Outcomes among adult survivors of total cavopulmonary Fontan palliation for single ventricle

Heart ◽  
2021 ◽  
pp. heartjnl-2021-319760
Author(s):  
Christopher Anigwe ◽  
Vidhushei Yogeswaran ◽  
Anita Moon-Grady ◽  
Sophie McAllister ◽  
Anika Aggarwal ◽  
...  
Keyword(s):  
Single Ventricle ◽  
Adverse Outcome ◽  
Survival Rates ◽  
Adult Survivors ◽  
Adverse Outcomes ◽  
Protein Losing Enteropathy ◽  
Tertiary Centre ◽  
Lateral Tunnel ◽  
Extracardiac Fontan ◽  
Fontan Palliation

ObjectiveThis tertiary centre study aims to identify factors associated with adverse outcomes in adult survivors with total cavopulmonary connection (TCPC) Fontan palliation for single ventricle.MethodsThis retrospective review of medical records identified adult (≥18 years) survivors of TCPC Fontan palliation who were followed at a single tertiary centre between 1 January 2000 and 1 July 2019. Adverse outcomes were defined as arrhythmia, pacemaker/implantable cardioverter defibrillator placement, liver cirrhosis, protein losing enteropathy, hospitalisation for heart failure, thromboembolic complication and/or death.Results160 adult TCPC patients met the inclusion criteria: 117 (73.1%) extracardiac and 43 (26.9%) lateral tunnel. The median (IQR) duration of follow-up since TCPC palliation was 17.5 (11.8–21.3) years. An adverse outcome occurred in 87 (54.4%) patients. Adverse outcome-free survival rates at 10, 20 and 25 years post TCPC were 89% (95% CI 82% to 93%), 60% (95% CI 50% to 69%) and 24% (95% CI 15% to 35%), respectively. On multivariate analysis, extracardiac Fontan (HR 2.21, 95% CI 1.20 to 4.08, p=0.011) was observed to be an independent risk factor for adverse outcomes after adjusting for age, race, morphology of the systemic ventricle and history of fenestration.ConclusionsIn this single-centre retrospective study of adult survivors of TCPC palliation, extracardiac Fontan was associated with an increased hazard for adverse outcomes. This finding could guide clinicians in developing risk modification strategies and management decisions to improve long-term outcomes in these patients.

scholarly journals A 45-year experience with the Fontan procedure: tachyarrhythmia, an important sign for adverse outcome

2019 ◽  
Vol 29 (3) ◽  
pp. 461-468 ◽  
Author(s):  
Friso M Rijnberg ◽  
Nico A Blom ◽  
Vladimir Sojak ◽  
Eline F Bruggemans ◽  
Irene M Kuipers ◽  
...  
Keyword(s):  
Adverse Outcome ◽  
Sinus Node ◽  
Fontan Procedure ◽  
Study Cohort ◽  
Protein Losing Enteropathy ◽  
Long Term Outcome ◽  
Extracardiac Conduit ◽  
Lateral Tunnel ◽  
Fontan Failure ◽  
Important Sign

Abstract OBJECTIVES This study aims to evaluate our 45-year experience with the Fontan procedure and to identify risk factors for late mortality and morbidity. METHODS Demographic, preoperative, perioperative and postoperative characteristics were retrospectively collected for all patients who underwent a Fontan procedure in a single centre between 1972 and 2016. RESULTS The study included 277 Fontan procedures (44 atriopulmonary connections, 28 Fontan-Björk, 42 lateral tunnels and 163 extracardiac conduits). Early failure occurred in 17 patients (6.1%). Median follow-up of the study cohort was 11.9 years (Q1–Q3 7.6–17.5). Longest survival estimates were 31% [95% confidence intervals (CI) 18–44%] at 35 years for atriopulmonary connection/Björk, 87% (95% CI 63–96%) at 20 years for lateral tunnel and 99% (95% CI 96–100%) at 15 years for extracardiac conduit. Estimated freedom from Fontan failure (death, heart transplant, take-down, protein-losing enteropathy, New York Heart Association III–IV) at 15 years was 65% (95% CI 52–76%) for atriopulmonary connection/Björk, 90% (95% CI 73–97%) for lateral tunnel and 90% (95% CI 82–94%) for extracardiac conduit. The development of tachyarrhythmia was an important predictor of Fontan failure [hazard ratio (HR) 2.6, 95% CI 1.2–5.8; P = 0.017], thromboembolic/neurological events (HR 3.6, 95% CI 1.4–9.4; P = 0.008) and pacemaker for sinus node dysfunction (HR 3.7, 95% CI 1.4–9.6; P = 0.008). Prolonged pleural effusion (>21 days) increased the risk of experiencing protein-losing enteropathy (HR 4.7, 95% CI 2.0–11.1; P < 0.001). CONCLUSIONS With modern techniques, survival and freedom from Fontan failure are good. However, Fontan patients remain subject to general attrition. Tachyarrhythmia is an important sign for an adverse outcome. Prevention and early treatment of tachyarrhythmia may, therefore, be paramount in improving the long-term outcome.

