Long term negative pressure ventilation: Rescue for the failing fontan?

Current treatment strategies for single ventricle patients include non-intervention strategy, surgical palliation or primary transplantation. Surgical palliation includes a staged operative course culminating in the Fontan operation. With progress in surgical techniques, the survival has been improving. However, almost all of these Fontan patients will demonstrate pathophysiologic changes that ultimately constitute "Fontan failure physiology". This article reviews the pathophysiologic changes, current approach to management of these patients and proposes a novel way of reversing some of the pathophysiologic changes by utilization of negative pressure ventilation.     

Discontinuous Pulmonary Arteries Do Not Preclude Good Fontan Outcomes

Objective. Discontinuous pulmonary arteries are believed to portend poor outcomes for a single ventricle palliation leading to Fontan's operation. This is a single institutional review of patients with single ventricle and discontinuous pulmonary arteries who underwent pulmonary artery centralization as part of staged surgical palliation. Design. The study is a retrospective case series. Patients. From November 1997 to December 2005, 12 centralization procedures were performed on 12 single ventricle patients with discontinuous pulmonary arteries. The diagnoses at surgery were as follows: heterotaxy 67%, pulmonary atresia 75%, a single morphologic right ventricle 58%, a single morphologic left ventricle 33%, and functional single ventricle with atrial situs inversus 8%. Outcome Measures. The outcome was assessed by hospital survival, actuarial survival, and New York Heart Association (NYHA) classification at follow-up. Results. The overall actuarial survival following centralization is 100% (95% confidence interval = 0.698 to 1). Seventy-five percent of the patients have undergone a Fontan procedure. Median McGoon ratio pre-Fontan = 1.65 (range: 1–2.1). Median follow-up after Fontan = 4.4 years (range: 1.2–9 years). Overall actuarial survival following Fontan is 100% (95% confidence interval = 0.428–0.911). Following the Fontan, there have been no thromboembolic complications, protein-losing enteropathy, nor Fontan takedowns. One hundred percent of the Fontan patients are NYHA class I. Conclusions. This experience indicates that a resuscitative strategy for discontinuous pulmonary arteries can result in good outcomes after the Fontan procedure. The presence of discontinuous pulmonary arteries in patients with single ventricle physiology should not preclude a management strategy with the goal of Fontan candidacy.     

Fontan Circulation: Success or Failure?

Fontan surgery represents a milestone in the evolution of congenital heart disease management. It achieved the seemingly improbable (ie, restoration of a noncyanotic state by entirely bypassing the subpulmonary ventricle). In so doing, it has allowed a generation of children who may have otherwise succumbed to their severe congenital heart defect to survive to adulthood. Perfect univentricular physiology is, however, an elusive goal. The Fontan circulation inherently represents a hemodynamic compromise that results in a catalog of potential multiorgan complications. In this review, we explore current knowledge regarding pathophysiology of the failing Fontan, its varied clinical manifestations, and potential therapeutic options. Failure of the Fontan circulation is broadly divided into 3 overlapping categories: ventricular dysfunction, systemic complications of Fontan physiology, and chronic Fontan failure. As long as the Fontan operation continues to serve as the paradigm of care for patients with univentricular hearts, efforts must be directed toward supporting this dynamic circulation that progressively declines in efficiency with age. Continued research in therapies is needed for univentricular dysfunction and systemic complications of Fontan palliation, including potential uses of mechanical support as a bridge to transplantation or as a neosubpulmonary ventricle. Fontan patients remain a major challenge to the medical and surgical community as a whole. Multicentre and multidisciplinary efforts to improve the density and depth of experiences might lead to a better appreciation for, and management of, Fontan failure and its ramifications.     

Transcatheter neoaortic valve replacement utilizing the melody valve in hypoplastic left heart syndrome

Percutaneous transcatheter pulmonary valve replacement with the Melody Valve is fast becoming an important adjunct in the treatment of older children and adults with failing right ventricular outflow tract conduits. Recently, the Melody Valve has also been successfully implanted in the tricuspid, mitral, and aortic positions, typically within a failing bioprosthetic valve. We present a patient who underwent Fontan palliation for hypoplastic left heart syndrome variant and subsequently developed severe neoaortic regurgitation, which was successfully treated with a transcatheter neoaortic valve replacement. To our knowledge, this is the first successful use of the Melody Valve in the neoaortic position in a patient with single‐ventricle physiology. Successful relief of neoaortic valve regurgitation using replacement with a transcatheter valve may allow avoidance of additional surgery, increase functional longevity of single‐ventricle palliation, and postpone the need for orthotopic heart transplantation. © 2014 Wiley Periodicals, Inc.     

