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 repair of Fontan baffle stenosis and fenestration in two patients using Cook Zenith aortic endografts

Patients with Fontan baffles for single ventricle may have cyanosis from right-to-left shunt through leaks in the baffle or due to intentionally created fenestrations. Typically this right-to-left shunt may be addressed with catheter-based occlusion devices. However, in narrowing of the Fontan baffle, placement of occluders within the Fontan baffle may additionally narrow the pathway and is therefore undesirable. We describe 2 patients with the combination of Fontan baffle stenosis and patent fenestration treated with a Zenith abdominal aortic aneurysm endograft (Cook Medical). The covered stent graft both occluded the right-to-left shunt and eliminated the baffle stenosis. Both patients have had symptomatic improvement.     

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.     

Transhepatic approach to create stent fenestration in the extracardiac fontan conduit in a child with dextrocardia and interrupted inferior vena cava with azygos continuation

Plastic bronchitis is a rare life‐threatening complication of Fontan operation. When medical treatment is ineffective in the setting of high systemic venous pressures, Fontan fenestration may be considered to decompress venous pressures and improve cardiac output by creation of the right‐to‐left shunting. However, transcatheter approach can be difficult in patients with complex venous anatomy. We report a 4‐year‐old girl born with hypoplastic left ventricle and heterotaxy syndrome, who developed plastic bronchitis following extracardiac Fontan procedure. Her venous anatomy was complex with dextrocardia and interrupted inferior vena cava with azygos continuation. Stent fenestration was successfully performed via transhepatic approach, which was selected based on the anatomical relationship (between extracardiac conduit, left atrium, and hepatic veins) delineated by pre‐catheterization cardiac MRI. Simultaneous transesophageal echocardiography guided the intervention. Her plastic bronchitis improved significantly in 3 months but slowly progressed after the stent fenestration. At her 8‐month follow‐up, stent fenestration remains open and she is currently under heart transplantation evaluation due to persistent plastic bronchitis. Treatment of plastic bronchitis can be undertaken with Fontan fenestration, with pre‐procedural MRI playing an essential role in patients with complex venous anatomy. © 2012 Wiley Periodicals, 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.     

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.     

NEW METHOD OF COVERED WALLSTENTS FOR DISTAL MALIGNANT BILIARY OBSTRUCTION TO REDUCE EARLY STENT‐RELATED COMPLICATIONS BASED ON CHARACTERISTICS

Aim: We previously reported a low occlusion rate with covered Wallstents for malignant biliary obstruction, but stent‐related complications other than occlusion posed a problem. A modified covered Wallstent insertion method based on stent characteristics was evaluated to reduce stent‐related complications. Methods: A total of 138 patients with distal malignant biliary obstruction received covered Wallstent placement. From October 2001 to October 2003, 69 patients received covered Wallstent placement (Group 1). Thereafter, we modified our stent insertion method and 69 patients received stent placement using this modified method from November 2003 to January 2007 (Group 2). The modified insertion method consists of endoscopic sphincterotomy carried out in patients without pancreatic duct invasion and longer stent placement with the center of the stent located in the center of the biliary stricture to prevent pancreatitis, kinking of the bile duct, and stent dislocation. A comparative analysis was carried out using prospectively collected data in these two cohorts. Results: Tumor ingrowth was not observed, and stent occlusion rate was 18.8% in Group 1 and 23.2% in Group 2. The overall rates of stent‐related complications did not differ (39.1% in Group 1 and 30.4% in Group 2), but stent‐related complications within 3 months decreased from 22 episodes in Group 1 to 13 episodes in Group 2. Median event‐free survival was prolonged by modified stent insertion method (125 days in Group 1 and 268 days in Group 2, P = 0.020), although cumulative survival and stent patency were not significantly different. Conclusions: Our modified method of covered Wallstent placement showed improved event‐free survival.     

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.     

De novo fenestration of extra‐cardiac fontan goretex conduit assisted by inoue balloon

De novo fenestration of Goretex conduit of extra‐cardiac total cavopulmonary connection in the postoperative period is challenging, and is rarely reported. We report a 17‐year‐old boy with failing Fontan circuit in whom fenestration was created, aided by an Inoue balloon. © 2012 Wiley Periodicals, Inc.     

