Fontan Takedown Research Articles

Outcomes of extracardiac Fontan operation: A single institution experience with 398 patients

ObjectiveThe study objective was to evaluate the outcomes of the extracardiac Fontan operation at a single institution. MethodsWe conducted a retrospective study of 398 patients from a single institution who underwent their initial extracardiac Fontan operation between 1997 and 2020. We determined the incidence of and risk factors for failure of the Fontan circulation, which includes death, Fontan takedown, heart transplantation, protein-losing enteropathy, plastic bronchitis, and functional status at the last follow-up. ResultsThe median follow-up time was 10.3 years (interquartile range, 6.4-14.6). The overall survival was 96% and 86% at 10 and 20 years after extracardiac Fontan operation, respectively. There were 6 early deaths (6/398, 1.5%) and 15 late deaths (15/398, 3.8%). Forty-nine patients (12.5%) developed failure of the Fontan circulation. Freedom from the failure of Fontan circulation was 88% at 10 years and 76% at 20 years. Risk factors for failure of the Fontan circulation were right ventricular dominance (hazard ratio, 4.7; P < .001; 95% CI, 2.1-10.5), aortic atresia (hazard ratio, 5.5; P < .001; 95% CI, 2.3-12.8), and elevated mean pulmonary artery pressure (hazard ratio, 2.3; P = .002; 95% CI, 1.2-6.7). ConclusionsRates of failure of the Fontan circulation are low after the contemporary extracardiac Fontan operation. Risk factors for failure of the extracardiac Fontan circulation include right ventricular dominance, aortic atresia, and elevated pulmonary artery pressures.

Vectorcardiography signs of a failing Fontan: Heart rate, PR interval, RtQRSvm, QRSvm and SPQRS-T angle as noninvasive markers of late Fontan complications and mortality

BackgroundLimited data exists on interpreting vectorcardiography (VCG) parameters in the Fontan population. ObjectiveThe purpose of this study was to demonstrate the associations between ECG/VCG parameters and Fontan failure (FF). Methods/results107 patients with a Fontan operation after 1990 and without significant ventricular pacing were included. FF and Fontan survival (FS) groups were compared. The average follow-up after Fontan operation was 11.8 years ±7.1 years. 14 patients had FF (13.1%) which was defined as having protein-losing-enteropathy (1.9%), plastic bronchitis (2.8%), Fontan takedown (1.9%), heart transplant (5.6%), NYHA class III-IV (2.8%) or death (0.9%). A 12‑lead ECG at last follow up or prior to FF was assessed for heart rate, PR interval, QRS duration, Qtc and left/right sided precordial measures (P-wave, QRS and T-wave vector magnitudes, spatial P-R and QRS-T angles). Transthoracic echocardiogram evaluated atrioventricular valve regurgitation and ventricular dysfunction at FF or last follow up. A cox multivariate regression analysis adjusted for LV dominance, ventricular dysfunction, HR, PR, QTc, Pvm, QRSvm, SPQRST-angle, RtPvm, RtQRSvm and RtTvm. Ventricular dysfunction, increased heart rate and prolonged PR interval were significantly associated to FF at the multivariate analysis. ROC analysis and Kaplan-meier analysis revealed an increased total mortality associated with a heart rate > 93 bpm, PR interval > 155 mv, QRSvm >1.91 mV, RtQRSvm >1.8 mV and SPQRST angle >92.3 mV with p values <0.001 to 0.018. ConclusionWe demonstrate the importance of ECG/VCG monitoring in the Fontan population and suggest specific indicators of late complications and mortality.

Alarming rate of liver cirrhosis after the small conduit extracardiac Fontan: A comparative analysis with the lateral tunnel

BackgroundThe association between the prevalence of cirrhosis and the types of Fontan operations remains unclear. MethodsWe conducted a retrospective chart review of 332 patients who underwent the Fontan procedure at our institution. Four patients who underwent the atriopulmonary connection Fontan were excluded from the analysis. Patients who had intracardiac-extracardiac conduit (126) between 1989 and 2021 were pooled with those having extracardiac conduit (ECC) (134). The 260 patients who underwent the ECC and the 68 patients who had the lateral tunnel (LT) Fontan constitute the core of the study. ResultsMedian age at the Fontan procedure was 23.7 months (interquartile range [IQR], 20.8-32.6) in the LT group, compared with 28.8 months (IQR, 24.6-39.5) in the ECC group (P < .01). The median follow-up was 14.8 years (IQR, 12.5-16.5) in the LT group and 7 years (IQR, 2.8-10.4) in the extracardiac conduit group. During the follow-up period, 3 patients (4.4%) with LT and 17 patients (6.5%) with ECC (11 patients with 16 mm or less conduit size) were diagnosed with cirrhosis. The prevalence of cirrhosis at 1, 5, 10, and 15 years was 0%, 0%, 0%, and 4.4% in the LT group, respectively, and 0%, 0.9%, 7.7%, and 29.8% in the ECC group (P < .01) Rates of mortality, Fontan revision, Fontan takedown, transplant, and complications were comparable between the 2 groups. ConclusionsThe extracardiac conduit Fontan seems to be associated with faster development of cirrhosis.