Recurrent Protein‐losing Enteropathy and Tricuspid Valve Insufficiency in a Transplanted Heart: A Causal Relationship?

This case report describes a toddler who developed a protein‐losing enteropathy (PLE) 4 years after orthotopic heart transplantation (OHT). He was born with a hypoplastic left heart syndrome for which he underwent a successful Norwood procedure, a Hemi–Fontan palliation, and a Fontan palliation at 18 months of age. Fifteen months following the Fontan operation, he developed a PLE and Fontan failure requiring OHT. Four years after OHT, he developed a severe tricuspid regurgitation and a PLE. His PLE improved after tricuspid valve replacement. It is now 2 years since his tricuspid valve replacement and he remains clinically free of ascites and peripheral edema with a normal serum albumin level. His prosthetic tricuspid valve is functioning normally.     

Staged Single‐ventricle Palliation in 2011: Outcomes and Expectations

Outcomes for staged palliation for single‐ventricle heart disease have improved over the past two decades. As outcomes improve, parental expectations for survival and quality of life have risen accordingly. Nevertheless, the number of interventions and complications these patients must endure remain high. The final surgical destination of the single‐ventricle patient, the total cavopulmonary connection (or Fontan operation) successfully separates systemic venous and pulmonary venous blood flow but does so at great cost. Fontan patients remain at significant risk of complications despite what are perceived to be “favorable” hemodynamics. The outcomes in this population are discussed in this review, with particular attention to the history behind our current strategies as well as to recent salient studies.     

Considerations in Critical-Care and Anesthetic Management of Adult Patients Living With Fontan Circulation

The Fontan procedure is a staged palliation for various complex congenital cardiac lesions, including tricuspid atresia, pulmonary atresia, hypoplastic left heart syndrome, and double-inlet left ventricle, all of which involve a functional single-ventricle physiology. The complexity of the patients’ original anatomy combined with the anatomic and physiologic consequences of the Fontan circulation creates challenges. Teens and adults living with Fontan palliation will need perioperative support for noncardiac surgery, peripartum management for labour and delivery, interventions related to their structural heart disease, electrophysiology procedures, pacemakers, cardioversions, cardiac surgery, transplantation, and advanced mechanical support. This review focuses on the anesthetic and intensive care unit (ICU) management of these patients during their perioperative journey, with an emphasis on the continuity of preintervention planning, referral pathways, and postintervention ICU management. Requests for recipes and doses of medications are frequent; however, as in normal anesthesia and ICU practice, the method of anesthesia and dosing are dependent on the presenting medical/surgical conditions and the underlying anatomy and physiologic reserve. A patient with Fontan palliation in their early 20s attending school full-time with a cavopulmonary connection is likely to have more reserve than a patient in their late 40s with an atriopulmonary Fontan at home waiting for a heart transplant. Each case will require an anesthetic and critical care plan tailored to the situation. The critical care environment is a natural extension of the anesthetic management of a patient, with complex considerations for a patient with Fontan palliation.     

Rare problems associated with the Fontan circulation

The Fontan operation, originally described for the surgical management of tricuspid atresia, is now the final surgery in the strategy of staged palliation for a number of different forms of congenital cardiac disease with a functionally univentricular heart. Despite the improved technical outcomes of the Fontan operation, staged palliation does not recreate a normal physiology. Without a pumping chamber delivering blood to the lungs, the cardiovascular system is less efficient; cardiac output is generally diminished, and the systemic venous pressure is increased. As a result, patients with "Fontan physiology" may face a number of rare but potentially life-threatening complications including hepatic dysfunction, abnormalities of coagulation, protein-losing enteropathy, and plastic bronchitis. Despite the staged palliation resulting in remarkable survival, the possible complications for this group of patients are complex, involve multiple organ systems, and can be life threatening. Identifying the mechanisms associated with each of the rare complications, and developing strategies to treat them, requires the work of many people at many institutions. Continued collaboration between sub-specialists and between institutions will be required to optimise the care for this group of survivors with functionally univentricular hearts.     

