Abstract 16822: Growth Curves Describing a Single Ventricle Population After Fontan Procedure

Circulation ◽  
2020 ◽  
Vol 142 (Suppl_3) ◽  
Author(s):  
Bethany Runkel ◽  
Vincent Staggs ◽  
Chelsea Hosey Cojocari ◽  
Jonathan B Wagner
Keyword(s):  
Single Ventricle ◽  
Multiple Stressors ◽  
Pediatric Population ◽  
Fontan Procedure ◽  
Fontan Operation ◽  
Growth Patterns ◽  
Growth Charts ◽  
Genetic Syndrome ◽  
Adiposity Rebound ◽  
Extracardiac Conduit

Introduction: Complex single ventricle congenital heart disease (CHD) patients are subject to multiple stressors early in life that affect somatic growth. Pre-Fontan growth patterns are well-described, but post-Fontan growth has not been extensively studied in the current era. We sought to describe post-Fontan growth patterns by creating sex-specific body mass index (BMI)-for-age curves. Hypothesis: There is no difference in the growth of post-Fontan pediatric patients when compared to published normative data. Methods: A single-center, preexisting database was retrospectively queried for all patients who underwent Fontan procedure between 2006 and 2018. Patients with a genetic syndrome, a primary endocrine disorder, or significant prematurity were excluded. BMI-for-age curves were created for both male and female patients using anthropometric data extracted from the electronic medical record. Curves were then visually compared with Centers for Disease Control and Prevention (CDC) growth charts. Results: Of 227 patients who underwent primary Fontan operation, 37 were excluded. Of the remaining 190 patients, 59% were male. All had an extracardiac conduit, half had right-ventricular dominant CHD, and 15% had a Fontan fenestration. Median age and weight at surgery were 3.9 years and 15 kilograms, respectively. BMI curves were similar in appearance to CDC BMI-for-age growth charts, with adiposity rebound at age 6. BMI increased more rapidly in teen boys compared with girls, and a total of five patients (2.6%) had BMI values greater than 25 kg/m 2 at 16 years of age. Conclusion: Post-Fontan patients at our institution demonstrate BMI-for-age patterns similar to those of the general population during childhood and early adolescence, with adiposity rebound occurring at a typical age. Though excess weight gain does not completely spare patients with single ventricle CHD, overweight status appears to be less common than in the general pediatric population.

scholarly journals Factors Related to Pleural Effusions After Fontan Procedure in the Era of Fenestration

Circulation ◽  
2001 ◽  
Vol 104 (suppl_1) ◽  
Author(s):  
Raymond T. Fedderly ◽  
Beth N. Whitstone ◽  
Stephanie J. Frisbee ◽  
James S. Tweddell ◽  
S. Bert Litwin
Keyword(s):  
Hospital Stay ◽  
Chest Tube ◽  
Single Ventricle ◽  
Fontan Procedure ◽  
Fontan Operation ◽  
Univariate Analysis ◽  
Ordinary Least Squares ◽  
Pleural Effusions ◽  
Tube Output ◽  
Chest Tube Output

Background Significant pleural effusions after the Fontan operation prolong hospital stay, may increase the risk of infection, and may necessitate a pleurodesis procedure. Methods and Results From February 1991 to April 2000, 98 consecutive patients under the age of 18 years underwent the fenestrated Fontan procedure at Children’s Hospital of Wisconsin. Ninety-four patients who survived at least 30 days after surgery were retrospectively evaluated for the following factors: age, ventricular morphology (right single ventricle, left single ventricle [RV/LV]), fenestration open (FO) or closed (FC) at end of operation, intracardiac Fontan (IF) or extracardiac Fontan (EF), days with chest tube output per day >5, 10, and/or 20 mL · kg −1 · d −1 (CTO5, CTO10, and CTO20, respectively), need for pleurodesis, length of hospital stay (LOS), operation during winter respiratory viral season of November through March (ReVS+, ReVS−), and pre-Fontan mean pulmonary artery pressure (PAP) and pulmonary vascular resistance (PVR). In univariate analysis, the ReVS+ patients had prolonged LOS, greater chest tube output, and more pleurodesis ( P <0.05), and PAP was related to CTO5 and CTO10 but not to CTO20 or LOS. No significant differences were found in LOS, CTO5, CTO10, CTO20, and need for pleurodesis between patients in RV/LV, FO/FC, IF/EF, or PVR groups. Patients <4 years of age had more instances of CTO20 ( P <0.05). When we used ordinary least squares regression analysis with age, FO or FC, RV or LV, PAP, and ReVS+ or ReVS− to predict each of CTO5, CTO10, CTO20, and LOS, only ReVS+ or ReVS− and age were statistically significant in all models. Conclusions Use of the Fontan procedure during the respiratory viral season appeared to be related to significant, prolonged pleural effusions and longer hospitalizations.

