Extracardiac Fontan fenestration using the SafeSept transseptal guidewire and snare‐controlled diabolo‐shaped covered‐stent placement

Transcatheter Fontan fenestration is a valuable option in situations of prolonged pleural drainage or low cardiac output in patients with failing Fontan circulation. This procedure relies on controlled baffle perforation without separation of the Fontan circuit from the pulmonary venous atrium, and placement of an accurately sized covered stent. We report a novel technique for transcatheter extracardiac Fontan fenestration using the SafeSept transseptal guidewire® and snare‐controlled diabolo‐shaped covered stent placement. © 2015 Wiley Periodicals, Inc.     

Novel technique to reduce the size of a Fontan Diabolo stent fenestration

Objectives : To develop an effective catheter technique to reduce the size of a Diabolo stent fenestration in the failing Fontan circulation. Background : Diabolo stent fenestration is employed by many centers in the treatment of the failing Fontan patient. With subsequent recovery, exercise tolerance may be impaired by significant desaturation secondary to the right to left shunt across the fenestration. Complete fenestration closure carries the risk of recurrence of the initial symptoms and, hence, reduction of the size of fenestration should be the preferred technique. Methods : Twenty‐eight patients with failing Fontan circulations (16 early and 12 late) underwent Diabolo stent fenestration for relief of symptoms. Five of these patients remained very limited by severe desaturation even at rest, after complete recovery from symptoms. Further cardiac catheterization with crimping/reduction of the size of the waist of the stent was carried out using a technique whereby a snare catheter was placed over the waist of the stent aided by an arterio‐venous guidewire loop and a balloon catheter placed within the stent. Results : All 5 patients had successful stent reduction with improvement in saturations, whilst still maintaining a small residual fenestration. No complications were encountered. Conclusion : This novel technique of reduction of a diabolo stent fenestration, in a failing Fontan circulation, offers the advantages of avoidance of implanting further devices in the circulation and the ability to redilate the stent should symptoms recur. © 2010 Wiley‐Liss, Inc.     

Transcatheter interventions in the early postoperative period after the Fontan procedure

Objective : To analyze the safety and clinical impact of interventional cardiac catheter procedures in the management of early postoperative problems after completion of an extracardiac Fontan procedure. Background : The mortality after Fontan procedure has consistently decreased over the last decade. The role of interventional catheterization to address early postoperative problems in this setting has not been studied systematically. Methods : Over a 9.7‐year period, 289 patients underwent an extracardiac fenestrated Fontan procedure with two early deaths (0.7%) and takedown in four (1.4%). Twenty‐seven patients (9.3%) underwent 32 interventional cardiac catheter procedures at a median interval of 12.2 (1–30) days. The median weight was 14.5 (13.5–25) kg. The case notes and procedure records were reviewed retrospectively. Results : Fontan pathway obstructions were treated in 11 patients with stent implantation with good results and no complications. Stent fenestration of the Fontan circulation was performed in 16 patients with one episode of transient hemiparesis and one episode of pericardial effusion. Three patients underwent initial balloon dilatation of branch pulmonary arteries or fenestration with little effect and underwent stent treatment 6 (5–9) days later. One patient had device closure of a large atrial fenestration. In one patient, residual anterograde pulmonary blood flow was occluded using a device. There were no deaths and in‐hospital course was improved in all. Conclusion : Interventional cardiac catheter procedures can be performed safely and effectively in the early postoperative period after Fontan completion to address hemodynamic problems. These techniques contribute significantly to achieve a very low mortality and address morbidity after Fontan completion. © 2010 Wiley‐Liss, Inc.     

Results of transcatheter Fontan fenestration to treat protein losing enteropathy.