Association of patient-level characteristics with long-term outcomes after Fontan palliation: Rationale, design, and baseline characteristics of the Pediatric Cardiac Care Consortium Fontan cohort study

BackgroundThe Fontan operation is used to palliate single ventricle congenital heart defects (CHD) but poses significant morbidity and mortality risks. We present the design, planned analyses, and rationale for a long-term Fontan cohort study aiming to examine the association of patient characteristics at the time of Fontan with post-Fontan morbidity and mortality. Methods and ResultsWe used the Pediatric Cardiac Care Consortium (PCCC), a US-based, multicenter registry of pediatric cardiac surgeries to identify patients who underwent the Fontan procedure for single ventricle CHD between 1 and 21 years of age. The primary outcomes are in-hospital Fontan failure (death or takedown) and post-discharge mortality through 2022. A total of 1461 (males 62.1%) patients met eligibility criteria and were included in the analytical cohort. The median age at Fontan evaluation was 3.1 years (IQR: 2.4–4.3). While 95 patients experienced in-hospital Fontan failure (78 deaths and 17 Fontan takedown), 1366 (93.5%) survived to discharge with Fontan physiology and formed the long-term analysis cohort. Over a median follow-up of 21.2 years (IQR: 18.4-24.5) 184 post-discharge deaths occurred. Thirty-year post Fontan survival was 75.0% (95% CI: 72.3%-77.8%) for all Fontan types with higher rates for current techniques such as lateral tunnel and extracardiac conduit 77.1% (95% CI: 73.5-80.8). ConclusionThe PCCC Fontan study aims to identify predictors for post-Fontan morbidity and mortality, enabling risk- stratification and informing surveillance practices. Additionally, the study may guide therapeutic interventions aiming to optimize hemodynamics and enhance Fontan longevity for individual patients.

Does leaving native antegrade pulmonary blood flow at the time of the superior cavopulmonary connection impact long-term outcomes after the Fontan?

ObjectivesAntegrade pulmonary blood flow (APBF) may be left or eliminated at the time of the superior cavopulmonary connection (SCPC). Our aim was to assess the impact of leaving native APBF at the SCPC on long-term Fontan outcomes. MethodsIn the Australia and New Zealand Fontan Registry (1985-2021), 587 patients had pre-existing native APBF at the SCPC. At the SCPC, 302 patients had APBF eliminated (APBF−) and 285 patients had APBF maintained (APBF+). The incidence of Fontan failure (composite end point of Fontan takedown, transplant, plastic bronchitis, protein losing enteropathy and death) and atrioventricular (AV) valve repair/replacement post SCPC was compared between the 2 groups. ResultsSex, predominant-ventricle morphology, isomerism, primary diagnosis, and age/type of Fontan were similar between groups. APBF− versus APBF+ had a higher incidence of arch obstruction/coarctation (17% vs 7%) and previous pulmonary artery band (54% vs 45%) and a lower rate of Fontan fenestration (27% vs 41%). The risk of Fontan failure was similar between the 2 groups (hazard ratio [HR], 1.01; 95% confidence interval [CI], 0.58-1.78; P = .96). The risk of AV-valve repair/replacement was greater in APBF+ versus APBF– (HR, 2.32; CI, 1.13-4.75; P = .022). The risk of AV-valve repair/replacement remained after adjustment for arch obstruction/coarctation, previous pulmonary artery band and Fontan fenestration (HR, 2.27; CI, 1.07-4.81; P = .033). ConclusionsMaintaining APBF at the time of the SCPC does not impact the risk of Fontan failure but may increase the incidence of AV-valve repair and/or replacement post-SCPC.

Tachyarrhythmias after extracardiac conduit Fontan procedure in the long-term follow-up: may native anatomy of single ventricle predict their occurrence?

