scholarly journals Mid Term Results of Fenestrated Intra-Extracardiac Fontan Procedure: Insights, Experiences and Expectations

2021 ◽  
Author(s):  
Arda Ozyuksel ◽  
Baran Simsek ◽  
Sener Demiroluk ◽  
Murat Saygi ◽  
Mehmet Bilal
Keyword(s):  
Fontan Procedure ◽  
Arterial Oxygen Saturation ◽  
Atrioventricular Septal Defect ◽  
Double Outlet Right Ventricle ◽  
Arterial Oxygen ◽  
Extracardiac Conduit ◽  
Lateral Tunnel ◽  
Cavopulmonary Anastomosis ◽  
Mid Term Results

Background: Intraextracardiac Fontan procedure aimed to combine the advantages of lateral tunnel and extracardiac conduit modifications of the original technique. Herein, we present our experience in our patients with intraextracardiac fenestrated Fontan Procedure. Methods: A retrospective analysis was performed in order to evaluate intraextracardiac fenestrated Fontan patients between 2014 and 2021. Seventeen patients were operated on with a mean age and body weight of 9.1 ± 5.5 years and 28.6 ± 14.6 kg. Results: Sixteen patients (94%) were palliated as univentricular physiology with hypoplasia of one of the ventricles. One patient (6%) with well-developed two ventricles with double outlet right ventricle and complete atrioventricular septal defect had straddling of the chordae prohibiting a biventricular repair. All of the patients had cavopulmonary anastomosis prior to Fontan completion, except one case. Fenestration was performed in all cases. Postoperative mean pulmonary artery pressures and arterial oxygen saturation levels at follow up were 10 ± 2.4 mmHg and 91.3 ± 2.7 %, respectively. Mean duration of pleural drainage was 5.4 ± 2.3 days. All of the fenestrations are patent at a mean follow up period of 4.8 ± 7.7 years, except one case. Any morbidity and mortality were not encountered. Conclusions: The mid-term results of intraextracardiac fenestrated Fontan procedure are encouraging. This procedure may improve the results in a patient population who should be palliated as univentricular physiology, especially in cases with complex cardiac anatomy.

Late outcomes after the Fontan procedure in patients with single ventricle: a meta-analysis

Heart ◽  
2018 ◽  
Vol 104 (18) ◽  
pp. 1508-1514 ◽  
Author(s):  
Ilana Schwartz ◽  
Courtney E McCracken ◽  
Christopher J Petit ◽  
Ritu Sachdeva
Keyword(s):  
Single Ventricle ◽  
Meta Analysis ◽  
Fontan Procedure ◽  
Time Intervals ◽  
Protein Losing Enteropathy ◽  
Extracardiac Conduit ◽  
Lateral Tunnel ◽  
Fontan Physiology ◽  
Pacemaker Insertion

ObjectiveMore patients with Fontan physiology are reaching adulthood. The purpose of this meta-analysis was to evaluate the late outcomes of patients palliated with Fontan procedure and to assess the risk factors for mortality.MethodsPubMed, Embase and Web of Science were queried to retrieve observational studies of survival in patients following the Fontan procedure with ≥5 years of follow-up. A random-effects model was used to determine pooled survival estimates at 5, 10 and 15 years. Meta-regression was used to assess potential moderators for death.ResultsNineteen articles with a total of 5859 patients were included. The weighted mean follow-up time was 8.94±2.64 years with overall 8.3% deaths and 1.5% transplants. Pooled survival estimates at 5, 10 and 15 years were 90.7%, 87.2% and 87.5%, respectively; and 88.4%, 85.7% and 84.1%, respectively, for studies that included all three time intervals (n=4). Earliest surgical year included in the study, proportion of atriopulmonary connections versus extracardiac conduit or lateral tunnel, and older age at Fontan were associated with higher rates of death, but ventricular morphology was not. Protein-losing enteropathy, reoperation and pacemaker insertion were reported in 2.1%, 5.6% and 6.8% patients, respectively.ConclusionsSurvival following the Fontan procedure has improved with time and is influenced by Fontan type and age at the time of Fontan. At a mean follow-up of 8.9 years, there was no significant association between survival and ventricular morphology, not taking into account the mortality prior to Fontan.

