Creation Of Fenestration Research Articles

The Initial Results of Physician-Modified Fenestrated-Branched Endovascular Repairs of the Aortic Arch and Lessons Learned From the First 21 Cases.

Physician-modified fenestrated-branched endovascular aortic repair (PM-FBEVAR) for the aortic arch provides a minimally invasive treatment option for patients who are too high-risk for open repair. Improvements in technique are gained with ongoing experience with these complex repairs. This study aims to describe outcomes of arch PM-FBEVAR and technical lessons. A retrospective review of consecutive patients who underwent PM-FBEVAR with zone 0 proximal sealing at a single institution between January 2019 and July 2023 was performed. Cases completed using initial techniques (early technique) were compared with cases using the current techniques (current technique). Modification technique changed to include a self-orienting spine trigger wire and anatomically specific fenestrations or inner branches in the current group. The primary outcome was in-hospital mortality. Secondary outcomes included technical success and 30 day stroke. A total of 21 patients underwent arch PM-FBEVAR, with 7 in the early group and 14 in the current group. Severe comorbidities were present in both groups including chronic obstructive pulmonary disease (COPD) (43% vs 36%), prior open ascending aortic repair (57% vs 43%), and prior stroke (86% vs 21%), respectively. Technical success was the same (86% vs 86%, p=1.0). Fluoroscopy time (56 vs 24 min, p=0.012) and in-hospital death (43% vs 0%, p=0.026) were significantly lower in the current group. A 30 day stroke rate (29% vs 7%, p=0.247) was non-significantly decreased in the current group. All-cause mortality was 100% vs 7% during median follow-up of 8 and 6 months (p<0.001). Three deaths in the early group were related to their aortic arch repair including aortic rupture during endograft advancement and 2 postoperative strokes. There is a significant learning curve associated with aortic arch PM-FBEVAR. This study suggests that gained experience, use of the spine trigger wire technique, and precise creation of fenestrations or inner branches can lead to a shorter procedure time and lower complications. Physician modified fenestrated branched endografting is feasible for the aortic arch. The high rate of stroke and perioperative mortality was reduced with incorporation of self-orienting spine trigger wire and anatomically specific inner branch creation.

Mechanical Comparison between Fenestrated Endograft and Physician-Made Fenestrations.

A fenestrated endograft (FE) is the first-line endovascular option for juxta and pararenal abdominal aortic aneurysms. A physician-modified stent-graft (PMSG) and laser in situ fenestration (LISF) have emerged to circumvent manufacturing delays, anatomic standards, and the procedure's cost raised by FE. The objective was to compare different fenestrations from a mechanical point of view. In total, five Zenith Cook fenestrations (Cook Medical, Bloomington, IN, USA) and five Anaconda fenestrations (Terumo Company, Inchinnan, Scotland, UK) were included in this study. Laser ISF and PMSG were created on a Cook TX2 polyethylene terephthalate (PET) cover material (Cook Medical, Bloomington, IN, USA). In total, five LISFs and fifty-five PMSG were created. All fenestrations included reached an 8 mm diameter. Radial extension tests were then performed to identify differences in the mechanical behavior between the fenestration designs. The branch pull-out force was measured to test the stability of assembling with a calibrated 8 mm branch. Fatigue tests were performed on the devices to assess the long-term outcomes of the endograft with an oversized 9 mm branch. The results revealed that at over 2 mm of oversizing, the highest average radial strength was 33.4 ± 6.9 N for the Zenith Cook fenestration. The radial strength was higher with the custom-made fenestrations, including both Zenith Cook and Anaconda fenestrations (9.5 ± 4.7 N and 4.49 ± 0.28 N). The comparison between LISF and double loop PMSG highlighted a higher strength value compared with LISF (3.96 N ± 1.86 vs. 2.7 N ± 0.82; p= 0.018). The diameter of the fenestrations varied between 8 and 9 mm. As the pin caliber inserted in the fenestration was 9 mm, one could consider that all fenestrations underwent an "elastic recoil" after cycling. The largest elastic recoil was observed in the non-reinforced/OC fenestrations (40%). A 10% elastic recoil was observed with LISF. In terms of mechanical behavior, the custom-made fenestration produced the highest results in terms of radial and branch pull-out strength. Both PMSG and LISF could be improved with the standardization of the fenestration creation protocol.