Rescue cardiac transplantation for failing staged palliation in patients with hypoplastic left heart syndrome

OBJECTIVE: Orthotopic heart transplantation is considered a rescue option for children with failing staged palliation or repair of hypoplastic left heart syndrome. We present our strategy for management, and outcomes, for these complex patients. METHODS: We transplanted 68 consecutive children, with diagnoses of hypoplastic left heart syndrome in 31, cardiomyopathy in 20, and post-operative complex congenital heart disease in 17. Of these patients, 9 (13.2%) were neonates, and 46 (67.6%) were infants. Median age was 118.5 days. Operative technique involves bicaval cannulation and anastamoses with continuous low flow bypass, and either short periods of circulatory arrest or continuous low flow antegrade cerebral perfusion for reconstruction of the aortic arch. Initial reperfusion of the donor heart utilizes glutamate and aspartate substrate enriched white blood cell filtered cardioplegia. Immunosuppressive therapy includes induction (pulse steroids, gamma globulin, and polyclonal rabbit antithymocyte globulin) and initial maintenance (calcineurin inhibitor, an anti-proliferative agent, and a weaning steroid protocol). Of the 31 patients with hypoplastic left heart syndrome, 23 underwent primary transplantation, and 8 underwent rescue transplantation from failing staged palliation in seven, or attempted biventricular repair in one. Of the seven patients who had failing staged palliation, three had undergone only the Norwood Stage 1 operation, 2 had undergone a Norwood Stage 1 operation and a Glenn superior cavopulmonary anastomosis and two had undergone a Norwood Stage 1 operation, a Glenn superior cavopulmonary anastomosis, and a completion Fontan operation. RESULTS: The group undergoing primary transplantation was younger (p equals 0.007), weighed less (p equals 0.003), and waited longer for an appropriate donor heart (p equals 0.021) compared to those requiring rescue transplantation. No significant difference exists between the groups with regards to donor heart ischaemic time or post-transplant length of hospital stay. Thirty day survival (p equals 0.156) and overall survival (p equals 0.053) was better in those having primary transplantation, although these differences were not statistically significant when a p value of less than 0.05 is considered to be significant. In those having primary transplantation, no patients had elevated panel reactive antibody greater than 10%. Half of the 8 requiring rescue transplantation had panel reactive antibody greater than 10%, and this subgroup did especially poorly. CONCLUSION: Cardiac transplantation can offer children with failing staged palliation their only chance of survival. Transplantation, however, carries a high risk in this subgroup, especially in the setting of elevated panel reactive antibody.     

Langzeitergebnisse nach modifizierter Fontan-Operation

Since the introduction of palliative surgical therapy for single ventricle defect more and more patients have reached adulthood. Thus, nowadays the long-term results of large cohorts can be analyzed. The results of the modified Fontan operation performed with modern surgical technique are characterized by low morbidity and mortality rates. Therefore in the past 40 years a new patient population with very specific anatomical and physiological principles has arisen and continues to increase in size. The long-term follow-up reveals stable hemodynamics under non-cyanotic conditions. The preconditions for optimal long-term outcome are strict preoperative selection and subject-specific postoperative supervision. The long-term results can be significantly improved if therapy is started early enough. Nevertheless the Fontan circulation is limited through the non-physiological flow principle and decreased function of the single ventricle. Essential for the long-term stability of the hemodynamics are the reduction of pulmonary vascular resistance and improvement of ventricular filling. The conventional medication for heart failure is not applicable in treatment of the failing Fontan circulation. Severe Fontan complications, such as protein-losing enteropathy with chronic ascites and chronic Fontan failure are rare. Nevertheless the possibility for conventional treatment in such cases is limited and heart transplantation is the ultima ratio therapy.     

Salvage of an epicardial lead in a pacemaker‐dependent patient with Fontan palliation using an IS‐1 extender

We present a case report of severed epicardial atrial lead salvage using an IS‐1 lead extender. A 37‐year‐old male with single ventricle physiology, Fontan palliation, sinus node dysfunction, recurrent atrial tachycardias, and atrial fibrillation resulting in failing Fontan physiology presented with failure of the atrial pacing lead. The patient was initially paced with an epicardial system that had to be removed due to pocket infection, and the epicardial leads were cut and abandoned. Given his significant sinus node dysfunction he required atrial pacing to allow for rhythm control. The failing Fontan physiology of the patient precluded him from undergoing surgery for epicardial lead placement or a complex intravascular lead placement procedure (although anatomically feasible). We considered the option of salvaging the existing epicardial atrial leads to provide atrial pacing, allowing for rhythm control and improvement of his failing Fontan physiology as a bridge to a more permanent pacing solution. This case report is important because it demonstrates how a lead extender can be used to salvage a severed pacemaker lead. This may be useful for patients in whom implantation of new leads is not promptly feasible due to patient anatomy and/or clinical status.     

