Methods: Between January 2014 and December 2022, among a total of 187 patients undergoing the Fontan operation, 10 (6 males, 4 females; median age: 10.9 years; range, 4.2 to 23 years) who underwent transcatheter creation or dilation of a Fontan fenestration due to the development of Fontan failure were retrospectively analyzed. Demographic data, laboratory results, echocardiographic findings, and catheterization parameters of the patients were recorded. The patients were classified into two groups based on the timing of Fontan failure: those who underwent fenestration before hospital discharge constituted the early-stage group, while those who underwent fenestration after discharge were assigned to the late-stage group. These two groups were compared in terms of hemodynamic parameters, changes in oxygen saturation, and complication rates.
Results: Transcatheter fenestration was performed in five patients due to Fontan failure in the early postoperative period, and in five patients in the late period. The median follow-up duration was 2.2 (range, 0.1 to 6) years. Following the procedure, pleural effusion regressed in four patients; median systemic venous pressure decreased by 3.4 (range, 2 to 9) mmHg, while median oxygen saturation dropped by 5.9% (range, 3 to 9%). Clinical improvement was observed in 70% of the patients within the first month. However, one patient died on postoperative Day 44 due to low cardiac output. Fenestration was performed in three patients with protein-losing enteropathy, and clinical improvement was achieved in all of them.
Conclusion: Transcatheter Fontan fenestration is a reliable therapeutic option for reducing systemic venous pressure and achieving hemodynamic improvement in symptomatic Fontan patients. Our study highlights the low complication rates and the potential of this procedure to yield favorable clinical outcomes. Transcatheter fenestration plays an important role in the management of high-risk Fontan patients.
Sequential surgeries and complex interventions play a crucial role in ensuring the survival of patients with congenital heart disease who have single ventricle physiology. The original atriopulmonary "classic" connection has undergone modifications to a lateral tunnel due to issues such as atrial hypertrophy and arrhythmias.[3, 5] The Fontan operation has evolved through various technical modifications since its introduction. With the development of the lateral tunnel technique in 1987, an intracardiac Fontan connection incorporating the native atrial wall to allow for growth became the preferred approach. However, this technique was not always feasible. In 1990, the extracardiac Fontan technique was introduced, along with the use of extracardiac conduits, intra-extracardiac conduits, intra-atrial tube grafts, and tunnels, leading to the development of various surgical modifications. During the same period, the concept of fenestration was introduced for high-risk Fontan candidates who exhibited certain criteria, including mean pulmonary artery pressure (mPAP) >18 mmHg, ventricular end-diastolic pressure >12 mmHg, atrioventricular (AV) valve insufficiency, pulmonary artery distortion, PVR >2 Wood Units (WU), and systemic ventricular outflow tract obstruction.[6]
There is a significant disparity in survival and complication rates between fenestrated and nonfenestrated Fontan patients. While studies have not consistently demonstrated a significant difference in long-term survival rates, complication rates are notably lower in fenestrated patients.[7] Fenestration has been associated with a reduced incidence of long-term complications such as PLE, hepatic dysfunction, and low cardiac output syndrome (LCOS), particularly in high-risk groups.[8]
In the management of elevated PVR, phosphodiesterase-5 inhibitors and/or endothelin receptor blockers are commonly used as the first-line medical treatment to reduce PVR. However, in the presence of PLE, pleural effusion, and/or ascites, drug therapy alone may not be sufficient to halt clinical deterioration and improve the patient's condition. In such cases, if there are no anatomical abnormalities which can be corrected, transcatheter techniques can be used to create or expand a Fontan baffle or conduit fenestration. The main goal of this procedure is to decrease PAP and increase cardiac output, leading to hemodynamic improvement in complex Fontan patients, even if it causes desaturation.[2, 9, 10]
The percutaneous creation of a Fontan fenestration with an additional intracardiac tunnel in a total cavopulmonary connection was first described in 2005, aiming to improve hemodynamics by reducing systemic venous pressure and increasing cardiac output in failing Fontan circulation.[11] T his w as followed by several small case series.[12, 13] The literature has previously highlighted the importance of creating or enlarging transcatheter Fontan fenestration.[9, 14] I n t his study, we a imed to evaluate clinical outcomes of patients who underwent transcatheter fenestration for the management of Fontan circulation failure at our center.