Extra-cardiac and complex Fontan baffle fenestration using radio frequency current via surgical electrocautery

Fontan baffle punctures and creation of Fontan fenestration for cardiac catheterisation procedures remain challenging especially due to the heavy calcification of prosthetic material and complex anatomy. We sought to evaluate our experience using radiofrequency current via surgical electrocautery needle for Fontan baffle puncture to facilitate diagnostic, electrophysiology, and interventional procedures. A retrospective chart review of all Fontan patients (pts) who underwent Fontan baffle puncture using radiofrequency energy via surgical electrocautery from three centres were performed from January 2011 to July 2021. A total of 19 pts underwent 22 successful Fontan baffle puncture. The median age and weight were 17 (3-36 years) and 55 (14-88) kg, respectively. The procedural indications for Fontan fenestration creation included: diagnostic study (n = 1), atrial septostomy and stenting (n = 1), electrophysiology study and ablation procedures (n = 8), Fontan baffle stenting for Fontan failure including protein-losing enteropathy (n = 7), and occlusion of veno-venous collaterals (n = 2) for cyanosis. The type of Fontan baffles included: extra-cardiac conduits (n = 12), lateral tunnel (n = 5), classic atrio-pulmonary connection (n = 1), and intra-cardiac baffle (n = 1). A Fontan baffle puncture was initially attempted using traditional method in 6 pts and Baylis radiofrequency trans-septal system in 2 pts unsuccessfully. In all pts, Fontan baffle puncture using radiofrequency energy via electrocautery needle was successful. The radiofrequency energy utilised was (10-50 W) and required 1-5 attempts for 2-5 seconds. There were no vascular or neurological complications. Radiofrequency current delivery using surgical electrocautery facilitates Fontan baffle puncture in patients with complex and calcified Fontan baffles for diagnostic, interventional, and electrophysiology procedures.

In Situ Fenestrated Thoracic Endovascular Repair Using the Upstream Peripheral Go BackTM Re-Entry Catheter

Purpose: The aim is to present a case of in situ fenestrated thoracic endovascular repair, using a re-entry peripheral catheter, needing urgent repair due to recurrent episodes of hemoptysis because of an aorto-bronchial fistula after previous thoracic endovascular aortic repair (TEVAR). Technique: A 74-year-old male with a previous TEVAR presented with hemoptysis, fever, and cachexia. An aorto-bronchial fistula and type Ia endoleak were depicted on computed tomography angiography (CTA). Due to the patient’s frail general condition, a proximal TEVAR extension was planned, prior to thoracotomy for debridement, and long-term antibiotics. An in situ fenestrated TEVAR was performed to provide proximal sealing and revascularize the left common carotid and subclavian arteries. For fenestration creation, an Upstream Peripheral Go Back catheter (Bentley, Hechingen, Germany) was used. The Go Back catheter has been approved as a re-entry tool for complex peripheral arterial revascularization. In this case, both fenestrations were successfully created. Proximal seal and target vessel patency were confirmed on CTA. Thoracotomy confirmed aortic exclusion. A wedge lobectomy was performed in combination with pediculated omentectomy to exclude the exposed endograft. Conclusion: In urgent cases, where custom-made thoracic devices are unavailable, in situ fenestrations with the Go Back catheter are an alternative to laser fenestrations. Clinical Impact Fenestrations are often required in acute TEVAR to achieve a proximal seal in the arch. In situ fenestrations with the Go Back catheter are an alternative to laser fenestrations and PMEGs in this setting.

A Case of Failing Fontan with Pulmonary Vein Occlusion Obtained Decrease in the Central Venous Pressure by Fenestration Creation after Embolization of Minor Aortopulmonary Collateral Artery and Pulmonary Artery

A Case of Failing Fontan with Pulmonary Vein Occlusion Obtained Decrease in the Central Venous Pressure by Fenestration Creation after Embolization of Minor Aortopulmonary Collateral Artery and Pulmonary Artery

Pre-Fontan Cardiac Catheterization Data as a Predictor of Prolonged Hospital Stay and Post-Discharge Adverse Outcomes Following the Fontan Procedure: A Single-Center Study.