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.     

TEMPORARY USE OF A NEW FULLY‐COVERED SELF‐EXPANDING METAL STENT FOR THE MANAGEMENT OF POST‐ESOPHAGECTOMY STRICTURES

Perioperative morbidity rates following esophagectomy for esophageal cancer remain quite high (26–41%) even at high‐volume centers. Complications may include stricture at the esophagogastric (EG) anastomosis, as well as tracheo‐esophageal or tracheo‐gastric fistula formation. Fully‐covered self‐expanding metal stents (FCSEMS) have only recently been described for use in benign esophageal disease. The use of FCSEMS for the management of postoperative complications following esophagectomy has not been well studied. We report our observations in three consecutive patients that underwent placement and subsequent removal of a new, fully‐covered metal stent (Wallflex^® esophageal stent) for treatment of dysphagia due to a persistent stricture at the EG anastomosis.     

"Taking in the waist": adjusting the size of a stented fontan fenestration

Early spontaneous closure of a fenestration following Fontan palliation may complicate the postoperative management of such patients. The creation of a fenestration in the catheterization laboratory with an intravascular stent may improve these patients' hemodynamic status. The aim of this study is to present a new technique to reduce the diameter of stented Fontan fenestrations in those patients in whom the stent diameter is functionally made too large.     

Percutaneous transcatheter communication between the pulmonary artery and atrium following an extra‐cardiac Fontan

In a nonfenestrated modified Fontan, transcatheter creation of a fenestration presents technical difficulties, especially with the extra‐cardiac modification where a Gore‐Tex tube is placed between the inferior caval vein and the pulmonary arteries. The authors describe an alternative approach to perforating the Gore‐Tex tube to create a fenestration by making a communication between the pulmonary artery and the atrial chamber, thus bridging the two circulations through native tissue. © 2008 Wiley‐Liss, 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.     

Topic controversies in the endoscopic management of malignant hilar strictures using metal stent: side‐by‐side versus stent‐in‐stent techniques

Endoscopic management of unresectable hilar malignant biliary stricture (MBS) is currently challenging, and the best approach is still controversial. Liver volume is the key to adequate biliary drainage in hilar MBS and multiple stenting is mandatory to drain over 50% of liver volume in most cases. The self‐expandable metallic stent (SEMS) has shown superior patency to plastic stents in recent reports. There are two methods of multiple stenting for hilar MBS: stent‐in‐stent (SIS) and side‐by‐side (SBS). Advantages of SIS include multiple SEMS placement in one stent caliber at the common bile duct (CBD), which is considered physiologically ideal. The through‐the‐mesh (TTM) technique with guidewires and the SEMS delivery system can be technically difficult in SIS, although the recent development of dedicated SEMSs having a loose portion facilitating the TTM technique makes SIS technically feasible both at stent deployment and re‐interventions. Conversely, the SBS technique, if placed across the papilla, is technically simple at initial placement and re‐intervention at stent occlusion. However, SBS has potential disadvantages of overexpansion of the CBD because of parallel placement of multiple SEMS, which can lead to portal vein thrombosis. Given the limited evidence available, a well‐designed randomized controlled trial comparing these two techniques is warranted.     

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.     

A new Amplatzer device to maintain patency of Fontan fenestrations and atrial septal defects.

This study evaluated use of Amplatzer fenestrated device to maintain patency of the Fontan fenestration and atrial septal defect. Fenestrations are routinely created in patients with lateral tunnel or extracardiac Fontan. Spontaneous closure of the fenestration can lead to Fontan circulation failure. Other patients without single-ventricle physiology may benefit from a small communication between the left and right atria for decompression if closure of the atrial septal defect leads to failure of a dysfunctional ventricle. Amplatzer septal occluder device was modified to create a fenestration through the disks. Three patients with modified Fontan and one patient with a large atrial septal defect underwent placement of the device by transcatheter technique. The device deployment was guided by transesophageal echocardiography. The procedure was successful in all patients. Contrast injection after placement revealed patent fenestration with free flow. Follow-up ranged from 3 months to 1 year. All devices were patent by transthoracic echocardiography. These preliminary results suggest that the Amplatzer fenestrated device can serve as a valuable tool in failing Fontan circulation and may help to avoid surgical intervention. More studies are needed to assess long-term efficacy of the device.     

