Risk stratification of adults with congenital heart disease during the COVID-19 pandemic: insights from a multinational survey among European experts.

OBJECTIVE: Adults with congenital heart disease (ACHD) may be at a higher risk of a fatal outcome in case of COVID-19. Current risk stratification among these patients relies on personal experience and extrapolation from patients with acquired heart disease. We aimed to provide an expert view on risk stratification while awaiting results from observational studies. METHODS: This study was an initiative of the EPOCH (European Collaboration for Prospective Outcome Research in Congenital Heart disease). Among nine European countries (Austria, Belgium, Denmark, France, Germany, Italy, the Netherlands, Spain and Switzerland), 24 experts from 23 tertiary ACHD centres participated in the survey. ACHD experts were asked to identify ACHD-specific COVID-19 risk factors from a list of potential outcome predictors and to estimate the risk of adverse COVID-19 outcomes in seven commonly seen patient scenarios. RESULTS: 82% of participants did not consider all ACHD patients at risk of COVID-19 related complications. There was a consensus on pulmonary arterial hypertension, Fontan physiology and cyanotic heart disease as risk factors for adverse outcomes. Among different ACHD scenarios, a patient with Eisenmenger syndrome was considered to be at the highest risk. There was a marked variability in risk estimation among the other potential outcome predictors and ACHD scenarios. CONCLUSIONS: Pulmonary arterial hypertension, Fontan palliation and cyanotic heart disease were widely considered as risk factors for poor outcome in COVID-19. However, there was a marked disparity in risk estimation for other clinical scenarios. We are in urgent need of outcome studies in ACHD suffering from COVID-19.

End-Organ Function and Exercise Performance in Patients With Fontan Circulation: What Characterizes the High Performers?

Background The physiologic hallmarks of the Fontan circulation-chronically elevated central venous pressures and low cardiac output-have significant effects not only on cardiovascular status but also impact other organ systems. Exercise capacity is limited in many and declines with age, accelerating in adolescence, but with wide variability. We explore the relationship between exercise performance and end-organ function in outpatient subjects with a Fontan circulation. Methods and Results This is a cross-sectional analysis of subject end-organ characterization from our outpatient Fontan circulation clinic with peak oxygen consumption (peak Vo2) at cardiopulmonary exercise testing as the primary outcome. We perform linear regression to assess associations between clinical characteristics and peak Vo2 as well as the magnitude of the association of clinical characteristics with peak Vo2. Of 265 subjects age 12.8 (9.5-16.4) years, there is a negative correlation between age and peak Vo2 (-0.49, P<0.001). Of those undergoing ramp cycle exercise testing, 34% perform above 80% predicted peak Vo2. Variables positively associated with peak Vo2 and their effect size include vitamin D sufficiency (+3.00, P=0.020) and absolute lymphocyte count (+0.23, P=0.005). Status as overweight/obese (-3.91, P=0.003) and hemoglobin (-0.77, P=0.003) are negatively associated. Neither ventricular morphology, timing of Fontan palliation, nor Fontan circulation type affect peak Vo2. Conclusions Higher peak Vo2 in those with a Fontan circulation is associated with younger age, vitamin D sufficiency, absence of overweight/obese, lower hemoglobin, and a healthier hepatic profile. Whether exercise training or other initiatives can modify organ characteristics in those with a Fontan circulation is worthy of exploration.

Socioeconomic Status and Long-term Outcomes in Single Ventricle Heart Disease.

BACKGROUND: Low socioeconomic status (SES) has emerged as an important risk factor for higher short-term mortality and neurodevelopmental outcomes in children with hypoplastic left heart syndrome and related anomalies; yet little is known about how SES affects these outcomes over the long-term. METHODS: We linked data from the Single Ventricle Reconstruction trial to US Census Bureau data to analyze the relationship of neighborhood SES tertiles with mortality and transplantation, neurodevelopment, quality of life, and functional status at 5 and 6 years post-Norwood procedure (N = 525). Cox proportional hazards regression and linear regression were used to assess the association of SES with mortality and neurodevelopmental outcomes, respectively. RESULTS: Patients in the lowest SES tertile were more likely to be racial minorities, older at stage 2 and Fontan procedures, and to have more complications and fewer cardiac catheterizations over follow-up (all P < .05) compared with patients in higher SES tertiles. Unadjusted mortality was highest for patients in the lowest SES tertile and lowest in the highest tertile (41% vs 29%, respectively; log-rank P = .027). Adjustment for patient birth and Norwood factors attenuated these differences slightly (P = .055). Patients in the lowest SES tertile reported lower functional status and lower fine motor, problem-solving, adaptive behavior, and communication skills at 6 years (all P < .05). These differences persisted after adjustment for baseline and post-Norwood factors. Quality of life did not differ by SES. CONCLUSIONS: Among patients with hypoplastic left heart syndrome, those with low SES have worse neurodevelopmental and functional status outcomes at 6 years. These differences were not explained by other patient or clinical characteristics.