Transcatheter fenestration to create an interatrial communication has been used to treat patients with protein losing enteropathy (PLE) after Fontan operation. No systematic data have been reported assessing the results of this procedure. Our institutional database was queried to identify patients after Fontan operation who had transcatheter fenestration to treat PLE. Clinical notes, laboratory data, echocardiograms, and cardiac catheterization data were reviewed. From 1995 to 2005, 16 transcatheter fenestration procedures were performed in seven patients. Median age at fenestration was 18 years (range 13-41 years). Median duration of follow-up was 3.6 years (range 0.2-10.4 years). Techniques for fenestration included blade/balloon septostomy, stent placement, Amplatzer-fenestrated ASD device, and balloon dilation of previous stent. Size of the fenestration created was 5.2 +/- 1.1 mm. Systemic venous pressure remained unchanged after fenestration. Cardiac index increased significantly. Reduction of ascites and edema was noted after 9 of the 16 procedures. Ten of 16 (63%) of fenestrations spontaneously occluded. Three patients are free of ascites although recurrence of PLE occurred in all. One patient with a patent fenestration continues to have ascites. Two patients had Fontan takedown. One patient had conversion to a fenestrated extracardiac conduit Fontan and died postoperatively. The results of transcatheter Fontan fenestration are often disappointing. Maintaining fenestration patency is difficult. Even after "successful" fenestration, resolution of PLE may be incomplete and recurrences have occurred in all. Early consideration should be given to Fontan takedown or cardiac transplant in severely symptomatic patients with PLE who do not respond to fenestration. Transcatheter fenestration may be a bridge to a definitive procedure.     

Atriale Isomerie und viszerale Heterotaxie

Atrial isomerism and visceral heterotaxy describe complex pathoanatomic findings with defects in the determination of lateralization. Differentiation of right and left atrial isomerism was based on the anatomy of the atrial appendages and bronchial tree and often associated with asplenia in right atrial isomerism and polysplenia in left atrial isomerism. In these pa tients complex cardiac anomalies determine clinical symptoms and diagnostic procedures.At primary diagnosis in the neonatal period, systemic venous and pulmonary venous connections and intracardiac anatomy are identified using the sequential segmental approach. Therapeutic decisions and palliative procedures can usually be based on these initial echocardiographic findings. As many of the patients were found to have a functionally univentricular heart, surgical procedures following the Fontan principle (lateral tunnel or extracardiac conduit procedure) were introduced in patients with heterotaxy syndrome and successfully performed. Early survival and long-term outcome vary depending on associated cardiac and systemic and pulmonary venous anomalies.The therapeutic options and mid-term results in patients with heterotaxy syndrome undergoing the Fontan procedure are reported.     

Transcatheter fontan takedown

Early failure of the Fontan circulation is rare in the current era but remains associated with a high mortality rate. Surgical Fontan takedown has evolved as one of the strategies to stabilize the circulation, improve survival, and allow for a future attempt at Fontan completion. We have completed Transcatheter fontan takedown in three patients with extracardiac conduits 0.8–6 months following their Fontan operations. Superior vena cava flow was redirected into only the pulmonary arteries by occluding the conduit with a vascular plug between the pulmonary arteries and fenestration and unrestrictive inferior vena cava flow was redirected into only the atrium by stenting and enlarging the fenestration. There were no procedure related complications. All patients had resolution of large‐volume chylous pleural effusions. One patient had resolution of protein‐losing enteropathy, two patients had improvement of plastic bronchitis. Two of three patients remain alive at latest follow‐up (4–24 months). This early experience suggests that Transcatheter fontan takedown is technically feasible and may be an alternative to surgical takedown in select patients with early failure of the Fontan circulation. © 2015 Wiley Periodicals, Inc.     

Late fenestration of the extracardiac conduit in a Fontan circuit by sequential stent flaring.

Recent Fontan circuits frequently involve an extracardiac conduit. We report on a new technique to create a late fenestration in such an extracardiac circuit by sequential flaring of a stent in the fenestration.     

