Fate of Patients With Single Ventricles Who Do Not Undergo the Fontan Procedure

Zheng, W.; Lee, Melissa; d’Udekem, Yves

doi:10.1016/j.athoracsur.2021.02.011

Cited by 17 publications

(11 citation statements)

References 36 publications

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“…Moreover, it was established that the trabeculations found in ET are closer to the normal human setting than to the axolotl setting. A few case series of DILV survivors are described in the literature so far [7][8][9] and cardiac imaging studies of single ventricle amphibians have been conducted 13 , however, this is the first study to directly compare human functionally ventricular hearts to those of the axolotl. Analyses of blood gasses [36][37][38] , injection of X-ray contrast 39 , and UV-light activated fluorescence 12 have been used previously to show degrees of streaming in the salamander and anuran amphibian ventricles.…”

Section: Discussionmentioning

confidence: 99%

“…Fontan patients suffer from the long-term consequences of this un-physiological circulation, which severely limits their quality of life and often leads to an untimely death 5,6 . Some patients with functionally univentricular hearts live without the standard Fontan palliation [7][8][9] . Long-term survival is probably related to favorable intraventricular streaming of blood flows with disparate oxygen saturation and a pulmonary artery stenosis 10 .Functionally univentricular hearts resemble the setting in the amphibians, where there is a single ventricle only [11][12][13] .…”

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confidence: 99%

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Opportunities and short-comings of the axolotl salamander heart as a model system of human single ventricle and excessive trabeculation

Lauridsen

Pedersen

Andersson

et al. 2022

Sci Rep

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Few experimental model systems are available for the rare congenital heart diseases of double inlet left ventricle (DILV), a subgroup of univentricular hearts, and excessive trabeculation (ET), or noncompaction. Here, we explore the heart of the axolotl salamander (Ambystoma mexicanum, Shaw 1789) as model system of these diseases. Using micro-echocardiography, we assessed the form and function of the heart of the axolotl, an amphibian, and compared this to human DILV (n = 3). The main finding was that both in the axolotl and DILV, blood flows of disparate oxygen saturation can stay separated in a single ventricle. In the axolotl there is a solitary ventricular inlet and outlet, whereas in DILV there are two separate inlets and outlets. Axolotls had a lower resting heart rate compared to DILV (22 vs. 72 beats per minute), lower ejection fraction (47 vs. 58%), and their oxygen consumption at rest was higher than peak oxygen consumption in DILV (30 vs. 17 ml min−1 kg−1). Concerning the ventricular myocardial organization, histology showed trabeculations in ET (n = 5) are much closer to the normal human setting than to the axolotl setting. We conclude that the axolotl heart resembles some aspects of DILV and ET albeit substantial species differences exist.

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Section: Discussionmentioning

confidence: 99%

mentioning

confidence: 99%

Opportunities and short-comings of the axolotl salamander heart as a model system of human single ventricle and excessive trabeculation

Lauridsen

Pedersen

Andersson

et al. 2022

Sci Rep

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“…For patients referred at any subsequent steps towards a univentricular pathway, after Norwood, bidirectional Glenn or Fontan completion, the possibility for a bi-ventricular conversion is ruled out only after all investigations have denied this surgical option (45)(46)(47)(48)(49)(50)(51)(52)(53).…”

Section: Review Methodsmentioning

confidence: 99%

A narrative review of modern approach and outcomes evaluation in congenital heart defects