Prevalence of Sleep-Disordered Breathing in a Cohort of Adult Fontan Patients.

Sleep-disordered breathing (SDB) is described in patients with acquired heart failure but its prevalence in adults with congenital heart disease is not well documented. It is likely that single-ventricle patients who have undergone Fontan palliation poorly tolerate the additional stress of SDB on their fragile cardiac physiology. This study sought to determine the prevalence of SDB in a population of adult Fontan patients. We performed a retrospective chart review of adult Fontan patients followed in our program (n = 55). All charts were evaluated for documentation of polysomnography (PSG). SDB was characterized as nocturnal hypoxemia without apnea or obstructive sleep apnea (OSA). Baseline demographics and clinical characteristics were documented, as were PSG findings including Apnea Hypopnea Index (AHI, events/hour) and desaturation events of > 5% from baseline saturation. Twenty-two of our 55 patients (40%) had undergone PSG (45% male, mean age 29 years). Of these patients, 17 had a PSG positive for SDB (77%). Nine patients (41%) had nocturnal hypoxemia without apnea, while 8 patients (36%) had a PSG positive for OSA. Lower baseline oxygen saturations, but not BMI or age, were statistically significantly correlated with higher AHI. SDB was present in the majority of our Fontan cohort who had undergone PSG, occurring at a higher prevalence than what is described in the general population, suggesting that screening should be considered for all Fontan patients. However, further study is needed to confirm this prevalence in a larger population, and if therapy has an impact on clinical outcomes.

Y-graft modification to the Fontan procedure: Increasingly balanced flow over time.

OBJECTIVE: The use of Y-grafts for Fontan completion is hypothesized to offer more balanced hepatic flow distribution (HFD) and decreased energy losses. The purpose of this study was to evaluate the hemodynamic performance of Y-grafts over time using serial cardiac magnetic resonance data and to compare their performance with extracardiac Fontan connections. METHODS: Ten Fontan patients with commercially available Y-graft connections and serial postoperative cardiac magnetic resonance data were included in this study. Patient-specific computational fluid dynamics simulations were used to estimate HFD and energy losses. Y-graft performance was compared with 3 extracardiac conduit Fontan groups (n = 10 for each) whose follow-up times straddle the Y-graft time points. RESULTS: Y-graft HFD became significantly more balanced over time (deviation from 50% decreased from 18% ± 14% to 8% ± 8%; P = .015). Total cavopulmonary connection resistance did not significantly change. Y-grafts at 3-year follow-up showed more balanced HFD than the extracardiac conduit groups at both the earlier and later follow-up times. Total cavopulmonary connection resistance was not significantly different between any Y-graft or extracardiac conduit group. CONCLUSIONS: Y-grafts showed significantly more balanced HFD over a 3-year follow-up without an increase in total cavopulmonary connection resistance, and therefore may be a valuable option for Fontan completion. Additional follow-up data at longer follow-up times are still needed to thoroughly characterize the potential advantages of Y-graft use.

Reintervention and survival in 1428 patients in the Australian and New Zealand Fontan Registry.

