Outcomes of the Non-fenestrated Fontan Procedure at High Altitude

2019 ◽  
Vol 10 (5) ◽  
pp. 590-596 ◽  
Author(s):  
Alexis Palacios-Macedo ◽  
Héctor Díliz-Nava ◽  
Orlando Tamariz-Cruz ◽  
Luis García-Benítez ◽  
Fabiola Pérez-Juárez ◽  
...  
Keyword(s):  
Risk Factor ◽  
Oxygen Saturation ◽  
High Altitude ◽  
Fontan Procedure ◽  
Fontan Operation ◽  
Hospital Death ◽  
Medium Term ◽  
Drainage Time ◽  
Extracardiac Fontan

Background: Although high altitude has been considered a risk factor for the Fontan operation, and an indication for fenestration, there is a paucity of data to support its routine use. Fenestration, with its necessary right to left induced shunt, together with the lower partial pressure of oxygen found with progressive altitude, can significantly decrease hemoglobin oxygen saturation, and therefore, it would be desirable to avoid it. Objective: To analyze immediate and medium-term results of the non-fenestrated, extracardiac, Fontan procedure at high altitude. Methods: Retrospective analysis of data from consecutive patients who underwent non-fenestrated, extracardiac, Fontan procedure at two institutions located in Mexico City at 2,312 m (7,585 ft) and 2,691 m (8,828 ft) above sea level. High altitude was not considered a risk factor. Results: Thirty-nine patients were included, with a mean age of 6.7 years. Mean preoperative indexed pulmonary vascular resistance was 1.7 Wood units. Seventy-nine percent of the patients extubated in the operating room. There was one in-hospital death (2.56%) and one at follow-up. Median chest tube drainage time was 6.5 and 6 days for the right and left pleural spaces. Median oxygen saturation at discharge was 90%. At a median follow-up of six months, all survivors, except one, had good tolerance to daily life activities. Conclusions: The present study shows good short- and medium-term results for the non-fenestrated, extracardiac, Fontan operation at altitudes between 2,300 and 2,700 m and might favor this strategy over fenestration to improve postoperative oxygen saturation. Further studies to examine the long-term outcomes of this approach need to be considered.

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>

Cardiopulmonary reponse to exercise after the Fontan Operation—a cross-sectional and longitudinal evaluation

1996 ◽  
Vol 6 (2) ◽  
pp. 136-142 ◽  
Author(s):  
Luc Mertens ◽  
Ralph Rogers ◽  
Tony Reybrouck ◽  
Monique Dumoulin ◽  
Luc Vanhees ◽  
...  
Keyword(s):  
Carbon Dioxide ◽  
Heart Rate ◽  
Dead Space ◽  
Ventilatory Threshold ◽  
Fontan Operation ◽  
Aerobic Performance ◽  
Medium Term ◽  
Physiological Dead Space ◽  
End Tidal

AbstractThe purpose of this study was to assess cardiorespiratory responses to submaximal exercise in patients with univentricular atrioventricular connection after the Fontan operation, and to evaluate whether changes occur during medium-term follow-up. Eighteen patients (age 12.1±5.5 years) underwent graded exercise test on a treadmill 2.3±1.4 year after the Fontan repair. Ventilatory gases were measured using breath-by-breath analysis. Results were compared to gender/age-matched controls. Twelve patients (age 14.2±5.4 years) were reevaluated 2.4±2.1 years after the first test. Aerobic exercise performance was subnormal in all patients during the first test. At the lowest level of exercise, the ventilatory threshold was already surpassed in 6/18 patients, while it was reduced in all other patients (p<0.001). All patients were in stable sinus rhythm throughout the test. Heart rate at all exercise levels was ±10% below normal (p<0.05). The respiratory frequency was increased at all exercise levels (p<0.001). The ventilatory equivalent for oxygen was increased (p<0.001), and the end-tidal tension of carbon dioxide was decreased (p<0.001). The ratio of physiological dead space/tidal volume was increased in all patients (p<0.001), while the normal decrease of this ratio during exercise was not observed. Upon reevaluation heart rate, respiratory rate, oxygen uptake, venti latory equivalent for oxygen, end-tidal carbon dioxide tension and physiological dead space did not change signifi cantly. Only a slight further decrease in ventilatory threshold was observed. Aerobic performance after the Fontan procedure ranges widely from just above resting metabolic rate to the lower limit of normal. Dyspnea during exercise is exacerbated by a decreased ventilatory threshold, increased physiological dead space, and decreased respir-atory efficiency. Cardiorespiratory response to exercise, nonetheless, remains relatively stable during medium-term follow-up.

