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.

Investigation of Vessel Growth and its Impact on Hemodynamics in Patients With Lateral Tunnel Total Cavopulmonary Connection

2012 ◽  
Author(s):  
Maria Restrepo ◽  
Lucia Mirabella ◽  
Elaine Tang ◽  
Chris Haggerty ◽  
Mark A. Fogel ◽  
...  
Keyword(s):  
Heart Defects ◽  
Fontan Procedure ◽  
Pulmonary Arteries ◽  
Vena Cava ◽  
Total Cavopulmonary Connection ◽  
Lateral Tunnel ◽  
Vessel Growth ◽  
Original Size ◽  
The Right ◽  
Cavopulmonary Connection

Single ventricle heart defects affect 2 per 1000 live births in the US and are lethal if left untreated. The Fontan procedure used to treat these defects consists of a series of palliative surgeries to create the total cavopulmonary connection (TCPC), which bypasses the right heart. In the last stage of this procedure, the inferior vena cava (IVC) is connected to the pulmonary arteries (PA) using one of the two approaches: the extra-cardiac (EC), where a synthetic graft is used as the conduit; and the lateral tunnel (LT) where part of the atrial wall is used along with a synthetic patch to create the conduit. The LT conduit is thought to grow in size in the long term because it is formed partially with biological tissue, as opposed to the EC conduit that retains its original size because it contains only synthetic material. The growth of the LT has not been yet quantified, especially in respect to the growth of other vessels forming the TCPC. Furthermore, the effect of this growth on the hemodynamics has not been elucidated. The objective of this study is to quantify the TCPC vessels growth in LT patients from serial magnetic resonance (MR) images, and to understand its effect on the connection hemodynamics using computational fluid dynamics (CFD).

Effect of Flow Pulsatility on 2nd Stage Fontan Hemodynamics: An In Vitro Investigation

2009 ◽  
Author(s):  
Christopher M. Haggerty ◽  
Lakshmi P. Dasi ◽  
Jessica Kanter ◽  
Ajit P. Yoganathan
Keyword(s):  
Congenital Heart ◽  
Single Ventricle ◽  
Heart Defects ◽  
Superior Vena ◽  
Fontan Procedure ◽  
Pulmonary Arteries ◽  
Vena Cava ◽  
Second Stage ◽  
In Vitro Investigation

The Fontan procedure [1] is the staged, palliative surgical approach used to treat patients suffering from single ventricle congenital heart defects. The second stage of this procedure involves the connection of the superior vena cava (SVC) to the pulmonary arteries (PAs) in either an end-to-side (known as the Bi-Directional Glenn (BDG)) or side-to-side (or Hemi-Fontan (HF)) fashion. Because of obvious disparities at the connection site, there are understandable differences in the fluid dynamics between the two geometries.

Virtual Design for the Fontan Procedure: From Idealized to Patient Specific Models Using CFD and Derivative-Free Optimization

2010 ◽  
Author(s):  
Weiguang Yang ◽  
Guillaume Troianowski ◽  
Alexandre Birolleau ◽  
Irene Vignon-Clementel ◽  
Jeffrey A. Feinstein ◽  
...  
Keyword(s):  
Single Ventricle ◽  
Heart Defects ◽  
Superior Vena ◽  
Fontan Procedure ◽  
Pulmonary Arteries ◽  
Vena Cava ◽  
Patient Specific ◽  
Virtual Design ◽  
Modeling Tools ◽  
Patient Specific Modeling

Single ventricle congenital heart defects are among the most challenging for pediatric cardiologists to treat. Children born with these defects are cyanotic, and these conditions are nearly uniformly fatal without treatment. A series of surgeries is performed to palliate single ventricle defects. The first stage consists of aortic reconstruction in a Norwood procedure. In the second stage, the Bidirectional Glenn procedure, the superior vena cava (SVC) is disconnected from the heart and redirected into the pulmonary arteries (PA’s). In the third and final stage, the Fontan procedure, the inferior vena cava (IVC) is connected to the PA’s via a straight Gore-Tex tube, forming a T-shaped junction with or without offset. Patient specific modeling tools provide a means to evaluate new designs with the goal of lowering long-term morbidity and improving patients’ quality of life.