OBJECTIVE: Patients undergoing single-ventricle palliation have experienced significant improvement in survival in the recent era. However, a substantial proportion of these patients undergo reoperations. We performed a review of the Australia and New Zealand (ANZ) Fontan Registry to determine the overall reintervention and reoperative burden in these patients. METHODS: A retrospective longitudinal cohort study was performed using data from patients who underwent a Fontan operation between 1975 and 2016 from the ANZ Fontan Registry. The data obtained included Fontan operation, reinterventions and most recent follow-up status. We examined the type and timing of reinterventions and survival. RESULTS: Of the 1428 patients identified, 435 (30%) underwent at least one reintervention after the Fontan operation: 110 patients underwent early reintervention and 413 underwent late reinterventions. Excluding Fontan conversion and transplantation, 220 patients underwent at least one interventional procedure and 209 patients underwent at least one reoperation. Fenestration closure and pacemaker-related procedures were the most common catheter and surgical interventions, respectively. The cumulative incidence of reintervention following Fontan was 23%, 37% and 55% at 10, 20 and 30 years, respectively. Survival and freedom from failure were worse in patients requiring later reintervention after Fontan surgery (51% vs 83% and 42% vs 69%, respectively at 30 years, p<0.001). This difference persisted after excluding pacemaker-related procedures (p<0.001). Operative mortality for non-pacemaker late reoperations after Fontan was 6%. CONCLUSIONS: A substantial proportion of Fontan patients require further intervention to maintain effective single-ventricle circulation. Patients undergoing reoperation after Fontan have higher rates of mortality and failure, despite intervention.

Does pregnancy impact subsequent health outcomes in the maternal Fontan circulation?

BACKGROUND: Pregnancy in women with a Fontan circulation is understood to have a significantly increased risk of maternal morbidity and mortality. Potential longer-term effects on health outcomes remain unknown. AIM: Ascertainment of adverse events, post-delivery, in women from the Australia and New Zealand (ANZ) Fontan Registry. Comparator data from women without pregnancy and men was utilised. METHODOLOGY/RESULTS: Living adults were identified (n = 263 women, 280 men) with Registry analysis ascertaining cardiac morphology, type of Fontan procedure, pregnancy and significant cardiac adverse events. Data are described descriptively for morphology and as median (inter quartile range) for other variables. Thirty women reported 45 pregnancies beyond 20 weeks' gestation. Despite being older, these women, pre-delivery, had the lowest cardiac morbidity burden, compared to both male and female controls (OR = 3.25 (95% CI 1.44-8.35, p = 0.008 and OR = 2.59 (95% CI 1.14-6.70), p = 0.03). These differences were not present post-delivery. Median follow-up time post-delivery was 3.6 (1.2-7.5) years. Thrombus documentation and thromboembolic events were more common post-delivery, although differences were not significant in a propensity analysis model (adjusting for post-Fontan follow-up time, age and type of Fontan). There were no differences in arrhythmia, cardioversion, heart transplant, Fontan conversion or mortality between women post-delivery and either male or female controls. CONCLUSION: The apparent increase in thromboembolic events post pregnancy requires further investigation in a larger group of women over a longer period of follow-up time. This may have potential implications for post-partum anticoagulation, and for future health outcomes.

Competing risks analysis of time-to-event data for cardiovascular surgeons.

OBJECTIVES: The purpose of this article is to provide thoracic and cardiovascular surgeons with a practical step-by-step strategy to use in collaboration with a biostatistician for implementation of competing risks analysis when analyzing time-to-event data. Patients may have an outside event that precludes the event of interest. Traditional time-to-event analysis incorrectly assumes noninformative censoring in this scenario, which will lead to invalid results and conclusions. METHODS: The steps are (1) to determine whether competing risks analysis is needed, (2) to perform a nonparametric analysis, (3) to perform a model-based analysis, (4) to interpret the results, and (5) to compare to traditional survival analysis methods. We apply our approach to a hypothetical cardiovascular surgery example in determining the hazard of mortality after the stage 3 Fontan operation associated with prematurity among patients with hypoplastic left heart syndrome who had successful completion of Norwood stage 1 while incorporating mortality during the stage 2 bidirectional Glenn procedure as a competing risk. We apply nonparametric, semiparametric, and parametric methods. RESULTS: Although Cox regression establishes prematurity as a significant risk factor of mortality after stage 3 (hazard ratio, 1.26; 95% confidence interval, 1.06-1.50; P = .009), the competing risks analysis with the Fine-Gray model accounting for mortality after stage 2 determines that prematurity is not a significant predictor (hazard ratio, 1.07; 95% confidence interval, 0.90-1.27; P = .467). CONCLUSIONS: This article provides a practical step-by-step approach for making competing risks more accessible for cardiac surgeons collaborating with a biostatistician in analyzing and interpreting time-to-event data.

Surgery of single ventricles in humanitarian practice: surgery for which patients?