Long-term outcome after atrioventricular valve surgery following modified Fontan operation

2010 ◽  
Vol 21 (1) ◽  
pp. 83-88 ◽  
Author(s):  
Shaji C. Menon ◽  
Joseph A. Dearani ◽  
Frank Cetta
Keyword(s):  
Ventricular Function ◽  
Fontan Procedure ◽  
Fontan Operation ◽  
Valve Surgery ◽  
Atrioventricular Valve ◽  
Term Outcome ◽  
Protein Losing Enteropathy ◽  
Long Term Outcome

AbstractObjectiveThe objective of this study was to evaluate the early and late results of atrioventricular valve surgery after Fontan operation.BackgroundAtrioventricular valve regurgitation is a known perioperative risk factor for Fontan operation. There are limited data on the outcomes of late atrioventricular valve surgery following Fontan operation.MethodsPatients who underwent atrioventricular valve surgery following Fontan procedure were identified from the Mayo Clinic Fontan database. Medical records were reviewed for pre-operative, operative, and post-operative clinical and haemodynamic data. All patients not known to be deceased were sent health status questionnaires.ResultsA total of 61 patients (28 females) underwent atrioventricular valve surgery following Fontan procedure. The median age at atrioventricular valve surgery was 14 years. The median duration between Fontan and atrioventricular valve surgery was 4.7 years. Median follow-up was 9 years. There were a total of 32 (52%) deaths with 8 (13%) within 30 days of surgery. The 5-, 10-, and 15-year survival rates were 67%, 57%, and 45%, respectively. On follow-up, 44 of 61 (72%) had arrhythmias, 21 of 29 (72%) were symptomatic, and 12 of 61 (20%) developed protein-losing enteropathy. On multivariate analysis, reduced ventricular function and development of protein-losing enteropathy were associated with decreased survival.ConclusionAtrioventricular valve surgery after Fontan procedure is associated with substantial late morbidity and mortality. Atrioventricular valve surgery in this cohort of patients portends poor long-term outcome and is associated with a high incidence of protein-losing enteropathy. Reduced ventricular function and development of protein-losing enteropathy were associated with decreased survival.

Noninvasive Assessment of Liver Stiffness in Patients Undergoing the Fontan Procedure

2020 ◽  
Vol 11 (5) ◽  
pp. 572-577 ◽  
Author(s):  
Sachin Talwar ◽  
Vasubabu Gudala ◽  
Raja Joshi ◽  
DM Shalimar ◽  
Kumble Seetharama Madhusudhan ◽  
...  
Keyword(s):  
Liver Injury ◽  
Transient Elastography ◽  
Early Recognition ◽  
Fontan Procedure ◽  
Fontan Operation ◽  
Liver Stiffness ◽  
Shear Wave Elastography ◽  
Noninvasive Methods ◽  
Over Time

Background: Hepatic derangements and liver injury following the Fontan operation (FO) may progress in some cases to hepatic fibrosis (HF) and cirrhosis. The respective roles of transient elastography (TE) or FibroScan, shear wave elastography (SWE), and aspartate transaminase to platelet ratio index (APRI) in assessing liver stiffness (LS) and possible liver injury in these patients are unclear. Methods: Thirty-nine patients (31 males), mean age 11.8 ± 5.7 years, median 11 years (interquartile range: 7-14.7 years), undergoing the FO between November 2017 and December 2018 were included. Liver stiffness and HF assessment was done with TE, SWE, and APRI in the preoperative period and postoperatively at 3-, 6-, and 12-month interval. Results: The LS values increased over time (postoperative values at 3, 6, and 12 months) as compared to preoperative values by both FibroScan and SWE. The change in liver stiffness measurement (LSM) was statistically significant: LSM0 to LSM3 ( P ≤ .0001), LSM0 to LSM6 ( P ≤ .0001), and LSM0 to LSM12 ( P = .001). Similarly, significant changes were observed on SWE: SWE0 to SWE3 ( P ≤ .0001), SWE0 to SWE6 ( P ≤ .0001), and SWE0 to SWE12 ( P = .001). There was no significant change in the APRI values over time. Conclusion: Noninvasive methods such as FibroScan and SWE may be of use to assess LS in follow-up of patients undergoing the FO for early recognition of hepatic changes.