Computational Model for the Study of Fontan Circulation

2018 ◽  
pp. 587-606
Author(s):  
Alejandro Talaminos-Barroso ◽  
Laura María Roa-Romero ◽  
Javier Reina-Tosina
Keyword(s):  
Computational Model ◽  
Cardiovascular System ◽  
Fontan Procedure ◽  
Fontan Operation ◽  
Univentricular Heart ◽  
Physiological Basis ◽  
Hemodynamic Variables ◽  
Extracardiac Conduit ◽  
Lateral Tunnel ◽  
Key Factor

In this chapter, the design and development of a computational model of the cardiovascular system is presented for patients who have undergone the Fontan operation. The model has been built from a physiological basis, considering some of the mechanisms associated to the cardiovascular system of patients with univentricular heart disease. Thus, the model allows the prediction of some hemodynamic variables considering different physiopathological conditions. The original conditions of the model are changed in the Fontan procedure and these new dynamics force the hemodynamic behaviours of the different considered variables. The model has been proved considering the classic Fontan procedure and the techniques from the lateral tunnel and the extracardiac conduit. The results compiled knowledge of several cardiovascular surgeons with many years of experience in such interventions, and have been validated by using other authors' data. In this sense, the participation of a multidisciplinary team has been considered as a key factor for the development of this work.

Comparison of Clinical and Simulation Results for the Stanford Y-Graft Fontan Pilot Trial

2012 ◽  
Author(s):  
Weiguang Yang ◽  
Jeffrey A. Feinstein ◽  
V. Mohan Reddy ◽  
Frandics P. Chan ◽  
Alison L. Marsden
Keyword(s):  
Inferior Vena ◽  
Single Ventricle ◽  
Heart Defects ◽  
Fontan Procedure ◽  
Pilot Trial ◽  
Pulmonary Arteries ◽  
Vena Cava ◽  
Extracardiac Conduit ◽  
Lateral Tunnel ◽  
Third Stage

Without surgical palliation, single ventricle heart defects are uniformly fatal. A three-staged surgical repair is typically performed on these patients, who are otherwise severely cyanotic. In the third stage, the Fontan procedure, the inferior vena cava (IVC) is connected to the pulmonary arteries (PAs) via a lateral tunnel or extracardiac conduit. Following Fontan completion, deoxygenated blood from the upper and lower body is redirected to the PAs, bypassing the heart.

Morphology of hearts undergoing Fontan repair

1998 ◽  
Vol 8 (2) ◽  
pp. 165-171 ◽  
Author(s):  
Vivek Murari ◽  
Rajesh Sharma ◽  
Balram Airan ◽  
Anil Bhan ◽  
Shiv K. Choudhary ◽  
...  
Keyword(s):  
Ventricular Septal Defect ◽  
Septal Defect ◽  
Single Ventricle ◽  
Pulmonary Stenosis ◽  
Fontan Procedure ◽  
Fontan Operation ◽  
Definitive Treatment ◽  
Precise Description ◽  
Biventricular Repair

AbstractBackgroundNotwithstanding the controversies evoked by the term “single ventricle”, most patients with this condition would undergo the Fontan procedure. In addition, there is a large group of patients in whom a biventricular repair would be abandoned in favour of a univentricular one because of the presence of unfavourable morphologic features. There is a need for a uniformly acceptable system of nomenclature that would permit precise description and classification of hearts with complex malformations to facilitate reporting and help in understanding the reasons for choosing a univentricular repair.MethodsEchocardiographic, angiographic and operative records of 240 patients undergoing the Fontan procedure were analysed.ResultsOut of the 104 patients with univentricular atrioventricular connections, 2 ventricles were discernible in all but 3 patients. A Fontan repair was performed in 136 patients with biventricular atrioventricular connections because of the presence of a hypoplastic ventricle in 52 patients and a non-committed ventricular septal defect in the remaining 84.ConclusionsThe Fontan operation is probably the only definitive treatment option for patients with univentricular atrioventricular connections. The decision to perform a univentricular repair in preference to a biventricular one in hearts with biventricular atrioventricular connections is based on the presence of a hypoplastic ventricle or a non-reroutable ventricular septal defect. This decision is subjective. In hearts with discordant atrioventricular connections and pulmonary stenosis, we prefer the Fontan operation to the classical repair.

