Midterm Prognosis and Effectiveness of Fontan Procedure with Total Cavopulmonary Connection Performed in Adults

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).

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Brain natriuretic peptide assessed at long-term follow-up before and after maximal exercise in surgically palliated patients with functionally univentricular hearts

Cardiology in the Young ◽

10.1017/s1047951107000686 ◽

2007 ◽

Vol 17 (5) ◽

pp. 505-511 ◽

Cited By ~ 6

Author(s):

Daniel Holmgren ◽

Eva Strömvall-Larsson ◽

Per-Arne Lundberg ◽

Bengt O. Eriksson ◽

Håkan Wåhlander

Keyword(s):

Brain Natriuretic Peptide ◽

Ventricular Function ◽

Natriuretic Peptide ◽

Maximal Exercise ◽

Fontan Procedure ◽

Control Group ◽

Total Cavopulmonary Connection ◽

Before And After ◽

Long Term Follow Up ◽

Cavopulmonary Connection

AbstractWe evaluated the concentrations of brain natriuretic peptide in the plasma as a marker of systolic ventricular function before and after maximal exercise in 15 surgically palliated patients with functionally univentricular hearts, with apparently good ventricular function. Of the patients, 6 with median age of 14.6 years, and a range from 12.5 to 17.9 years, had been palliated by construction of a total cavopulmonary connection, while the other 9 patients, with a median age of 32.1 years, and a range from 15.6 to 54.2 years, had undergone the classical Fontan procedure. We used 8 healthy individuals, with a median age of 13.9 years, and a range from 12.8 to 14.2 years, as a control group for the measurements of brain natriuretic peptide. The values of the peptide were significantly higher in those with the classical Fontan procedure, both before, when the median value was 131.8 nanogram per litre, with a range from 0.5 to 296.4, and after maximal exercise, when the median value was 108.1, with a range from 0.1 to 235.9. The comparable values in those with a total cavopulmonary connection were a median of 12.8, and a range from 0.5 to 39.1 before, and a median of 9.7, with a range from 2.7 to 26.2 after maximal exercise. The median value for the control group was 13.1, with a range from 2.6 to 38.7 before exercise (p = 0.016), and a median of 24.1, with a range from 5.8 to 66.7 after maximal exercise (p = 0.03), respectively. In the control subjects, the level of the peptide increased by a median of 9.7 nanograms per litre, with a range from 1.2 to 28.0 after maximal exercise (p = 0.008). The level was unchanged after maximal exercise in those with classical Fontan procedures and total cavopulmonary connections, with a difference between levels before and after exercise of a median of 5.9 nanogram per litre, and a range from −23.7 to 31.0 (p = 0.96), and a median of −1.0 nanogram per litre, with a range from −12.0 to 3.9 (p > 0.99), respectively. We conclude that maximal exercise did not increase the level of brain natriuretic peptide level in those patients with the classical Fontan procedure, nor those with a total cavopulmonary connection, findings which may indicate that systolic ventricular dysfunction is not the major cause of the decreased working capacity observed in patients with well functioning palliated functionally univentricular hearts.

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Formation of thrombus and thromboembolism after the bidirectional Glenn anastomosis, total cavopulmonary connection and the Fontan operation

Cardiology in the Young ◽

10.1017/s1047951100006107 ◽

1998 ◽

Vol 8 (2) ◽

pp. 211-216 ◽

Cited By ~ 7

Author(s):

Katarina Hanséus ◽

Gudrun Björkhem ◽

Peeter Jögi ◽

Sven-Erik Sonesson

Keyword(s):

Clinical Characteristics ◽

Fontan Procedure ◽

Fontan Operation ◽

Significant Risk ◽

Sudden Onset ◽

Pulmonary Trunk ◽

Total Cavopulmonary Connection ◽

Cerebrovascular Accidents ◽

Bidirectional Glenn ◽

Cavopulmonary Connection

AbstractAlthough patients undergoing surgery with the Fontan procedure or its modifications are increasingly recognised to be at risk for thromboembolism, further knowledge is needed to minimise this complication and its sequels. To address this issue, we reviewed 100 patients operated with the Fontan procedure, the bidirectional Glenn anastomosis and/or the total cavopulmonary connection to describe our incidence and clinical characteristics of postoperative formation of thrombus. Symptomatic thrombosis or cerebrovascular accidents were found in 5 patients. Asymptomatic thrombus were found in another 5 patients. Three patients had venous thrombi. In 6 patients the thrombosis was found on the arterial side. In all these cases, the thrombus was located in the stump of the divided pulmonary trunk. In 3 of these patients, sudden onset of hemiparesis preceded the diagnosis of the thrombus while the remaining 3 patients were asymptomatic. In 3 cases, the formation of thrombus in the stump of the divided pulmonary trunk occurred after a bidirectional Glenn anastomosis. One patient developed severe neurological symptoms 2 months after a total cavopulmonary connection. No thrombus was found, but the patient had a small right-to-left shunt and embolization could not be excluded. The incidence of thrombosis after Fontan-type surgery in this study is 10%. Although not all episodes of thrombosis are symptomatic, there is a significant risk of severe sequels due to embolization to the pulmonary or cerebral circulations. The stump of the divided pulmonary trunk is one of the main sites for intracardiac formation of thrombus, even after a bidirectional Glenn anastomosis.

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Fontan Hemodynamics Investigation via Modeling and Experimental Characterization of Idealized Pediatric Total Cavopulmonary Connection

Applied Sciences ◽

10.3390/app10196910 ◽

2020 ◽

Vol 10 (19) ◽

pp. 6910

Author(s):

Andrey Porfiryev ◽

Aleksandr Markov ◽

Andrey Galyastov ◽

Maxim Denisov ◽

Olga Burdukova ◽

...

