the Fontan circuit to gain access into the pulmonary venous atrium. These attempts were unsuccessful and transseptal puncture of the RA was again performed. Repeat blade and static balloon atrial septostomy (BBAS) was performed as previously described. At the end of the procedure, the mean CVP = 11 mmHg, saturation measurement remained in the low 80s, and the fenestration was patent angiographically and by TEE (Figure 5). The TS was positioned in the inferior vena cava and the dilator was reintroduced into the sheath. Contrast was injected through the dilator, as both the sheath and dilator were pulled back into the liver parenchyma. With both the sheath and dilator positioned within the liver parenchyma, Surgifoam (Absorbable Gelatin Sponge, Ethicon Sommerville, New Jersey), diluted with normal saline and contrast, was injected through the dilator into the hepatic parenchymal tract until the edge of the liver capsule was reached. Pressure was held over the transhepatic puncture site for 45 minutes. The child was admitted to the pediatric intensive care unit for overnight observation. Follow-up transthoracic echocardiography the following morning demonstrated a widely patent fenestration. The patient is now 7 months post-fenestration creation with a room air saturation of 84% and a widely patent fenestration demonstrated by transthoracic echocardiography. More importantly, the anasarca has resolved and the patient has normal total protein and albumin levels. Discussion. The autologous extracardiac lateral tunnel utilizing pedicled pericardium is a recent modification of the Fontan operation.6 The major theoretical advantage of this modification is that it utilizes one’s own pericardium (with its own blood supply) to create the extracardiac lateral tunnel, thereby retaining its potential for growth. When this proposed modification was first described, none of the patients required fenestration.6,7 Surgical fenestration has since been reported in 2 cases.8 In both cases, surgical fenestration was required for elevated CVP at the time of the Fontan operation. We describe what we believe to be the first reported transcatheter fenestration creation in a patient who developed PLE with elevated CVP after undergoing the APEF operation. When the APEF operation was first described, it was postulated that a fenestration could be created through the RA in the catheterization laboratory.6 This report describes transcatheter fenestration creation via the transhepatic approach. As this report describes, it required several right atrial punctures and performance of several BBAS procedures to create a patent fenestration. While there was no difficulty puncturing the RA, keeping the fenestration patent required several BBAS procedures. The authors postulate that because the RA forms the medial wall of the extracardiac lateral tunnel in the APEF modification, the pectinate muscles within the RA may have played a part in closing the fenestration. While a patent fenestration can be created as described above, other alternatives that could have been utilized to keep the fenestration from closing are the utilization of a cutting balloon (limited by availability of larger diameter balloons), creation of a butterfly stent,9 use of a fenestrated Amplatzer device,10 or possibly utilizing a larger-diameter balloon dilation catheter to enlarge the fenestration after performance of blade septostomy. In retrospect, stent implantation after a single transseptal puncture of the right atrial wall would probably have been technically simpler and could have achieved better control of the size of the fenestration. In summary, we describe the first reported case of successful transcatheter fenestration of APEF in a patient with PLE via the transhepatic approach. Early- to medium-term follow-up demonstrates continued patency of the fenestration 7 months after successful creation of the fenestration in the catheterization laboratory. The authors recommend continued close follow-up.
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