The study involved reviewing clinical reports, laboratory data, echocardiographic findings, and cardiac catheterization results obtained from the Filemaker® database (Claris International Inc., CA, USA). Early mortality and complications were defined as those occurring within 30 days after the Fontan operation or before hospital discharge. In the early post-Fontan group, patients who underwent the procedure had ongoing pleural effusion, ascites, and LCOS despite treatment and were still hospitalized. In the late post-Fontan group, recurrent pleural effusions, ascites, and PLE were the predominant findings.
Perioperative hemodynamic variables such as systemic venous, pulmonary artery, ventricular end-diastolic, and systemic arterial pressures, as well as arterial oxygen saturations, were evaluated. However, consistent measurement of all variables during cardiac catheterizations for Fontan fenestration was not feasible in some patients due to their critical condition. We implemented a postoperative Fontan procedure follow-up protocol to all patients at our center.[15],[16] This protocol also involves pre-procedural optimization with diuretics, pulmonary vasodilators, and nutritional support, which have significantly reduced the incidence of prolonged pleural effusions and postoperative ascites. These measures help stabilize hemodynamics, reduce perioperative risks, and promote early recovery. They also allow for continuous assessment of therapeutic response to guide further management. Prolonged pleural effusion was defined as the need for a chest tube for more than seven days. According to this protocol, if the patient was evaluated through preoperative cardiac computed tomography (CT)/magnetic resonance imaging (MRI) and catheterization and found to be at high risk (older age (>8 to 10 years) and elevated mPAP (>15 to 18 mmHg), impaired lymphatic distribution, reduced systemic ventricular function, abnormal pulmonary artery anatomy and arborization, or significant extracardiac anomalies), surgical fenestration was performed. Otherwise, it was not routinely performed. In complex cases that do not meet high-risk criteria, non-fenestrated Fontan procedures are performed, and a potential fenestration site is marked intraoperatively, usually with a pacing wire, to facilitate possible future fenestration if needed.
Procedural details
General anesthesia was administered to all patients for the procedure. Since transesophageal echocardiography (TEE) was utilized during the fenestration procedure, all patients were intubated and mechanically ventilated. Initially, patients were evaluated hemodynamically, with a focus on mPAP and ventricular end-diastolic pressures. Then, anatomical problems that could be addressed (such as stenosis in the pulmonary artery branches, presence of antegrade flow, etc.) were assessed. After resolving anatomical problems, fenestration was created or dilated in patients who still had elevated Fontan pressure. As a precautionary measure, prior to traversing the Fontan conduit, a cautery plate was meticulously positioned to prepare for potential radiofrequency (RF) energy utilization via surgical electrocautery during the procedure.
In all cases, femoral venous access was the preferred approach, except for a single instance where the jugular vein was utilized due to bilateral occlusion of femoral veins. Simultaneously, femoral arterial access was established for continuous monitoring. Following the induction of general anesthesia, vascular cannulation was performed, and prior to the fenestration procedure, a bolus of 100 international units (IU) of heparin per kg of body weight was administered and activated clotting time (ACT) was maintained between 200 and 250 sec. The Fontan conduit and atrium were visualized using angiography. Contrast material injections were performed into the Fontan tube in anteroposterior and lateral positions, concurrently with injections through the artery into the right atrium to assess the anatomical proximity between the Fontan conduit and the atrium.
Figure 2 provides a stepwise depiction of the transcatheter fenestration creation procedure. Initially, following the visualization of the Fontan conduit and atrium, a Brockenbrough needle was carefully advanced through a 6Fr/7Fr long sheath (Cook Medical, Copenhagen) to the designated area located between the conduit and atrium. Notably, an Ampere® RF ensemble (St. Jude Medical, MN, USA) was employed to create the fenestration in only one patient.
After puncturing the septal and atrial walls using a needle, the needle's position within the atrial cavity was verified through a hand-made contrast injection. A 0.014-inch × 300-cm coronary guidewire (Abbott Laboratories, IL, USA) was, then, threaded through the needle and directed into a pulmonary vein or aorta. After confirming the proper position with the assistance of TEE, the needle and dilator from the long sheath were meticulously replaced with a low-profile balloon catheter (Mozec? NC; Meril Life Sciences, India), ranging from 2 to 3.5 mm in diameter, to navigate the channel and dilate the atrial wall. Subsequently, the balloon catheter was gradually replaced to achieve further expansion of the area. The deflated balloon within the long sheath, situated in the atrium, was withdrawn along with the guidewire, and it was replaced with a covered metal stent (The Advanta? V12 LD, Atrium Medical Corp., NH, USA); Express? SD renal stent, Boston Scientific, MA, USA; Myra? BMS peripheral stent, Meril Life Sciences, India) which could be expanded with a balloon on a 0.035'' exchange wire, with sizes ranging from 6 to 8 mm × 16 t o 2 2 m m. T he stent was carefully positioned to traverse both the conduit and atrial walls. Precise stent placement into the septum was guided by repeated angiograms facilitated through manual contrast injections as necessary, aiming for a target window diameter of 4 to 6 mm. Following the creation of the right-to-left shunt windows, a decrease in both oxygen saturation and systemic venous pressure was observed. A final angiogram was performed to ensure accuracy and confirm the stent"s placement.