OBJECTIVES: To analyse the feasibility and effectiveness in humanitarian practice of surgical management of children with single-ventricle heart condition. METHODS: Retrospective study of children with a single ventricle, managed by the association Mécénat-Chirurgie Cardiaque since 1996, with long-term follow-up after their return home. RESULTS: Of the 138 children in our cohort, 119 had one or more surgeries (180 procedures): palliative surgery alone (systemic-pulmonary anastomosis or banding), 41; partial cavo-pulmonary connection, 47; total cavo-pulmonary connection (mean age 8.5 years), 31. Operative mortality is 5.5%. After a mean follow-up of 5.6 years, 18 children (13%) were lost to follow-up. Survival at 10 years is 79% in children receiving surgery (palliative only, 72%; partial cavo-pulmonary connection, 77%; total cavo-pulmonary connection, 97%) versus 29% in children with no surgical intervention. The prognosis is better for tricuspid atresia and double-inlet left ventricle (86 and 83% survival at 10 years) than for double-outlet right ventricle or complete atrio-ventricular canal defect (64 and 68% at 5 years). CONCLUSION: The surgery of the single ventricle in humanitarian medicine allows a very satisfactory survival after one or more surgeries tending towards a total cavo-pulmonary connection as soon as possible.

MRI Evaluation of Lymphatic Abnormalities in the Neck and Thorax after Fontan Surgery: Relationship with Outcome.

Background The Fontan operation is performed for surgical palliation of single ventricle physiology. This operation is usually preceded by a superior cavopulmonary connection (SCPC); lymphatic abnormalities after SCPC may be demonstrated at MRI and prior to the Fontan operation. Purpose To determine if the degree of neck and thoracic lymphatic abnormalities at T2-weighted MRI in patients after superior cavopulmonary connection (SCPC) correlated with surgical outcomes from the Fontan procedure. Materials and Methods Patients for whom SCPC was performed for palliation of single ventricle disease who underwent chest MRI between July 2012 and May 2015 at a single institution were retrospectively reviewed. T2-weighted images were scored as lymphatic type 1 (little or no T2 mediastinal and supraclavicular signal) to type 4 (T2 signal into both the mediastinum and the lung parenchyma). Fontan takedown, duration of post-Fontan hospitalization and pleural effusion, postoperative plastic bronchitis, need for transplant, and mortality were tabulated. The relationship between lymphatic type and clinical outcomes was evaluated by using analysis of variance (ANOVA), the Kruskal-Wallis H test, and the Fisher exact test. Results A total of 83 patients (mean age, 7.9 years ± 2.6) were evaluated. Among these 83 patients, 53 (64%) were classified with type 1 or 2 lymphatic abnormalities, 17 (20%) with type 3, and 12 (16%) with type 4. The rate of failure of Fontan completion was higher in patients with type 4 than in type 1 or 2 (54% vs 2%, respectively; P = .004). Need for cardiac transplant (one of 13 [8%]) and death (three of 13 [23%]) occurred only in type 4. Median postoperative length of stay was longer for patients with type 4 than for those with types 1 or 2 (29 days vs 9 days, respectively; P < .01). Conclusion Greater MRI-based severity of lymphatic abnormalities in patients prior to planned Fontan procedure was associated with failure of Fontan completion and longer postoperative stay. © RSNA, 2019 Online supplemental material is available for this article.

The clinical spectrum of Fontan-associated liver disease: results from a prospective multimodality screening cohort.

AIMS: Liver fibrosis and cirrhosis are a consequence of a Fontan physiology, and determine prognosis. It is unclear whether non-invasive assessment of liver pathology is helpful to provide clinically relevant information. The aims of this study were to assess the spectrum of Fontan-associated liver disease (FALD) and usefulness of non-invasive methods to assess biopsy confirmed liver fibrosis. METHODS AND RESULTS: Hepatic screening of consecutive patients consisted of a blood panel, ultrasonography, elastography, contrast-enhanced magnetic resonance imaging (MRI)/computed tomography (CT) scan, and liver biopsy (scored with Fontan specific fibrosis scores and collagen proportionate area; CPA). Fibrosis parameters, varices, ascites, and splenomegaly were measured on imaging. Thirty-eight of 49 referred patients (27 ± 6.6 years, 73.7% male) underwent the complete screening protocol. Liver fibrosis on biopsy was present in all patients, and classified as severe (Stages 3-4) in 68%. Median CPA was 22.5% (16.9-29.5) and correlated with individual fibrosis scores. ELF® and liver stiffness were elevated, but MELD-XI scores were low in all patients. Fibrosis severity neither correlated to ELF® and liver stiffness, nor to (semi-) quantitative fibrosis parameters on MRI/CT. Varices were present in 50% and hyperenhancing nodules in 25% of patients, both independent of fibrosis stage, but varices were associated with higher CPA values. CONCLUSION: The FALD spectrum includes both hepatic congestion and severe fibrosis, with signs of portal hypertension and hyperenhancing nodules as significant manifestations. Routine imaging, transient elastography, and serum biomarkers are unable to accurately assess severity of liver fibrosis in this cohort. Future research should focus on validating new diagnostic tools with biopsy as the reference standard.