Successful childbirth in a Fontan with protein losing enteropathy

2019 ◽  
Vol 29 (4) ◽  
pp. 547-548
Author(s):  
Kristi N. Ryan ◽  
Michael R. Leonardi ◽  
Ty E. Hasselman
Keyword(s):  
Functional Status ◽  
Single Ventricle ◽  
Survival Rates ◽  
Protein Losing Enteropathy ◽  
Healthy Baby

AbstractAs survival rates and functional status of the adult single ventricle population have grown, some may become pregnant inadvertently or against our advice. The outcomes are often poor, being worse for the fetus/baby rather than the mother with a Fontan circuit. No reports of a successful delivery of a healthy baby to a Fontan mother with protein losing enteropathy were found in the literature. We present one such case.

Late outcomes after the Fontan procedure in patients with single ventricle: a meta-analysis

Heart ◽  
2018 ◽  
Vol 104 (18) ◽  
pp. 1508-1514 ◽  
Author(s):  
Ilana Schwartz ◽  
Courtney E McCracken ◽  
Christopher J Petit ◽  
Ritu Sachdeva
Keyword(s):  
Single Ventricle ◽  
Meta Analysis ◽  
Fontan Procedure ◽  
Time Intervals ◽  
Protein Losing Enteropathy ◽  
Extracardiac Conduit ◽  
Lateral Tunnel ◽  
Fontan Physiology ◽  
Pacemaker Insertion

ObjectiveMore patients with Fontan physiology are reaching adulthood. The purpose of this meta-analysis was to evaluate the late outcomes of patients palliated with Fontan procedure and to assess the risk factors for mortality.MethodsPubMed, Embase and Web of Science were queried to retrieve observational studies of survival in patients following the Fontan procedure with ≥5 years of follow-up. A random-effects model was used to determine pooled survival estimates at 5, 10 and 15 years. Meta-regression was used to assess potential moderators for death.ResultsNineteen articles with a total of 5859 patients were included. The weighted mean follow-up time was 8.94±2.64 years with overall 8.3% deaths and 1.5% transplants. Pooled survival estimates at 5, 10 and 15 years were 90.7%, 87.2% and 87.5%, respectively; and 88.4%, 85.7% and 84.1%, respectively, for studies that included all three time intervals (n=4). Earliest surgical year included in the study, proportion of atriopulmonary connections versus extracardiac conduit or lateral tunnel, and older age at Fontan were associated with higher rates of death, but ventricular morphology was not. Protein-losing enteropathy, reoperation and pacemaker insertion were reported in 2.1%, 5.6% and 6.8% patients, respectively.ConclusionsSurvival following the Fontan procedure has improved with time and is influenced by Fontan type and age at the time of Fontan. At a mean follow-up of 8.9 years, there was no significant association between survival and ventricular morphology, not taking into account the mortality prior to Fontan.

scholarly journals Frailty is Associated with an Increased Risk of Major Adverse Outcomes in Elderly Patients Following Surgical Treatment of Hip Fracture