In addition, two patients who underwent the classic Fontan operation had their atrial septum dilated, and peripheral stents (Express?) measuring 7×15 mm and 7×17 mm were deployed. Balloon dilatation was performed in one patient due to narrowing of the previously surgically opened fenestration.
All patients were initiated on warfarin sodium following the procedure, with a target international normalized ratio (INR) of 2.0 to 2.5. Post-procedural rhythm monitoring was routinely performed in all patients using telemetry during the early postoperative period and 24-h Holter monitoring prior to discharge. For long-term rhythm surveillance, patients were advised to undergo regular Holter assessments every six to 12 months, particularly in patients with a known arrhythmia history.
Statistical analysis
Statistical analysis was performed using the IBM SPSS version 23.0 software (IBM Corp., Armonk, NY, USA). Continuous data were presented in mean ± standard deviation (SD) or median (min-max), while categorical data were presented in number and frequency. The Wilcoxon signed-rank test was used to compare pre- and post-procedural hemodynamic parameters within groups. The Mann-Whitney U tests were used to compare pre- and post-procedural changes in pressure and saturation values between early and late intervention groups. The chi-square test was used to assess differences in complication rates between groups. Mixed-way repeated measures analysis of variance (ANOVA) was employed for comparing interaction effects between time (pre/post) and intervention groups. A p v alue o f < 0.05 w as considered statistically significant.
Table 1: Diagnosis and characteristics of patients
Transcatheter fenestration was performed in five patients in the early post-Fontan period (range, 19 to 57 days) and in five patients in the late period (range, 84 to 2,160 days).
Pre-procedural hemodynamic parameters showed a median inferior vena cava (IVC) pressure of 20.5 (range, 16 to 25) mmHg. Median systemic arterial oxygen saturation was 89.5% (range, 83 to 98%). Following the creation and stenting of a Fontan fenestration, the median IVC pressure decreased to 17 (range, 11 to 21) mmHg (p<0.01), while the median systemic arterial oxygen saturation decreased from 89.5 to 84.5% (range, 75 to 90%) (p<0.01) (Table 2). However, considering the differences in these changes between the early and late intervention groups, no statistically significant difference was found (p=0.70 and p=0.58). Additionally, there was no significant difference in the pre- and postprocedural pressure and oxygen saturation values and complication rates between the early and late groups (p>0.05).
Table 2: Comparison of hemodynamic data obtained before and after intervention
In two patients, significant systemic AV valve regurgitation was noted; however, no intervention was performed before the fenestration procedure. None of the patients had more than mild aortic valve insufficiency. All patients had normal systolic and diastolic ventricular function.
Among these 10 patients, additional transcatheter procedures were performed in two cases during the main procedure and in five patients prior to it. These additional interventions included: one patient with major aortopulmonary collateral artery (MAPCA) embolization, one patient with right internal mammary artery (RIMA) closure, two patients with left pulmonary artery (LPA) stenting alongside antegrade flow closure, and three patients with antegrade flow closure alone. Among those performed during the fenestration session, one patient underwent RIMA closure to address complex aortopulmonary collateral artery formation contributing to elevated pulmonary pressures, while another received antegrade flow closure and LPA stenting. Addressing these anatomical defects contributed to improved hemodynamic status in all affected patients.
However, in those undergoing both fenestration and additional interventions within the same procedure, PAP remained elevated even after anatomical corrections were completed, thereby necessitating the creation of fenestration.
