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.     

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.     

Tie a yellow ribbon around a papillary muscle

An 8‐year‐old boy with tetralogy of Fallot, pulmonary atresia, and major aortopulmonary collaterals status post complete repair including a fenestrated ventricular septal defect patch presented to the catheterization laboratory for fenestration closure. During the procedure, the catheterization wire was found to have an unusual intracardiac loop and was unable to be straightened within the heart. Three‐dimensional transesophageal echocardiography revealed the wire was looped around a right ventricular papillary muscle. The wire was readjusted, and the fenestration was successfully closed. Three‐dimensional imaging was essential in unambiguously defining the catheter course and assisting in fenestration closure.     

A modified technique of stent fenestration of the interatrial septum improves patients with pulmonary hypertension

Aims: A significant number of patients with pulmonary hypertension are resistant to medical therapy. We wanted to evaluate whether the modified technique of stent fenestration of the interatrial septum would be feasible and safe, and offer clinical benefit. Methods and Results: The medical records of all patients with pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension who underwent a stent fenestration of the interatrial septum between 2001 and 2008 were reviewed. In all fifteen patients (12 female, mean age 48.2 ± 20.5 years) a successful fenestration procedure could be performed. Median follow‐up time between diagnosis and fenestration was 2.3 years (range from 0.5 to 18.6 years). Mean event free survival since diagnosis and after septostomy was 9.8 ± 2.9 and 3.2 ± 0.8 years, respectively. When one extreme outlier was excluded, the 6 min walk distance improved significantly from 309 ± 69 m immediately before fenestration to 374 ± 84 m, 3–4 months after fenestration (n = 8, paired t‐test, P = 0.03). No stent occlusion occurred. Conclusion: The modified stent fenestration technique is feasible and safe in patients with severe pulmonary hypertension. In a selected group of patients, functional capacity might improve although disease progression continues. © 2008 Wiley‐Liss, Inc.     

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.     

Vascular endothelial growth factor increases fenestral permeability in hepatic sinusoidal endothelial cells

Abstract: Vascular endothelial growth factor (VEGF) is an important regulator of vasculogenesis and vascular permeability. Hepatic sinusoidal endothelial cells (SECs) possess sieve‐like pores that form an anastomosing labyrinth structure by the deeply invaginated plasma membrane. Caveolin is the principal structural protein in caveolae. In this study, we examined the role of VEGF on the fenestration and permeability of SECs and the relation with caveolin‐1. SECs isolated from rat livers by collagenase infusion method were cultured for 24 h with (10 or 100 ng/ml) or without VEGF. The cells were then examined by transmission and scanning electron microscopy (EM). The expression of caveolin was investigated by confocal immunofluorescence, immunogold EM, and Western blot. Endocytosis and intracellular traffic was studied using horseradish peroxidase (HRP) reaction as a marker of fluid phase transport in SECs. Both transmission and scanning EM showed an increased number of sinusoidal endothelial fenestrae (SEF) in SECs cultured with VEGF. By confocal immunofluorescence, SECs cultured with VEGF displayed prominent caveolin‐1‐positive aggregates in the cytoplasm, especially surrounding the nucleus region. Immunogold EM depicted increased caveolin‐1 reactivity on vesicles and vacuoles of VEGF‐treated SECs compared with VEGF‐nontreated cells. However, there was no change in the level of caveolin‐1 protein expression on Western blot. After HRP injection, an increase of electron‐dense tracer filled the SEF in cells treated with VEGF. Our results suggested that VEGF induced fenestration in SECs, accompanied by an increased number of caveolae‐like vesicles. Increased caveolin‐1 might be associated with vesicle formation but not with fenestration. Increased fenestration may augment hepatic sinusoidal permeability and trans‐endothelial transport.     

Effects of persistent Fontan fenestration patency on cardiopulmonary exercise testing variables

Cardiopulmonary exercise testing (CPET) aids in clinical assessment of patients with Fontan circulation. Effects of persistent fenestration on CPET variables have not been clearly defined. Associations between fenestration and CPET variables at anaerobic threshold (AT) and peak exercise were explored in the Pediatric Heart Network Fontan Cross‐Sectional Study cohort. Fenestration patency was associated with a greater decrease in oxygen saturation from rest to peak exercise (fenestration ?4.9 ± 3.8 v. nonfenestration ?3 ± 3.5; P < .001). Physiological dead space at peak exercise was higher in fenestrated v. nonfenestrated (25.2 ± 16.1 v. 21.4 ± 15.2; P = .03). There was a weak association between fenestration patency and maximal work and heart rate. Fenestration patency was also weakly correlated with oxygen pulse, work and VE/VCO2 at AT. The effect of persistent fenestration on CPET measurements was minimal in this study, likely due to the cross‐sectional design.     

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.     

Fate of a modified fenestration of atrial septal occluder device after transcatheter closure of atrial septal defects in elderly patients

Background: Data on closure of atrial septal defects (ASD) in elderly patients with a fenestrated Amplatzer septal occluder (ASO) device is limited. Methods: A hemodynamically significant ASD was closed with a fenestrated ASO in 3 patients with ages >62 years. Prior to implant a 4‐mm fenestration was created by balloon dilatation without additional suture fixation just adjacent to the stent part of the device. Indications for fenestration were restrictive left ventricular physiology and/or pulmonary hypertension. Heparin had been administered during and for 48 hours after the procedure. Two patients were maintained on phenprocoumon because of chronic atrial fibrillation, the remaining patient on aspirin and clopidogrel for 3 months after implant. Transesophageal echocardiography (TEE) and hemodynamic evaluation were performed 4–18 months after ASD closure. Results: A trace or small fenestration through the ASO with left‐to‐right shunt was detected by TEE in all 3 patients without any hemodynamic significance. No thrombus formation was observed. Pulmonary hypertension improved in the affected patient. Pulmonary arterial wedge pressure and cardiac index improved in the second patient with improvement in heart failure symptoms and of quality of life in both. The third patient, after initial improvement for 6 months, developed significant comorbidities and clinical deterioration at 18 months follow‐up. Conclusion: The modified fenestration of the ASO decreased significantly in size at follow‐up. Applying this technique to selected patients judged to be at risk for ASD closure avoids acute decompensation and allows gradual diminuition of right ventricular volume overload during mid‐term follow‐up. (J Interven Cardiol 2011;24:485–490)     

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.     