Outcome of single ventricle and total anomalous pulmonary venous connection

Management of patients with single-ventricle physiology is significantly affected by anomalies of pulmonary venous return at all stages, whether primary palliation, bidirectional Glenn shunt, or completion of Fontan circulation. We treated 25 patients with pulmonary venous anomalies and single ventricle by staged palliation, from June 1996 to May 2005. Visceral heterotaxy with atrial isomerism was present in 19 of them. Primary palliation with a systemic-to-pulmonary artery shunt was undertaken in 15 patients. There were 5 early deaths, of which 4 were due to obstruction of pulmonary venous return. A bidirectional Glenn shunt was constructed in 17 patients including 10 who had it as a primary palliative procedure. There were 7 early deaths after the bidirectional Glenn procedure; only one was due to pulmonary venous obstruction. Five patients attained completion of the Fontan procedure. There was one early death after the Fontan operation. Anomalous pulmonary venous return can significantly complicate the management of patients with single ventricle, with an impact on survival in early infancy. Palliation with the aim of instituting extracardiac conduit Fontan circulation allows greater latitude and more streamlined management.     

Single ventricle palliation: greater risk of complications with the Fontan procedure than with the bidirectional Glenn procedure alone

BACKGROUND: This study was performed to evaluate and compare the early, intermediate, and long-term outcomes of the bidirectional Glenn procedure and Fontan procedure in patients who live at moderately high altitude. METHODS: The outcome of each method of palliation for patients with a functionally single ventricle was retrospectively evaluated from a review of medical records. RESULTS: The bidirectional Glenn procedure was performed in 177 patients from October 1984 to June 2004. The Fontan procedure was performed in 149 patients from June 1978 to June 2004. Cardiovascular death or heart transplantation occurred in 8% of patients after the bidirectional Glenn procedure and 17% of patients after the Fontan procedure. Complications of systemic thromboembolic events, bleeding associated with anticoagulation therapy, protein losing enteropathy, and arrhythmias requiring implantation of a pacemaker, cardioversion, or radiofrequency ablation occurred in 7% of patients after the bidirectional Glenn procedure and 47% of patients after the Fontan procedure. Cardiovascular deaths and heart transplantation occurred less frequently when the Fontan procedure was performed in patients with a previous bidirectional Glenn procedure. However, the actuarial transplant-free survival and freedom from complications was not superior for a subgroup of patients who had a Fontan procedure after a bidirectional Glenn procedure in comparison to a subgroup of patients who had a bidirectional Glenn procedure alone. CONCLUSIONS: The bidirectional Glenn procedure can be used for long-term palliation of patients with a functionally single ventricle. Additional palliation with a Fontan procedure may increase the risk of stroke, protein losing enteropathy and arrhythmias without improving survival.     

Amplatzer occlusion of accessory ventriculopulmonary connections

Background: Children with complex congenital heart disease often require staged palliation to regulate systemic and pulmonary blood flow. Accessory sources of pulmonary blood flow including aortopulmonary collaterals, aortopulmonary shunts, and ventriculopulmonary connections following Glenn or Fontan palliation can be associated with elevated central venous pressures and persistent pleural drainage. Occlusion of accessory ventriculopulmonary connections in this setting has traditionally been accomplished surgically. Objective: To review the efficacy of Amplatzer devices in transcatheter occlusion of accessory ventriculopulmonary connections in children with complex congenital heart disease. Methods: Patients were identified and their records retrospectively reviewed for indication, procedural details, and clinical efficacy and outcome. Results: Between December 2004 and March 2008, seven patients underwent occlusion of accessory ventriculopulmonary connections using an Amplatzer Septal Occluder (3), an Amplatzer Duct Occluder (3), or an Amplatzer Vascular Plug (1). Underlying single ventricle physiology was present in six of these patients. The site of occlusion was the right ventricle to pulmonary artery (Sano) conduit in two patients, the native main pulmonary artery in three patients, the pulmonary valve in one patient, and a left ventricle to pulmonary artery homograft in one patient with biventricular physiology. There were no complications associated with these procedures. Conclusions: Amplatzer occlusion devices provide a safe and effective means of eliminating accessory ventriculopulmonary connections in children who have undergone surgical palliation of congenital heart disease. © 2008 Wiley‐Liss, Inc.     