Despite improved outcomes following modifications to the Fontan technique, significant morbidity and mortality persist. We sought to determine if abnormal pre-Fontan catheterization hemodynamic data will predict postoperative prolonged hospital stay (PHLOS) and adverse post-discharge outcomes. This is a retrospective study of patients who underwent the Fontan procedure at Children's Hospital of New Orleans from 2008 to 2018. PHLOS was defined as ≥ 14 and ≥ 21days to discharge post Fontan. We defined post-discharge adverse outcomes as thromboembolic phenomena requiring anticoagulation therapy, protein-losing enteropathy, plastic bronchitis, transplantation, persistent chylous effusion requiring fenestration creation, or death. Statistical analysis was performed using student t test, Chi-square test, and multivariable logistic regression analysis using IBM SPSS version 22. Ninety-seven patients underwent extracardiac Fontan. Forty-one patients (42.3%) experienced hospitalization ≥ 14days, 31 patients (32%) experienced hospitalization ≥ 21days, and 14 patients (14.4%) experienced adverse post-discharge outcome. Elevated end-diastolic pressure (EDP) ≥ 10mmHg (p = 0.005, OR 4.2, CI 1.5-11.4) was independently associated with ≥ 14days of hospitalization, while a CI < 4 L/minute/meters2 combined with one abnormal catheterization variable was associated with PHLOS and post-discharge adverse outcomes (p = 0.03, OR 2.8, CI 1.1-7.3 and p = 0.043, OR 6.42, OR 1.1-38.9, respectively). The absence of fenestration was also associated with post-discharge adverse outcomes (p = 0.007, OR 5.8, CI1.6-20.7). Elevated EDP may be associated with PHLOS, while CI < 4 L/minute/meters2 combined with abnormal catheterization hemodynamics may be associated with PHLOS and adverse post-discharge outcomes, while absence of fenestration may be associated with post-discharge adverse events.

Use of 3D Printing to Guide Creation of Fenestrations in Physician-Modified Stent-Grafts for Treatment of Thoracoabdominal Aortic Disease

Purpose: To summarize the experience and outcomes of total endovascular repair of thoracoabdominal aortic disease using 3-dimensional (3D) printed models to guide on-site creation of fenestrations in aortic stent-grafts. Materials and Methods: From April 2018 to March 2019, 34 patients (mean age 58±14 years; 24 men) with thoracoabdominal aortic disease were treated in our department. Nineteen patients had thoracoabdominal aortic dissection and 15 had thoracoabdominal aortic aneurysm. Preoperatively, a 3D printed model of the aorta was made according to computed tomography images. In the operating room, the main aortic stent-graft was completely released in the 3D printed model, and the position of each fenestration or branch was marked on the stent-graft. The fenestrations were then made using an electric pen. Wires were sewn to the edge of the fenestrations using nonabsorbable sutures. After customization, the aortic stent-graft was reloaded into the delivery sheath and deployed. Results: The printing process took ~5 hours (1 hour for image reconstruction, 3 hours for printing, and 1 hour for postprocessing). The physician-modified stent-grafts had a total of 107 fenestrations secured by 102 bridging stent-grafts, including 73 covered stents and 29 bare stents. The average procedure time was 5.6±1.2 hours, including a mean 1.3 hours for stent-graft customization. No renal insufficiency or paraplegia occurred. Two branch arteries were lost during the operation. One patient (3%) died 1 week after surgery from a retrograde dissection rupture. One patient developed a minor cerebral infarction postoperatively. The mean follow-up time was 8.5 months. There was 1 endoleak from a fenestration (coil embolized) and 4 distal ruptures of the aortic dissection (3 treated and 1 observed). Conclusion: Three-dimensional printing can be used to guide creation of fenestrated stent-grafts for the treatment of thoracoabdominal aortic diseases involving crucial branches. This technique appears to be more accurate than the traditional measurement method, with short-term follow-up demonstrating the safety and reliability of the method. However, further research and development are needed.