The Modified Fontan Procedure and Prolonged Pleural Effusions

2009 ◽  
Vol 75 (2) ◽  
pp. 175-177 ◽  
Author(s):  
Christopher E. Mascio ◽  
Matthew Wayment ◽  
Tarah T. Colaizy ◽  
Larry T. Mahoney ◽  
Harold M. Burkhart
Keyword(s):  
Pulmonary Artery ◽  
Pulmonary Artery Pressure ◽  
Fontan Procedure ◽  
Pulmonary Atresia ◽  
Double Outlet Right Ventricle ◽  
Pleural Effusions ◽  
Artery Pressure ◽  
Mean Pulmonary Artery Pressure ◽  
Extracardiac Conduit ◽  
Lateral Tunnel

The modified Fontan procedure may be complicated by prolonged pleural drainage. Predisposing factors are not fully understood. This study examines perioperative variables associated with prolonged effusions. We examined the Fontan procedure in 41 patients. Mean age was 45 months (range, 9 to 113 months). Mean weight was 14.7 kg (range, 6.9 to 30.4 kg). Diagnoses included tricuspid atresia in 29 per cent, pulmonary atresia in 12 per cent, and double-outlet right ventricle in 12 per cent. Fontan revisions were excluded. The extracardiac Fontan was performed in 22 patients (54%) and the lateral tunnel (LT) in 18 (44%). Fenestration was performed in 56 per cent. Ten patients (24%) had prolonged pleural effusions. Mean chest tube output (24.2 vs 14.1 mL/kg/d, P < 0.01), days with chest tubes (10.2 vs 5.8 days, P < 0.01), and length of stay (20.8 vs 8.13 days, P < 0.01) were significantly greater in those with effusions. Preoperative mean pulmonary artery pressure was higher in patients with prolonged effusions (12.7 vs 9.90 mmHg, P = 0.001). No other factors were identified as risk factors. Prolonged pleural effusions (greater than 14 days) are common after the modified Fontan procedure. A higher preoperative mean pulmonary artery pressure may be predictive of these effusions. Fenestration, type of Fontan reconstruction, and size of extracardiac conduit did not predispose to postoperative effusions.

Outcomes of the Extracardiac Fontan Procedure Using Cardiopulmonary Bypass: Early Results

2014 ◽  
Vol 17 (3) ◽  
pp. 173 ◽  
Author(s):  
Murat Ugurlucan ◽  
Eylem Yayla Tuncer ◽  
Fusun Guzelmeric ◽  
Eylul Kafali ◽  
Omer Ali Sayin ◽  
...  
Keyword(s):  
Cardiopulmonary Bypass ◽  
Fontan Procedure ◽  
Fontan Operation ◽  
Arterial Oxygen Saturation ◽  
Vena Cava ◽  
Arterial Oxygen ◽  
Shunt Operation ◽  
Early Results ◽  
The Mean ◽  
Extracardiac Fontan

