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.     

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.     

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.     

Characterization and treatment of systemic venous to pulmonary venous collaterals seen after the Fontan operation

OBJECTIVES: To determine the anatomical characteristics of systemic venous collaterals formed after the Fontan operation, and the efficacy of a transcatheter strategy for management. METHODS: We reviewed retrospectively the data from cardiac catherization of 50 persistently cyanotic patients after the Fontan operation. RESULTS: A total of 54 transcatheter interventions were performed, at a mean age of 6.3 +/- 3.5 years, a mean interval of 2.7 +/- 2.9 years from completion of the Fontan circulation. Of 38 patients who had fenestration of the baffle at the time of surgery, 25 had patency of the fenestration, and 24 had the fenestration occluded with a device at the time of interventional treatment for associated venous collaterals. We identified a total of 68 systemic venous collateral channels, of which 36 (53%) were supracardiac, 12 (18%) cardiac, and 20 (29%) infracardiac in origin. The most common site of origin was the brachiocephalic vein (44%), followed by the left phrenic vein (25%). A longer time from surgery, at 3.3 +/- 3.4 years, was associated with the identification of collaterals having a diameter larger than 4 mm (p < 0.01). The mean pulmonary arterial pressure was higher in those with larger compared to those with smaller collaterals (13.3 +/- 2.8 versus 11.1 +/- 2.0 mmHg, p < 0.01). Coils were used for occlusion of 61 vessels, and a Rashkind occluder for the remaining 7. After exclusion of the patients undergoing simultaneous closure of their fenestration, systemic saturation of oxygen increased from 89 +/- 6% to 95 +/- 3% (p < 0.01). CONCLUSION: Venous collateral channels are common in patients suffering progressive cyanosis in the setting of the Fontan circulation. The collaterals increase in size with time, and are associated with higher pulmonary arterial pressures. Transcatheter treatment is feasible, and results in resolution of cyanosis. Only continuing follow-up will show whether further collateralization occurs in time.     

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.     

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.     

Feasibility and clinical impact of transcatheter closure of interatrial communications after a fenestrated Fontan procedure: medium-term outcomes.

OBJECTIVE: This study was to review an institutional experience with transcatheter closure of Fontan fenestrations and its impact on clinical care. BACKGROUND: An interatrial fenestration improves postoperative outcomes in high-risk children undergoing a Fontan repair. While technical feasibility has been well defined, the clinical impact of subsequent closure is not well defined. METHODS: Transcatheter closure of a surgically created or additional interatrial communication was attempted in 152 children at a median interval of 13.8 months after surgery. The clinical records were reviewed for demographic and anatomical characteristics, previous surgeries; catheterization data, and status at latest follow-up. RESULTS: Mean oxygen saturation and right atrial pressure increased acutely from 87% +/- 5% to 96% +/- 3% (P < 0.001) and 12 +/- 2 mm Hg to 13 +/- 3 mm Hg (P < 0.001), respectively. Higher systemic venous atrial pressures after occlusion correlated with higher pulmonary artery pressures (P = 0.05) before the Fontan procedure and with higher right (P < 0.001) and left atrial (P = 0.001) and ventricular end-diastolic pressures (P < 0.001) immediately before occlusion. Complications included device malposition in 2 children, 1 child each had an air embolism and post-procedural bleeding, and each self-limiting and 1 child had acute ST elevation in inferior ECG leads because of occlusion of the acute marginal branch which was treated with angioplasty and placement of a stent. At follow-up (median 4.5 years), the mean oxygen saturation was 95% +/- 3%. Residual interatrial leaks were noted echocardiographically in 9%. Two children developed protein-losing enteropathy after fenestration closure. No deaths or strokes were observed in follow-up. CONCLUSIONS: Transcatheter occlusion of Fontan fenestrations is safe with acute and persistent improvements in oxygen saturations.     

Dilatation and stenting of the fontan pathway: impact of the stenosis treatment on chronic ascites