Abstract Funding Acknowledgements Type of funding sources: None. Background Arrhythmic complications, especially tachycardias, represent a common and important comorbidity during long-term follow up (FU) in patients with Fontan palliation for complexes congenital heart diseases (CHDs) with univentricular hearts. Surgical Fontan technique has evolved from direct atrio-pulmonary connection via the intracardiac lateral tunnel to the latest extracardiac conduit (ECC) procedure. Despite its shorter FU compared to previous techniques, the ECC variation is nowadays the main approach for many improvements, including lower incidence of arrhythmic complications (especially atrial arrhythmias). Purpose The aim of this study was to evaluate, during a long-term FU, the incidence of tachyarrhythmias and their correlation with the morphology of CHDs’ single ventricle native anatomy in a large cohort of patients with previous ECC Fontan procedure. Methods In this single centre observational cohort study, we retrospectively evaluated 351 patients treated with ECC Fontan palliation in our Institution between January 1987 and October 2017. Exclusion criteria were ECC Fontan take-down and a FU less than 5 years. CHD diagnosis, single ventricle native anatomy and surgical history were reviewed. Patients had cardiac examination once a year. We divided the overall study population in 2 groups based on unique ventricle's morphology: left-type (LV group) or right-type (RV group). Results A total of 303 patients [56% males; mean age at last FU 20 (IQR 15-28) years; 64.7% LV group] with ECC Fontan were enrolled. The mean FU was 16 (IQR 11-20, the longest 33) years. No sudden arrhythmic cardiac death occurred. Ten patients (6 of the LV group) underwent cardiac transplantation for Fontan failure. During FU, 34 patients of the LV (17.3%) and 22 of the RV group (20.2%) showed at least one tachyarrhythmia after a mean time of 12.1 and 12.9 years from ECC procedure, respectively. One patient (LV group) had one tachyarrhythmia in the early time period, &amp;lt;30 days from procedure. LV group showed a higher incidence of supraventricular tachyarrhythmias (SVTs) than RV group (68% vs 41% of total arrhythmias, p=0.08); in contrast, in RV group we found a higher number of ventricular tachyarrhythmias (VTs) compared to the other group (73% vs 41%, p=0.04). We observed both types of tachyarrhythmias (SVT and VT) in 3 patients of LV and 2 of RV group. Conclusions This study, with one of the largest cohort and longest FU after ECC Fontan, shows that ECC is associated with higher arrhythmic complications than previously reported, even though with longer latency period than other Fontan procedures. Moreover, our results suggest that patients with right-type single ventricle native anatomy are significantly more prone to develop VTs compared to those with left-type ventricle. This may reflect the drawbacks of the right ventricle physiology within Fontan circulation where the right ventricle acts for long time as the main pump of the circulatory system.

Long-term outcomes of patients with single ventricle who do not undergo Fontan palliation

BackgroundPatients with single ventricle (SV) without Fontan palliation are uncommon, and their long-term outcomes remain unclear. MethodsRetrospective study of 35 adult patients with SV without Fontan from two tertiary centers. Primary outcome was mortality. ResultsMedian age at first follow-up was 31 years (IQR: 20–40). Most common defect was double inlet left ventricle (34%), and 69% had left ventricular morphology. Patients were unoperated (46%), had systemic-to-pulmonary artery shunt (31%) or bidirectional cavopulmonary shunt (23%) as final palliation. Most common reasons for not progressing to Fontan palliation were pulmonary vascular disease (54%), patient refusal (17%), Fontan takedown (14%), and hypoplastic pulmonary arteries (11%). Baseline mean hemoglobin was 195 ± 29 g/L, mean O2 saturation 83 ± 6.9%, and 4 patients in NYHA Class III‒IV. After a mean follow-up of 10 ± 8.3 years, there were 9 deaths with heart failure being the leading cause (n = 6). Age-adjusted survival of these adult SV survivors was 73% and 53% at 40 and 50 years of age, respectively. Deceased patients more frequently had renal impairment (50% vs 0%) and QRS prolongation (75% vs 16%) at baseline (all p < 0.05). During follow-up, 40% had a new arrhythmia (atrial: n = 14, ventricular: n = 3), 34% had one or more hospitalizations for heart failure, and 17% had a stroke. A greater proportion of patients with pre-existing or new atrial/ventricular arrhythmia died compared to those without (42% vs 6%, p = 0.02). ConclusionsPatients with SV without Fontan have high mortality and a substantial burden of cardiovascular complications, particularly arrhythmia. QRS prolongation and renal impairment were associated with mortality.

Body Mass Index Trajectory and Outcome Post Fontan Procedure.