Corno¹,

LaPar²,

Wen³

et al. 2021

Transl Pediatr

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Objective: The purpose of this review is to highlight critical advances and innovative approaches to the most challenging clinical situations in congenital heart surgery, to establish a new perspective from which to evaluate current clinical practice patterns and contemporary United States program ranking systems. Background:The past decades have witnessed substantial advances in the treatment of congenital heart defects. New strategies are deeply rooted in calculated risk-taking innovations. Pioneer surgeons developed, improved and refined critical operative skills and techniques to optimize cardiovascular physiology, decrease operative mortality and improve clinical outcomes.Methods: Unfortunately, in the modern surgical era, supportive environments to allow surgeons to make similar gains and innovative contributions remain scarce. In the current practice, overall procedure volume is prioritized to safeguard quality metrics, including hospital survival and length of stay, surgical complications, and neurocognitive outcomes. As a result, exceptional surgical results have become translated and defined by public ranking systems such as the US News and World Report Best Children's Hospital National Ranking (USNWR) and the Congenital Heart Surgery Database of the Society for Thoracic Surgeons (CHSD-STS), primary based upon early post-operative mortality. This reality places surgeons in a vulnerable position where pressure to achieve a high clinical ranking contrasts with a surgeon definition of "acceptable" surgical risk. Currently, the most frequently used risk stratification tools do not factor in important differences in strategies, such as staged palliation versus complete repair, or bi-ventricular versus uni-ventricular physiology. This favors hospitals pursuing multistage surgical approaches, even if the result is worse long-term morbidity, mortality and increased resource utilization. This economy of ranking-based decision-making causes surgeons either avoid operating altogether or accept less advantageous multi-staged treatment strategies for patients with elevated expected mortality. Such an environment also might present much farther-reaching negative impacts on the growth and development of junior surgeons and trainees, as well as on the pursuit of new surgical innovations to aid future generations of patients.Conclusions: Risk aversive surgical behavior is creating an environment not favorable for the children born with truly complex congenital heart defects.

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“…The simplest final product of the cardiac dysfunction in single ventricle is expressed by cyanosis and dyspnea [1][2][3][4][5][6]28]. It is likely that the pulmonary vascular resistance are variable in different functional states, especially in frogs and salamanders, with the resulting QP:QS, ration between the pulmonary blood flow (QP) and systemic blood flow (QS), depending upon variable circumstances and environments [7,24], as in human beings with single ventricle.…”

Section: Excessively Trabeculated Ventricular Chambermentioning

confidence: 99%

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confidence: 99%

Single ventricle: amphibians and human beings

2022

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Survival to adult life in patients born with "functionally" single ventricle, without any surgical treatment, as natural history, is extremely rarely reported [1][2][3][4][5][6][7]. In general, these group of patients with "functionally" single ventricle are symptomatic from the first weeks of life, and require two to three staged operations to obtain that the single ventricle pumps the oxygenated blood to the systemic circulation, while the less oxygenated blood is deviated by gravity from the superior and inferior vena cava directly to the lungs [1,8,9]. The result of this artificial "Fontan circulation" is high systemic venous pressure, and the chronic elevation of the systemic venous pressure may results in liver failure, renal failure, protein-losing enteropathy, and plastic bronchitis [10][11][12][13][14][15][16][17]. As a consequence, the long-term surgical outcomes of these patients are complicated by the combination of heart failure and cyanosis, substantially reducing their life expectancy and severely compromising their quality of life [10][11][12][13][14][15][16][17][18][19]. Mathematical and computational fluid dynamic models were used to study the blood flow distribution in single ventricle [20,21], and a better design of circulation, altering the traditional surgical options [22]. Research could greatly benefit from studies performed on animals born with single ventricle, even if the only animal models available in nature are amphibians, like axolotl salamander (Ambystoma mexicanum) [7] and frogs (Xenopus laevis) [23,24], and reptiles.These experimental studies on amphibians, performed with echocardiography and cardiac magnetic resonance imaging, revealed that salamanders and frogs have two elements in common in their double inlet form of single ventricle: intact interatrial septum and excessively trabeculated ventricular chamber.

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Fate of Patients With Single Ventricles Who Do Not Undergo the Fontan Procedure

Cited by 17 publications

References 36 publications

Opportunities and short-comings of the axolotl salamander heart as a model system of human single ventricle and excessive trabeculation

Opportunities and short-comings of the axolotl salamander heart as a model system of human single ventricle and excessive trabeculation

A narrative review of modern approach and outcomes evaluation in congenital heart defects

Single ventricle: amphibians and human beings

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