OBJECTIVE: Low pulmonary artery pressure (PAP) and unobstructed flow in the pulmonary arteries (PA) and the systemic veins are the most important factors for optimal long-term Fontan hemodynamics. We retrospectively analyzed the impact of transcatheter interventions for treatment of stenoses in the Fontan pathway to optimize the circulation, with special attention to chronic ascites. PATIENTS AND DESIGN: Sixty-three of a total of 112 patients underwent heart catheterization 4.6 (1.0-11.4) years after Fontan operation. The median age of the patients at the end of follow-up was 11 (3.1-45) years. Patients were divided into two groups, one with chronic ascites and one without. The impact of elevated PAP and of morphologic stenoses in the Fontan pathway for the development of chronic ascites was analyzed. RESULTS: Ascites was observed in 11 patients; 5 of them had stenosis in the Fontan pathway (P = 0.010). High PAP of 15 mmHg or more (n = 13) was correlated positively with the development of ascites (5 of 13, P = 0.040). Twenty interventions for removal of stenosis in the Fontan pathway were performed in 14 patients. We observed temporary relief of ascites in all and complete resolution of ascites after alleviation of the stenosis in 2 patients with PAP <12 mmHg. CONCLUSION: Transcatheter treatment of stenosis can effectively improve the hemodynamics in Fontan circulation, even in patients without measurable pressure gradient. In those with low PAP, it may lead to the disappearance of chronic ascites.     

Protein-Losing Enteropathy Following Fontan Palliation

Protein-losing enteropathy (PLE) is a chronic condition involving multiple organ systems that may develop any time following Fontan completion. The pathogenesis of PLE is complex and multifactorial. Chronic venous hypertension, low cardiac output, and abnormal lymphatics may all play a role in the pathogenesis of PLE. Common signs and symptoms include chronic diarrhea, abdominal pain, and ascites. Diagnosis is based on the presence of signs and symptoms in addition to hypoalbuminemia and elevated stool alpha 1 antitrypsin. Early identification and a comprehensive approach to evaluation and treatment are important, as they may affect survival. The initial evaluation should include cardiac catheterization for hemodynamic assessment. Although an evidence base for treatment is lacking, various medical, interventional, and surgical approaches have been described with variable degrees of success. Commonly used therapies include nutritional support, diuretics, subcutaneous unfractionated heparin, budesonide, and sildenafil. Limited data exist for Fontan conversion or takedown. Assessment for heart transplantation should be considered. PLE mortality is high—approximately 50%—but may be mitigated by aggressive investigation and management. The evolving understanding of the role of lymphatics in the pathophysiology of PLE and the emerging role of interventional lymphatic procedures may further improve outcomes in this patient population.     

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.     

Hybrid approach to surgical correction of tetralogy of Fallot in all patients with functioning Blalock Taussig shunts.

BACKGROUND: In total surgical correction of tetralogy of Fallot (TOF) with functioning Blalock Taussig shunts (BTS), shunt take down increased surgical time, bleeding, and might injure phrenic and recurrent laryngeal nerve and thoracic duct. OBJECTIVES: A routine hybrid approach using transcatheter BTS closure immediately before total surgical correction of TOF in all patients might reduce these problems. We analyze the safety and feasibility of this approach. METHODS: Transcatheter BTS closure was achieved using single or multiple stainless steel embolization coils, Amplatzer vascular plugs, or duct occluders. When coils were released without control by bioptome forceps, coil migration in larger shunts was prevented by proximal or distal balloon occlusion. RESULTS: This routine hybrid strategy was followed in 22 consecutive patients aged 1-13 years over 4-year-period and 21 procedures were successful. Among the 16 patients attempted with coils, 13 had successful closure, 2 needed Amplatzer duct occluder devices, and 1 sent for surgical shunt takedown due to acute angulation of the shunt. New Amplatzer vascular plugs were used in six patients. Bioptome was used in six patients and proximal or distal balloon occlusion of flow was used in three patients. Four patients had closure of associated aortopulmonary or chest wall collaterals. CONCLUSION: Hybrid approach using routine transcatheter closure of all BTS immediately before surgical correction of TOF shunts with coils/plugs/devices is safe, feasible, and reproducible.     

Recurrent Protein‐losing Enteropathy and Tricuspid Valve Insufficiency in a Transplanted Heart: A Causal Relationship?

This case report describes a toddler who developed a protein‐losing enteropathy (PLE) 4 years after orthotopic heart transplantation (OHT). He was born with a hypoplastic left heart syndrome for which he underwent a successful Norwood procedure, a Hemi–Fontan palliation, and a Fontan palliation at 18 months of age. Fifteen months following the Fontan operation, he developed a PLE and Fontan failure requiring OHT. Four years after OHT, he developed a severe tricuspid regurgitation and a PLE. His PLE improved after tricuspid valve replacement. It is now 2 years since his tricuspid valve replacement and he remains clinically free of ascites and peripheral edema with a normal serum albumin level. His prosthetic tricuspid valve is functioning normally.     