Background Patients with a single ventricle who experience early life growth failure suffer high morbidity and mortality in the perisurgical period. However, long-term implications of poor infant growth, as well as associations between body mass index (BMI) and outcome in adulthood, remain unclear. We aimed to model BMI trajectories of patients with a single ventricle undergoing a Fontan procedure to determine trajectory-based differences in baseline characteristics and long-term clinical outcomes. Methods and Results We performed a retrospective analysis of medical records from patients in the Australia and New Zealand Fontan Registry receiving treatment at the Royal Children's Hospital, The Children's Hospital at Westmead, Royal Melbourne Hospital, and Royal Prince Alfred Hospital from 1981 to 2018. BMI trajectories were modeled in 496 patients using latent class growth analysis from 0 to 6 months, 6 to 60 months, and 5 to 16 years. Trajectories were compared regarding long-term incidence of severe Fontan failure (defined as mortality, heart transplantation, Fontan takedown, or New York Heart Association class III/IV heart failure). Three trajectories were found for male and female subjects at each age group-lower, middle, higher. Subjects in the lower trajectory at 0 to 6 months were more likely to have an atriopulmonary Fontan and experienced increased mortality long term. No association was found between higher BMI trajectory, current BMI, and long-term outcome. Conclusions Poor growth in early life correlates with increased long-term severe Fontan failure. Delineation of distinct BMI trajectories can be used in larger and older cohorts to find optimal BMI targets for patient outcome.

Predictive factors contributing to prolonged recovery in patients after Fontan operation

BackgroundProlonged recovery is a severe issue in patients after Fontan operation. However, predictive factors related to this issue are not adequately evaluated. The present study aimed to investigate potential predictive factors which can predict Fontan postoperative recovery.MethodsWe retrospectively reviewed the perioperative medical records of patients with Fontan surgery between January 2015 and December 2018, and divided patients with > 75%ile cardiac intensive care unit stay into prolonged recovery group. The others were assigned to standard recovery group. Patients that died or underwent a Fontan takedown were excluded. Statistical analysis was performed to compare data difference of the two groups.Results282/307 cases fulfilled the inclusion criteria. Seventy patients were considered in prolonged recovery and 212 patients were defined as standard recovery. Pre- and intra-operative data showed a higher incidence of heterotaxy syndrome, longer cardiopulmonary bypass and aortic cross-clamp time in the prolonged recovery group. Postoperative information analysis displayed that ventilation time, oxygen index after extubation, hemodynamic data, inotropic score (IS), drainage volume, volume resuscitation, pulmonary hypertension (PH) treatment, and surgical reintervention were significantly different between the two groups. Higher IS postoperatively, and PH treatment and higher fluid resuscitation within two days were independent predictive factors for prolonged recovery in our multivariate model. C-statistic model showed a high predictive ability in prolonged recovery by using the three factors.ConclusionsVentilation time, higher IS in postoperative day, and PH treatment and higher fluid resuscitation within two days were independent risk factors and have a high predictability for Fontan prolonged recovery.

Fontan failure: phenotypes, evaluation, management, and future directions.

Management of "failing" and "failed" Fontan circulation, particularly the indications, timing, and type of re-intervention, currently remains nebulous. Factors contributing to pathogenesis and mortality following Fontan procedure differ between children and adults. Since organ systems in individual patients are affected differently, we searched the extant literature for a "failing" and "failed" Fontan reviewing the clinical phenotypes, diagnostic modalities, pharmacological, non-pharmacological, and surgical techniques employed, and their outcomes. A total of 410 investigations were synthesised. Although proper candidate selection, thoughtful technical modifications, timely deployment of mechanical support devices, tissue-engineered conduits, and Fontan takedown have decreased the peri-operative mortality from 9 to 15% and 1 to 3% per cent in recent series, pernicious changes in organ function are causing long-term patient attrition. In the setting of a failed Fontan circulation, literature documents three surgical options: Fontan revision, Fontan conversion, or cardiac transplantation. The reported morbidity of 25% and mortality of 8-10% among Fontan conversion continue to improve in select institutions. While operative mortality following cardiac transplantation for Fontan failure is 30% higher than for other CHDs, there is no difference in long-term survival with actuarial 10-year survival of around 54%. Mechanical circulatory assistance, stem cells, and tissue-engineered Fontan conduit for destination therapy or as a bridge to transplantation are in infancy for failing Fontan circulation. An individualised management strategy according to clinical phenotypes may delay the organ damage in patients with a failing Fontan circulation. At present, cardiac transplantation remains the last stage of palliation with gradually improving outcomes.

Impact of Fontan Fenestration on Long-Term Outcomes: A Propensity Score-Matched Analysis.