2019 ◽  
Vol 9 (1) ◽  
Author(s):  
Chiu-Liang Chen ◽  
Chun-Min Chen ◽  
Chun-Yi Wang ◽  
Po-Wei Ko ◽  
Chung-Hwan Chen ◽  
...  
Keyword(s):  
Hip Fracture ◽  
Cox Regression ◽  
Adverse Outcome ◽  
Medical Center ◽  
Survival Rates ◽  
Adverse Outcomes ◽  
Emergency Department Visits ◽  
Clinical Frailty Scale ◽  
Increased Risk ◽  
Cohort Study Design

AbstractWe intended to explore the effect of level of frailty on, and relationship with, 1-, 3-, and 6-month postoperative emergency department visits, readmissions, and mortality. This is a prospective multicenter observational cohort study design. Patients aged 50 years or older treated for hip fracture (n = 245) were taken from Orthopedic wards in one medical center (n = 131) and one district hospital (n = 114) in Changhua County, Taiwan. Frailty was defined as measured by the validated Clinical Frailty Scale and categorized as robust, pre-frail, and frail. We used Kaplan-Meier analysis to estimate survival rates and Cox regression to estimate the risk of frailty associated with adverse outcomes. To examine the longitudinal associations between frailty and adverse outcome, the cross-lagged models were explored. Of the 245 patients, 55 (22.4%) were classified as frail, 113 (46.1%) as pre-frail, and 77 (31.4%) as robust. More cumulative events occurred for frail than for robust patients for each adverse outcome. Frailty has long-term effect on each adverse outcome after discharge, rather than the effect simultaneously. Targeting pre-frailty and frailty is essential for prevent adverse outcomes and improving the overall health of older adults after discharge for hip fracture.

Severe conduit stenosis in a patient with Fontan circulation with a Y-shaped Dacron conduit

2021 ◽  
pp. 1-3
Author(s):  
Daiji Takajo ◽  
Preetha L. Balakrishnan ◽  
Sanjeev Aggarwal
Keyword(s):  
Energy Loss ◽  
Polyethylene Terephthalate ◽  
Rare Complication ◽  
Flow Distribution ◽  
Fontan Circulation ◽  
Distribution Data ◽  
Extracardiac Fontan ◽  
Fontan Palliation

Abstract Conduit stenosis is a major, albeit rare, complication following the Fontan palliation. A single-baffle conduit with polytetrafluoroethylene is widely used for an extracardiac type Fontan palliation. A polyethylene terephthalate conduit (Dacron) is sometimes used for the conduit when more flexibility is required. A Y-shaped conduit is rarely used, but it may reduce the energy loss and achieve better hepatic flow distribution. Data on the long-term patency and complications when using a Y-shaped Dacron conduit is lacking. We report a case of a severely stenotic Y-shaped Dacron conduit in a patient who underwent extracardiac Fontan palliation.

Developing an adolescent and adult Fontan Management Programme

2021 ◽  
pp. 1-6
Author(s):  
Adam M. Lubert ◽  
Tarek Alsaied ◽  
Andrew T. Trout ◽  
Jonathan R. Dillman ◽  
Joseph J. Palermo ◽  
...  
Keyword(s):  
Single Ventricle ◽  
Fontan Procedure ◽  
Multiple Organ ◽  
Fontan Circulation ◽  
Management Programme ◽  
Adverse Outcomes ◽  
Cardiac Complications ◽  
Organ Systems ◽  
Adolescents And Adults ◽  
Care Models

Abstract Patients with single-ventricle CHD undergo a series of palliative surgeries that culminate in the Fontan procedure. While the Fontan procedure allows most patients to survive to adulthood, the Fontan circulation can eventually lead to multiple cardiac complications and multi-organ dysfunction. Care for adolescents and adults with a Fontan circulation has begun to transition from a primarily cardiac-focused model to care models, which are designed to monitor multiple organ systems, and using clues from this screening, identify patients who are at risk for adverse outcomes. The complexity of care required for these patients led our centre to develop a multidisciplinary Fontan Management Programme with the primary goals of earlier detection and treatment of complications through the development of a cohesive network of diverse medical subspecialists with Fontan expertise.