Use of covered stents to occlude extracardiac Fontan fenestration

INTRODUCTION: A fenestration is usually surgically created to improve the post-operative course of patients undergoing total cavopulmonary connection. It, however, has a potentially deleterious effect on the long-term period. Closure of these fenestrations is usually performed by interventional catheterization. No device has been specially designed and closure of extracardiac fenestration, in particular, can be challenging. We report our experience in occlusion of such fenestrations using covered stents (Numed Inc). METHODS: From July 2005 to October 2005, we attempted to occlude extracardiac Fontan fenestration using CP covered stents in 4 consecutive patients. RESULTS: All patients had a successful occlusion of the fenestration. The procedure was performed from femoral or jugular vein respectively in three and one patient. Mean central venous pressure did not increase significantly (from 11.25 to 12.75 mmHg) whereas mean oxygen saturation increased significantly from 92% to 99% (p=0.0047). Abolition of shunt was obtained immediately after insertion of the covered stent in all patients. No early complications were observed. All patients were ambulatory the day after the procedure. CONCLUSION: The insertion of a covered stent inside the extracardiac Fontan conduit allowed the exclusion of the fenestration in all patients without the need of crossing the fenestration and with no early mortality or morbidity. Long-term follow-up are needed before considering the use of such device as the device of choice in that application.     

Use of stents to maintain atrial defects and fontan fenestrations in congenital heart disease

Maintaining patent atrial septal communications or fenestrations can be vital in conditions requiring adequate decompression of the atria or Fontan baffle. We have recently deployed stents for this purpose, and the aim of this retrospective analysis is to describe our experience. All 26 patients undergoing such stent placement were retrospectively studied and for neonates with hypoplastic left heart syndrome (HLHS) and patients with Fontan fenestrations, their data were compared to controls undergoing transseptal static balloon dilation during the same time period. All 7 stented neonates with HLHS survived to their Norwood procedure and 57% survived to hospital discharge, similar to those who had static balloon dilation. Complications occurred in both HLHS groups but transient complete heart block was only seen in the control group, which also had larger balloons used (10.3 mm vs 7 mm, P=0.002). The success rate for patients undergoing stent placement in Fontan fenestrations was 64% compared to 76% with dilation alone. Complications were seen in 64% of the Fontan stented group compared to 39% for controls. There were 5 other patients with complex lesions (3 of whom were on the Extracorporeal Membrane Oxygenator) in whom stent placement successfully maintained atrial communication patency. Atrial septal stent placement in neonates with HLHS with restrictive defects is effective and appears at least as safe as static balloon dilation. On the other hand, initial fenestration stent placement is indicated only after extracardiac Fontan procedures in which the previous fenestration location cannot be found.     

Closure of extrocardiac Fontan fenestration by using the Amplatzer duct occluder

INTRODUCTION: A direct or tubular communication between the systemic venous system and the systemic atrium, generally called fenestration, is surgically created to improve the postoperative period of patients undergoing total cavopulmonary connection. However, a fenestration prompts a potentially deleterious right to left shunt, and is generally closed after the postoperative period. Direct fenestrations can be closed using coils, or devices designed for atrial septal defect closure. However, no devices have been designed for closure of extracardiac fenestrations. We report our experience concerning the closure of extracardiac Fontan fenestration by the Amplatzer duct occluder (ADO). METHODS: From January 2001 to December 2002, we closed extracardiac Fontan fenestrations using the ADO device in 10 consecutive patients. Indications to fenestration closure were: low velocity shunt through the fenestration, mild desaturation, and absence of effusions. RESULTS: All patients had a successful closure of the fenestration. The procedure was performed through the femoral vein in 7 cases and through the right jugular vein in 3. Mean central venous pressure increased not significantly from 12 to 13-mmHg. Mean oxygen saturation increased significantly from 90 to 97% (p<0.001). Immediate shut abolition was obtained in 9 cases. No complications were observed. At a median follow-up of 12 months (range 6-18 months), all patients are free of symptoms and have a normal oxygen saturation at rest as well as at exertion. CONCLUSION: The ADO device allowed closing the extracardiac Fontan fenestration in all patients with no mortality, no morbidity and a rate of 100% of complete closure at mid-term follow-up.     