BackgroundThe long‐term impact of fenestration at the time of Fontan operation remains unclear. We aimed to review the early and long‐term impact of Fontan fenestration in the Australia and New Zealand cohort.Methods and ResultsWe reviewed 1443 patients (621 fenestrated, 822 nonfenestrated) from the Australia and New Zealand Fontan registry. Data were collected on preoperative demographics, operative details, and follow‐up. Propensity‐score matching was performed to account for the various preoperative and operative differences and risk factors. Primary outcomes were survival and freedom from failure. Median follow‐up was 10.6 years. After propensity‐score matching (407 matched pairs), there was no difference in survival (87% versus 90% at 20 years; P=0.16) or freedom from failure (73% versus 80% at 20 years; P=0.10) between patients with and without fenestration, respectively. Although patients with fenestration had longer bypass and cross‐clamp times (P<0.001), there was no difference in hospital length of stay or prolonged pleural effusions (P=0.80 and P=0.46, respectively). Freedom from systemic and Fontan circuit thromboembolism was higher in the nonfenestrated group (89%; 95% CI, 88%–95%) than the fenestrated group (84%; 95% CI, 77%–89%; P=0.03). There was no difference in incidence of plastic bronchitis, protein‐losing enteropathy, New York Heart Association Class III/IV symptoms, or Fontan takedown.ConclusionsIn the propensity score–matched analysis we have demonstrated no difference in long‐term survival or freedom from Fontan failure in patients with and without fenestration. There was a higher incidence of long‐term thromboembolic events in patients with fenestration. Overall, it appears that fenestration in Fontan circulation does not bring long‐term benefits.

Preoperative N-terminal pro-brain natriuretic peptide is associated with Fontan outcomes

ObjectiveThe role of preoperative N-terminal pro-brain natriuretic peptide level in patient outcomes after the Fontan operation remains unclear. MethodsThe medical records of all patients who underwent their first Fontan operation from June 2011 to October 2019 in our tertiary referral pediatric cardiac center were retrospectively reviewed. Preoperative hemodynamic factors and N-terminal pro-brain natriuretic peptide were analyzed to test the association of mortality and morbidity. ResultsWe enrolled 110 patients (men/women 62/48; median age, 4.1 [3.4, 5.8] years; median follow-up period, 4.28 [2.31, 6.71] years). Almost all operations were extracardiac conduits (98.2%). Primary outcomes of death, Fontan takedown, and heart transplantation were observed in 9 patients (8.2%). Abnormal ventricular contractility, elevated preoperative pulmonary artery pressure, high pulmonary vascular resistance index, and high log10 N-terminal pro-brain natriuretic peptide level were associated with poor outcomes. Secondary outcomes: atrioventricular valve regurgitation moderate or greater, elevated pulmonary artery pressure, high pulmonary vascular resistance index, and high log10 N-terminal pro-brain natriuretic peptide level were associated with rehospitalization due to heart failure. Multivariable Cox regression analysis revealed that log10 N-terminal pro-brain natriuretic peptide was the only significant predictor of all primary and secondary outcomes. A scoring system including factors of pulmonary artery pressure, pulmonary vascular resistance index, and N-terminal pro-brain natriuretic peptide was established, and the risk stratification is associated with outcomes after the Fontan operation. ConclusionsHigh preoperative N-terminal pro-brain natriuretic peptide was associated with poor outcomes after the Fontan operation.

Abstract 9701: Hemodynamic Parameters Deteriorate Over Time, and Relate to Adverse Clinical Outcomes, in Children and Adults with Fontan Circulation

Introduction: In patients with univentricular heart disease, Fontan palliation allows for improved survival. However, Fontan circulatory function deteriorates over time, marked by the onset of functional limitations, morbidities, and mortality. Invasive surveillance offers insights into hemodynamic conditions and may facilitate identification of at-risk patients. Objective: To characterize longitudinal changes in invasive Fontan hemodynamics and evaluate for associations with adverse clinical outcomes. Methods: Single-center retrospective study of serial invasive hemodynamics (≥2 catheterizations) in Fontan patients from 2006-2020. The first and last procedures per patient were analyzed for Fontan pressure, ventricular end diastolic pressure (EDP), cardiac output (CO), systemic (SVR), pulmonary (PVR) and total vascular resistance (TVR). The composite clinical endpoint included death, heart transplant or listing, Fontan takedown, protein losing enteropathy, plastic bronchitis, admission for arrhythmia or congestive heart failure and Fontan revision. Results: Serial hemodynamic evaluations were analyzed in 71 Fontan patients, with first catheterization occurring at median age of 7.1 years (IQR 4.1, 14) and 8.4 years (4.7, 12.3) between assessments. The most common diagnosis was hypoplastic left heart syndrome (45%). Fontan pressure, CO and PVR did not significantly change between assessments, whereas EDP increased by 1.9±3.6 mmHg ( p &lt;0.001). The annualized rate of change in EDP was 0.2±0.14 mmHg/year. SVR (2.0±7.5 U x m 2 , p =0.012) and TVR (2.16 ±7.8 U x m 2 , p =0.031) also increased significantly between assessments. The clinical endpoint was met in 43 (61%) patients and was associated with a greater change in transpulmonary gradient (0.13±1.9 vs -0.87±2.0 mmHg, p =0.046) and Fontan pressure (1.1±5.4 vs -0.7±2.7 mmHg, p =0.066) but not EDP, CO, SVR, PVR or TVR. Conclusion: Over a relatively brief time period, this single-center cohort of Fontan patients demonstrated clinically significant longitudinal increases in EDP, SVR and TVR. Adverse clinical outcomes were common and associated with greater hemodynamic deterioration between assessments. Serial interrogation of Fontan hemodynamics is likely to prove clinically useful.