scholarly journals Prolonged Emergency Department Length of Stay as a Predictor of Adverse Outcomes in Patients with Intracranial Hemorrhage

2015 ◽  
Vol 2015 ◽  
pp. 1-5 ◽  
Author(s):  
Erica M. Jones ◽  
Amelia K. Boehme ◽  
Aimee Aysenne ◽  
Tiffany Chang ◽  
Karen C. Albright ◽  
...  
Keyword(s):  
Emergency Department ◽  
Length Of Stay ◽  
Adverse Outcome ◽  
Independent Predictor ◽  
Adverse Outcomes ◽  
Stroke Patients ◽  
Significant Independent Predictor ◽  
Prolonged Length ◽  
Stroke Center ◽  
Shift Change

Objectives. Extended time in the emergency department (ED) has been related to adverse outcomes among stroke patients. We examined the associations of ED nursing shift change (SC) and length of stay in the ED with outcomes in patients with intracerebral hemorrhage (ICH). Methods. Data were collected on all spontaneous ICH patients admitted to our stroke center from 7/1/08–6/30/12. Outcomes (frequency of pneumonia, modified Rankin Scale (mRS) score at discharge, NIHSS score at discharge, and mortality rate) were compared based on shift change experience and length of stay (LOS) dichotomized at 5 hours after arrival. Results. Of the 162 patients included, 60 (37.0%) were present in the ED during a SC. The frequency of pneumonia was similar in the two groups. Exposure to an ED SC was not a significant independent predictor of any outcome. LOS in the ED ≥5 hours was a significant independent predictor of discharge mRS 4–6 (OR 3.638, 95% CI 1.531–8.645, and P = 0.0034) and discharge NIHSS (OR 3.049, 95% CI 1.491–6.236, and P = 0.0023) but not death. Conclusions. Our study found no association between nursing SC and adverse outcome in patients with ICH but confirms the prior finding of worsened outcome after prolonged length of stay in the ED.

scholarly journals Routine surveillance of patients post Fontan palliation: lessons learnt from cardiac catheterisation

2021 ◽  
Vol 5 (1) ◽  
Author(s):  
Maria Victoria Ordonez ◽  
Giovanni Biglino ◽  
Radwa Bedair
Keyword(s):  
Clinical Management ◽  
Liver Stiffness ◽  
Adult Patients ◽  
Cardiac Catheterisation ◽  
Cross Sectional ◽  
Lateral Tunnel ◽  
Cross Sectional Imaging ◽  
Sectional Imaging ◽  
Diagnostic Catheter ◽  
Fontan Palliation

Abstract Background There is no consensus on the clinical utility of ‘routine’ diagnostic cardiac catheterisation in patients with Fontan palliation in the absence of symptoms or haemodynamic lesions. Objective We sought to evaluate whether diagnostic cardiac catheterisation for a variety of indications led to a change in the clinical management of patients with a Fontan circulation. Methods All adult patients (≥16 years) with Fontan palliation undergoing diagnostic cardiac catheterisation at our institution from 2016 to 2019 were included retrospectively. Patients undergoing electrophysiological studies were excluded as haemodynamic measurements were not taken. Routine cardiac catheterisation at our institution is considered in adult patients who have not had a diagnostic cardiac catheter for more than 5 years. Results Thirty-eight patients, mean age 27 ± 7 years, 60% NYHA I, 31% NYHA II, 8% NYHA III, at mean duration post Fontan of 20 ± 6 years, lateral tunnel (LT) n = 20, extracardiac (EC) n = 14 and atriopulmonary (AP) n = 4, underwent 41 diagnostic cardiac catheterisation procedures. Indication for cardiac catheterisation was as follows: haemodynamic lesion identified on cross-sectional imaging in 12; routine catheterisation in 9; cyanosis in 8; dyspnoea in 8; significant liver stiffness on ultrasound hepatic elastography in 2; and arrhythmia in 2. Of the 9 patients undergoing routine diagnostic catheterisation, 3 had not had any diagnostic catheterisation since their Fontan completion and, in the remaining six, the mean time lapsed since the last diagnostic catheter was 8 ± 3 years. The diagnostic catheterisation led to a recommended change in clinical management on 24 occasions (59%): catheter intervention in 17 (40%); surgery in 4 (10%); medication change in 3 (17%); and transplant referral in 2 (5%). The clinical indications that led to changes in clinical management were: cyanosis (8/8), dyspnoea (7/8), haemodynamic lesions on cross-sectional imaging (8/11) and arrhythmia (1/2). None of the 9 patients listed for routine diagnostic catheterisation or as a result of findings on ultrasound hepatic elastography had a recommended change in clinical management. Conclusion Diagnostic cardiac catheterisation frequently leads to changes in the clinical management of patients with Fontan palliation presenting with dyspnoea, cyanosis, and for further evaluation of potential haemodynamic lesions identified on cross-sectional imaging. Routine cardiac catheterisation in the absence of the above indications had limited impact on clinical management in our cohort.