<p><strong>Background</strong>: Although the avoidance of cardiopulmonary bypass during the Fontan procedure has potential advantages, using cardiopulmonary bypass during this procedure has no adverse effects in terms of morbidity and mortality rates. In this study, we assessed the postoperative outcomes of our first 9 patients who have undergone extracardiac Fontan operation by the same surgeon using cardiopulmonary bypass.</p><p><strong>Methods</strong>: Between September 2011 and April 2013,  9 consecutive patients (3 males and 6 females) underwent extra-cardiac Fontan operation. All operations were performed under cardiopulmonary bypass at normothermia by the same surgeon.  The age of patients ranged between 4 and 17 (9.8 ± 4.2) years. Previous operations performed on these patients were modified Blalock-Taussig shunt procedure in 2 patients, bidirectional cavopulmonary shunt operation in 6 patients, and pulmonary arterial banding in 1 patient. Except 2 patients who required intracardiac intervention, cross-clamping was not applied. In all patients, the extracardiac Fontan procedure was carried out by interposing an appropriately sized tube graft between the infe-rior vena cava and right pulmonary artery.</p><p><strong>Results</strong>: The mean intraoperative Fontan pressure and transpulmonary gradient were 12.3 ± 2.5 and 6.9 ± 2.2 mm Hg, respectively. Intraoperative fenestration was not required. There was no mortality and 7 patients were discharged with-out complications. Complications included persistent pleural effusion in 1 patient and a transient neurological event in 1 patient. All patients were weaned off mechanical ventila-tion within 24 hours. The mean arterial oxygen saturation increased from 76.1% ± 5.3% to 93.5% ± 2.2%. All patients were in sinus rhythm postoperatively. Five patients required blood and blood-product transfusions. The mean intensive care unit and hospital stay periods were 2.9 ± 1.7 and 8.2 ±  1.9 days, respectively.</p><p><strong>Conclusions</strong>: The extracardiac Fontan operation per-formed using cardiopulmonary bypass provides satisfactory results in short-term follow-up and is associated with favor-able postoperative hemodynamics and morbidity rates.</p>

scholarly journals Massive haemorrhagic complications of ruptured pulmonary arteriovenous malformations: outcomes from a 12 years’ retrospective study

2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Xu Ma ◽  
Bing Jie ◽  
Dong Yu ◽  
Ling-Ling Li ◽  
Sen Jiang
Keyword(s):  
Arteriovenous Malformations ◽  
Arterial Oxygen Saturation ◽  
Arterial Oxygen ◽  
Hereditary Haemorrhagic Telangiectasia ◽  
Pulmonary Arteriovenous Malformations ◽  
Imaging Data ◽  
Peripheral Lung ◽  
Life Threatening ◽  
The Mean

Abstract Background The life-threatening haemorrhagic complications of pulmonary arteriovenous malformations (PAVMs) are extremely rare, and only described in isolated cases. This study was designed to comprehensively investigate management of ruptured PAVMs. Methods We retrospectively assessed clinical and imaging data of ruptured PAVMs to summarize incidence, clinical characteristics, and outcomes following embolisation between January 2008 and January 2021. Results Eighteen of 406 (4.4%) patients with PAVMs developed haemorrhagic complications. Twelve of 18 patients were clinically diagnosed with hereditary haemorrhagic telangiectasia (HHT). Haemorrhagic complications occurred with no clear trigger in all cases. Eight of 18 patients (44.4%) were initially misdiagnosed or had undergone early ineffective treatment. 28 lesions were detected, with 89.3% of them located in peripheral lung. Computed tomography angiography (CTA) showed indirect signs to indicate ruptured PAVMs in all cases. Lower haemoglobin concentrations were associated with the diameter of afferent arteries in the ruptured lesions. Successful embolotherapy was achieved in all cases. After embolotherapy, arterial oxygen saturation improved and bleeding was controlled (P < 0.05). The mean follow-up time was 3.2 ± 2.5 years (range, 7 months to 10 years). Conclusions Life threatening haemorrhagic complications of PAVMs are rare, they usually occur without a trigger and can be easily misdiagnosed. HHT and larger size of afferent arteries are major risk factors of these complications. CTA is a useful tool for diagnosis and therapeutic guidance for ruptured PAVMs. Embolotherapy is an effective therapy for this life-threatening complication.

Lymphatic Decompression Concomitant With Fontan/Kreutzer Procedure: Early Experience

2020 ◽  
Vol 11 (3) ◽  
pp. 284-292
Author(s):  
Christian Kreutzer ◽  
Daniel Alberto Klinger ◽  
Benjamin Chiostri ◽  
Santiago Sendoya ◽  
Mariana Lopez Daneri ◽  
...  
Keyword(s):  
Magnetic Resonance Imaging ◽  
Magnetic Resonance ◽  
Arterial Oxygen ◽  
Type I ◽  
Resonance Imaging ◽  
Lymphatic Abnormalities ◽  
Cavopulmonary Anastomosis ◽  
Late Morbidity ◽  
Group A