Screening for Fontan-associated liver disease: are we doing enough?

Abstract Introduction Fontan-associated Liver Disease (FALD) is an extracardiac complication with a substantial disease burden that can reduce quality of life, increase healthcare demand and result in premature mortality. Effective FALD screening is paramount in preventing late-stage complications. Purpose To assess the adherence of a tertiary adult congenital heart disease referral centre to FALD screening. Methods 1. A total of 256 Fontan patients reviewed at our centre between 1st January 1977 and 31st December 2020 were identified from the congenital cardiac surgical database and the adult congenital heart disease clinical nurse specialist database. 2. The following patients were excluded: aged under 18 years on 31st December 2020 (103 cases), out of region or loss to follow-up (35 cases), death (30 cases), cardiac or liver transplantation (4 cases) and Fontan takedown (3 cases.) The mean endpoint (death, transplant or Fontan takedown) was 30 years. 3. The final study population included 81 patients. 4. Data was collected by review of clinic letters, blood test and imaging results from electronic records between 1st January 2018 and 31st December 2020. 5. The data were analysed to determine whether the screening recommendations from “Fontan-Associated Liver Disease: Proceedings from the American College of Cardiology Stakeholders Meeting, October 1 to 2, 2015, Washington DC” (ACC Stakeholders Meeting) were performed. Results The ACC Stakeholders Meeting recommended history taking and physical examination at least once a year with Fontan blood tests (full blood count, urea and electrolytes, liver function tests, alpha-fetoprotein and international normalized ratio) and imaging (abdominal ultrasound, computerised tomography or magnetic resonance imaging) at least once every three years. Of the 81 patients (47 male, mean age 27±8.3 years), the mean age of Fontan completion was 6.6±5.3 years. The mean follow-up period since Fontan completion was 20±8.1 years. History taking and physical examination were documented in 84% (68/81) and 79% (64/81) of patients respectively. 60% (59/81) had a full set of Fontan blood tests taken which increased to 73% (49/81) if alpha-fetoprotein was excluded while imaging was performed in 80% (65/81) of cases. Overall, 46% (37/81) cases had abnormal Fontan blood test or imaging. Hepatology referral was made in only 21% (17/81) of cases and MELD-XI score was not documented. Conclusions FALD screening tests were frequently underperformed compared to international guidance. Adherence may be improved by 1) promoting a Fontan blood test bundle; 2) implementing a standardised letter template which includes an investigation date list; 3) advocating MELD-XI score; and 4) exploring a joint cardiac and hepatology Fontan clinic. Funding Acknowledgement Type of funding sources: None. Figure 1Figure 2

Fontan operation at less than 3 years of age is not a risk factor for long-term failure.

The age at which the Fontan operation is performed varies globally. Over the last decade, the median age of patients having the Fontan in Australia and New Zealand has been 4.6 years, including 6% of patients younger than 3 years. Long-term outcomes of an early Fontan operation are unclear and are described in this study. Patients from the Australian and New Zealand Fontan Registry were grouped by age at Fontan. A Fontan before 3 years (early Fontan) was compared to the combined second and third quartiles by age at surgery in the Registry (3.6-6.1 years; control). Outcomes included Fontan failure (death, transplant, New York Heart Association functional group III/IV heart failure, Fontan takedown or conversion, protein losing enteropathy and plastic bronchitis), arrhythmias, thromboembolism and reinterventions. A total of 191 patients who had early Fontan operations were compared to 781 controls. Profound or progressive cyanosis was noted more frequently in the early than in the control group (63% vs 23%; P < 0.001). The early group was followed up for a median 22.1 years. The incidence of long-term failure was similar between the 2 groups (early, 1.08 failures per 100 patient-years of follow-up vs control, 0.99; log-rank P = 0.79). Adjusted for risk factors, early age at Fontan was not a risk factor for long-term failure [hazard ratio (HR) 1.16, 95% confidence interval (CI) 0.77-1.76; P = 0.48], new-onset arrhythmia (HR 0.93, 95% CI 0.63-1.39; P = 0.73), thromboembolism (HR 0.50, 95% CI 0.28-0.91; P = 0.024) or reintervention (HR 1.08, 95% CI 0.80-1.45; P = 0.62). Having the Fontan operation at an early age was not a risk factor for short- or long-term adverse outcomes in our cohort.