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.

Outcomes of the Extracardiac Fontan Procedure Using Cardiopulmonary Bypass: Early Results

2014 ◽  
Vol 17 (3) ◽  
pp. 173 ◽  
Author(s):  
Murat Ugurlucan ◽  
Eylem Yayla Tuncer ◽  
Fusun Guzelmeric ◽  
Eylul Kafali ◽  
Omer Ali Sayin ◽  
...  
Keyword(s):  
Cardiopulmonary Bypass ◽  
Fontan Procedure ◽  
Fontan Operation ◽  
Arterial Oxygen Saturation ◽  
Vena Cava ◽  
Arterial Oxygen ◽  
Shunt Operation ◽  
Early Results ◽  
The Mean ◽  
Extracardiac Fontan

<p><strong>Background</strong>: Although the avoidance of cardiopulmonary bypass during the Fontan procedure has potential advantages, using cardiopulmonary bypass during this procedure has no adverse effects in terms of morbidity and mortality rates. In this study, we assessed the postoperative outcomes of our first 9 patients who have undergone extracardiac Fontan operation by the same surgeon using cardiopulmonary bypass.</p><p><strong>Methods</strong>: Between September 2011 and April 2013,  9 consecutive patients (3 males and 6 females) underwent extra-cardiac Fontan operation. All operations were performed under cardiopulmonary bypass at normothermia by the same surgeon.  The age of patients ranged between 4 and 17 (9.8 ± 4.2) years. Previous operations performed on these patients were modified Blalock-Taussig shunt procedure in 2 patients, bidirectional cavopulmonary shunt operation in 6 patients, and pulmonary arterial banding in 1 patient. Except 2 patients who required intracardiac intervention, cross-clamping was not applied. In all patients, the extracardiac Fontan procedure was carried out by interposing an appropriately sized tube graft between the infe-rior vena cava and right pulmonary artery.</p><p><strong>Results</strong>: The mean intraoperative Fontan pressure and transpulmonary gradient were 12.3 ± 2.5 and 6.9 ± 2.2 mm Hg, respectively. Intraoperative fenestration was not required. There was no mortality and 7 patients were discharged with-out complications. Complications included persistent pleural effusion in 1 patient and a transient neurological event in 1 patient. All patients were weaned off mechanical ventila-tion within 24 hours. The mean arterial oxygen saturation increased from 76.1% ± 5.3% to 93.5% ± 2.2%. All patients were in sinus rhythm postoperatively. Five patients required blood and blood-product transfusions. The mean intensive care unit and hospital stay periods were 2.9 ± 1.7 and 8.2 ±  1.9 days, respectively.</p><p><strong>Conclusions</strong>: The extracardiac Fontan operation per-formed using cardiopulmonary bypass provides satisfactory results in short-term follow-up and is associated with favor-able postoperative hemodynamics and morbidity rates.</p>

Normal cerebral ventricular volume growth in childhood

2020 ◽  
Vol 26 (5) ◽  
pp. 517-524
Author(s):  
Noah S. Cutler ◽  
Sudharsan Srinivasan ◽  
Bryan L. Aaron ◽  
Sharath Kumar Anand ◽  
Michael S. Kang ◽  
...  
Keyword(s):  
Brain Mri ◽  
Volume Growth ◽  
Ventricular Volume ◽  
Growth Patterns ◽  
Diagnostic Process ◽  
Growth Charts ◽  
Normal Brain ◽  
Mr Images ◽  
Ventricular Volumes ◽  
Brain Mr Images