Keyword(s):

Blood Flow ◽

Heart Transplantation ◽

Human Body ◽

High Speed ◽

Fontan Procedure ◽

Operating Conditions ◽

Univentricular Heart ◽

Total Cavopulmonary Connection ◽

Different Types ◽

Cavopulmonary Connection

Simulation of the human body normal operating conditions is the important issue in the engineering process of designing biomedical devices intended for implantation. As an example of such process the Fontan procedure aims to support the human body function. It is a standard palliative treatment method for patients with a functionally univentricular heart. Nevertheless, this procedure has significant drawbacks. For instance, overload of the only functional ventricle leads to the inevitability of the heart transplantation. Herein, we perform simulation and experimental characteristics of the pediatric total cavopulmonary connection (TCPC) influence on the blood flow. We investigate and design three different types of pediatric TCPC configurations; we detect fluorescent particles via a high-speed camera in order to analyze distribution of the blood flow velocity modulus in different types of TCPCs. Finally, we evaluate hydraulic power losses for various cases. This work is particularly relevant for the improvement of existing TCPCs quality that can extend the life of Fontan patients. Moreover, it also applies to the reduction of morbidity and mortality of the patients waiting for a heart transplantation.

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Fontan completions over 10 years after Glenn procedures

Cardiology in the Young ◽

10.1017/s1047951113000280 ◽

2013 ◽

Vol 24 (2) ◽

pp. 290-296 ◽

Cited By ~ 4

Author(s):

Koichi Sughimoto ◽

Kozo Matsuo ◽

Koichiro Niwa ◽

Yasutaka Kawasoe ◽

Shigeru Tateno ◽

...

Keyword(s):

Fontan Procedure ◽

Pacemaker Implantation ◽

Early Period ◽

Maze Procedure ◽

Total Cavopulmonary Connection ◽

Atrioventricular Valve ◽

Atrioventricular Valve Regurgitation ◽

Lateral Tunnel ◽

Cavopulmonary Connection ◽

Glenn Procedure

AbstractObjective: Despite the broadened indications for Fontan procedure, there are patients who could not proceed to Fontan procedure because of the strict Fontan criteria during the early period. Some patients suffer from post-Glenn complications such as hypoxia, arrhythmia, or fatigue with exertion long after the Glenn procedure. We explored the possibility of Fontan completion for those patients.Methods: Between 2004 and 2010, five consecutive patients aged between 13 and 31 years (median 21) underwent Fontan completion. These patients had been followed up for more than 10 years (10 to 13, median 11) after Glenn procedure as non-Fontan candidates. We summarise these patients retrospectively in terms of their pre-operative physiological condition, surgical strategy, and problems that these patients hold.Results: Pre-operative catheterisation showed pulmonary vascular resistance ranging from 0.9 to 3.7 (median 2.2), pulmonary to systemic flow ratio of 0.3 to 1.6 (median 0.9), and two patients had significant aortopulmonary collaterals. Extracardiac total cavopulmonary connections were performed in three patients, lateral tunnel total cavopulmonary connection in one patient, and intracardiac total cavopulmonary connection in one patient, without a surgical fenestration. Concomitant surgeries were required including valve surgeries – atrioventricular valve plasty in three patients and tricuspid valve replacement in one patient; systemic outflow tract obstruction release – Damus–Kaye–Stansel procedure in two patients and subaortic stenosis resection in one patient; and anti-arrhythmic therapies – maze procedure in two patients, cryoablation in two patients, and pacemaker implantation in two patients. All patients are now in New York Heart Association category I.Conclusion:Patients often suffer from post-Glenn complications. Of those, if they are re-examined carefully, some may have a chance to undergo Fontan completion and benefit from it. Multiple lesions such as atrioventricular valve regurgitation, systemic outflow obstruction, or arrhythmia should be surgically repaired concomitantly.

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Recanalisation of the left superior caval vein after Fontan procedure: not so rare complication: possibilities of percutaneous closure using various devices

Cardiology in the Young ◽

10.1017/s104795111400016x ◽

2014 ◽

Vol 25 (3) ◽

pp. 485-490

Author(s):

Jacek Kusa ◽

Leslaw Szydlowski ◽

Ewa Nowakowska ◽

Agnieszka Skierska

Keyword(s):

Rare Complication ◽

Fontan Procedure ◽

Arterial Oxygen Saturation ◽

Percutaneous Closure ◽

Arterial Oxygen ◽

Total Cavopulmonary Connection ◽

Sudden Increase ◽

Caval Vein ◽

Superior Caval Vein ◽

Cavopulmonary Connection

AbstractAim: Evaluation of possibilities of percutaneous closure of recanalised left superior caval vein after total cavopulmonary connection.Methods and Results: We analysed 19 patients after total cavopulmonary connection catheterised because of a sudden increase of desaturation. In four of them, the recanalisation of the left superior caval vein was identified. For this reason, the balloon occlusion tests of the veins were made temporarily. In all cases, the haemodynamic status of patients did not change, and arterial oxygen saturation increased significantly. Thus, using different types of implants, these veins were closed effectively in all patients. During the short-term follow-up, the effectiveness of treatments and constantly maintaining a high level of saturation were confirmed.Conclusions: Meticulous investigation of unclear causes of desaturation in cyanotic patients after Fontan completion is necessary. Almost all causes of desaturation, including recanalised additional left superior caval vein, can be effectively treated percutaneously.

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