Long-term outcomes following Fontan takedown in Australia and New Zealand

ObjectiveFontan takedown remains an option for the management of Fontan failure. We sought to evaluate early and late outcomes after Fontan takedown. MethodsThe Australia and New Zealand Fontan Registry was interrogated to identify all patients who had a Fontan takedown. ResultsOver a 43-year study period (1975-2018), 36 of 1540 (2.3%) had a Fontan takedown. The median age at takedown was 5.1 years (interquartile range [IQR], 3.7, 7.0). Nine (25%) patients had a takedown within 48 hours, 6 (16%) between 2 days and 3 weeks, 14 (39%) between 3 weeks and 6 months, whereas 7 (19%) had a late takedown (>6 months). Median interval to takedown was 26 days (IQR, 1.5, 127.5). Sixteen (44%) patients died at a median of 57.5 days (IQR, 21.8, 76.8). The greatest mortality occurred between 3 weeks and 6 months (<2 days: 1/9, 11%; 2 days to 3 weeks: 2/6, 33%; 3 weeks to 6 months: 11/14, 79%; >6 months: 2/7, 28%; P = .007). At median follow-up of 9.4 years (IQR, 4.5, 15.3), 11 (31%) patients were alive with an intermediate circulation (10 in New York Heart Association class I/II). Five (14%) patients underwent a successful second Fontan. Freedom from death/transplant after Fontan takedown was 59%, 56%, and 52% at 1, 5, and 10 years, respectively. ConclusionsThe incidence of Fontan takedown is low, but mortality is high. The majority of takedowns occurred within 6 months. Mortality was lowest when takedown occurred <2 days and highest between 3 weeks and 6 months. A second Fontan is possible in a small proportion of survivors.

24-Year Results of Nonfenestrated Extracardiac Fontan Including Fontan Conversions

There is active debate regarding the optimal method of Fontan palliation. In light of this, we reviewed our experience with the nonfenestrated extracardiac Fontan including Fontan conversion. We performed a retrospective review of all nonfenestrated extracardiac Fontan and Fontan conversion operations at our institution from December 1, 1994 to December 31, 2018. Standard demographic data were collected, including underlying anatomy, preoperative ventricular and valvular function, operative details, perioperative data, and clinical outcomes. Statistical analysis included comparison between initial extracardiac Fontan patients and Fontan conversions, as well as analysis of risk factors for adverse outcomes. There were 341 patients with an overall operative mortality of 4 patients (1.2%). Of these, 193 were extracardiac nonfenestrated Fontan completion operations (57%) and 148 were Fontan conversions (43%). Length of stay was 11 days (SD, 6 days) with ventilator duration of 28 hours (SD, 26 hours). Six of the completion Fontan patients (3%) required Fontan takedown at a median time of 2.5 days. Upon multivariable analysis, risk factors associated with adverse events (mortality, Fontan takedown, and/or transplant) included increased cardiopulmonary bypass time, preoperative decreased dominant ventricular function, and length of stay. Kaplan-Meier curves demonstrated that mild or greater preoperative ventricular dysfunction decreased survival as well as freedom from adverse events for both initial extracardiac Fontan and Fontan conversion patients. Over the past 24 years, our strategy of nonfenestrated extracardiac Fontan has achieved low operative mortality for both initial Fontan and Fontan conversion. There is a steady attrition of Fontan patients to cardiac transplantation; the key risk factor is preoperative ventricular dysfunction.

Fontan completion during winter season is not associated with higher mortality or morbidity in the early post-operative period.

