Current treatment options for the Failing Fontan Circulation

Resistance Protein ◽

Abstract: The Fontan operation was introduced in 1968. For congenital malformations where biventricular repair is not suitable, the Fontan procedure has provided a long-term palliation strategy with improved outcome compared to the initially developed procedures. Despite these improvements, several complications merely as a result of a failing Fontan circulation (including myocardial dysfunction, arrhythmias, increased pulmonary vascular resistance, protein losing enteropathy, hepatic dysfunction, plastic bronchitis and thrombo-embolism) will limit life-expectancy in this patient cohort. This review provides an overview of the most common complications of the Fontan circulation and the currently available treatment options.

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

10.1017/s1047951110001538 ◽

2010 ◽

Vol 21 (1) ◽

pp. 83-88 ◽

Cited By ~ 19

Author(s):

Shaji C. Menon ◽

Joseph A. Dearani ◽

Frank Cetta

Keyword(s):

Ventricular Function ◽

Fontan Procedure ◽

Fontan Operation ◽

Valve Surgery ◽

Atrioventricular Valve ◽

Term Outcome ◽

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.

Surgical revisions and mechanical support of the failing Fontan

10.1017/s1047951113001674 ◽

2013 ◽

Vol 23 (6) ◽

pp. 847-851 ◽

Cited By ~ 3

Author(s):

Stephanie Fuller

Keyword(s):

Single Ventricle ◽

Fontan Operation ◽

Mechanical Support ◽

Management Of Complications ◽

Fontan Failure ◽

Mechanical Devices ◽

Surgical Treatments ◽

AbstractDespite its many anatomic modifications, Fontan failure remains prevalent in adult patients living with single-ventricle physiology. Although the Fontan operation has created a generation of survivors of complex single-ventricle disease, recognition of the limitation of this circulation is increasing. Most commonly, survivors suffer from ventricular dysfunction, protein-losing enteropathy, plastic bronchitis and arrhythmias. Although several medical therapies exist to palliate these long-term complications, surgical treatments are becoming increasingly useful, including the introduction of mechanical devices and cardiac transplantation. Currently, there are a variety of options available for mechanical support of the failing Fontan. Further developments in mechanical support technology will be required to tailor-make specific devices for this unique population. Owing to the fact that availability of donor hearts for transplantation is limited, mechanical support will play an increasingly important role in the management of complications of the Fontan circulation.

Long term follow-up after pregnancy in Fontan patients

European Heart Journal ◽

10.1093/ehjci/ehaa946.2212 ◽

2020 ◽

Vol 41 (Supplement_2) ◽

Author(s):

E Gribaudo ◽

A Constantine ◽

A Pires ◽

I Ahmed ◽

R Patel ◽

...

Keyword(s):

Ventricular Function ◽

Nyha Class ◽

Fontan Procedure ◽

Fontan Circulation ◽

Atrial Arrhythmia ◽

Class I ◽

Fontan Patients

Abstract Background Patients with single ventricle physiology who have benefited from the Fontan procedure are reaching childbearing age. In this population, complications are common around pregnancy, but the long term effect of pregnancy is unclear. Purpose To evaluate functional and structural changes several years after pregnancy in patients with Fontan circulation. Methods Women with Fontan circulation who had a pregnancy beyond the 20 weeks of gestation followed at our centre between 2005–2019 were included. The following data were collected before pregnancy and at the last follow up (f-up): cardiac anatomy, type of Fontan procedure, resting O2 saturation (O2sat), NYHA class, systemic atrioventricular valve (SAVV) regurgitation, ventricular function, history of arrhythmias, heart failure (HF), thromboembolism, Fontan-related liver disease (FLD), protein-losing enteropathy (PLE), plastic bronchitis and cardiopulmonary test data. Results 12 patients had 18 pregnancies, giving birth to 17 live newborns (1 newborn death at 24 weeks of gestation). 7 (58.3%)patients had a total cavo-pulmonary connection, 3 (25%)an atrio-pulmonary connection and 2 (16.7%)a Bjork type Fontan. Tricuspid atresia was the most common anatomy (6, 50%). Before pregnancy, O2sat was 95.4±1.9%and all patients were in NYHA class I or II. All patients (12, 100%)had a normal or mildly impaired ventricular function and 3 (25%)had a moderate or severe SAAV regurgitation. 6 (50%)patients had 2 pregnancies. Mean age at first pregnancy was 29.9±4.4 years. Cardiac events occurred in 7 (38%)pregnancies, most commonly atrial arrhythmia (4, 57%), 2 (16.6%)patients developed new cyanosis and 1 had a thromboembolic event. No maternal death occurred and 1 patient had severe haemorrhage. After a mean f-up of 84±48 months, there was no significant worsening in O2sat (94.3±3.1 at f-up, p=1) and all patients remained in NYHA class I or II (12, 100%, p=1). At f-up all patients (12, 100%)continued to have a normal or mildly impaired ventricular function (p=1) and there was no significant worsening in the SAAV regurgitation (3 patients with moderate or severe regurgitation, p=1). 3 (25%)patients developed FLD (p=0.3), 3 (25%) a new episode of atrial arrhythmia (p=0.3). There were no significantly differences in peak VO2 (22.5±3.8 pre-pregnancy, 22.8±5.5 at f-up, p=0.4) and VE/VCO2 slope (34.9±11.2 pre-pregnancy, 36.3±11.8 at f-up, p=0.2)before pregnancy and during f-up. Conclusions In this small cohort of Fontan patients who successfully completed at least 1 pregnancy, there was no significant decline in functional or structural cardiac parameters long term after delivery. Funding Acknowledgement Type of funding source: None