Determination of the Frequency of Right and Left Internal Mammary Artery Embolization in Single Ventricle Patients: A Two-Center Study.

Embolization of systemic to pulmonary artery collaterals to regulate pulmonary arterial flow or pressure of the cavopulmonary circulation in patients with single ventricle is a common practice. The relative incidence and impact of this practice on future interventions like coronary artery bypass grafting is poorly understood. This study aims to evaluate the frequency and implications of internal mammary artery (IMA) embolization in the single ventricle (SV) population. A retrospective chart review was performed of SV patients who underwent cardiac catheterization before and after Fontan procedure between February 2007 and 2017. Data were collected from two tertiary care centers in the Midwest. Of the 304 SV patients, 62 (20.4%) underwent embolization of one or more IMAs, whereas 242 (79.6%) did not. The rate of embolization of IMA was 40.5% in one center and 14.5% in the second center. Among patients who received IMA embolization, left internal mammary artery (LIMA) embolization was seen in 6 (9.7%) patients. Majority of patients underwent either right internal mammary artery (RIMA) embolization (n = 25; 40.3%) or RIMA and LIMA embolization (n = 27; 43.5%). IMA embolization in SV patients is common. Embolizing IMAs early in life will likely eliminate a valuable graft option for coronary artery bypass grafting should it be required in the future care of these patients. Multi-center, prospective, nation-wide studies are warranted to examine coronary artery disease in the SV population and true frequency of IMA embolization. Delineation of which IMAs were embolized is a necessary in surgical and cardiac intervention national data, such as Society of Thoracic Surgeons (STS) database. All measures should be taken to preserve IMAs patency, if deemed feasible and safe.

Long-term outcomes of single-ventricle palliation for pulmonary atresia with intact ventricular septum: Fontan survivors remain at risk of late myocardial ischaemia and death.

OBJECTIVES: The specific outcomes of patients with pulmonary atresia with intact ventricular septum late after Fontan palliation are unknown. Patients with smaller right ventricles and myocardial sinusoids are known to have worse survival in the first years of life. Whether the potential for coronary ischaemia affects the long-term outcomes of these patients after Fontan palliation is still unknown. METHODS: All patients with pulmonary atresia with intact ventricular septum who underwent the Fontan procedure from 1984 to 2016 in Australia and New Zealand were identified, and preoperative, perioperative and follow-up data were collected. RESULTS: Late follow-up data were available for 120 patients. The median length of follow-up after the Fontan procedure was 9.1 years (interquartile range 4.2-15.4 years). Late death occurred in 9% of patients (11/120). Six were sudden, unexpected deaths; 4 of those occurred in patients known to have right ventricle-dependent coronary circulation (RVDCC). Those with RVDCC had a higher incidence of sudden death (4/20 vs 2/100; P = 0.007). RVDCC was associated with late death (P = 0.01) and the development of myocardial ischaemia after Fontan completion (P < 0.001). The 10-year survival rate was 77% (95% confidence interval 56-100%) for patients with RVDCC vs 96% (95% confidence interval 92-100%) for patients without RVDCC. CONCLUSIONS: Long-term survival of patients with pulmonary atresia with intact ventricular septum after the Fontan procedure is excellent, but patients with RVDCC remain susceptible to coronary ischaemia and sudden death. Closer surveillance and investigation for exercise-induced ischaemia may be necessary.

Five decades of the Fontan operation: A systematic review of international reports on outcomes after univentricular palliation.