Three-dimensional (3d) Printing Technology for Aligning Fenestrations in Surgeon Modified Endografts

Introduction: Custom built fenestrated endografts are the preferred option for treating complex paravisceral abdominal aortic aneurysms (AAA). However, when resources and time are unavailable surgeon modified endografts (SMEG) have been reported to be a safe and effective solution. Creation of fenestrations is approximated based on measurements of the physician using imaging software. We sought to determine the applicability of 3D printing a patients' aortic segment in achieving perfect matching of fenestrations with target vessels in SMEGs and to detect factors affecting time needed to achieve this for urgent. Methods: The same vascular surgeon/operator used free open-source software (3DSlicer, MeshMixer) and contrast enhanced computed tomography (CT) series to manually segment the celiac to renal arteries part of the aorta and the first 3 mm of each branch. This volume was then transformed into a hollow 1:1 sized model to be printed with a fused deposition modelling printer (Prusa MK3S). Dedicated 3D-printer software was used to calculate estimated print time with sterilizable co-polyester filament (nGen Flex, ColorFabb). The time for each step of the procedure was recorded along with the 3D model dimensions/volume and filament volume used, CT characteristics and patient details. Continuous variables are expressed as mean +/- SD. Kendall's tau multiple correlations and multiple linear regression were employed to seek relationships between variables and detect independent predictors of time taken. Results: Thirty-one consecutive AAA patients' preoperative CTs were analyzed (7 juxtarenal, 24 infrarenal, 29 men). Each patient's hollow 3D aortic model segmentation needed 13.5+/-4.3 minutes while preparation for 3D printing took 1.3+/-0.9 minutes. Print time was calculated to be 131.3+/-35.2 minutes. Segmentation time displayed a negative correlation with the number of CTs previously segmented by the operator (b = - 0.484, p< 0.001). Print time showed a positive correlation with filament volume (b = 0.6, p< 0.001) and 3D model volume (b = 0.406, p=0.001). 3D model volume was also found to have a positive association with CT interslice distance (CTID) (b=0.628, p< 0.001) and a negative relationship to female sex (b=-0.329, p=0.03). Multiple regression analysis indicated that segmentation time was independently predicted (R squared =0.849, p< 0.001) by CTID, maximum Hounsfield value of the segmentation, number of CTs previously segmented by the operator and the height and sex of the patient. In turn, print time was independently predicted (R squared =0.945, p< 0.001) by filament volume, model dimensions, CTID and the height and sex of the patient. Conclusion: Free open-source software and 3D printers can be used to perfectly align fenestrations on surgeon modified endografts by deploying the endograft within the sterilized 3D printed aortic segment of each patient in an ideally short time frame for urgent cases. Besides aortic volume printed, operator experience and small CT interslice distance can independently reduce time needed.Figure 2Visceral Aortic segment model - top view.View Large Image Figure ViewerDownload Hi-res image Download (PPT) Disclosure: Consulting / Proctoring regional Endologix distributor

Contemporary Outcomes of the Fontan Operation: A Large Single-Institution Cohort

The Fontan procedure is the accepted standard for single-ventricle palliation. The goal of this study was to determine short- and midterm outcomes of patients undergoing a Fontan operation at a single institution and to identify contemporary risk factors for acute and chronic failure. All patients undergoing a Fontan operation between 1995 and 2016 were included. Failure was defined as death, transplantation, Fontan takedown or revision, fenestration creation or enlargement, plastic bronchitis, protein-losing enteropathy, or major perioperative reintervention. Multivariable logistic and Cox regression models were used to identify risk factors for acute (perioperative) and chronic failure (after hospital discharge or 30 days postoperatively, or both). The cohort included 610 patients. Median age at surgery was 4 years. Median follow-up was 6.8 years. Trends showed increasing use of extracardiac conduits, nonfenestrated Fontan, and extubation in the operating room. Perioperative mortality was 0.5% (n= 3). Transplant-free survival at 5, 10, and 15 years was 97%, 94%, and 92%, respectively; freedom from failure was 91%, 89%, and 87%, respectively. Extubation in the operating room was associated with lower risk of acute failure (odds ratio, 0.30; 95% confidence interval [CI], 0.11 to 0.87). Independent risk factors for chronic failure included genetic syndrome (hazard ratio [HR], 2.54; 95% CI, 1.11 to 5.83), ventricular dysfunction (HR, 3.86; 95% CI, 1.81 to 8.24), cardiopulmonary bypass time in 30-minute intervals (HR, 1.242; 95% CI, 1.100 to 1.402), and persistent pleural effusions (HR, 4.26; 95% CI, 2.25 to 8.07). Moderate or severe atrioventricular valve regurgitation (HR, 2.61; 95% CI, 1.13 to 6.02) and cardiopulmonary bypass time (HR, 1.22; 95% CI, 1.03 to 1.45) were associated with reduced long-term transplant-free survival. Contemporary midterm outcomes for Fontan patients are reassuring. Lifelong follow-up is mandatory to determine long-term outcomes and need for additional surgery as patients reach adulthood.