Growth of children with a functionally single ventricle following palliation at moderately increased altitude

The bidirectional Glenn and Fontan procedures are empirically performed as interim and definitive procedures in children with a functionally single ventricle. The optimal stage of palliation, nonetheless, remains unknown. During childhood, growth is a fundamental measure of response to therapy. Growth may be influenced by the degree of cyanosis, the volume load on the ventricle, and cardiac performance. Thus, the weight and stature of children with a functionally single ventricle who underwent a bidirectional Glenn procedure or a Fontan procedure were studied to determine the effect of each intervention on growth. Z scores for weight and stature were retrospectively determined prior to palliation, at yearly intervals for 4 years, and from long-term measurements until 18 years of age in all patients with at least 2 years of observation following palliation. Growth was evaluated in 54 patients with a bidirectional Glenn procedure, and 65 patients with a Fontan procedure. The Z scores for weight were improved after each method of surgical palliation. Stature, however, was improved only following the bidirectional Glenn procedure. Growth was impaired in patients who developed protein losing enteropathy. Weight improved only during the initial 2 years after the Fontan procedure in patients who had a surgical fenestration. Over the long-term, patients who underwent a Fontan procedure were more likely to have a Z score less than -2.0 for weight and stature than patients who underwent only a bidirectional Glenn procedure. Late mortality and the incidence of heart transplantation were increased in patients who experienced a decrease in their rate of growth, defined as a negative change of more than one Z score in weight or stature, following the Fontan procedure. In conclusion, at moderately increased altitude, children with a functionally single ventricle grow more appropriately following the bidirectional Glenn procedure than following the Fontan procedure. A decrease in the rate of growth is associated with a poor prognosis following the Fontan procedure.     

Resuscitation and Perioperative Management of the High‐risk Single Ventricle Patient: First‐stage Palliation

Infants born with hypoplastic left heart syndrome or other lesions resulting in a single right ventricle face the highest risk of mortality among all forms of congenital heart disease. Before the modern era of surgical palliation, these conditions were universally lethal; recent refinements in surgical technique and perioperative management have translated into dramatic improvements in survival. Nonetheless, these infants remain at a high risk of morbidity and mortality, and an appreciation of single ventricle physiology is fundamental to the care of these high‐risk patients. Herein, resuscitation and perioperative management of infants with hypoplastic left heart syndrome are reviewed. Basic neonatal and pediatric life support recommendations are summarized, and perioperative first‐stage clinical management strategies are reviewed.     

Percutaneous catheter aspiration thrombectomy for the occluded stents of pulmonary artery in children with single ventricle physiology after fontan surgery

Successful surgical palliation with the Fontan procedure allows survival into adulthood for many patients born with single ventricle (SV) physiology, but the limited studies reported incidence of perioperative and long‐term complications including thromboembolic events. Chronic pulmonary embolism is a common complication in patients with Fontan circulation, and may have serious consequences. Percutaneous intervention may be less invasive option for such a high‐risk population than surgery is. We described two patients who developed complete thrombosis of the left pulmonary artery following catheter placement of a stent in this vessel shortly after Fontan surgery. Percutaneous catheter aspiration thrombectomy was successfully performed. Percutaneous catheter aspiration thrombectomy may be considered as a viable option in acute thrombus in children with SV physiology after Fontan surgery. © 2014 Wiley Periodicals, Inc.     

Frontiers in Fontan failure: Innovation and improving outcomes: A conference summary

The initial “Frontiers in Fontan Failure” conference in 2015 in Atlanta, GA, provided an opportunity for experts in the field of pediatric cardiology and adult congenital heart disease to focus on the etiology, physiology, and potential interventions for patients with “Failing Fontan” physiology. Four types of “Fontan Failure” were described and then published by Dr Book et al. The acknowledgment that even Dr Fontan himself realized that the Fontan procedure “imposed a gradually declining functional capacity and premature late death after an initial period of often excellent palliation.” The purpose of the second “Frontiers in Fontan Failure” was to further the discussion regarding new data and technologies as well as novel interventions. The 2017 “Frontiers in Fontan Failure: Innovation and Improving Outcomes” was sponsored by Children’s Healthcare of Atlanta, Sibley Heart Center Cardiology, and Emory University School of Medicine. Future directions in the management of Fontan failure include further investigations into the risk of sudden cardiac death and how to properly prevent it, achievable interventions in modifying the Fontan physiology to treat or prevent late complications, and improved and refined algorithms in Fontan surveillance. Finally, further research into the interventional treatment of lymphatic‐related complications hold the promise of marked improvement in the quality of life of advanced Fontan failure patients and as such should be encouraged and contributed to.     