Almost fifty years after its first clinical application, the modified Fontan operation is among the most frequently performed procedures in congenital heart disease surgery in children today. The objective of this review is to systematically summarize the international evolution of outcomes in regard to morbidity and mortality of patients with Fontan palliation. All studies published over the past five decades with more than 100 Fontan patients included were screened. In eligible studies, information concerning preoperative patients' characteristics, Fontan modifications employed, early mortality, long-term survival and frequency of relevant complications was extracted. Ultimately, thirty-one studies published by the largest surgical centers with an overall number of 9390 patients were included in this review. The extracardiac total cavopulmonary anastomosis is the most frequently used Fontan modification. Hemodynamic data demonstrate a rigorous overall adherence to suggested Fontan selection criteria. The analysis showed a clear trend toward improved early and long-term survival over the time period covered. Although inconsistently reported, severe complications such as arrhythmias, thromboembolic events and protein-losing enteropathy as well as reoperations and reinterventions were frequent. In conclusion, patients palliated for complex univentricular heart malformations nowadays benefit from the experience and technical developments of the past decades and have a significantly improved long-term prognosis. However, important issues concerning postoperative long-term morbidity and mortality are still unsolved and clear intrinsic limitations of the Fontan circulation are becoming evident as the population of Fontan patients ages.

Imagen hepática de pacientes con cirugía de Fontan / Liver imaging in patients with fontan circulation

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Imagen hepática de pacientes con cirugía de Fontan. Respuesta / Liver imaging in patients with fontan circulation. Response

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Unplanned cardiac reoperations and interventions during long-term follow-up after the Norwood procedure†.

OBJECTIVES: Patients with hypoplastic left heart syndrome or related malformations are predominantly treated with a 3-stage palliation. Anatomic or physiologic problems can lead to unplanned additional surgical or catheter interventions during single ventricle palliation. Changes in operative technique may have an impact on the reoperation rate. METHODS: Between 1997 and 2014, 317 Norwood procedures were performed at our centre. A retrospective single centre investigation was carried out concerning incidence, timing, indication and type of unplanned interstage cardiac reoperations and catheter interventions during follow-up of Norwood patients. Patients were followed from birth until the end of 2015. Cardiac procedures taking place at the time of the bidirectional Glenn or Fontan procedure or heart transplantation were not included. RESULTS: Sixty-five of the Norwood patients (20.5%) had at least one additional surgical cardiac procedure. Nine patients (2.8%) needed open procedures prior to the Norwood operations, 11.0% had procedures in the interstage I, 3.5% in the interstage II and 9.1% of the Fontan patients had cardiac reoperations afterwards. Main indications for unplanned surgery were insufficient pulmonary perfusion and tricuspid regurgitation. Eighty-one patients (25.6%) had at least one interstage catheter intervention during follow-up mainly addressing stenosis of the pulmonary arteries, aortic arch stenosis or aortopulmonary collaterals. CONCLUSIONS: The number of unplanned reoperations and interventions during staged palliation is remarkably high showing surgical peaks in the interstage I and after the Fontan procedure and an interventional peak in the interstage II. Thorough early information of the parents about possibly anticipated additional procedures is necessary.

Common atrioventricular valve failure during single ventricle palliation†.

OBJECTIVES: To determine the risk of atrioventricular valve failure (valve intervention or moderate or greater regurgitation) during the lifetime of patients with single ventricle physiology and common atrioventricular valve. METHODS: Patients' data were extracted from an existing bi-national, population based registry. A retrospective review of their medical records was undertaken to determine the incidence of atrioventricular valve repair/replacement or moderate or greater regurgitation. RESULTS: From a registry of 1468 Fontan survivors, 136 patients with common atrioventricular valve were identified. Complete echocardiographic follow-up was available for 114 patients. Median length of follow-up was 10.2 years (interquartile range 5-15 years). Twenty-five year survival and freedom from Fontan failure were 94% [95% confidence interval (CI), 88-100%] and 74% (95% CI, 64-87%), respectively. Twenty-eight patients underwent 24 initial repairs and 4 replacements. The 24 patients undergoing repair subsequently needed 6 re-repairs, 2 replacements and 8 had moderate or greater regurgitation at last follow-up. Four-year freedom from atrioventricular valve repair failure was 50% (95% CI, 34-75%). An additional 30 patients developed moderate or greater atrioventricular valve regurgitation (6 New York Heart Association ≥3, 10 Fontan failures, 0 deaths). Cumulative incidence of the composite endpoint of atrioventricular valve failure at 28 years was 62% (95% CI, 49-74%). CONCLUSIONS: Patients with single ventricle physiology and common atrioventricular valve experience a continuous decline in valve function. The majority of patients experience valve failure in the first 30 years of life.