In Vitro Laser Fenestration of Aortic Stent-Grafts: A Qualitative Analysis Under Scanning Electron Microscope.

In situ fenestration of stent-grafts allows patients with life threatening aortic pathologies to be amenable to emergent "off the shelf indications for use" percutaneous treatments as a bail out technique. Three types of aortic stent-grafts were subjected to laser fenestration in a physiological saline solution followed by balloon angioplasty using 8, 10 or 12 mm in diameter noncompliant balloons. The morphology and the size of fenestrations were observed under optical and scanning electron microscopy. The damage to the fabrics was analyzed and quantified. The creation of fenestrations was feasible in all devices, with varying degrees of fraying and/or tearing. The monofilament twill weave (Medtronic Valiant) tore in two directions (warp and weft) while the multifilament weave fenestrations showed more fraying (Anaconda Vascutek and Zenith TX2 Cook). The size and directions of tearing were more predictable with the 8 mm diameter balloon whereas the results obtained with the 10 and 12 mm diameter balloons were more unpredictable. The fenestrations were free of melting of the yarns and blackening of the filaments. The in situ fenestration is feasible but the observed damage to the fabric constructions must be carefully considered. This procedure must currently be limited to urgent and emergent life threatening cases because it is off indications for use for approved devices.

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

BackgroundPatients with protein-losing enteropathy (PLE) following the Fontan operation have a reported 50% mortality at 5 years after diagnosis. ObjectivesThe aim of this study was to review outcomes in patients with PLE following the Fontan operation. MethodsFrom 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. ResultsMean 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%]). ConclusionsPLE 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.

Impact of fenestration creation on managing patients with protein loosing enteropathy complicating Fontan procedure

Protein loosing enteropathy (PLE) is well known complication following Fontan procedure and one way of managing such complication is to create fenestration if it is not present or enlarging it if it is small to reduce the Fontan pressure and reduce venous congestion which end up by intestinal protein loss. To evaluate the effect of such procedure in our population. From February 2006 through October 2011 9 patients who underwent fenestration creation due to development of PLE was assessed in regard to clinical, laboratory result and hemodynamic effect prior and post procedure. Median age is 7 years(4–21) , median weight is 23 kg (15–52) , male: female ratio is 3.5:1 ( male & 2 female), median saturation pre and post procedure was 93% and 82% respectively, median albumin pre and post procedure was 18 gm/dl and 31 gm/dl during the first 2 wks and 36 gm/dl thereafter, median pulmonary artery pressure before and after was 25 mmhg ( 17–32 mmhg) and 16 mmhg (14–19 mmhg), transpulmonary pressure gradient reduced from a median of 11 mmhg to 5 mmhg, No immediate deaths , 2 patients need re-dilatation, there was 2 (22%) late deaths (one t has stent thrombosis one month after followed by fulminant pulmonary embolism, though all patients were on anticoagulant other one with sudden arrest came to the emergency could not be resuscitated ) 3 (33%) patients have persistent low albumin though the fenestration is patent. Transcatheter fenestration creation as a management of PLE following Fontan procedure is feasible procedure, can be done in the cath. lab with little morbidity and mortality and with beneficial effect, however late complication and complete resolving of PLE is of concern especially if done late.