Rebuttal

10.1017/s1047951104006432 ◽

2004 ◽

Vol 14 (S1) ◽

pp. 114-114 ◽

Cited By ~ 1

Author(s):

Leonard L. Bailey

Keyword(s):

Coronary Disease ◽

Reconstructive Surgery ◽

Fontan Circulation ◽

Actuarial Survival ◽

Term Survival ◽

Long Term Survival ◽

Fontan Physiology ◽

The portrayal of a beautiful youngster performing uninhibited acrobatics based on Fontan physiology, as presented by Marshall Jacobs, is a brilliant and beautiful thing for us to see. It is, perhaps, all about will over physiology. But it is, nevertheless, happening for that child. Marshall mentioned the need for re-transplantation, whether the beginning strategy was transplantation or reconstructive surgery. Indeed, a relatively small percentage of children transplanted will require re-transplantation because of severe graft coronary disease. Remarkably, in the Loma Linda experience, 10-year actuarial survival for 26 patients following elective re-transplantation is over 85%, exceeding overall actuarial survival at 10 years for children following primary transplantation. Many of the transplanted infants, however, seem to be realistically hopeful that one heart will last their entire lifetime. Of course, the hope is that their's will be a long and healthy lifetime. The requirement for late transplantation following Fontan procedures, however, seems almost inevitable. We'll simply have to keep these children with Fontan circulation under surveillance to see when, in the course of their lives, transplantation will become necessary. Unfortunately, operative and long-term survival among children who are transplanted for failing Fontan physiology have, as yet, been somewhat suboptimal.

Abstract 14773: Biventricular Conversion After Fontan Completion: A Preliminary Experience

Circulation ◽

10.1161/circ.142.suppl_3.14773 ◽

2020 ◽

Vol 142 (Suppl_3) ◽

Author(s):

Ilias P Doulamis ◽

Supreet P Marathe ◽

Breanna L Piekarski ◽

Rebecca S Beroukhim ◽

Gerald R Marx ◽

...

Keyword(s):