Objective: To present a strategy for identifying patients at risk of lymphatic failure in the setting of planned Fontan/Kreutzer completion, allowing a tailored surgical approach. Methods: Since January 2017, clinical evaluation before performance of the Fontan/Kreutzer procedure included T2-weighted magnetic resonance imaging (MRI) lymphangiography. Thoracic lymphatic abnormalities were categorized using a scale of I to IV according to progression of severity. Patients with severe lymphatic abnormalities (types III and IV) underwent Fontan/Kreutzer with lymphatic decompression via connection of the left jugular–subclavian junction containing the thoracic duct to the systemic atrium (group A). Results: Thirteen patients were enrolled. Magnetic resonance imaging showed type I abnormalities in four cases (30.7%), II in four (30.7%), III in two (15.3%), and IV in three (23.3%). Patients in types III and IV underwent a Fontan/Kreutzer with lymphatic decompression (group A, n = 5), while patients in types I and II underwent a fenestrated extracardiac Fontan/Kreutzer procedure without lymphatic decompression (group B, n = 8). Preoperatively, there were no differences in age, weight, ventricular dominance (right vs left), superior vena cava pressure, incidence of chylothorax after previous superior cavopulmonary anastomosis (Glenn), or need for concomitant procedures at Fontan/Kreutzer completion. There were no differences in procedural times between the groups, nor were there differences in mortalities and Fontan/Kreutzer takedowns. There were no statistically significant differences in early and late morbidity between the two groups with the exception of total volume of effusions output postoperatively. At median follow-up of 18 months (range, 4-28 months), all patients in group A are in New York Heart Association class 1 with no differences between groups in arterial oxygen saturation. Conclusions: Lymphatic decompression during Fontan/Kreutzer procedure was successfully performed in patients identified by MRI as predisposed to lymphatic failure. A larger cohort of patients and longer follow-up are required to determine the efficacy of this approach in preventing early- and long-term Fontan/Kreutzer failure.

Computational Model for the Study of Fontan Circulation

2018 ◽  
pp. 587-606
Author(s):  
Alejandro Talaminos-Barroso ◽  
Laura María Roa-Romero ◽  
Javier Reina-Tosina
Keyword(s):  
Computational Model ◽  
Cardiovascular System ◽  
Fontan Procedure ◽  
Fontan Operation ◽  
Univentricular Heart ◽  
Physiological Basis ◽  
Hemodynamic Variables ◽  
Extracardiac Conduit ◽  
Lateral Tunnel ◽  
Key Factor

In this chapter, the design and development of a computational model of the cardiovascular system is presented for patients who have undergone the Fontan operation. The model has been built from a physiological basis, considering some of the mechanisms associated to the cardiovascular system of patients with univentricular heart disease. Thus, the model allows the prediction of some hemodynamic variables considering different physiopathological conditions. The original conditions of the model are changed in the Fontan procedure and these new dynamics force the hemodynamic behaviours of the different considered variables. The model has been proved considering the classic Fontan procedure and the techniques from the lateral tunnel and the extracardiac conduit. The results compiled knowledge of several cardiovascular surgeons with many years of experience in such interventions, and have been validated by using other authors' data. In this sense, the participation of a multidisciplinary team has been considered as a key factor for the development of this work.

Use of the bidirectional cavopulmonary shunt in patients under one year of age

1995 ◽  
Vol 5 (1) ◽  
pp. 28-30
Author(s):  
Juan Calderón-Colmenero ◽  
Samuel Ramírez ◽  
Maria Rijlaarsdam ◽  
Alfonso Buendia ◽  
Carlos Zabal ◽  
...  
Keyword(s):  
Right Ventricle ◽  
Oxygen Saturation ◽  
Pulmonary Stenosis ◽  
Arterial Oxygen Saturation ◽  
Double Outlet Right Ventricle ◽  
Arterial Oxygen ◽  
Cardiothoracic Ratio ◽  
Bidirectional Cavopulmonary Shunt ◽  
One Year ◽  
Cavopulmonary Shunt