Systemic ventricle morphology impact on ten-year survival after Fontan surgery

2018 ◽  
Vol 26 (9) ◽  
pp. 677-684 ◽  
Author(s):  
Mira Fauziah ◽  
Oktavia Lilyasari ◽  
Lies Dina Liastuti ◽  
Budi Rahmat
Keyword(s):  
Survival Rate ◽  
Fontan Procedure ◽  
Fontan Operation ◽  
Survival Rates ◽  
Univentricular Heart ◽  
Thromboembolic Events ◽  
Term Survival ◽  
Systemic Ventricle ◽  
The Impact

Background The Fontan procedure has been applied in many patients with single-ventricle physiology, with quite low mortality rates all over the world, and a 8.6%–9.1% rate in Indonesia. Structural and characteristic differences in the morphologically left and right ventricles influence the role of the systemic ventricle in the functionally univentricular heart and impact on postoperative outcomes. Mid- and long-term survival based on systemic ventricle morphology remains controversial. This study aimed to investigate the impact of systemic ventricle morphology on ten-year survival after the Fontan procedure. Methods The 162 patients who underwent a Fontan operation at our institution between 2008 and February 2018 and survived to discharge were reviewed and followed up until March 2018. Data were extracted from the registry and pediatric surgical conferences, medical records, surgery reports, echocardiography and catheterization reports, and follow-up to the end of the study period. Median follow-up was 26.5 months (range 10.75–54 months). Results The patients were divided into 2 groups based on systemic ventricle morphology. Seventy-four patients were included in the left morphology group and 88 in the right morphology group. There was no difference in 10-year survival rates between the two type of systemic ventricle morphology. Postoperative thromboembolic events influenced the 10-year survival rate after the Fontan procedure (hazard ratio 4.84, 95% confidence interval: 1.26–18.55, p = 0.021). Conclusion Systemic ventricle morphology was not associated with the 10-year survival rate after the Fontan procedure. Postoperative thromboembolic events accounted for a 4.84-times higher mortality risk after the Fontan procedure.

Mid-Term Follow-Up After Extracardiac Fontan Operation

2004 ◽  
Vol 52 (4) ◽  
pp. 218-224 ◽  
Author(s):  
V. Alexi-Meskishvili ◽  
S. Ovroutski ◽  
P. Ewert ◽  
J.-H. Nürnberg ◽  
B. Stiller ◽  
...  
Keyword(s):  
Fontan Operation ◽  
Extracardiac Fontan

scholarly journals Incidence, Predictors, and Mortality in Patients With Liver Cancer After Fontan Operation

2021 ◽  
Vol 10 (4) ◽  
Author(s):  
Hideo Ohuchi ◽  
Yohsuke Hayama ◽  
Kimiko Nakajima ◽  
Kenichi Kurosaki ◽  
Isao Shiraishi ◽  
...  
Keyword(s):  
Liver Fibrosis ◽  
Liver Cancer ◽  
Hospice Care ◽  
Fontan Procedure ◽  
Fontan Operation ◽  
Late Complication ◽  
Asymptomatic Patients ◽  
Median Period ◽  
New Onset