Catheter closure of tube graft fenestration in extracardiac Fontan operation

As the extracardiac Fontan operation evolves, a reliable method for creating and subsequently closing communications between the systemic and pulmonary venous chambers would become useful. We describe a simple and safe technique of fenestration amenable to coil occlusion, which can be carried out in most cardiac catheterization laboratories.     

Left‐sided Scimitar Vein Causing Cyanosis after Fontan Operation: Successful Transcatheter Device Occlusion Using Magnetic Resonance Imaging X‐ray Fusion

We present the case of a 3‐year‐old boy with asplenia‐type heterotaxy syndrome and functionally single ventricle congenital heart disease who developed cyanosis early after the Fontan operation. Combined cardiac magnetic resonance imaging (MRI) and catheterization identified a large hepatic vein to pulmonary vein connection as the source of right to left shunt. The anatomy was quite unusual, suggesting an underlying diagnosis of mixed total anomalous pulmonary venous connection with left‐sided scimitar vein. This pattern of pulmonary venous return has not been previously reported in a patient with asplenia. MRI x‐ray fusion was used to guide transcatheter device occlusion of the scimitar vein, resulting in marked clinical improvement.     

Transhepatic Approach for Extracardiac Inferior Cavopulmonary Connection Stent Fenestration

We report on a 3‐year‐old male who underwent transcatheter stent fenestration of the inferior portion of an extracardiac total cavopulmonary connection in the setting of hypoplastic left heart syndrome. Transhepatic approach, following an unsuccessful attempt from the femoral vein facilitated delivery of a diabolo‐shaped stent.     

Incidence and factors influencing the spontaneous closure of Fontan fenestration

Introduction: The Fontan operation is the final stage of single ventricle palliation in patients with complex congenital heart disease. Fenestration in the Fontan conduit, providing an atrial level right to left shunt, has been shown to reduce early postoperative morbidity. However, there is limited data on the long‐term fate of this fenestration. The aim of this study is to define the rate of spontaneous closure of the fenestration in the Fontan conduit and factors predictive of the fate of the fenestration.
Methods: This was a retrospective study reviewing the medical records of the patients who underwent fenestrated Fontan operation at our center. Preoperative, intraoperative and postoperative variables including the status of the Fontan fenestration were extracted and analyzed.
Results: Of 67 patients included in the study, 15 (22%) had spontaneous closure of the fenestration. Of the remaining 52 patients, 11 (20%) had procedural closure of this fenestration (10 via cardiac catheterization and 1 via surgery) at a median duration of 3 months after the Fontan operation. Patients with higher preoperative pulmonary vascular resistance and a history of postoperative systemic venous thromboembolism had higher likelihood of having persistence of the fenestration with P value of .045 and .037, respectively.
Conclusions: The rate of spontaneous closure of the Fontan fenestration was 22% in our study. Elevated preoperative pulmonary vascular resistance and history of systemic venous thromboembolism are predictive of persistent Fontan fenestration.     

Stent implantation to create interatrial communications in patients with complex congenital heart disease

A restrictive interatrial communication can complicate the management of complex congenital heart disease. The purpose of this report is to present a new technique to achieve a patent and reliable interatrial communication by using an endovascular stent. A stent was successfully implanted across a fenestrated extracardiac conduit in two patients with low cardiac output after Fontan operations and across the interatrial septum in a patient with double inlet left ventricle and severe left atrioventricular stenosis. The procedures were uncomplicated and all patients showed immediate hemodynamic improvement. Cathet. Cardiovasc. Intervent. 47:310–313, 1999. © 1999 Wiley-Liss, Inc.     

First implantable hemodynamic monitoring device placement in single ventricle fontan anatomy

The Fontan anatomy leads to elevated central venous pressure along with chronic venous congestion and low cardiac output; this is felt to be responsible for deterioration of exercise tolerance and functional capacity over time. Real‐time hemodynamic evaluation of the Fontan anatomy has not been evaluated until now. Here, we report the technical aspects of the first two adult Fontan patients to undergo placement of an invasive hemodynamic monitor (IHM). We validate IHM readings with invasive pulmonary artery catheter derived hemodynamics in the Fontan and show successful home transmission of pulmonary artery hemodynamic tracings. This technology has the capacity to change current understanding of Fontan hemodynamics and treatment in patients with complex single‐ventricle anatomy. © 2016 Wiley Periodicals, Inc.     

