Correspondence - Univentricular heart and Fontan circulation should not be mixed together

Many patients with a functional univentricular heart were treated in the 1970s and 1980s, using an atriopulmonary connection to create the Fontan circulation.1–3Although this procedure, in many patients, was initially successful, and provided arterial saturations of oxygen close to normal, as these patients were followed over the years, in some cases they developed significant complications. One complication of the atriopulmonary connection is progressive right atrial dilation, which leads to atrial arrhythmias, such as atrial flutter or fibrillation.4,5The combination of these two problems leads to low cardiac output, diminished quality of life, and poor categorization within the classification of the New York Heart Association. This, and other issues, has led most centres to abandon the atriopulmonary connection as a means of creating the Fontan circulation in favour of the lateral tunnel with cavopulmonary connections,6or the extracardiac conduit.7

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Ventricular dysfunction of the functionally univentricular heart: management and outcomes

Cardiology in the Young ◽

10.1017/s1047951105001605 ◽

2005 ◽

Vol 15 (S3) ◽

pp. 31-34 ◽

Cited By ~ 14

Author(s):

Marc Gewillig

Keyword(s):

Congenital Malformation ◽

Working Conditions ◽

Ventricular Dysfunction ◽

Fontan Circulation ◽

Univentricular Heart ◽

Stress Strain ◽

Cross Sectional ◽

Surgical Conditions ◽

Contraction And Relaxation

The ventricle of a functionally univentricular heart is dilated, hypertrophic, and hypocontractile, certainly after the completion of a Fontan circulation (reviewed in Reference). It is difficult, however, to determine the reasons for these abnormal ventricular conditions. The abnormal size of the ventricle, along with the less than adequate function, can be due to the congenital malformation itself, the previous surgical conditions, or the very abnormal working conditions of the ventricle during the various stages of palliation. Moreover, because of the varying morphology of the pumping ventricle in patients with a functionally univentricular arrangement, it is difficult to assess size, shape, hypertrophy, stress, strain, contraction and relaxation, both in cross sectional and serial follow-up.

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State of the art of the Fontan strategy for treatment of univentricular heart disease

F1000Research ◽

10.12688/f1000research.13792.1 ◽

2018 ◽

Vol 7 ◽

pp. 935 ◽

Cited By ~ 17

Author(s):

Jelle P. G. van der Ven ◽

Eva van den Bosch ◽

Ad J.C.C. Bogers ◽

Willem A. Helbing

Keyword(s):

State Of The Art ◽

Surgical Techniques ◽

Volume Overload ◽

Ventricular Volume ◽

Fontan Circulation ◽

Circulatory Support ◽

Univentricular Heart ◽

High Morbidity ◽

Fontan Patient ◽

Protein Losing Enteropathy

In patients with a functionally univentricular heart, the Fontan strategy achieves separation of the systemic and pulmonary circulation and reduction of ventricular volume overload. Contemporary modifications of surgical techniques have significantly improved survival. However, the resulting Fontan physiology is associated with high morbidity. In this review, we discuss the state of the art of the Fontan strategy by assessing survival and risk factors for mortality. Complications of the Fontan circulation, such as cardiac arrhythmia, thromboembolism, and protein-losing enteropathy, are discussed. Common surgical and catheter-based interventions following Fontan completion are outlined. We describe functional status measurements such as quality of life and developmental outcomes in the contemporary Fontan patient. The current role of drug therapy in the Fontan patient is explored. Furthermore, we assess the current use and outcomes of mechanical circulatory support in the Fontan circulation and novel surgical innovations. Despite large improvements in outcomes for contemporary Fontan patients, a large burden of disease exists in this patient population. Continued efforts to improve outcomes are warranted. Several remaining challenges in the Fontan field are outlined.

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Functionally univentricular hearts and Fontan circulation

10.1093/med/9780199228188.003.0025 ◽

2011 ◽

Keyword(s):

Hypoplastic Left Heart Syndrome ◽

Congenital Heart ◽

Complex Form ◽

Fontan Circulation ◽

Univentricular Heart ◽

Left Heart ◽

Hypoplastic Left Heart ◽

Ventricular Cavity ◽

Staged Approach ◽

Left Heart Syndrome

The functionally univentricular heart 172Staged approach to achieve definitive palliation 174Fontan circulation 176Hypoplastic left heart syndrome (HLHS) 186Rare and highly complex form of cyanotic congenital heart disease.The term describes a variety of cardiac malformations in which: • There is functionally a single ventricular cavity....

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Anatomy of the Functionally Univentricular Heart

World Journal for Pediatric and Congenital Heart Surgery ◽

10.1177/2150135118800694 ◽

2018 ◽

Vol 9 (6) ◽

pp. 677-684 ◽

Cited By ~ 2

Author(s):

Robert H. Anderson ◽

Rodney C. G. Franklin ◽

Diane E. Spicer

Keyword(s):