Seven of the 10 patients showed an improvement in their clinical condition in the days following the fenestration procedure while two of them experienced delayed recovery exceeding four weeks. Three patients underwent a procedure due to PLE, and clinical improvement was observed in all three cases. Among the three patients, two showed a return to normal levels of alpha-1 antitrypsin in their stool, while no significant change was detected in one patient. Only one patient died due to LCOS on Day 44 after the Fontan operation, although transcatheter fenestration was successful. Post-procedure, the patient continued to experience persistent pleural effusion, which required pleurodesis 10 days after fenestration. Despite this intervention, the patient developed LCOS. Intensive medical therapy was initiated; however, the patient did not survive (Table 3).
Table 3: Pre- and postoperative hemodynamic data of patients, additional procedures and outcomes
There were no procedure-related deaths or major complications. In our analysis of transcatheter fenestration procedures in 10 patients, serious complications such as pericardial effusion, tamponade, or acute cardiac decompensation were not observed. One patient experienced recurrent supraventricular tachycardia, which was successfully treated with intravenous adenosine injection. Another patient developed post-procedural pleural effusion, which resolved with medical treatment during follow-up.
In general, it was observed that in 90% (n=9) of the discharged and followed patients in our hospital, the fenestrations remained open for at least 12 months. The fenestration was closed in only one patient, whose mPAP was within acceptable limits. Twenty-four months after the procedure, due to persistently low oxygen saturation (<85%) despite clinical stability, the fenestration was closed using an 18-mm Amplatzer? multi-fenestrated septal occluder (Abbott Cardiovascular, IL, USA). Following the closure, the patient"s oxygen saturation levels improved (>95%), and no complications were observed during subsequent follow-up. During the follow-up period, only one patient experienced a transient thromboembolic event accompanied by dizziness and seizure. The condition resolved with medical treatment, and the symptoms did not recur.
While our study focused on transcatheter fenestration, alternative strategies such as intra-extracardiac Fontan procedures (fenestrated or not) or routine fenestration with planned percutaneous closure when necessary, as highlighted in the literature, may offer safer options for selected patients.[18] For early intervention groups, these surgical alternatives could represent viable options to achieve hemodynamic stability while minimizing the risks associated with transcatheter techniques.
In the late period, interventions are more commonly prompted by chronic complications such as PLE, recurrent ascites, or progressive cyanosis due to the loss of fenestration patency. Late fenestration has shown efficacy in addressing these issues, particularly when combined with medical therapies targeting elevated PVR.[17] In our study, five patients underwent late fenestration, with a median interval of 18 months post-Fontan surgery. Among these, sustained symptomatic relief was observed in four patients, while one required additional interventions due to recurrent PLE.
Timing also plays a critical role in the decision to perform transcatheter fenestration. Identifying patients at risk before the onset of overt failure requires careful evaluation of hemodynamic parameters, imaging studies, and clinical indicators such as reduced exercise tolerance or unexplained fluid retention.[10, 17] Additionally, incorporating biomarkers such as brain natriuretic peptide (BNP) levels or systemic venous oxygen saturation into routine follow-up may provide earlier signals for intervention. A proactive approach to timing could improve the success rate and reduce procedural complications. At our center, we have established a dedicated follow-up clinic specifically for Fontan patients. Along with clinical assessments, we routinely monitor laboratory markers such as BNP, stool alpha-1 antitrypsin, and liver enzymes and renal function tests to detect early signs of Fontan circulation failure. This comprehensive approach helps us tailor interventions promptly and maintain better long-term outcomes.
The treatment priorities during transcatheter fenestration must be clearly defined to optimize outcomes. First, ensuring that anatomical factors such as stenosis or residual antegrade pulmonary blood flow are addressed prior to fenestration is essential. This minimizes unnecessary shunting and ensures that fenestration serves its intended purpose of reducing systemic venous pressures and improving cardiac output. Second, managing comorbidities such as elevated PVR or arrhythmias with targeted therapies (e.g., phosphodiesterase inhibitors, endothelin receptor antagonists) before the procedure may enhance the patient's ability to adapt to the fenestration.[11, 19] In cases where these measures fail to provide benefit, transcatheter fenestration can offer a solution.
In Fontan patients, the effects of fenestration have been well-defined.[6] Success in transcatheter fenestration depends on several procedural and patient-specific factors. From a technical perspective, precise imaging guidance during the procedure is critical, particularly in cases with complex anatomies or calcified Fontan baffles. Real-time TEE or intracardiac echocardiography (ICE) can significantly improve the accuracy of needle placement and reduce the risk of complications.[20] While adjusting the stent position, we focused on maintaining proper orientation and alignment to prevent distortion of adjacent structures, such as valve annuli or vascular junctions. During TEE monitoring, real-time visualization guided our wire and catheter placement, while continuous assessment of hemodynamics helped detect potential complications such as interference with valves or inadvertent perforation. Patient-specific factors, such as pre-existing coagulopathies, hemodynamic instability, or prior interventions, may also influence outcomes. Tailoring peri-procedural management to address these variables, such as optimizing fluid status and correcting coagulopathy, is crucial.