OBJECTIVENormal percentile growth charts for head circumference, length, and weight are well-established tools for clinicians to detect abnormal growth patterns. Currently, no standard exists for evaluating normal size or growth of cerebral ventricular volume. The current standard practice relies on clinical experience for a subjective assessment of cerebral ventricular size to determine whether a patient is outside the normal volume range. An improved definition of normal ventricular volumes would facilitate a more data-driven diagnostic process. The authors sought to develop a growth curve of cerebral ventricular volumes using a large number of normal pediatric brain MR images.METHODSThe authors performed a retrospective analysis of patients aged 0 to 18 years, who were evaluated at their institution between 2009 and 2016 with brain MRI performed for headaches, convulsions, or head injury. Patients were excluded for diagnoses of hydrocephalus, congenital brain malformations, intracranial hemorrhage, meningitis, or intracranial mass lesions established at any time during a 3- to 10-year follow-up. The volume of the cerebral ventricles for each T2-weighted MRI sequence was calculated with a custom semiautomated segmentation program written in MATLAB. Normal percentile curves were calculated using the lambda-mu-sigma smoothing method.RESULTSVentricular volume was calculated for 687 normal brain MR images obtained in 617 different patients. A chart with standardized growth curves was developed from this set of normal ventricular volumes representing the 5th, 10th, 25th, 50th, 75th, 90th, and 95th percentiles. The charted data were binned by age at scan date by 3-month intervals for ages 0–1 year, 6-month intervals for ages 1–3 years, and 12-month intervals for ages 3–18 years. Additional percentile values were calculated for boys only and girls only.CONCLUSIONSThe authors developed centile estimation growth charts of normal 3D ventricular volumes measured on brain MRI for pediatric patients. These charts may serve as a quantitative clinical reference to help discern normal variance from pathologic ventriculomegaly.

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.

Functional state of patients with heterotaxy syndrome following the Fontan operation

2007 ◽  
Vol 17 (S4) ◽  
pp. 44-53 ◽  
Author(s):  
Andrew M. Atz ◽  
Meryl S. Cohen ◽  
Lynn A. Sleeper ◽  
Brian W. McCrindle ◽  
Minmin Lu ◽  
...  
Keyword(s):  
Health Status ◽  
Functional State ◽  
Exercise Performance ◽  
Fontan Procedure ◽  
Fontan Operation ◽  
Pulmonary Veins ◽  
Functional Health ◽  
Health State ◽  
Ventricular Performance ◽  
Health Status Questionnaires

AbstractBackgroundChildren born with heterotaxy syndromes have poorer outcomes compared with children born with comparable cardiac lesions requiring similar surgical palliation. Heterotaxy has been reported as a separate risk factor for mortality and increased morbidity in a series of Fontan operations reported from single centres. Little is known, however, about the functional state of surviving patients with heterotaxy following a Fontan operation.MethodsIn the multicentric cross-sectional study carried out by the Pediatric Heart Network of 546 survivors of the Fontan procedure, the patients, aged from 6 to 18 years, underwent evaluation by echocardiography, exercise testing, electrocardiography, magnetic resonance imaging, and functional health status questionnaires compiled by the patients and their parents. Heterotaxy was identified in 42 patients (8%). Medical and patient characteristics were compared between those with heterotaxy and the remaining 504 patients who did not have heterotaxy.ResultsPatients with heterotaxy had their Fontan procedure performed at a later age, with a median of 3.9 years versus 2.8 years (p = 0.001) and had volume-unloading surgery performed later, at a median age of 1.4 versus 0.9 years (p = 0.008). These patients had significantly different ventricular and atrioventricular valvar morphology, as well as a higher incidence of systemic and pulmonary venous abnormalities. They had a higher incidence of prior surgery to the pulmonary veins, at 21 versus 0.4%. The type of Fontan procedure was different, but no difference was detected in length of stay in hospital, or the number of postoperative complications. Sinus rhythm was less common, at 44 versus 71%, (p = 0.002), and history of atrial arrhythmias more common, at 19 versus 8%, (p = 0.018) in those with heterotaxy. Echocardiography revealed a greater degree atrioventricular valvar regurgitation, lower indexed stroke volume, and greater Tei index. Exercise performance, levels of brain natriuretic peptide in the serum, and summary and domain scores from health status questionnaires, were not different from those not having heterotaxy.ConclusionsThe study illustrates a profile of characteristics, medical history, functional health state, and markers of ventricular performance in patients with heterotaxy after the Fontan procedure. Despite obvious anatomic differences, and some differences in echocardiography and heart rhythm, there were no important differences in exercise performance or functional health state between these patients and other survivors of the Fontan procedure.

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