Endovascular interventions in the perioperative management of the Fontan procedure in a developing congenital heart surgery program

Results Two patients underwent the first Fontan palliations while participating in a development program for congenital heart surgery. Both presented at 32-33 months of age with baseline oxygen saturations of 82-84%. Patient 1 underwent an extracardiac Fontan with a GoreTex conduit larger than the IVC due to limited supplies. Postoperatively, the patient decompensated to a low cardiac output state with elevated Fontan pressures and significant edema that did not resolve despite fenestration creation. Catheterization demonstrated inferior conduit anastomotic stenosis and right pulmonary artery (RPA) obstruction by compression. Angioplasty of the extracardiac conduit distal anastomosis and RPA stent implant reduced Fontan pressures. The patient was extubated in 3 days and discharged home at 21 days. Patient 2 underwent a lateral tunnel Fontan and extubated 2 hours after surgery, but developed desaturation and low cardiac output within 24 hours. Catheterization revealed elevated Fontan pressures and a large SVC to pulmonary vein collateral. After coil embolization, the patient slightly improved, but underwent subsequent catheterization several days later where an Amplatzer duct occluder was used to obstruct significant antegrade flow through a previously banded pulmonary valve resulting in acute clinical improvement.

A surgeon’s perspective regarding the regulatory, compliance, and legal issues involved with physician-modified devices

Physician-modified endovascular devices are becoming commonplace in a modern climate where innovation outpaces regulated technological advancement. Off-label use of medical devices occurs on a daily basis throughout many institutions across the United States and when performed by physicians, is both legal and unregulated. The purpose of this invited commentary is to review the regulatory, compliance, and legal issues regarding the practice of medical device modification.

Impact of fenestration creation on managing patients with protein losing enteropathy complicating Fontan procedure

Protein losing enteropathy (PLE) is a well known complication following Fontan procedure and one way of managing such complication is to create fenestration if it is not present or enlarging it if it is small to reduce the Fontan pressure and reduce venous congestion which end up by intestinal protein loss. To evaluate the effect of such procedure in our population. From February 2006 through October 2011, 9 patients who underwent fenestration creation due to development of PLE were assessed in regard to clinical, laboratory results and hemodynamic effect prior and post-procedure. Median age is 7 years (4–21), median weight is 23 kg (15–52), male:female ratio is 3.5:1 (male and 2 female), median saturation pre- and post-procedure was 93% and 82%, respectively, median albumin pre- and post-procedure was 18 g/dl and 31 g/dl during the first 2 weeks and 36 g/dl thereafter, median pulmonary artery pressure before and after was 25 mmHg (17–32 mmHg) and 16 mmHg (14–19 mmHg), transpulmonary pressure gradient reduced from a median of 11 mmHg to 5 mmHg, no immediate deaths, 2 patients need re-dilatation, there was 2 (22%) late deaths (one has stent thrombosis 1 month after followed up by fulminant pulmonary embolism, though all patients were on anticoagulant, other one with sudden arrest came to the emergency could not be resuscitated) 3 (33%) patients have persistent low albumin though the fenestration is present in patient. Transcatheter fenestration creation as a management of PLE following Fontan procedure is a feasible procedure, can be done in the cath. lab with little morbidity and mortality and with beneficial effect, however late complication and complete resolving of PLE is of concern especially if done late.

Atrial Septal Stent Fenestration in the Management of Idiopathic Pulmonary Hypertension

Objectives: Assess the clinical impact of atrial septal stent fenestration in the management of patients with severe idiopathic pulmonary hypertension. Methods: Three patients (median age 12 years; range 11–13 years; weight 34 kg; range 28–41 kg) with severe idiopathic pulmonary hypertension accompanied by exertional syncope were catheterised with the creation of a fenestration between the left and right atria. Clinical symptoms, 6-min walk test (6MW) with resting and post exercise saturations and echocardiographic data were reviewed pre and post fenestration creation. Results: Two patients were stented and one underwent static balloon dilation; due to recurrent bacterial infections a stent was not implanted. Follow-up was complete at a median of 126 days (range 109–362 days). There were no complications. All patients had resolution of exertional syncope; 2/3 were asymptomatic. Mean 6MW distance increased from 447m to 538m (p value = NS); resting saturations fell from 96% to 94% (p value = NS) and post exercise saturations fell from 84% to 76% as expected (p value = NS). No significant change in levels of pulmonary hypertension estimated by tricuspid regurgitant gradient (87 mmHg to 76 mmHg) or left ventricular fractional shortening (33–37%). Conclusions: Atrial septal fenestration was well tolerated in our patients. It appeared to prevent collapse in severe idiopathic pulmonary hypertension and improved exercise tolerance as measured by 6MW with the expected fall in exercise saturations. Stent fenestration may allow longer term patency and titratability of the fenestration. Performed earlier in the course of the illness it may be better tolerated, facilitating a gradual tolerance of desaturation.