经导管封堵全腔静脉肺动脉连接术外管道开窗的初步临床应用

目的 探讨经导管封堵全腔静脉肺动脉连接术外管道开窗的方法及疗效.方法 2007年3至7月共行3例外管道开窗封堵术,男2例,女1例,年龄分别为7、14及8岁,术前诊断均为发绀,属复杂先天性心脏病,不能行双心室矫治,而行全腔静脉肺动脉连接术,术中均加行外管道开窗.术后患者持续存在口唇发绀,血氧饱和度降低.3例患者行心导管及造影检查后证实上、下腔静脉与肺动脉吻合口通畅,无狭窄改变,肺动脉平均压均小于15 mm Hg(1 mm Hg=0.133 kPa),分别采用AGA公司Amplatzer动脉导管未闭封堵器及房间隔缺损封堵器对外管道与右房之间的交通(开窗)进行封堵.结果 所有患者均封堵成功,封堵后即刻、15 min、30 min及1 h分别测肺动脉及腔静脉压,与封堵前比较无明显升高,且患者无明确不适症状,血氧饱和度较封堵术前均有提高,发绀减轻,超声心动图及造影证实封堵器形态及位置良好.无术中及术后并发症,术后随访3至6个月,经超声心动图证实封堵器形态及位置良好,无残余分流.结论 经导管外管道开窗封堵术技术成功率高、疗效确切,但远期疗效尚需密切随访.     

Occlusion of Fontan fenestrations using the Amplatzer septal occluder

Objective—To evaluate the efficacy and safety of the Amplatzer septal occluder device for occlusion of Fontan fenestrations.
Subjects—Five children aged 5-10 years who had undergone a fenestrated Fontan operation.
Setting—Tertiary paediatric cardiology centre.
Methods—Each patient had right and left heart catheterisation to assess haemodynamic suitability for fenestration closure. Sizing of the defect was achieved with a balloon wedge catheter and transoesphageal echocardiography. Transcatheter occlusion of the fenestration was accomplished using a 4 mm device in three patients, and 5 mm or 9 mm devices in the other two patients. Residual shunting following occlusion was assessed using angiography and echocardiography.
Results—100% occlusion rate of the fenestration was achieved in all patients. No complications or device failures were seen during the three month follow up period.
Conclusion—The Amplatzer septal occluder device is safe, and effectively occludes the Fontan fenestration.

Keywords: Fontan circulation;  fenestration occlusion;  Amplatzer septal occluder device;  congenital heart disease     

Clinical Outcomes and Improved Survival in Patients With Protein-Losing Enteropathy After the Fontan Operation

Background

Patients with protein-losing enteropathy (PLE) following the Fontan operation have a reported 50% mortality at 5 years after diagnosis.

Objectives

The aim of this study was to review outcomes in patients with PLE following the Fontan operation.

Methods

From 1992 to 2010, 42 patients (55% male) with PLE following the Fontan operation were identified from clinical databases at the Mayo Clinic. Data were collected retrospectively.

Results

Mean age at PLE diagnosis was 18.9 ± 11.0 years. Initial Fontan operation was performed at 10.1 ± 10.8 years of age. Mean time from Fontan operation to PLE diagnosis was 8.4 ± 14.2 years. Survival was 88% at 5 years. Decreased survival was seen in patients with high Fontan pressure (mean >15 mm Hg; p = 0.04), decreased ventricular function (ejection fraction <55%; p = 0.03), and New York Heart Association functional class >2 at diagnosis (p = 0.04). Patients who died had higher pulmonary vascular resistance (3.8 ± 1.6 Wood units [WU] vs. 2.1 ± 1.1 WU; p = 0.017), lower cardiac index (1.6 ± 0.4 l/min/m² vs. 2.7 ± 0.7 l/min/m²; p < 0.0001), and lower mixed venous saturation (53% vs. 66%; p = 0.01), compared with survivors. Factors were assessed at the time of PLE diagnosis. Treatments used more frequently in survivors with PLE included spironolactone (21 [68%]), octreotide (7 [21%]), sildenafil (6 [19%]), fenestration creation (15 [48%]), and relief of Fontan obstruction (7 [23%]).

Conclusions

PLE remains difficult to treat; however, in the current era, survival has improved with advances in treatment. Further study is needed to better understand the mechanism of disease and ideal treatment strategy.     