Fontan Circulation ◽

Exercise Intolerance ◽

Optimal Timing ◽

Atrioventricular Canal ◽

Systolic Volume ◽

Hepatic Congestion ◽

Atrioventricular Canal Defect ◽

Fontan Physiology ◽

Hypothesis: Biventricular conversion (BiVC) following takedown of Fontan circulation is feasible and results in improved hemodynamics. Methods: Retrospective analysis of patients who had takedown of Fontan circulation and conversion to BiV circulation at a single center from September 2007 to April 2020. Failing Fontan physiology was defined as Fontan circulation pressure >15 mmHg. Results: There were 23 patients (median age: 10.0 (7.5-13.0) years); 15 (65%) had failing Fontan physiology and 8 (35%) underwent elective takedown of their Fontan circulation. Of the 15 patients with failing Fontan physiology, 4 had exercise intolerance or cyanosis, 3 had hepatic congestion or cirrhosis, 3 had end-organ damage and 1 patient had protein losing enteropathy; the rest 4 patients had no other sign of SVP complications. A subset of patients (n=6) underwent recruitment of the non-dominant ventricle prior to conversion. HLHS (p<0.01) and sub-/aortic stenosis (p<0.01) were more common in these patients. BiVC with or without staged ventricular recruitment led to a significant increase in indexed end-diastolic volume (p<0.01), indexed end-systolic volume (p<0.01) and ventricular mass (p<0.01) of the non-dominant ventricle (14 RV, 9 LV). There were 1 (4%) early and 4 (17%) late deaths. All who underwent elective BiVC survived, while 2-year survival rate for patients with a failing Fontan circulation was 72.7% (95% CI: 37-90%) (Figure 1). The overall, 1-year reintervention free survival was 44.1% (95% CI: 21-65%). Left dominant atrioventricular canal defect (p<0.01) and early year of BiVC (p=0.02) were significant predictors for mortality. Conclusions: BiVC is feasible in patients with failing Fontans, and has promising outcomes after elective takedown of Fontan circulation. A staged approach for ventricular recruitment does not seem inferior to primary BiVC. The optimal timing for BiVC in Fontan patients needs further evaluation.

Protein-Losing Enteropathy and Plastic Bronchitis After the Fontan Operation

Critical Care Nurse ◽

10.4037/ccn2018784 ◽

2018 ◽

Vol 38 (6) ◽

pp. e5-e12 ◽

Cited By ~ 1

Author(s):

Christine Peyton

Keyword(s):

Heart Failure ◽

Congestive Heart Failure ◽

Complex Disease ◽

Fontan Procedure ◽

Fontan Operation ◽

Venous Pressure ◽

Venous Hypertension ◽

Plastic Bronchitis ◽

Fontan Physiology

Protein-losing enteropathy and plastic bronchitis remain challenging to treat despite recent treatment advances. Protein-losing enteropathy and plastic bronchitis have been diagnosed in patients with cardiomyopathy, constrictive pericarditis, and congestive heart failure. This article focuses on patients with protein-losing enteropathy or plastic bronchitis following the Fontan procedure. Patients with single-ventricle physiology who have undergone the Fontan procedure are at risk for these conditions. Fontan physiology predisposes patients to chronically low cardiac output, increased central venous pressure, and congestive heart failure. These altered hemodynamics lead to increased mesenteric vascular resistance, resulting in venous hypertension and congestion in protein-losing enteropathy. Plastic bronchitis is a complex disease in which chronic high lymphatic pressures from Fontan physiology cause acellular bronchial casts to develop. These entities may also occur in patients with normal Fontan hemodynamics. This article also covers medical and surgical interventions for protein-losing enteropathy and plastic bronchitis. (Critical Care Nurse. 2018;38[6]:e5–e12)

MICROSURGICAL TREATMENT OF SYMPTOMATIC SACRAL PERINEURIAL CYSTS

Neurosurgery ◽

10.1227/01.neu.0000255457.12978.78 ◽

2007 ◽

Vol 60 (6) ◽

pp. 1059-1066 ◽

Cited By ~ 54

Author(s):

Dongsheng Guo ◽

Kai Shu ◽

Rudong Chen ◽

Changshu Ke ◽

Yanchang Zhu ◽

...

Keyword(s):

Patient Outcomes ◽

Treatment Options ◽

Current Treatment ◽

Cerebrospinal Fluid Leakage ◽

Local Defect ◽

Microsurgical Treatment ◽

The Past ◽

Return Visits

Abstract OBJECTIVE The aim of this study was to investigate the microsurgical results of symptomatic sacral perineurial cysts of 11 patients and to discuss the treatment options of the past 10 years. METHODS We retrospectively reviewed the records of 11 patients with symptomatic sacral perineurial cysts who underwent microsurgical treatment at Tongji Hospital, Huazhong University of Science and Technology from 1993 through 2006. The philosophy was to perform total or partial cyst wall removal, to imbricate the remaining nerve sheath if possible, and to repair local defect with muscle, Gelfoam (Pharmacia & Upjohn, Kalamazoo, MI), and fibrin glue. Patient outcomes were assessed by comparing the preoperative and postoperative examination results. The average follow-up time obtained from return visits to the neurosurgery clinic or by telephone questionnaires ranged from 2 months to 13 years. A literature search and analysis of current treatment options were performed. RESULTS Nine of the 11 patients (82%) experienced complete or substantial relief of their preoperative symptoms. One patient (Patient 4) experienced worsening of bladder dysfunction after surgery and recovered slowly to subnormal function during the subsequent 2 months. The symptoms of Patient 9 did not resolve, and magnetic resonance imaging showed that the cyst had reoccurred. The patient underwent reoperation 3 months later without any improvement. One patient (Patient 11) experience a cerebrospinal fluid leakage complication. Neither new postoperative neurological defects nor infection were observed in our series. In the literature, there are six different treatment options under debate and controversially discussed. CONCLUSION Microsurgical treatment yielded the best long-term resolution of patient symptoms to date and should be recommended to appropriately selected patients.