Effect of Flow Pulsatility on Modeling the Total Cavopulmonary Hemodynamics: A Numerical Investigation

2012 ◽  
Author(s):  
Reza H. Khiabani ◽  
Maria Restrepo ◽  
Elaine Tang ◽  
Diane De Zélicourt ◽  
Mark Fogel ◽  
...  
Keyword(s):  
Boundary Conditions ◽  
Heart Defects ◽  
Superior Vena ◽  
Fontan Procedure ◽  
Pulmonary Arteries ◽  
Vena Cava ◽  
Venous Flow ◽  
Surgical Interventions ◽  
The Right

Single Ventricle Heart Defects (SVHD) are present in 2 per 1000 live births in the US. SVHD are characterized by cyanotic mixing between the de-oxygenated blood from the systemic circulation return and the oxygenated blood from the pulmonary arteries. Palliative surgical repairs (Fontan procedure) are performed to bypass the right ventricle in these patients. In current practice, the surgical interventions commonly result in the total cavopulmonary connection (TCPC). In this configuration the systemic venous returns (inferior vena cava, IVC, and superior vena cava, SVC) are directly routed to the right and left pulmonary arteries (RPA and LPA), bypassing the right heart. The resulting anatomy has complex and unsteady hemodynamics characterized by flow mixing and flow separation. Pulsation of the inlet venous flow during a cardiac cycle results in complex and unsteady flow patterns in the TCPC. Although various degrees of pulsatility have been observed in vivo, non-pulsatile (time-averaged) flow boundary conditions have traditionally been assumed in modeling TCPC hemodynamics, and only recently have pulsatile conditions been incorporated without completely characterizing their effect or importance. In this study, 3D numerical simulations were performed to predict TCPC hemodynamics with both pulsatile and non-pulsatile boundary conditions and to investigate the accuracy of applying non-pulsatile boundary conditions. Flow structures, energy dissipation rate and pressure drop were compared under rest and estimated exercise conditions. The results show that TCPC hemodynamics can be strongly influenced by the presence of pulsatile flow. However, there exists a minimum pulsatility threshold, identified by defining a weighted pulsatility index (wPI), above which the influence is significant.

In Vitro Study of Pulmonary Vascular Resistance in Fontan Circulation With Respiration Effects

2012 ◽  
Author(s):  
Marija Vukicevic ◽  
Timothy A. Conover ◽  
Jian Zhou ◽  
Tain-Yen Hsia ◽  
Richard S. Figliola
Keyword(s):  
Pulmonary Vascular Resistance ◽  
Vascular Resistance ◽  
Heart Defects ◽  
Superior Vena ◽  
Fontan Procedure ◽  
Fontan Operation ◽  
Venous Pressure ◽  
Pulmonary Arteries ◽  
Pulmonary Regurgitation ◽  
Vena Cava

The Fontan operation is the final stage of palliative surgery for children born with single ventricle heart defects. The most common configuration is called total cavopulmonary connection (TCPC), wherein the inferior vena cava and superior vena cava are anastomosed directly to the pulmonary arteries; therefore the pulmonary circulation is driven by venous pressure only. The Fontan procedure, although successful in the early postoperative period, with time can decrease in efficiency or even fail within several years after the operation. The reasons of different clinical outcomes for some of the Fontan patients are not clear enough, even though it is commonly accepted that certain factors such as low pulmonary vascular resistance and proper shape and size of the TCPC construction are crucial for the succesful long term outcomes. Accordingly, one of the major problems is the increase in pulmonary vascular resistance due to altered hemodynamics after the surgery, causing venous hypertension and respiratory-dependent pulmonary regurgitation [1]. The main pulmonary arteries may also see increased resistance due to congenital malformations, surgical scarring, or deliberate surgical banding. Thus, the consequence of the increased pulmonary vascular resistance at both proximal and distal locations with respect to the TCPC junction, and its effect on the systemic pressures and flow rates, is the main objective of this study.

Kawashima by Fenestrated Hemi-Fontan for Palliation Following Prior Stage I Norwood Operation

2018 ◽  
Vol 9 (4) ◽  
pp. 451-453 ◽  
Author(s):  
Jenny E. Zablah ◽  
Michael Ross ◽  
Neil Wilson ◽  
Brian Fonseca ◽  
Max B. Mitchell
Keyword(s):  
Single Ventricle ◽  
Superior Vena ◽  
Fontan Procedure ◽  
Venous Blood ◽  
Young Infant ◽  
Pulmonary Arteries ◽  
Vena Cava ◽  
Stage I ◽  
Azygos Continuation ◽  
Cavopulmonary Shunt

Single ventricle patients with interrupted inferior vena cava (IVC) and azygos continuation to the superior vena cava (SVC) are typically palliated with a bidirectional cavopulmonary shunt (BCPS), known as the Kawashima operation in this setting. Because the volume of venous blood directed to the pulmonary arteries is substantially greater in the presence of interrupted IVC, Kawashima procedures are commonly delayed to older age compared to other single ventricle patients undergoing BCPS. We report two young infant single ventricle patients with interrupted IVC and azygos continuation to the SVC who underwent stage I Norwood procedures for initial palliation. In both cases, a fenestrated hemi-Fontan procedure achieved successful Kawashima circulations.