Cardiac Surgery ◽

Left Ventricle ◽

Right Ventricle ◽

Congenital Heart ◽

Operative Procedure ◽

Fontan Circulation ◽

Pediatric Cardiac Surgery ◽

Univentricular Heart ◽

Ventricular Mass ◽

The World

The world of pediatric cardiac surgery and cardiac surgery as a whole lost one of the great pioneers with the passing, at the beginning of 2018, of Francis Fontan. Hence to add to the recognition of his achievements, the European Congenital Heart Surgeons Association (ECHSA) has established a lecture to be given in his memory at their annual meetings. It was a significant honor and privilege to be invited to present the initial lecture. In this report, we describe the essence of the presentation. Many patients are now palliated by construction of the Fontan circulation. Very few of those put forward for this operative procedure have anatomically univentricular hearts. It remains frequent, nonetheless, to find accounts of many patients allegedly having “single” ventricles. We discuss the background to this illogical approach to description of hearts having one big and one small ventricle, showing that those with normal hearts have a single left ventricle, albeit co-existing with a single right ventricle. We show that analysis of the ventricular mass in tripartite fashion produces much needed clarity in the appropriate description of the ventricular mass in those increasingly submitted for construction of the Fontan circulation. We emphasize that although it was patients with univentricular atrioventricular connections who were the first to benefit from the procedure, the majority of patients now have biventricular atrioventricular connections, although the hypoplastic ventricle possesses all three of its normal components. We show that description of the ventricular arrangement as being functionally, or physiologically, univentricular provides logic in what had previously been an illogical environment.

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The Influence of Respiration on Blood Flow in the Fontan Circulation: Insights for Imaging-Based Clinical Evaluation of the Total Cavopulmonary Connection

Frontiers in Cardiovascular Medicine ◽

10.3389/fcvm.2021.683849 ◽

2021 ◽

Vol 8 ◽

Author(s):

Séline F. S. van der Woude ◽

Friso M. Rijnberg ◽

Mark G. Hazekamp ◽

Monique R. M. Jongbloed ◽

Sasa Kenjeres ◽

...

Keyword(s):

Blood Flow ◽

Pulmonary Circulation ◽

Fluid Dynamic ◽

Heart Defects ◽

Venous Pressure ◽

Pulmonary Arteries ◽

Fontan Circulation ◽

Univentricular Heart ◽

The Impact ◽

Fontan Patients

Congenital heart disease is the most common birth defect and functionally univentricular heart defects represent the most severe end of this spectrum. The Fontan circulation provides an unique solution for single ventricle patients, by connecting both caval veins directly to the pulmonary arteries. As a result, the pulmonary circulation in Fontan palliated patients is characterized by a passive, low-energy circulation that depends on increased systemic venous pressure to drive blood toward the lungs. The absence of a subpulmonary ventricle led to the widely believed concept that respiration, by sucking blood to the pulmonary circulation during inspiration, is of great importance as a driving force for antegrade blood flow in Fontan patients. However, recent studies show that respiration influences pulsatility, but has a limited effect on net forward flow in the Fontan circulation. Importantly, since MRI examination is recommended every 2 years in Fontan patients, clinicians should be aware that most conventional MRI flow sequences do not capture the pulsatility of the blood flow as a result of the respiration. In this review, the unique flow dynamics influenced by the cardiac and respiratory cycle at multiple locations within the Fontan circulation is discussed. The impact of (not) incorporating respiration in different MRI flow sequences on the interpretation of clinical flow parameters will be covered. Finally, the influence of incorporating respiration in advanced computational fluid dynamic modeling will be outlined.

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Spinal surgery in the univentricular heart – is it viable?

Cardiology in the Young ◽

10.1017/s1047951112002235 ◽

2013 ◽

Vol 24 (1) ◽

pp. 73-78 ◽

Cited By ~ 4

Author(s):

C. Pérez-Caballero Macarrón ◽

E. Sobrino Ruiz ◽

J. Burgos Flores ◽

JL. Vázquez Martínez ◽

A. Coca Pérez ◽

...

Keyword(s):

Hospital Stay ◽

Spinal Surgery ◽

Anaesthetic Management ◽

Massive Bleeding ◽

Fontan Circulation ◽

Univentricular Heart ◽

Scoliosis Surgery ◽

Post Operative Period ◽

The Mean ◽

Fontan Physiology

AbstractIntroductionThe management of patients with Fontan physiology who undergo scoliosis surgery is difficult. The purpose of this article was to describe our experience in the management of patients with Fontan circulation undergoing spinal surgery for correction of scoliosis.Materials and methodsThis was a retrospective study including patients with Fontan physiology who underwent spinal orthopaedic surgery. Anaesthetic management, post-operative complications, paediatric intensive care unit and total hospital stay, and the need for blood transfusions were analysed.ResultsWe identified eight children with Fontan physiology who had undergone spinal surgery from 2000 to 2010. All patients were receiving cardiac medications at the time of spinal surgery. The mean age at surgery was 14.8 years (range 12–21). In all, three patients needed inotropic support with dopamine (3, 5, and 8 μg/kg/min), which was started during surgery. During the immediate post-operative period, one patient died because of hypovolaemic shock caused by massive bleeding and dysrythmia. Mean blood loss during the post-operative period was 22.2 cc/kg (7.8–44.6). Surgical drainages were maintained for a mean time of 3 days (range 1–7). The mean hospital stay was 9.2 days (range 6–19). Pleural effusions developed in two patients. On follow-up, one patient presented with thoracic pseudarthrosis and another with a serohaematoma of the surgical wound.ConclusionsSpinal surgery in patients with Fontan circulation is a high-risk operation. These patients must be managed by a specialised team.

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