Background Liver cancer (LC) is a serious late complication after the Fontan operation. However, the incidence, predictors, and prognosis remain unknown. The purpose of our study was to determine these clinical characteristics. Methods and Results We assessed liver function in 339 consecutive patients who had undergone the Fontan procedure from 2005 to 2019. LC was histologically diagnosed in 10 patients after a median period of 2.9 years (range: 0.3–13.8; median age: 29.9 years [range: 14.4–41.5 years]; overall median post–Fontan procedure follow‐up: 25.6 years [range: 13–32.1 years]), and the annual incidence was 0.89%. Over the entire post‐Fontan follow‐up period, the annual incidences of new‐onset LC in the second, third, and fourth decades were 0.14%, 0.43%, and 8.83%, respectively. The patients with LC had longer follow‐up periods, higher levels of AFP (α‐fetoprotein), and higher values of liver fibrosis indices ( P <0.01–0.0001). Moreover, all indices were predictive of new‐onset LC ( P <0.01–0.0001). The LC treatments were surgical resection (n=3), transarterial chemoembolization (n=3), radiofrequency ablation (n=2), and hospice care (n=2). During a median follow‐up of 9.4 months, 4 patients died; the survival rate at 1 year was 60%, and it was better among asymptomatic patients ( P <0.01). Conclusions The LC incidence rapidly increased ≥30 years after the Fontan procedure, and liver fibrosis indices and AFP were predictive of new‐onset LC. These LC‐predictive markers should be monitored closely and mandatorily for early LC detection and better prognosis.

Extracardiac Fontan Fenestration Device Closure with Amplatzer Vascular Plug II and Septal Occluder: Procedure Results and Medium-Term Follow-Up

2020 ◽  
Vol 41 (4) ◽  
pp. 703-708
Author(s):  
Melissa K. Webb ◽  
Lyndsey E. Hunter ◽  
Theodore R. Kremer ◽  
Charles B. Huddleston ◽  
Andrew C. Fiore ◽  
...  
Keyword(s):  
Amplatzer Vascular Plug ◽  
Device Closure ◽  
Medium Term ◽  
Vascular Plug ◽  
Extracardiac Fontan ◽  
Amplatzer Vascular Plug Ii ◽  
Fontan Fenestration

scholarly journals Outcomes following modified extracardiac Fontan procedure of direct total cavopulmonary connection with autologous vessels: a single-centre 10-year experience

2019 ◽  
Author(s):  
Xiaobing Liu ◽  
Haiyun Yuan ◽  
Jimei Chen ◽  
Jianzheng Cen ◽  
Zhiqiang Nie ◽  
...  
Keyword(s):  
Fontan Operation ◽  
Growth Potential ◽  
Total Cavopulmonary Connection ◽  
Long Term Outcomes ◽  
Protein Losing Enteropathy ◽  
Significant Difference ◽  
Cavopulmonary Connection ◽  
Extracardiac Fontan

AbstractOBJECTIVESThe modified extracardiac Fontan of direct total cavopulmonary connection (dTCPC) with entirely autologous vessels is a better solution in selected patients with functionally univentricular hearts because it combines the advantages of a tubular connection and of full growth potential. We investigated the mid-term to long-term outcomes of the physical condition of patients who had the dTCPC and assessed the growth potential of the autologous dTCPC pathway.METHODSFrom July 2005 to June 2014, 31 patients, aged 1.8–14.0 years, underwent a modified extracardiac Fontan with dTCPC at our institution. Twenty-two patients underwent a 1-stage dTCPC and 9 patients underwent a 2-stage dTCPC. The preoperative and postoperative data were reviewed retrospectively.RESULTSThere were 17 (54.8%) boys and 14 (45.2%) girls, with a median age of 6.4 years (range 1.8–14.0 years) and a mean weight of 17.8 kg (range 9–41 kg). The mean follow-up period was 6.0 years (range 2.1–10.2 years). There was 1 early death and 2 late deaths. The event-free survival rate for the 31 patients was 88.9% at 10 years during the follow-up period. Late protein-losing enteropathy, thromboembolism, arrhythmia or heart failure were not observed. There was a significant difference between the preoperative and postoperative data for body mass index and the diameters of the autologous dTCPC pathway and pulmonary artery branches, confirming that the growth potential of the autologous dTCPC pathway was realized.CONCLUSIONSThe dTCPC procedure could be performed with satisfactory midterm to long-term outcomes in selected patients. It has the potential of retaining the advantage of the extracardiac Fontan operation together with the potential for growth and the avoidance of prosthetic materials.

Mid-term follow-up after extracardiac fontan operation

2004 ◽  
Vol 52 (S 1) ◽  
Author(s):  
V Alexi-Meskishvili ◽  
S Ovroutski ◽  
P Ewert ◽  
JH N�rnberg ◽  
B Stiller ◽  
...  
Keyword(s):  
Fontan Operation ◽  
Extracardiac Fontan
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