Perioperative Care of the Infant With Single Ventricle Physiology

Among patients with congenital heart defects, neonates with single ventricle disease continue to challenge clinicians despite significant improvements in survival over the past 30 years. The cardiac anatomical variants associated with the term “single ventricle” are characterized by severe hypoplasia (or absence) of either ventricle, typically in association with obstruction or atresia of either the pulmonary or systemic outflow tracts. Physiologically, the single ventricle receives both pulmonary and systemic venous blood and ejects simultaneously into the pulmonary and systemic circulations, a pattern commonly referred to as single ventricle physiology. Medical and surgical management strategies, though palliative, are aimed at achieving the optimal balance of systemic blood flow and pulmonary blood flow to maximize oxygen delivery. Patients with single ventricle physiology have a greater risk of dying than those with biventricular circulations and are generally committed to multiple palliative interventions throughout childhood with considerable risk. Surgical intervention in the newborn period involves Norwood Stage I palliation, placement of a systemic-to-pulmonary artery shunt, or banding of the pulmonary artery, depending on the status of the outflow tracts. Heart transplantation is offered as the initial approach in some centers. The management strategy and the actual delivery of care from the time of birth (or at time of diagnosis) through the postoperative period is crucial to optimize the short-term and long-term outcomes. Whereas survival following initial palliation in experienced centers is as high as 95%, emphasis is now appropriately shifting toward the control of in-hospital morbidity and optimizing long-term functional outcome. Centers are continually striving to gather and apply new knowledge related to the underlying anatomical and physiologic problems while seeking to improve decision making and care of the patient with single ventricle physiology.     

Prevalence and Risk Factors Associated with Renal Dysfunction in Patients with Single Ventricle Congenital Heart Disease after Fontan Palliation

Objectives: The Fontan operation has increased survival in patients with single-ventricle congenital heart defects. However, Fontan survivors are at risk of other organ dysfunctions, such as renal dysfunction (RD). The objectives of this study are to assess the prevalence of and potential risk factors for RD among Fontan survivors. Design, setting, and patients: We performed a two-center, cross-sectional study that included Fontan survivors evaluated in outpatient-clinics for routine follow up between 01/08-12/16. Outcome measures: The primary outcome was the presence of RD defined by estimated glomerular filtration rate (eGFR) of <90 ml/min/1.73 m² derived using the serum creatinine-based Full Age Spectrum equation. Chi-square and t-tests were used to compare groups with and without RD. A multivariable logistic regression model was derived to identify risk factors associated with the presence of RD using stepwise variable selection methods. Additionally, using eGFR as a continuous variable, a linear regression model was derived to evaluate risk factors that negatively correlate with eGFR. Results: We included 402 Fontan survivors; 61% male; median age 13.7 (2.3–49.9) years; median time since initial Fontan 9.8 (0.1–36.9) years. RD was present in 27.4% (110/402) of patients. Risk factors for RD included single ventricle with right ventricular morphology [odds ratio 2.04; 95% CI (1.26,3.3)], ascites [2.99 (1.04,8.59)] and sildenafil therapy [2.22 (1.05,4.67)]. Risk factors that negatively correlate with eGFR included history of Stage 1 Norwood palliation (−7.6 ml/min/ 1.73 m²; p = 0.003); “failing Fontan physiology” defined by ascites, protein-losing enteropathy and/or plastic bronchitis (−8.9 ml/min/1.73 m²; p = 0.01) and moderate or greater ventricular dysfunction (−16.7 ml/min/1.73 m²; p = 0.02). Conclusions: One-fourth of Fontan survivors demonstrate RD within ten years after Fontan. Risk factors for RD included right ventricular morphology of the single ventricle, history of Stage 1 Norwood palliation, “failing Fontan physiology,” or ventricular dysfunction. Therefore, comprehensive screening for RD in Fontan survivors is needed, particularly in those identified at a higher risk for RD.