Irreparable rotator cuff tears: Current treatment options

Orthopedic Reviews ◽

10.4081/or.2019.8146 ◽

2019 ◽

Vol 11 (3) ◽

Cited By ~ 1

Author(s):

Tristan Juhan ◽

Michael Stone ◽

Omid Jalali ◽

Will Curtis ◽

John Prodromo ◽

...

Keyword(s):

Rotator Cuff ◽

Reverse Shoulder Arthroplasty ◽

Treatment Options ◽

Current Treatment ◽

Rotator Cuff Tears ◽

Biceps Tenotomy ◽

Common Causes ◽

Cuff Repair ◽

Term Data

Rotator cuff disease is one of the most common causes of shoulder pain, yet controversy still exists regarding treatment of “irreparable” tears. Nonoperative management, including physical therapy and steroid injections, should be reserved for those without significant pain or functional impairment. Debridement may be used for low-demand patients, and should be performed with partial cuff repair, subacromial decompression, and/or acromioplasty to maximize outcomes. Biceps tenotomy and/or tenodesis have been shown to reduce postoperative pain and improve satisfaction when performed in conjunction with rotator cuff repairs, with no difference in functional outcome comparatively. Tendon transfers have been advocated with the potential benefit to improve function and decrease pain. More recently, extracellular matrix and human-derived dermal allografts have been used off-label as patch grafts in irreparable tears. Superior capsular reconstructive techniques and subacromial balloon spacers serve a similar function by acting to depress the humeral head in a cuff-deficient shoulder, however long-term data is needed before widespread adoption of these procedures. Finally, reverse shoulder arthroplasty serves as a salvage option for low demand elderly patients.

The effect of bosentan in patients with a failing Fontan circulation

10.1017/s1047951109990023 ◽

2009 ◽

Vol 19 (4) ◽

pp. 331-339 ◽

Cited By ~ 64

Author(s):

Caroline Ovaert ◽

Daisy Thijs ◽

Daniel Dewolf ◽

Jaap Ottenkamp ◽

Hugues Dessy ◽

...

Keyword(s):

Statistical Significance ◽

Fontan Operation ◽

Tertiary Care ◽

Blood Chemistry ◽

Hepatic Function ◽

Fontan Circulation ◽

Controlled Study ◽

Open Label ◽

AbstractObjectivesTo investigate the effect of bosentan in patients with a failing Fontan circulation.DesignA multicentric open label, non-controlled study.Setting5 tertiary care centres for congenital cardiology.PatientsWe included 10 patients with a failing Fontan circulation. Their median age at inclusion was 12.12 years, with a range from 4.41 to 33,41 years. The median interval between the Fontan operation and inclusion was 7.84 years, with a range from 1.96 to 12,18 years. Participants received half the usual dose of bosentan for 4 weeks, and then the full dose for a further 12 weeks.Main measures of outcomesWe assessed saturations of oxygen at rest and during exercise, using a 6 minutes walk test, at baseline, and during and after 16 weeks of treatment. At each visit, we assessed blood chemistry and hepatic function, and asked the patients to complete a questionnaire concerning quality of life. All medical events and possible side effects were recorded.ResultsOf the cohort, 1 patient withdrew. The changes in saturations of oxygen, exercise performance, and scores for the questionnaire did not reach statistical significance for the whole group. We noted, nonetheless, that saturations of oxygen and/or exercise capacity improved in 5 of the patients. This was further confirmed when those patients deteriorated again when the drug was discontinued.ConclusionsOur study failed to show significant improvement after 3 months of treatment with bosentan in a small group of patients with failing Fontan circulations. Some individuals, nonetheless, did improve. When planning larger trials, it would be better to identify those patients who might potentially benefit from the treatment prior to commencing the trial.