An increase in PAP was observed in all patients in whom transcatheter fenestration was planned. In seven out of 10 patients, despite addressing anatomical factors contributing to this pressure increase, such as antegrade flow, LPA stenosis, presence of MAPCAs, either before or during the procedure, fenestration was required despite anatomical correction, due to persistently elevated pressure. However, as suggested in a recent publication,[21] p otential d esaturation f ollowing fenestration can be anticipated by carefully assessing factors such as fenestration size, PVR, and residual shunts. This underscores the importance of thorough patient selection and tailored fenestration sizing to minimize desaturation risk. At our center, the target fenestration diameter of 4 to 6 mm was determined based on previous reports highlighting its efficacy in balancing improved hemodynamics and minimizing desaturation. Smaller fenestration sizes have been associated with insufficient relief of systemic venous pressure, while larger sizes can lead to excessive right-to-left shunting and oxygen desaturation.[18] The observed reduction in IVC pressure (from 20.5 to 17 mmHg) was associated with significant improvements in venous congestion-related symptoms, such as pleural effusion and ascites, which were resolved in most patients. Despite the decrease in oxygen saturation (from 89.5 to 84.5%), clinical improvement was noted due to the increased cardiac output resulting from the right-to-left shunt created by fenestration. As highlighted in the literature, this physiological adaptation reflects a delicate balance between systemic venous unloading and controlled desaturation, underscoring the importance of patient-specific hemodynamic goals.[18]
Acute clinical improvement was observed in seven of our patients. Additionally, in 70% of patients followed at our hospital, the stented fenestration remained patent for at least 24 months. In some patients with adapted Fontan circulation, stented fenestration can be completely or partially closed over time.[22] In one particular case, the fenestration was closed via a transcatheter approach two years after the initial procedure, as the patient"s saturation levels, initially quite low, improved significantly once the Fontan circulation had adequately adapted, thereby allowing for safe closure. The duration of fenestration patency in our study (90% of patients maintained patency beyond 12 months) is a particularly noteworthy outcome. In comparison, the study by Rupp et al.[9] reported a patency rate of 85% at 24 months. This is consistent with the results of our study, reflecting similar outcomes in terms of fenestration durability. While our study does not include follow-up data beyond 24 months, longer-term studies in the literature have shown that fenestration patency can remain significant up to five years, although the patency rates tend to decline over time. Among 182 patients who underwent fenestrated Fontan procedures, spontaneous closure was reported in 20% of cases at an average follow-up of four years. While often asymptomatic, spontaneous closure was associated with acute life-threatening symptoms in 3 to 4% of patients, highlighting the need for careful monitoring.[14]
Endovascular transcatheter creation of a de novo stented Fontan tunnel is an important tool for treating patients with Fontan circulation failure, including those with extracardiac cavopulmonary connections. This approach is effective in both acute and chronic conditions. Additionally, in patients who have undergone atriopulmonary Fontan operation, expanding the interatrial septum with a stent contributes to increased cardiac output and decreased venous pressure. For extracardiac Fontan patients, the transseptal technique, which involves puncturing the PTFE tube with a needle, may suffice in most cases. However, the use of RF energy can be required in rare and challenging situations.[9] The delivery of RF current using surgical electrocautery has been shown in the literature to facilitate puncture of the Fontan baffle plate for diagnostic, interventional, and electrophysiology procedures in patients with complex and particularly heavily calcified Fontan baffle materials. In one of our cases, RF was utilized due to the presence of thickened and calcified tissue that made puncturing with a standard needle technically challenging. The advantages of RF include its ability to deliver precise, controlled energy, which minimizes the risk of excessive tissue injury or unintended perforation.[23] However, due to increased procedural complexity and equipment requirements, RF was reserved for select cases. In patients where the PTFE material was not significantly calcified or thickened, a standard needle was sufficient to achieve a safe and effective puncture. This highlights the importance of patient-specific decision-making when selecting the most appropriate technique for fenestration creation.
