The neuroendocrinal system of the heart after construction of a Glenn anastomosis or the Fontan circulation

Many complex cardiac defects produce a functionally single ventricle, in which there is mixing of the systemic and pulmonary circulations. The output from the functionally single ventricle is divided between the two circulations: the proportion going to the systemic and pulmonary vascular beds being determined by the relative resistance to flows within the respective circulations.

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Single Ventricle—A Comprehensive Review

Children ◽

10.3390/children8060441 ◽

2021 ◽

Vol 8 (6) ◽

pp. 441

Author(s):

P. Syamasundar Rao

Keyword(s):

Single Ventricle ◽

Management Strategies ◽

Fontan Circulation ◽

Cardiac Defects ◽

Comprehensive Review ◽

Single Ventricle Physiology ◽

Alternative Approaches

In this paper, the author enumerates cardiac defects with a functionally single ventricle, summarizes single ventricle physiology, presents a summary of management strategies to address the single ventricle defects, goes over the steps of staged total cavo-pulmonary connection, cites the prevalence of inter-stage mortality, names the causes of inter-stage mortality, discusses strategies to address the inter-stage mortality, reviews post-Fontan issues, and introduces alternative approaches to Fontan circulation.

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Developing an adolescent and adult Fontan Management Programme

Cardiology in the Young ◽

10.1017/s104795112100175x ◽

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.

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Flow profile characteristics in Fontan circulation are associated with the single ventricle dilation and function: principal component analysis study

AJP Heart and Circulatory Physiology ◽

10.1152/ajpheart.00686.2019 ◽

2020 ◽

Vol 318 (5) ◽

pp. H1032-H1040 ◽

Cited By ~ 1

Author(s):

Michal Schäfer ◽

Benjamin S. Frank ◽

Stephen M. Humphries ◽

Kendall S. Hunter ◽

Katherine L. Carmody ◽

...

Keyword(s):

Single Ventricle ◽

Principal Component ◽

Ventricular Volume ◽

Fontan Circulation ◽

Collateral Flow ◽

Right Pulmonary Artery ◽

Flow Through ◽

The Right ◽

And Function ◽

Flow Waveforms

The Fontan circulation is characterized as a nonpulsatile flow propagation without a pressure-generating ventricle. However, flow through the Fontan circulation still exhibits oscillatory waves as a result of pressure changes generated by the systemic single ventricle. Identification of discrete flow patterns through the Fontan circuit may be important to understand single ventricle performance. Ninety-seven patients with Fontan circulation underwent phase-contrast MRI of the right pulmonary artery, yielding subject-specific flow waveforms. Principal component (PC) analysis was performed on preprocessed flow waveforms. Principal components were then correlated with standard MRI indices of function, volume, and aortopulmonary collateral flow. The first principal component (PC) described systolic versus diastolic-dominant flow through the Fontan circulation, accounting for 31.3% of the variance in all waveforms. The first PC correlated with end-diastolic volume ( R = 0.34, P = 0.001), and end-systolic volume ( R = 0.30, P = 0.003), cardiac index ( R = 0.51, P < 0.001), and the amount of aortopulmonary collateral flow ( R = 0.25, P = 0.027)—lower ventricular volumes and a smaller volume of collateral flow—were associated with diastolic-dominant cavopulmonary flow. The second PC accounted for 19.5% of variance and described late diastolic acceleration versus deceleration and correlated with ejection fraction—diastolic deceleration was associated with higher ejection fraction. Principal components describing the diastolic flow variations in pulmonary arteries are related to the single ventricle function and volumes. Particularly, diastolic-dominant flow without late acceleration appears to be related to preserved ventricular volume and function, respectively. NEW & NOTEWORTHY The exact physiological significance of flow oscillations of phasic and temporal flow variations in Fontan circulation is unknown. With the use of principal component analysis, we discovered that flow variations in the right pulmonary artery of Fontan patients are related to the single ventricle function and volumes. Particularly, diastolic-dominant flow without late acceleration appears to be related to more ideal ventricular volume and systolic function, respectively.