Thrombosis of an extracardiac fontan tunnel: Combined treatment of thrombolysis and stenting

Obstruction of the so‐called Fontan tunnel represents a life‐threatening complication and requires immediate intervention. We describe the successful use of prolonged thrombolysis with rt‐PA, allowing stent placement in an extracardiac tunnel in a 7‐year‐old boy. © 2009 Wiley‐Liss, Inc.     

Large diameter fully covered self‐expanding metal stent placement for palliation of proximal malignant esophageal strictures

In patients with malignant esophageal strictures within 6 cm from the upper esophageal sphincter, self‐expanding metal stents placement represents a challenge because there is an increased risk of complications. The aim of this study was to assess the safety and effectiveness of large‐diameter WallFlex^® fully covered self‐expanding metal stents for palliation of patients with proximal malignant esophageal strictures. From March 2010 to December 2012, 12 patients with proximal strictures (4–6 cm from the upper esophageal sphincter) and six with very proximal strictures (<4 cm from the upper esophageal sphincter) were palliated with this fully covered self‐expanding metal stent and included in the study. Technical success was 100% and clinical success was 94%. The mean baseline dysphagia score was 3.2, and 1 week after stenting it improved significantly to 1.3 (P < 0.001). Early complications occurred in four patients, more frequently in patients with very proximal strictures as compared with patients with proximal strictures (P = 0.02). Late complications occurred in five patients, and there were no differences between patients with very proximal strictures or proximal strictures (P = 0.245). The mean survival after stent placement was 119 days, and no differences between patients with very proximal strictures versus proximal strictures were found (P = 0.851). There was no stent‐related mortality or 30‐day mortality. Our results suggested that a large‐diameter fully covered self‐expanding metal stent is an effective and secure device for palliation of patients with proximal malignant esophageal strictures.     

Safety and efficacy of a fully covered large‐diameter self‐expanding metal stent for the treatment of upper gastrointestinal perforations,anastomotic leaks,and fistula

Upper gastrointestinal perforations, fistula, and anastomotic leaks are severe conditions with high mortality. Temporary endoscopic placement of fully covered self‐expanding metal stent (fSEMS) has emerged as treatment option. Stent migration is a major drawback of currently used stents. Migration is often attributed to a relatively too small stent diameter as esophageal stents were initially intended for the treatment of strictures. This study aimed to investigate the safety and efficacy of a large‐diameter fSEMS for treatment of these conditions. Data were retrospectively collected from patients who received this stent in the Netherlands between March 2011 and August 2013. Clinical success was defined as sufficient leak closure after stent removal as confirmed by endoscopy or X‐ray with oral contrast without surgical intervention or placement of another type of stent. Adverse events were graded according a standardized grading system. Stent placement was performed in 34 patients for the following indications: perforation (n = 6), anastomotic leak (n = 26), and fistula (n = 2). Technical success rate was 97% (33/34). Clinical success rate was 44% (15/34) after one stent and 50% (17/34) after an additional stent. There were no severe adverse events and stent‐related mortality. The overall adverse event rate was 50% (all graded ‘moderate’). There were 14 (41%) stent migrations (complete n = 8, partial n = 6). Other adverse events were bleeding (n = 2) and aspiration pneumonia (n = 1). Reinterventions for failure of the large‐diameter fSEMS were placement of another type of fSEMS (n = 4), surgical repair (n = 3), or esophagectomy (n = 1). Eleven patients (32%) died in‐hospital because of persisting intrathoracic sepsis (n = 10) or preexistent bowel ischemia (n = 1). This study suggests that temporary placement of a large‐diameter fSEMS for the treatment of upper gastrointestinal perforations, fistula, and anastomotic leaks is safe in terms of severe adverse events and stent‐related mortality. The larger diameter does not seem to prevent stent migration.