After Fontan surgery, PLE remains a challenging complication to treat. Historically, the prognosis has been quite catastrophic, with a 50% mortality rate within the first five years following diagnosis.[24] In this context, transcatheter fenestration is increasingly recognized as a primary treatment option for PLE patients. Vyas et al.[10] reported 16 transcatheter fenestration procedures in seven patients from 1995 to 2005. Three out of the seven patients did not experience ascites, but all patients had a recurrence of PLE. In our study, fenestration was performed in three patients with PLE, and all showed improvement in ascites and edema symptoms.
Pleural effusions are commonly observed after Fontan surgery and reflect the hemodynamic changes resulting from elevated systemic venous pressures. Various strategies have been adopted for the management of pleural effusion following Fontan. These include fluid restriction, low-salt diet, diuretics, and the use of vasodilators such as sildenafil and bosentan.[25] I n t he l ateral F ontan era, fenestration of the atrial septum was developed in 1989 to reduce pleural effusion.[26] However, currently many centers report conflicting results with fenestrated and non-fenestrated approaches. Fenestration has been shown to significantly reduce drainage duration and hospital stay in patients with pleural effusion who do not respond to the aforementioned treatment measures, as demonstrated in various studies.[27] However, there are also studies that report no statistically significant difference in drainage duration and volume with fenestration.[28] In our study, four out of five patients who underwent fenestration due to prolonged chest drainage showed clinical improvement and were discharged from the hospital within a short period.
In addition, peri-procedural anticoagulation and monitoring strategies should be carefully optimized to manage the ongoing risks of thrombosis and bleeding, as the ideal anticoagulation regimen after the Fontan procedure remains uncertain.[29] It is known that the presence or persistence of a right-to-left shunt can increase the risk of stroke in patients.[30] I n a m eta-analysis i nvestigating t he efficacy of fenestration in Fontan procedures, no significant difference was found in the occurrence of stroke between fenestrated and non-fenestrated Fontan procedures.[31] I n a s tudy b y S yfiridis et al.,[32] lifetime treatment with warfarin was preferred as anticoagulant prophylaxis after Fontan procedure. In patients where it was difficult to control the INR, low-dose warfarin and low-dose aspirin or aspirin alone were used. We encountered a thromboembolic complication that did not leave any sequelae in only one of our patients. Nonetheless, this study has certain limitations. First, its retrospective nature and single-center setting may introduce selection bias and limit the generalizability of the findings. Second, the small sample size and lack of long-term follow-up data beyond 24 months restrict conclusions about the durability of the procedure. Further multi-center, large-scale, prospective studies are needed to draw more reliable conclusions on this subject. In conclusion, our study results demonstrate that transcatheter fenestration is a feasible and effective therapeutic option for selected patients with Fontan circulation failure. The procedure is associated with favorable hemodynamic improvements and clinical responses in most cases. These findings support the integration of fenestration into the individualized management strategies for patients experiencing Fontan-related complications.
Data Sharing Statement: The data that support the findings of this study are available from the corresponding author upon reasonable request.
Author Contributions: Concept: E.D., E.C., A.G.; Design, writing: E.D., E.C.; Data collection and processing: E.D., R.S.B., P.A., E.C., M.S., İ.C.T.;Analysis or Interpretation: E.D., E.C., İ.C.T., Y.E., A.G.;Literature Search: E.D., E.C., R.S.B., P.A., M.S., A.G.; Critical review: E.C., A.G.
Conflict of Interest: The authors declared no conflicts of interest with respect to the authorship and/or publication of this article.
Funding: The authors received no financial support for the research and/or authorship of this article.
1) Mori M, Aguirre AJ, Elder RW, Kashkouli A, Farris AB, Ford
RM, et al. Beyond a broken heart: Circulatory dysfunction
in the failing Fontan. Pediatr Cardiol 2014;35:569-79. doi: 10.1007/s00246-014-0881-y.
2) John AS, Johnson JA, Khan M, Driscoll DJ, Warnes CA,
Cetta F. Clinical outcomes and improved survival in
patients with protein-losing enteropathy after the Fontan
operation. J Am Coll Cardiol 2014;64:54-62. doi: 10.1016/j.
jacc.2014.04.025.
3) Fontan F, Baudet E. Surgical repair of tricuspid atresia.
Thorax 1971;26:240-8. doi: 10.1136/thx.26.3.240.