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Imaging complex congenital heart disease — functional single ventricle, the Glenn circulation and the Fontan circulation: A multimodality approach

Progress in Pediatric Cardiology ◽

10.1016/j.ppedcard.2009.10.003 ◽

2010 ◽

Vol 28 (1-2) ◽

pp. 45-58 ◽

Cited By ~ 6

Author(s):

David W. Brown ◽

Andrew J. Powell ◽

Tal Geva

Keyword(s):

Congenital Heart Disease ◽

Heart Disease ◽

Congenital Heart ◽

Single Ventricle ◽

Fontan Circulation ◽

Complex Congenital Heart Disease ◽

Multimodality Approach

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The Fontan outcomes network: first steps towards building a lifespan registry for individuals with Fontan circulation in the United States

Cardiology in the Young ◽

10.1017/s1047951120001869 ◽

2020 ◽

Vol 30 (8) ◽

pp. 1070-1075 ◽

Cited By ~ 2

Author(s):

Tarek Alsaied ◽

Kiona Y. Allen ◽

Jeffrey B. Anderson ◽

Julia S. Anixt ◽

David W. Brown ◽

...

Keyword(s):

Congenital Heart ◽

Single Ventricle ◽

Emotional Health ◽

The United States ◽

Fontan Circulation ◽

Systematic Design ◽

Neurodevelopmental Outcomes ◽

New Learning ◽

Outcomes For Children

AbstractThe Fontan Outcomes Network was created to improve outcomes for children and adults with single ventricle CHD living with Fontan circulation. The network mission is to optimise longevity and quality of life by improving physical health, neurodevelopmental outcomes, resilience, and emotional health for these individuals and their families. This manuscript describes the systematic design of this new learning health network, including the initial steps in development of a national, lifespan registry, and pilot testing of data collection forms at 10 congenital heart centres.

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Total cavopulmonary connection for functionally single ventricle with pulmonary atresia and abnormal arborization of pulmonary arteries - exclusion of overwhelmed area by collateral arteries from Fontan circulation

Interactive Cardiovascular and Thoracic Surgery ◽

10.1510/icvts.2007.174367 ◽

2008 ◽

Vol 7 (6) ◽

pp. 1180-1182 ◽

Cited By ~ 2

Author(s):

M. Nagashima ◽

N. Hibino ◽

E. Yamamoto ◽

T. Higaki

Keyword(s):

Single Ventricle ◽

Pulmonary Arteries ◽

Pulmonary Atresia ◽

Fontan Circulation ◽

Total Cavopulmonary Connection ◽

Collateral Arteries ◽

Cavopulmonary Connection

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Implantation of a Berlin Heart as Single Ventricle By-Pass on Fontan Circulation in Univentricular Heart Failure

ASAIO Journal ◽

10.1097/mat.0b013e31815a2500 ◽

2007 ◽

Vol 53 (6) ◽

pp. e1-e2 ◽

Cited By ~ 30

Author(s):

Davide F. Calvaruso ◽

Salvatore Ocello ◽

Nicoletta Salviato ◽

Diego Guardì ◽

David F. Petruccelli ◽

...

Keyword(s):

Heart Failure ◽

Single Ventricle ◽

Fontan Circulation ◽

Univentricular Heart ◽

Berlin Heart

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Fontan circulation and implications for future reproduction

Obstetric Medicine ◽

10.1177/1753495x16676727 ◽

2016 ◽

Vol 10 (3) ◽

pp. 104-106

Author(s):

Niharika Mehta ◽

Srilakshmi Mitta

Keyword(s):

Single Ventricle ◽

Fontan Procedure ◽

Fontan Circulation ◽

Cardiac Complications ◽

Preconception Counseling ◽

Passive Flow ◽

History Of ◽

Future Reproduction ◽

Long Term Prognosis

This article summarizes and critiques four recent publications looking at preconception counseling, pregnancy outcomes, and cardiac complications in women with history of Fontan circulation. The Fontan procedure is a palliative strategy for single-ventricle type congenital heart disease and involves passive flow of venous return into the pulmonary circulation, bypassing the ventricles. Pregnancy in these patients is not without risk and preconception counseling and contraception practices vary widely. High rates of miscarriage, prematurity, and small-for-gestational-age babies are reported. Cardiac complications include mainly arrhythmias. Whether long-term prognosis in these patients is affected by pregnancy is not yet known.

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