Hemodynamics of Fontan Failure

Egbe, Alexander C.; Connolly, Heidi M.; Miranda, William R.; Ammash, Naser M.; Hagler, Donald J.; Veldtman, Gruschen; Borlaug, Barry A.

doi:10.1161/circheartfailure.117.004515

Cited by 100 publications

(24 citation statements)

References 30 publications

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“…Finally, the thoracic IVC was replaced with a simulated Dacron shunt to mimic that typically implanted during the surgical procedure, [ 85,86 ] and pulmonary resistances were increased to model pulmonary hypertension, which is highly prevalent in Fontan patients. [ 87 ] The input parameters of this model and the schematic can be found in Table S2 and Figure S5 (Supporting Information), respectively.…”

Section: Resultsmentioning

confidence: 99%

Object‐Oriented Lumped‐Parameter Modeling of the Cardiovascular System for Physiological and Pathophysiological Conditions

Rosalia

Ozturk

Story

et al. 2021

Advcd Theory and Sims

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In this work, a lumped‐parameter Windkessel model of the cardiovascular system that simulates biomechanical parameters of the human physiology is presented. The object‐oriented platform provided by the MATLAB‐based modeling environment SIMSCAPE is employed to compute blood pressures and flows in each heart chamber and at various sites of the vascular tree. The hydraulic domain allows the determination of cardiovascular hemodynamics intuitively from geometrical and mechanical properties of the system, while custom elements model the pumping action of the heart and the effects of respiration on blood flow. The model is validated by comparing predicted hemodynamics with normal physiology during both systole and diastole, demonstrating that changes in arterial pressures with breathing are consistent with reported physiological effects of cardiorespiratory coupling. The capabilities of this platform are explored through two exemplary case studies: i) pressure‐overload heart failure due to aortic constriction, validated in vitro and via finite element analysis, and ii) single‐ventricle Fontan physiology, validated in vitro and compared with the clinical literature. This platform provides a practical tool for the calculation of cardiovascular hemodynamics from hydraulic parameters, enabling the intuitive creation of in silico representations of complex circulatory loops, the planning and optimization of medical interventions, and the prediction of clinically relevant patient‐specific hemodynamics.

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Section: Resultsmentioning

confidence: 99%

Object‐Oriented Lumped‐Parameter Modeling of the Cardiovascular System for Physiological and Pathophysiological Conditions

Rosalia

Ozturk

Story

et al. 2021

Advcd Theory and Sims

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“… 11 , 12 Published studies have variably used FCF as a composite endpoint to include death, transplantation or listing for transplantation. 11 , 13 Additionally, the development of patient‐experienced exercise intolerance, inability to carry out daily life activities and Fontan takedown have sometimes been included in the definition of FCF. 2 , 14 , 15 , 16 Other variables included within the spectrum of FCF were heart failure symptoms and other co‐morbidities including arrhythmias, thromboembolism, protein‐losing enteropathy (PLE), plastic bronchitis (PB), oedema, persistent effusions and liver dysfunction.…”

Section: Terms and Definitionsmentioning

confidence: 99%

Reaching consensus for unified medical language in Fontan care

Alsaied

Rathod

Aboulhosn³

et al. 2021

ESC Heart Failure

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AimsThe Fontan operation has resulted in improved survival in patients with single-ventricle congenital heart disease. As a result, there is a growing population of teenagers and adults with a Fontan circulation. Many co-morbidities have been increasingly recognized in this population due to the unique features of the Fontan circulation. Standardization of how Fontan co-morbid conditions are defined will help facilitate understanding, consistency and interpretability of research and clinical experience. Unifying common language usage in Fontan is a critical precursor step for data comparison of research findings and clinical outcomes and ultimately accelerating improvements in management for this growing group of patients. This manuscript aimed to create unified definitions for morbidities seen after the Fontan palliation. Methods In association of many congenital heart disease organizations, this work used Delphi methodology to reach a broad consensus among recognized experts regarding commonly used terms in Fontan care and research. Each definition underwent at least three rounds of revisions to reach a final definition through surveys sent to experts in the field of single-ventricle care. Results The process of reaching a consensus on multiple morbidities associated with the Fontan procedure is summarized in this manuscript. The different versions that preceded reaching the consensus are also presented in the Supporting Information. Table 1 represents the final definitions according to the consensus. Conclusions We propose the use of these definitions for clinical care, future research studies, registry development and clinical trials.

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“…Microvascular occlusion is common, as are arteriovenous malformations, leading to erroneous trans-pulmonary gradients. Although difficult to evaluate, elevated PVR is a key mechanism in FFP and unmasking can occur post-OHT following restoration of pulsatile flow with detrimental effects on right ventricular function (33,34). Early studies demonstrated higher post-transplant mortality with elevated © Annals of Cardiothoracic Surgery.…”

Section: Pulmonary Vascular Resistance (Pvr)mentioning

confidence: 99%

Transplantation in the single ventricle population

Kenny¹,

DeRita²,

Nassar³

et al. 2018

Ann. Cardiothorac. Surg.

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The single ventricle patient population comprises the most complex cohort presenting to the cardiac transplant team, in terms of demographics, anatomic substrate, and unique physiology. It is also the most rapidly growing diagnostic group presenting for heart transplantation. In this manuscript, we aim to describe the changing landscape of transplantation in single ventricle conditions through reflection on our own institution's practice and experience, alongside contemporary literature review. Single ventricle patients are heterogeneous in terms of age, anatomic diagnosis and physiology according to surgical stage of repair.Progress in surgical palliative strategies has impacted upon the present composition of the population, with growing numbers of hypoplastic left heart syndrome patients and those with late physiology failure following Fontan completion. Multiple prior surgeries, immunological sensitivity and multi-organ involvement impart high peri-operative risk but can be mitigated in part by careful pre-operative planning by a dedicated multidisciplinary team addressing issues such as planning of concurrent reconstructive surgery, minimizing the post-operative effect of collaterals, timely harvesting, oversizing of donor organs to minimize graft failure, and strategies to address anticipated post-operative elevation in pulmonary vascular resistance. Determining optimal timing for transplant in these patients remains unclear, but understanding the risk of alternative surgical options can help guide decision making with regards to listing.

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Hemodynamics of Fontan Failure

Cited by 100 publications

References 30 publications

Object‐Oriented Lumped‐Parameter Modeling of the Cardiovascular System for Physiological and Pathophysiological Conditions

Object‐Oriented Lumped‐Parameter Modeling of the Cardiovascular System for Physiological and Pathophysiological Conditions

Reaching consensus for unified medical language in Fontan care

Transplantation in the single ventricle population

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