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.     

Contemporary use of embolic protection devices in saphenous vein graft interventions: Insights from the stent of saphenous vein grafts trial

We report successful deployment of a fenestrated Helex Septal Occluder in a 46‐year‐old lady with a moderate‐sized secundum atrial septal defect and elevated left‐ventricular end diastolic pressure secondary to renovascular hypertension. Initial balloon occlusion of the defect lead to significant rise in left atrial pressure. Creation of a 4‐mm fenestration offered controlled decompression of the left atrium while reducing the atrial shunt considerably. © 2011 Wiley‐Liss, Inc.     

Unique Sail‐Like Structure of Cor Triatriatum Dexter in Three‐Dimensional Echocardiogram

Cor triatriatum dexter (CTD) is an extremely rare congenital condition arising from the persistence of the right valve of the sinus venosus. It divides the right atrium (RA) into 2 separate chambers. We report a case of a 50‐year‐old man who had an incidental finding of CTD on transesophageal echocardiogram. An incomplete membrane of the RA was seen, and three‐dimensional echocardiogram delineated the structure clearly as a triangular sail‐like structure with multiple orifices and a fenestration.     

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.     

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.     

Multimodality Imaging of Cor Triatriatum Sinister in an Octagenerian

Cor triatriatum sinister (CTS) is a rare congenital cardiac anomaly in which a fibromuscular membrane divides the atrium into two chambers. As CTS can occur as an isolated anomaly, it is frequently associated with other cardiac anomalies. Although symptoms are usually encountered in infancy, CTS may rarely present in adulthood when the membrane contains large fenestration or other escape drainage exists. We herein, present an 82‐year‐old patient with a typical late diagnosis of asymptomatic CTS.     

CT血管成像诊断颅内动脉成窗变异与血管异常

目的探讨CT血管成像(CTA)诊断颅内动脉成窗变异及合并其他血管异常。方法回顾性分析2013年1月—2014年1月中山大学附属中山医院653例经头部CTA检查,并诊断为颅内动脉成窗的39例患者的影像资料,分析颅内动脉成窗的发生率、部位、形态及其他血管异常。结果 (1)653例患者共检出39例(5.97%)41个成窗动脉,其中基底动脉成窗检出率3.37%(22个),椎动脉成窗检出率0.31%(2个),大脑前动脉成窗检出率1.22%(8个),前交通动脉成窗检出率0.61%(4个),大脑中动脉成窗检出率0.76%(5个)。后循环成窗以凸透镜型为主(66.67%,16/24),前循环成窗以裂隙型为主(76.47%,13/17),差异有统计学意义(χ2=7.411,P0.05)。(2)39例中22例成窗患者合并其他血管异常,以单侧或双侧胚胎型大脑后动脉最多(10例),其次为颅内动脉瘤(5例)。5例动脉瘤部位均远离成窗部位,位于颈内动脉交通段3例、基底动脉顶端1例、双侧大脑中动脉M2段1例。结论 CTA可以直观、清晰地显示颅内动脉成窗及合并其他血管异常,但血管成窗与血管异常之间无显著相关性。     

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.     

Thoracic aortic disease: Can we safely cover the branches?

• Thoracic aortic disease has usually been treated with surgery.
• Thoracic endovascular aortic repair is challenging if supra‐aortic vessels are involved.
• In situ fenestration of the main graft from a retrograde approach while using bare‐metal stents as bridge stents appears to be a safe and practical technique.

     

Effect of baffle fenestration on outcome of the modified Fontan operation.

BACKGROUND. The "fenestrated Fontan" (surgical baffle fenestration followed by transcatheter test occlusion and permanent closure after postoperative recovery) was adopted in an effort to reduce perioperative mortality and morbidity. This study assesses the effect of baffle fenestration on outcome. METHODS AND RESULTS. Patients having a modified Fontan operation with a cavocaval baffle and cavopulmonary anastomosis were retrospectively selected for study. Those with baffle fenestration (n = 91) were compared with those without baffle fenestration (n = 56) with respect to preoperative risk factors, age, anatomy, surgical date, and presence or absence of a previous bidirectional cavopulmonary anastomosis. Outcome variables were failure (death or take-down) and duration of postoperative pleural effusions and hospitalization. Survival and clinical status after hospital discharge were ascertained. The two groups did not appear to differ with respect to age or anatomic diagnosis. Patients having baffle fenestration were at significantly greater preoperative risk by univariate and multivariate analysis (p < 0.01). Operative failure was low in both groups (11% without and 7% with baffle fenestration, p = NS). Durations of pleural effusions and hospitalization were significantly shorter with baffle fenestration (p < 0.01). Neither date of surgery nor a previous bidirectional cavopulmonary anastomosis appeared to contribute to improved outcome. Patients with baffle fenestration had lower postoperative systemic venous pressure (p < 0.01). There were no late deaths. Functional status in both groups is good (82% in New York Heart Association class I). CONCLUSIONS. Baffle fenestration is associated with low mortality, significantly less pleural effusion, and significantly shorter hospitalization among high-risk patients having a modified Fontan operation.