Lumped parameter models for two-ventricle and healthy and failing extracardiac Fontan circulations

2021 ◽  
Author(s):  
Matthew G Doyle ◽  
Marina Chugunova ◽  
S Lucy Roche ◽  
James P Keener
Keyword(s):  
Single Ventricle ◽  
Left Ventricular ◽  
Sensitivity Analyses ◽  
Surgical Strategies ◽  
Lumped Parameter ◽  
Lumped Parameter Models ◽  
Fontan Failure ◽  
Existence Of Periodic Solutions ◽  
Mathematical Definitions ◽  
Extracardiac Fontan

Abstract Fontan circulations are surgical strategies to treat infants born with single ventricle physiology. Clinical and mathematical definitions of Fontan failure are lacking, and understanding is needed of parameters indicative of declining physiologies. Our objective is to develop lumped parameter models of two-ventricle and single-ventricle circulations. These models, their mathematical formulations and a proof of existence of periodic solutions are presented. Sensitivity analyses are performed to identify key parameters. Systemic venous and systolic left ventricular compliances and systemic capillary and pulmonary venous resistances are identified as key parameters. Our models serve as a framework to study the differences between two-ventricle and single-ventricle physiologies and healthy and failing Fontan circulations.

scholarly journals Management of colorectal polyp cancers

2012 ◽  
Vol 94 (8) ◽  
pp. 574-578 ◽  
Author(s):  
S Naqvi ◽  
S Burroughs ◽  
HS Chave ◽  
G Branagan
Keyword(s):  
Lymph Node ◽  
Lymph Node Metastases ◽  
Resection Margin ◽  
Adverse Outcome ◽  
Colorectal Polyps ◽  
Adverse Outcomes ◽  
Resection Margins ◽  
Residual Tumour ◽  
Clear Resection Margin ◽  
Node Metastases

INTRODUCTION Management of malignant colorectal polyps is controversial. The options are resection or surveillance. Resection margin status is accepted as an independent predictor of adverse outcome. However, the rate of adverse outcome in polyps with a resection margin of <1mm has not been investigated. METHODS A retrospective search of the pathology database was undertaken. All polyp cancers were included. A single histopathologist reviewed all of the included polyp cancers. Polyps were divided into three groups: clear resection margin, involved resection margin and unknown resection margin. Polyps were also analysed for tumour grade, morphology, Haggitt/Kikuchi level and lymphovascular invasion. Adverse outcome was defined as residual tumour at the polypectomy site and/or lymph node metastases in the surgical group and local or distant recurrence in the surveillance group. RESULTS Sixty-five polyps (34 male patients, mean age: 73 years, range: 50–94 years) were included. Forty-six had clear polyp resection margins; none had any adverse outcomes. Sixteen patients had involved polyp resection margins and twelve of these underwent surgery: seven had residual tumour and two of these patients had lymph node metastases. Four underwent surveillance, of whom two developed local recurrence. Three patients had resection margins on which the histopathologist was unable to comment. All patients with a clear resection margin had no adverse outcome regardless of other predictive factors. CONCLUSIONS Polyp cancers with clear resection margins, even those with <1mm clearance, can be treated safely with surveillance in our experience. Polyp cancers with unknown or involved resection margins should be treated surgically.

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