The aim of our study was to compare post-operative outcome after total cavopulmonary connection between patients operated during winter and summer season. We retrospectively studied 211 patients who underwent extracardiac total cavopulmonary connection completion at our institution between 1995 and 2015 (median age 4 (1-42) years). Seventy (33%) patients were operated during winter (November to March) and 141 (67%) patients during summer season (April to October). Patients operated during winter and summer season showed no difference in early mortality (7% versus 5%, p = 0.52) and severe morbidity like need for early Fontan takedown (1% versus 1%, p = 0.99) and need for mechanical circulatory support (9% versus 4%, p = 0.12). The post-operative course and haemodynamic outcome were comparable between both groups of patients (ICU (4 versus 3 days, p = 0.44) and hospital stay (15 versus 14 days, p = 0.28), prolonged pleural effusions (36% versus 31%, p = 0.51), need for dialysis (16% versus 11%, p = 0.37), ascites (37% versus 33%, p = 0.52), supraventricular tachyarrhythmia (16% versus 13%, p = 0.56) and chylothorax (26% versus 16%, p = 0.12), change of antibiotic treatment (47% versus 36%, p = 0.06), prolonged inotropic support (24% versus 14%, p = 0.05), intubation time (15 versus 12 hours, p = 0.33), and incidence of fast-track extubation (11% versus 22%, p = 0.06). Outcomes after total cavopulmonary connection completion during winter and summer season were comparably related to mortality, severe morbidity, or longer hospital stay in the early post-operative period. These results suggest that total cavopulmonary connection completion during winter season is as safe as during summer season.

Prevalence and Cause of Early Fontan Complications: Does the Lymphatic Circulation Play a Role?

BackgroundRecent studies suggest that lymphatic congestion plays a role in development of late Fontan complications, such as protein‐losing enteropathy. However, the role of the lymphatic circulation in early post‐Fontan outcomes is not well defined.Methods and ResultsThis was a retrospective, single‐center study of patients undergoing first‐time Fontan completion from 2012 to 2017. The primary outcome was early Fontan complication ≤6 months after surgery, a composite of death, Fontan takedown, extracorporeal membrane oxygenation, chest tube drainage >14 days, cardiac catheterization, readmission, or transplant. Complication causes were assigned to 1 of 4 groups: (1) Fontan circuit obstruction, (2) ventricular dysfunction or atrioventricular valve regurgitation, (3) persistent pleural effusions in the absence of Fontan obstruction or ventricular dysfunction, and (4) chylothorax or plastic bronchitis. T2‐weighted magnetic resonance imaging sequences were used to assess for lymphatic perfusion abnormality. The cohort consisted of 238 patients. Fifty‐eight (24%) developed early complications: 20 of 58 (34.5%) in group 1, 8 of 58 (14%) in group 2, 18 of 58 (31%) in group 3, and 12 of 58 (20%) in group 4. Preoperative T2 imaging was available for 126 (53%) patients. Patients with high‐grade lymphatic abnormalities had 6 times greater odds of developing early complications (P=0.001).ConclusionsThere is substantial morbidity in the early post‐Fontan period. Half of those who developed early complications had lymphatic failure or persistent effusions unrelated to structural or functional abnormalities. Preoperative T2 imaging demonstrated that patients with higher‐grade lymphatic perfusion abnormalities were significantly more likely to develop early complications. This has implications for risk stratification and optimization of patients before Fontan palliation.

Fontan-associated nephropathy: Predictors and outcomes

BackgroundNephropathy is a known complication of the Fontan circulation, but its determinants have not been identified and patient outcomes are also still unknown. MethodsThe Australia and New Zealand Fontan Registry was used to identify those who underwent Fontan operation before and survived beyond 16-years-old with an intact Fontan circulation. Serum creatinine values were collected for each patient between 16 and 25 years and at recent follow-up. The Modification of Diet in Renal Disease (MDRD) equation was used to calculate eGFR. Patient outcomes were obtained from the Registry. Fontan failure was defined as death, transplantation, plastic bronchitis, protein losing enteropathy, Fontan takedown and NYHA class III-IV. ResultsSerum creatinine measurements were available for 328 patients. Renal dysfunction was defined as eGFR <90 mL/min/1.72m2. Renal dysfunction was present in 67/328 (20%) and 3/328 (1%) patients had an eGFR <60 mL/min/1.72m2. The 10-year survival and 10-year freedom from death and transplantation were the same, 96% (95% CI: 0.9–1) for those with renal dysfunction, and 89% (0.83–0.95; p = 0.1) and 87% (95% CI: 0.81–0.94; p = 0.05) for patients without dysfunction. The 10-year freedom from failure were also similar, 83% (95% CI: 0.70–0.97) for those without renal dysfunction vs 80% (95% CI: 0.74–0.89; p = 0.84). There was no change in mean eGFR for the renal dysfunction group over a mean of 8 ± 5.5 years. ConclusionBy the time they reach adulthood, 20% of patients with a Fontan circulation have renal dysfunction by eGFR calculation. Over the course of one decade, Fontan-associated nephropathy appears well tolerated.