Late catheter interventions in hypoplastic left heart syndrome

Interventional cardiology plays a key role in the diagnosis and management of patients with functionally univentricular physiology after the various stages of surgical palliation. The interventions performed are widely variable in type, including angioplasty of stenotic vessels and implantation of stents in stenotic vessels; closure of defects such as collaterals, leaks in baffles, and fenestrations; creation of fenestration; and more. In the setting of venous hypertension associated with stenosis at the Fontan baffle, conduit, or pulmonary arteries, stent implantation is often preferred, as the aim is to eliminate completely the narrowing, given that relatively mild stenosis can have a significant detrimental hemodynamic effect in patients with functionally univentricular circulation. The procedure is highly successful. In patients who fail after Fontan procedure, creation of a fenestration is often performed, with variable technique depending on the underlying anatomic substrate. To increase chances of patency of the fenestration, implantation of a stent is often required, particularly in the setting of an extracardiac conduit. For those patients with cyanosis and favorable Fontan hemodynamics, closure of the fenestration is performed using atrial septal occluder devices with high success rate. Coils compatible with magnetic resonance imaging are used widely to treat collateral vessels, although on occasion other specific embolization tools are required, such as particles or vascular plugs. Postoperative arch obstruction is successfully managed with angioplasty at a younger age, while implantation of a stent in the aorta is reserved for older patients. Specifics of these interventional procedures as applied to the population of patients with functionally univentricular hearts are described in this manuscript.

Abstract 16642: Clinical Outcomes and Improved Survival in Patients With Protein Losing Enteropathy After the Fontan Operation

Background: Patients (pts) with protein losing enteropathy (PLE) following the Fontan operation (op) have a reported 50% mortality at 5 years (yrs) post diagnosis. We review our clinical outcomes in this pt population. Methods: From 1997 to 2010, 42 pts (55% M) with PLE following the Fontan op were identified from clinical databases. Data were collected by retrospective chart review. Results: Mean age at PLE diagnosis was 18.9 yrs + 11 (4.7-57.9 yrs) and initial Fontan op was 10.1 yrs + 10.8 (1.8-37.6 yrs). Mean time from Fontan op to PLE diagnosis was 8.4 yrs + 14.2 (30 days-27yrs). Types of Fontan op included: atriopulmonary connection (28, 67%), lateral tunnel or intraatrial conduit (6, 14%), or extracardiac conduit (8, 19%). Mean time from diagnosis to last follow-up was 8 yrs + 6 (1-22yrs). Survival was 88% at 5 yrs. There were 11 deaths, mean age 32 yrs + 7.7 (22-47 yrs). Mean time interval from diagnosis to death was 7.5 yrs + 5.4 (1-18yrs). Causes of death include: sepsis (7), complications after Fontan conversion (1), and unknown (3). Decreased survival was seen in pts with high Fontan pressure (mean &gt;15mmHg, p=0.04), decreased ventricular function (EF &lt; 55%, p=0.03), and NYHA class &gt;2 at diagnosis (p=0.04). Pts who died had higher pulmonary vascular resistance (3.8 + 1.6 WU vs 2.1 + 1.1 WU, p=0.017), lower cardiac index (1.6 L/min/m 2 + 0.4 vs 2.7 L/min/m 2 + 0.7, p&lt;0.0001), and lower mixed venous saturation (53% versus 66%, p=0.01) compared to survivors. All factors were obtained at initial PLE diagnosis. Pts treated with medical therapy only (n=15) resulted in symptomatic improvement in 7 pts (47%), no improvement in 5 (33%), and death in 3 (20%). Pts treated with medical + surgical/interventional catheterization procedures (n=27) resulted in symptomatic improvement in 10 pts (37%), no improvement in 9 (33%), and death in 8 (30%). Treatments used more in survivors included spironolactone (21, 68%), octreotide (7, 21%), sildenafil (6, 19%), fenestration creation (15, 48%), and relief of Fontan obstruction (7, 23%). Of the 31 survivors, 17 pts remain well-controlled. Conclusions: PLE remains difficult to treat, but in the current era, survival has improved with advances in treatment. Further study is needed to understand the mechanism of disease and ideal treatment strategy.