4) Pearl JM, Laks H, Stein DG, Drinkwater DC, George BL,
Williams RG. Total cavopulmonary anastomosis versus
conventional modified Fontan procedure. Ann Thorac Surg
1991;52:189-96. doi: 10.1016/0003-4975(91)91335-s.
5) Newburger JW, Sleeper LA, Frommelt PC, Pearson GD,
Mahle WT, Chen S, et al. Transplantation-free survival and
interventions at 3 years in the single ventricle reconstruction
trial. Circulation 2014;129:2013-20. doi: 10.1161/
CIRCULATIONAHA.113.006191.
6) Bridges ND, Lock JE, Castaneda AR. Baffle fenestration
with subsequent transcatheter closure. Modification of the
Fontan operation for patients at increased risk. Circulation
1990;82:1681-9. doi: 10.1161/01.cir.82.5.1681.
7) Li D, Li M, Zhou X, An Q. Comparison of the fenestrated
and non-fenestrated Fontan procedures: A meta-analysis.
Medicine (Baltimore) 2019;98:e16554. doi: 10.1097/
MD.0000000000016554.
8) Atz AM, Travison TG, McCrindle BW, Mahony L,
Quartermain M, Williams RV, et al. Late status of
Fontan patients with persistent surgical fenestration.
J Am Coll Cardiol 2011;57:2437-43. doi: 10.1016/j.
jacc.2011.01.031.
9) Rupp S, Schieke C, Kerst G, Mazhari N, Moysich A, Latus H,
et al. Creation of a transcatheter fenestration in children with
failure of Fontan circulation: Focus on extracardiac conduit
connection. Catheter Cardiovasc Interv 2015;86:1189-94. doi: 10.1002/ccd.26042.
10) Vyas H, Driscoll DJ, Cabalka AK, Cetta F, Hagler DJ.
Results of transcatheter Fontan fenestration to treat protein
losing enteropathy. Catheter Cardiovasc Interv 2007;69:584-9. doi: 10.1002/ccd.21045.
11) Michel-Behnke I, Luedemann M, Bauer J, Hagel KJ,
Akintuerk H, Schranz D. Fenestration in extracardiac
conduits in children after modified Fontan operation by
implantation of stent grafts. Pediatr Cardiol 2005;26:93-6.
doi: 10.1007/s00246-004-0693-6.
12) Gewillig M, Boshoff D, Delhaas T. Late fenestration of the
extracardiac conduit in a Fontan circuit by sequential stent
flaring. Catheter Cardiovasc Interv 2006;67:298-301. doi: 10.1002/ccd.20604.
13) Bar-Cohen Y, Perry SB, Keane JF, Lock JE. Use of stents
to maintain atrial defects and fontan fenestrations in
congenital heart disease. J Interv Cardiol 2005;18:111-8. doi: 10.1111/j.1540-8183.2005.04049.x.
14) Kreutzer J, Lock JE, Jonas RA, Keane JF. Transcatheter
fenestration dilation and/or creation in postoperative Fontan
patients. Am J Cardiol 1997;79:228-32. doi: 10.1016/s0002-9149(96)00723-0.
15) Ergün S, Yıldız O, Ayyıldız P, Çilsal E, Öztürk E, Onan İS,
et al. Parameters affecting pleural drainage and management
strategy after Fontan operation. J Card Surg 2020;35:1556-62. doi: 10.1111/jocs.14691.
16) Çınar B, Atik SU, Gökalp S, Çilsal E, Şahin M, Kamalı H,
et al. Predictors of prolonged pleural effusion after Fontan
operation. Cardiol Young 2023;33:2094-100. doi: 10.1017/
S1047951123000264.
17) Daley M, du Plessis K, Zannino D, Hornung T, Disney
P, Cordina R, et al. Reintervention and survival in
1428 patients in the Australian and New Zealand
Fontan Registry. Heart 2020;106:751-7. doi: 10.1136/
heartjnl-2019-315430.
18) Ahmad Z, Jin LH, Penny DJ, Rusin CG, Peskin CS, Puelz C.
Optimal fenestration of the Fontan circulation. Front Physiol
2022;13:867995. doi: 10.3389/fphys.2022.867995.
19) Fan F, Liu Z, Li S, Yi T, Yan J, Yan F, et al. Effect of
fenestration on early postoperative outcome in extracardiac
Fontan patients with different risk levels. Pediatr Cardiol
2017;38:643-9. doi: 10.1007/s00246-016-1561-x.