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.

Optimization of an Idealized Y-Graft for the Fontan Procedure Using CFD and a Derivative-Free Optimization Algorithm

2009 ◽  
Author(s):  
Weiguang Yang ◽  
Jeffrey A. Feinstein ◽  
V. Mohan Reddy ◽  
Alison L. Marsden
Keyword(s):  
Heart Defects ◽  
Superior Vena ◽  
Fontan Procedure ◽  
Survival Rates ◽  
Pulmonary Arteries ◽  
Vena Cava ◽  
Protein Losing Enteropathy ◽  
Second Stage ◽  
Derivative Free Optimization ◽  
Derivative Free

The Fontan procedure is a surgery performed to treat patients with single ventricle congenital heart defects. The Fontan is the final of three surgical stages. The first stage consists of aortic reconstruction, in a Norwood procedure or variant thereof. In the second stage, the Bidirectional Glenn procedure, the superior vena cava (SVC) is disconnected from the heart and redirected into the pulmonary arteries (PAs). In the third and final stage, the inferior vena cava (IVC) is connected to PAs via a straight Gore-Tex tube, forming a T-shaped junction. Although early survival rates following the Fontan procedure can exceed 90%, significant morbidity remains after surgery including venous hemodynamic abnormalities, diminished exercise capacity, thromboembolic complications, protein-losing enteropathy, heart transplant etc. [1].

Hemodynamic Impact of Stenting in the Total Cavopulmonary Connection With Lateral Tunnel Stenosis

2012 ◽  
Author(s):  
Elaine Tang ◽  
Doff B. McElhinney ◽  
Ajit P. Yoganathan
Keyword(s):  
Heart Defects ◽  
Superior Vena ◽  
Pulmonary Arteries ◽  
Vena Cava ◽  
Total Cavopulmonary Connection ◽  
Lateral Tunnel ◽  
The Us ◽  
Intravascular Stent ◽  
The Right ◽  
Cavopulmonary Connection

2 per 1000 children in the US are born with functionally single ventricle (SV) heart defects. To restore the separate systemic and pulmonary circulations, a Total Cavopulmonary Connection (TCPC) is carried out through a series of surgical steps, which result in the direct connection of the superior vena cava (SVC) and inferior vena cava (IVC) to the pulmonary arteries without an intervening pulmonary ventricle. One way to complete the TCPC is by placing a synthetic patch in the right atrium, forming an intracardiac lateral tunnel (LT) as the final step. As patients grow, some LT pathways become stenosed. The stenosis can impose extra resistance to flow in addition to the TCPC in the SV circulation. One method of treating LT stenosis is by placement of an intravascular stent.

Die Aplasie der Vena cava inferior als Ursache rezidivierender Bein- und Beckenvenenthrombosen

VASA ◽  
1999 ◽  
Vol 28 (4) ◽  
pp. 289-292 ◽  
Author(s):  
Tiesenhausen ◽  
Amann ◽  
Thalhammer ◽  
Aschauer
Keyword(s):  
Inferior Vena Cava ◽  
Inferior Vena ◽  
Heart Defects ◽  
Vena Cava ◽  
Clinical Signs ◽  
Vena Cava Inferior ◽  
Caval Vein ◽  
Rare Finding ◽  
Thrombotic Complications ◽  
Asplenia Syndrome

Congenital anomalies of the caval vein are often associated with other abnormities such as heart defects, situs inversus or a polysplenia-asplenia-syndrome. An isolated, congenital malformation like aplasia of the inferior vena cava is a rare finding. A review of the embryology and abnormities, diagnostics, clinical signs and treatment is given together with the histories of two patients having thrombosis of the lower extremities and pelvic veins, caused by aplasia of the inferior vena cava. After thrombotic complications caused by vena cava aplasia there is high risk of recurrence. Those patients should be anticoagulated for lifetime.

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