Invited Commentary

Protein-losing enteropathy (PLE) is an enigmatic ailment that strikes children and young adults after a Fontan operation. Over the past 20 years myriad treatments have been proposed ranging from ameliorating the circulatory failure of the Fontan circuit through fenestration creation to the use of medications such as steroidal agents, unfractionated heparin, and high-dose spironolactone. For each of these strategies, great initial enthusiasm has ultimately been tempered by the finding of only modest utility with limited long-term efficacy. Data suggest that inflammation may play an important role in PLE after a Fontan operation. On theoretical grounds, oral controlled-release budesonide is an ideal intestinal antiinflammatory agent. Unlike prednisone, it has high enteric affinity and is 90% metabolized at first pass through the liver, theoretically reducing the risk of steroid-related systemic side effects. Borrowing from a broad experience in children with inflammatory bowel disease, we reported in The Annals of Thoracic Surgery in 2010 on the successful use of budesonide in raising serum protein levels in patients treated at a median age of 15 years (range, 4 to 29 years). John and colleagues [1John A.S. Driscoll D.J. Warnes C.A. Phillips S.D. Cetta F. The use of oral budesonide in adolescents and adults with protein-losing enteropathy after the Fontan operation.Ann Thorac Surg. 2011; 92: 1451-1456Abstract Full Text Full Text PDF PubMed Scopus (30) Google Scholar] from the Mayo Clinic now report their experience with budesonide in a group of patients treated at a median age of 20 years (range, 16 to 32 years) They found a high rate of steroid-related side effects as well as a high rate of treatment failure. The high rate of side effects likely reflects indolent progressive hepatic dysfunction related to time after Fontan operation. The high failure rate suggests a resistance to disease treatment in those with long-standing illness, perhaps related to chronic changes in the gut lumen over time. The growing experience with oral budesonide as treatment for PLE provides a number of lessons. There is clearly a subgroup of patients who respond well, with the ability to wean to low doses with improvement in symptoms and quality of life, and a subgroup that does not respond. Budesonide is a potentially useful agent but should be tried with caution. If serious side effects are noted, or no response is seen within a limited time (3 to 6 months), weaning and discontinuation is indicated. More effective strategies for this debilitating disease await a clearer fundamental understanding of the mechanism of the disorder, which to date continues to elude us. The Use of Oral Budesonide in Adolescents and Adults With Protein-Losing Enteropathy After the Fontan OperationThe Annals of Thoracic SurgeryVol. 92Issue 4PreviewApproximately 5% to 15% of patients develop protein-losing enteropathy (PLE) after the Fontan operation. Oral controlled release (CR) budesonide has been used as a treatment strategy, but its use in the older Fontan population has not been described. Full-Text PDF

Clinical Experience with a Customized Fenestrated Endograft for Juxtarenal Abdominal Aortic Aneurysm Repair

Despite the numerous stent-graft devices available, unsuitable anatomy is still the greatest exclusion criterion for endovascular abdominal aortic aneurysm (AAA) repair (EVAR). The present report describes an on-site preprocedural customization of a conventional Zenith stent-graft device just before the endovascular procedure that includes the creation of fenestrations and scallops as necessary for the patient's anatomy. Three patients with difficult anatomy in whom conventional AAA repair posed a high degree of risk were treated with customization of the stent-graft device to fit disparate renal arteries. A single fenestration for the left renal artery was made in two cases, and a single scallop was made in the other case to accommodate the superior mesenteric artery. Gold beads were used to mark the location of the fenestration and scallop. The three cases were successfully performed without perceptible endoleaks in the follow-up period, which ranged from 4 to 14 months. No procedure-related complications were detected; however, pneumonia developed in one patient 3 weeks after EVAR. The initial results with this technique are encouraging, and the role of EVAR can be significantly increased with the use of this customization technique when the interventionalist does not have access to the commercially available devices or when the waiting time is too prolonged to accommodate the patient's clinical situation.