SummaryBetween January 1990 and March 1992, a bidirectional cavopulmonary shunt was performed in seven patients under one year of age for palliation of complex cyanotic congenital heart disease. Five cases had tricuspid atresia with a restrictive ventricular septal defect. One case presented with complete mirror imagery, right-sided heart, double inlet and double outlet right ventricle with pulmonary stenosis. Another case had isomerism of the left atrial appendages with common atrioventricular valve and double outlet right ventricle. All had a pulmonary arterial index greater than 250 mm/m2(Nakata index). Age and weights before surgery averaged five months (one to 11 months) and 5.6 kg (range 2.8 to 8.5 kg) respectively. Preoperative mean arterial oxygen saturation measured by cardiac catheterization was 60.05±14% and postoperative mean arterial oxygen saturation on room air was 79.85±6.03% (p<0.05). There were no deaths, although postoperative complications occurred in three patients, namely pleural effusion and pneumothorax. Mean follow-up was 10±4.2 months. The cardiothoracic ratio decreased in all cases. On subsequent echocardiographic studies with pulsed Doppler sampling, low velocity systolic-diastolic forward flow was registered in the pulmonary trunk. The velocity of flow increased clearly during inspiration. The bidirectional cavopulmonary shunt can be considered as primary palliation for complex cardiac malformations with restricted pulmonary flow independent of age and body weight.

scholarly journals A 45-year experience with the Fontan procedure: tachyarrhythmia, an important sign for adverse outcome

2019 ◽  
Vol 29 (3) ◽  
pp. 461-468 ◽  
Author(s):  
Friso M Rijnberg ◽  
Nico A Blom ◽  
Vladimir Sojak ◽  
Eline F Bruggemans ◽  
Irene M Kuipers ◽  
...  
Keyword(s):  
Adverse Outcome ◽  
Sinus Node ◽  
Fontan Procedure ◽  
Study Cohort ◽  
Protein Losing Enteropathy ◽  
Long Term Outcome ◽  
Extracardiac Conduit ◽  
Lateral Tunnel ◽  
Fontan Failure ◽  
Important Sign

Abstract OBJECTIVES This study aims to evaluate our 45-year experience with the Fontan procedure and to identify risk factors for late mortality and morbidity. METHODS Demographic, preoperative, perioperative and postoperative characteristics were retrospectively collected for all patients who underwent a Fontan procedure in a single centre between 1972 and 2016. RESULTS The study included 277 Fontan procedures (44 atriopulmonary connections, 28 Fontan-Björk, 42 lateral tunnels and 163 extracardiac conduits). Early failure occurred in 17 patients (6.1%). Median follow-up of the study cohort was 11.9 years (Q1–Q3 7.6–17.5). Longest survival estimates were 31% [95% confidence intervals (CI) 18–44%] at 35 years for atriopulmonary connection/Björk, 87% (95% CI 63–96%) at 20 years for lateral tunnel and 99% (95% CI 96–100%) at 15 years for extracardiac conduit. Estimated freedom from Fontan failure (death, heart transplant, take-down, protein-losing enteropathy, New York Heart Association III–IV) at 15 years was 65% (95% CI 52–76%) for atriopulmonary connection/Björk, 90% (95% CI 73–97%) for lateral tunnel and 90% (95% CI 82–94%) for extracardiac conduit. The development of tachyarrhythmia was an important predictor of Fontan failure [hazard ratio (HR) 2.6, 95% CI 1.2–5.8; P = 0.017], thromboembolic/neurological events (HR 3.6, 95% CI 1.4–9.4; P = 0.008) and pacemaker for sinus node dysfunction (HR 3.7, 95% CI 1.4–9.6; P = 0.008). Prolonged pleural effusion (>21 days) increased the risk of experiencing protein-losing enteropathy (HR 4.7, 95% CI 2.0–11.1; P < 0.001). CONCLUSIONS With modern techniques, survival and freedom from Fontan failure are good. However, Fontan patients remain subject to general attrition. Tachyarrhythmia is an important sign for an adverse outcome. Prevention and early treatment of tachyarrhythmia may, therefore, be paramount in improving the long-term outcome.

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