20) Agnoletti G, Gala S, Ferroni F, Bordese R, Appendini
L, Pace Napoleone C, et al. Endothelin inhibitors lower
pulmonary vascular resistance and improve functional
capacity in patients with Fontan circulation. J Thorac
Cardiovasc Surg 2017;153:1468-75. doi: 10.1016/j.
jtcvs.2017.01.051.
21) Kawasaki Y, Sasaki T, Forbes TJ, Ross RD, Kobayashi
D. Optimal criteria for transcatheter closure of Fontan
fenestration: A single-center experience with a review of
literature. Heart Vessels 2021;36:1246-55. doi: 10.1007/
s00380-021-01798-y.
22) Anderson B, Bhole V, Desai T, Mehta C, Stumper O. Novel
technique to reduce the size of a Fontan Diabolo stent
fenestration. Catheter Cardiovasc Interv 2010;76:860-4. doi: 10.1002/ccd.22661.
23) Khan A, Qureshi AM, Bansal M, Stapleton G, Webb MK,
Lam W, et al. Extra-cardiac and complex Fontan baffle
fenestration using radio frequency current via surgical
electrocautery. Cardiol Young 2023;33:2621-7. doi: 10.1017/
S1047951123000380.
24) Mertens L, Hagler DJ, Sauer U, Somerville J, Gewillig M.
Protein-losing enteropathy after the Fontan operation: An
international multicenter study. PLE study group. J Thorac
Cardiovasc Surg 1998;115:1063-73. doi: 10.1016/s0022-5223(98)70406-4.
25) Mori H, Park IS, Yamagishi H, Nakamura M, Ishikawa S,
Takigiku K, et al. Sildenafil reduces pulmonary vascular
resistance in single ventricular physiology. Int J Cardiol
2016;221:122-7. doi: 10.1016/j.ijcard.2016.06.322.
26) Bridges ND, Mayer JE Jr, Lock JE, Jonas RA, Hanley FL,
Keane JF, et al. Effect of baffle fenestration on outcome of
the modified Fontan operation. Circulation 1992;86:1762-9.
doi: 10.1161/01.cir.86.6.1762.
27) Lemler MS, Scott WA, Leonard SR, Stromberg D, Ramaciotti
C. Fenestration improves clinical outcome of the fontan
procedure: A prospective, randomized study. Circulation
2002;105:207-12. doi: 10.1161/hc0202.102237.
28) Talwar S, Paidi A, Sreeniwas V, Dutt Upadhyay A, Das S,
Choudhary SK. Comparison of pleural effusion between
fenestrated and nonfenestrated extracardiac Fontan: A
prospective randomized study. J Card Surg 2020;35:2688-94.
doi: 10.1111/jocs.14886.
29) Kaulitz R, Ziemer G, Rauch R, Girisch M, Bertram H, Wessel
A, et al. Prophylaxis of thromboembolic complications after
the Fontan operation (total cavopulmonary anastomosis).
J Thorac Cardiovasc Surg 2005;129:569-75. doi: 10.1016/j.
jtcvs.2004.08.045.
30) Day RW, Boyer RS, Tait VF, Ruttenberg HD. Factors
associated with stroke following the Fontan procedure.
Pediatr Cardiol 1995;16:270-5. doi: 10.1007/BF00798060.
Turkish Journal of Thoracic and Cardiovascular Surgery published orginal papers on topics in cardiovascular surgery, cardiovascular anesthesia,cardiology and thoracic surgery. These encompass all relevant clinical, surgical and laboratory specialities, editorials, current and collective reviews, tecnical knowhow papers, case reports, "How to Do It" papers. All copyrights of the articles that published or will be published belongs to Turkish Journal of Thoracic and Cardiovascular Surgery and without permission of editorial board whole articles or any part of articles table pictures and graphics could not be published. Turkish Journal of Thoracic and Cardiovascular Surgery is indexed by Science Citation Index - Expanded (SCIE)
Editor of the Journal of Turkish Thoracic and Cardiovascular Surgery
Ataşehir Mah. Ataşehir Bulvarı 48 Ada Mimoza 2-2 K:2 D:6 Ataşehir - İSTANBUL - TURKEY
Phone: +90 216 456 14 54 | GSM: 0549 456 14 54 | e - Mail : dergi@tkdcd.org
www.tkdcd.org
){kind=link}
){kind=link}
){kind=link}
){kind=link}
){kind=link}