Regression of Pulmonary Arteriovenous Malformations after Transcatheter Reconnection of the Pulmonary Arteries in Patients with Unidirectional Fontan

Objective. Pulmonary arteriovenous malformations (PAVM) develop in patients with a lung deprived of hepatic blood flow. Unidirectional Fontan patients have superior vena cava (SVC) blood directed to the left or right pulmonary artery (PA), and inferior vena cava (IVC) and hepatic blood baffled to the contra‐lateral PA. Cyanosis often develops due to PAVM in the lung deprived of hepatic blood. We report experiences with transcatheter PA reconnection in patients with unidirectional Fontan and PAVM. Design. Patients with unidirectional Fontan, cyanosis, and PAVM underwent transcatheter PA reconnection. Following simultaneous injection of contrast into PAs, a transseptal needle was used to puncture from one PA to the other. A balloon‐mounted, uncovered stent was positioned bridging the pulmonary arteries. The procedure provided a nonrestrictive, permanent communication between the PAs. Results were retrospectively reviewed. Catheterization data and oxygen saturations before and after procedure were analyzed. Results. Six patients with a median age of 14 years (range 13–35 years) were identified. All patients had functional single ventricle with unidirectional Fontan and PAVM in the lung supplied solely by SVC flow. The reconnection procedure was performed successfully without complications in all cases. The median follow‐up time for all 6 patients was 12.8 months (range 5.8–19.3 months). Median oxygen saturation was 0.86 (range 0.81–0.92) prior to the procedure, and 0.95 (range 0.92–0.98) on follow‐up, with a median increase of 9% (range 8–11%). Conclusion. Patients with unidirectional Fontan and PAVM demonstrate increased oxygen saturations following reconnection of PAs, suggesting regression of PAVM. This procedure can be performed safely using uncovered stents, and it is effective in improving systemic oxygen saturations.     

Reversal of protein losing enteropathy with prednisone in adults with modified Fontan operations: long term palliation or bridge to cardiac transplantation?

Protein losing enteropathy (PLE), defined as severe loss of serum protein into the intestine, occurs in 4-13% of patients after the Fontan procedure and carries a dismal prognosis with a five year survival between 46% and 59%. Chronically raised systemic venous pressure is thought to be responsible for the development of PLE in these patients, with perhaps superimposed immunological or inflammatory factors. The success rate of contemporary medical, transcatheter, and surgical treatments attempting to reduce systemic venous pressure ranges from 19% to 40%. Prednisone treatment for PLE has been tried, with variable success rates reported in children. The effect of prednisone in adult patients with PLE after the Fontan procedure is largely unknown. Two cases of PLE in adults (a 39 year old woman and a 25 year old man) after modified Fontan procedure who responded dramatically to oral prednisone treatment are reported, suggesting that a trial of this "non-invasive" treatment should be considered as long term palliation or bridge to cardiac transplantation.     

Exercise Capacity Improves after Transcatheter Closure of the Fontan Fenestration in Children

Objectives. This study evaluated the aerobic capacity, exercise capacity, and arterial oxygen saturation (O₂Sat) in children before and after transcatheter Fontan fenestration closure. Design. Observational study comparing exercise parameters and hemodynamics before and after transcatheter fenestration closure in Fontan patients. Outcome Measures. Working capacity, exercise duration, oxygen consumption (VO₂), and arterial O₂Sat were evaluated during aerobic exercise. Results. Twenty patients (mean age 11.4 years) underwent standardized exercise testing before and after fenestration closure. Twelve patients underwent cycle ergometry testing (mean age 14.8 years) (group1), and eight younger patients (mean age 6.4 years) underwent Bruce treadmill testing (group 2). The same exercise protocol was used in each patient before and after fenestration closure (interval between tests: 118 ± 142 days). Immediately following fenestration closure at cardiac catheterization, cardiac index decreased (3.0 to 2.1 L/minute/m²) and Fontan pressure increased (11 ± 2 to 12 ± 2 mm Hg) with an increased arterial saturation (92 to 96%) (P < .001). The total group demonstrated no significant change in pre‐ and postclosure maximal heart rates (164 ± 21 and 169 ± 19 bpm). Rest and exercise O₂Sat increased (89 and 82 to 95 and 92%) (P < .0001). Exercise duration increased (7.7 ± 1.9 to 9.2 ± 2.4 minutes) (P < .0005). Maximal VO₂, indexed maximal VO₂, and total working capacity in kilopond‐meters (kpm) increased (1.2 ± 0.5, 27 ± 7 and 2466 ± 1012 to 1.3 ± 0.4 L/minute, 31 ± 9 mL/kg/minute and 2869 ± 1051 kpm, respectively) (P < .005). Conclusion. In children with a univentricular heart after Fontan palliation, transcatheter fenestration closure improves exercise arterial O₂Sat and aerobic capacity despite a restricted resting cardiac output documented by catheterization immediately after the closure procedure.     

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.