New technique for fenestration of the interatrial septum

Achieving controlled flow between the systemic and pulmonary venous circulations is desirable in many complex congenital heart diseases. This includes the Fontan circulation, primary pulmonary hypertension, double inlet ventricles, or hypoplastic left heart with obstruction to the atrioventricular valve. As no specific device is available for this purpose, we developed a balloon-mounted stent technique to achieve a predetermined-sized fenestration of an atrial baffle in a patient with Fontan circulation. The details of the technique are described.     

Is it necessary to routinely fenestrate an extracardiac fontan?

OBJECTIVES: This study was conducted to assess the need for, and use of, fenestration of an extracardiac conduit Fontan. BACKGROUND: Fenestration of a Fontan connection has been proposed as a means of improving outcomes of single ventricle palliation. The benefit of fenestration is likely to be greatest in the early postoperative period when patients may experience increased pulmonary vascular resistance and decreased ventricular function due to the effects of cardiopulmonary bypass, aortic cross-clamping and positive pressure ventilation. However, there are potential drawbacks to fenestration. The utility of fenestration with extracardiac Fontan operation has not been determined. METHODS: Since 1992, 81 patients have undergone a modification of the Fontan procedure in which an extracardiac inferior cavopulmonary conduit is used in combination with a previously staged bidirectional Glenn anastomosis. We conducted a retrospective review of these patients. RESULTS: Fenestration was performed selectively in 32 patients (39%), including only 2 of the last 38 (5%). In seven patients, a fenestration was placed or clipped in the early postoperative period without cardiopulmonary bypass. There were two operative deaths. Prolonged (>2 weeks) pleural drainage occurred in 13 patients, 8 with fenestration and 5 without. In addition to undergoing earlier Fontan in our experience, patients who had a fenestration placed had significantly higher preoperative pulmonary vascular resistance, significantly higher common atrial pressure after Fontan and significantly lower post-Fontan systemic arterial oxygen saturation. Fontan pressure did not differ between nonfenestrated and fenestrated patients. At follow-up ranging to five years, there were two late deaths and no patients developed protein losing enteropathy. CONCLUSIONS: Fenestration is not necessary in most Fontan patients when an extracardiac conduit technique is performed as described in this article, and therefore, should not be performed routinely with the extracardiac conduit Fontan. The need for fenestration should be assessed after cardiopulmonary bypass when hemodynamics can be evaluated accurately. Fenestration can be placed and revised easily without bypass and with minimal intervention in patients with an extracardiac conduit Fontan.     

CT angiography in Fontans with implanted stents

BackgroundCT angiography is used as a non-invasive method in the evaluation of patients with Fontan circulation. For good visualization of patients having undergone the Fontan operation the optimal scan timing and adequate intravenous route is important.PurposeThe aim of this study was to confirm that computer tomography is very a good tool for assessment of patients after Fontan procedure with implanted stents in pulmonary arteries or in fenestration.Material and methodsSix patients with Fontan circulation and implanted stent in left pulmonary artery or in fenestration underwent CT angiography. The CT angiography was successfully performed to all patients. For homogenous enhancement of Fontan pulmonary arteries and Fontan tract we decided to use 1-minute delay scan with right antecubital application of contrast agent. The optimal enhancement was evaluated at the right pulmonary artery (RPA), left pulmonary artery (LPA), and Fontan tract. Optimal enhancement was defined when evaluation of stent was possible.ResultsOptimal enhancement when the stent was possible to evaluate intraluminally was achieved in seven CT examinations. The Bland–Altman test demonstrated good agreement between readers.ConclusionsThis study demonstrates that CT angiography is a fast, accurate and reproducible method in the evaluation of patients with Fontan circulation, and implanted stent in pulmonary arteries or in fenestration.