Fontan Circulation

Goldberg, David J.; Paridon, Stephen M.

doi:10.1161/circulationaha.114.013365

Cited by 10 publications

(6 citation statements)

References 24 publications

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“…Venous blood is diverted from the vena cava to the pulmonary artery bypassing the single ventricle. The success of this operation has resulted in an increasing population of adults living longer with congenital heart disease [1]. …”

Section: Introductionmentioning

confidence: 99%

“…The main limitation of the Fontan circulation is the absence of a sub-pulmonary ventricle. Blood flow through the pulmonary vasculature depends on the gradient between central venous pressure and ventricular end-diastolic pressure, as well as a low resistance to flow across the pulmonary vascular bed (pulmonary vascular resistance) [1]. …”

Section: Introductionmentioning

confidence: 99%

“…Eventually there is a decrease in exercise capacity, functional status and an increase in heart failure-related hospital admissions with an increase in mortality [1]. …”

Section: Introductionmentioning

confidence: 99%

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The use of Macitentan in Fontan circulation: a case report

Demetriades

Aziz

Condliffe

et al. 2017

BMC Cardiovasc Disord

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BackgroundThe Fontan circulation, a result of a palliative procedure in patients with single systemic ventricles, is defined by chronically elevated pulmonary vascular resistance. When traditional heart failure therapies fail, pharmacological agents that reduce pulmonary artery pressures may be used. These include endothelial-receptor antagonists, prostanoids and phosphodiesterase type 5 inhibitors. We report the first use of macitentan, an endothelin-receptor antagonist, in a patient with a Fontan circulation.Case presentationWe describe the case of a 50 year old female with tricuspid atresia and transposition of the great arteries. Following complex surgery as a child, she subsequently underwent a fenestrated modified atrial pulmonary Fontan operation which was later converted to a total cavopulmonary anastomosis Fontan circulation. Due to failure of various medications to relieve her worsening symptoms, she was commenced on macitentan in April 2016. Few months later, she demonstrated a significant symptomatic improvement and associated increase in her incremental shuttle walking test distance.ConclusionsMacitentan has slower receptor dissociation kinetics compared to other endothelin-receptor antagonists, leading to enhanced pharmacological activity with promising effects in patients with pulmonary arterial hypertension. The patient we report has shown considerable improvement in exercise capacity following introduction of this medication and thus we suggest further randomised trials to establish the role of different endothelin-receptor antagonists in the management of the Fontan circulation.

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Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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The use of Macitentan in Fontan circulation: a case report

Demetriades

Aziz

Condliffe

et al. 2017

BMC Cardiovasc Disord

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“…Third, although the potential impact of pulmonary vasodilators on Fontan physiology has been evaluated, no study to date has addressed the essential question of therapy for Fontan patients: will treatment with pulmonary vasodilators alter the long-term outcomes of patients with this imperfect physiology? 44 In addition, whether chronic daily use of pulmonary vasodilators will reduce or eliminate the risk of late consequences after Fontan surgery is a question that must be answered. 45 Therefore, larger sample sizes and RCTs with long-term follow-up are needed to evaluate the efficacy and safety of pulmonary vasodilator therapy in post-Fontan patients.…”

Section: Discussionmentioning

confidence: 99%

The efficacy and safety of pulmonary vasodilators in patients with Fontan circulation: a meta‐analysis of randomized controlled trials

Wang

Liao

et al. 2019

Pulm. circ.

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No previous meta-analysis has evaluated the efficacy and safety of pulmonary vasodilators in Fontan physiology. Recent relative trials have obtained conflicting results regarding improvements in peak oxygen consumption; the relatively small number of patients in each study may be a limiting factor. We aimed to evaluate the efficacy and safety of pulmonary vasodilators in Fontan patients. Relevant studies were identified by searching the PubMed, Embase, and Cochrane Library databases. Pooled outcomes were determined to assess the efficacy and safety of pulmonary vasodilators in Fontan patients. Nine randomized controlled studies involving 381 patients with Fontan circulation were included. Pulmonary vasodilator therapy led to significant improvement (mean difference = −0.39, 95% CI: [−0.72, −0.05]) in the New York Heart Association (NYHA) functional class. The 6-minute walking distance (6MWD) was significantly increased by 134 m (95% CI: [86.07, 181.94]), and the peak VO2 was also significantly improved (mean difference = 1.42 ml·(kg·min)-1, 95% CI: [0.21, 2.63]). Additionally, the mean pulmonary artery pressure (mPAP) was significantly reduced (mean difference = −2.25 mmHg, 95% CI: [−3.00, −1.50]). No significant change was found in mortality or in brain natriuretic peptide (BNP) or N-terminal pronatriuretic peptide (NT-proBNP). Four studies reported no side effects and good drug tolerance, and two studies reported mild adverse effects. The present meta-analysis indicated that pulmonary vasodilators (primarily the PDE-5 inhibitor and endothelin-1 receptor antagonist) significantly improved the hemodynamics of Fontan patients, reduced the NYHA functional class and increased the 6MWD. The peak oxygen consumption was also improved. No significant change was observed in mortality or in the BNP or NT-proBNP level. Overall, the pulmonary vasodilators were well tolerated. This finding needs to be confirmed in future studies.

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“…The Fontan procedure shows perioperative success, but suffers from several long-term complications 2,14,17,18,29 . These long-term complications and comorbidities are correlated to TCPC hemodynamics 10,12,16,20,23,25,27,30,37 . The fact that a relationship exists between Fontan hemodynamics and patient outcomes promotes the modeling and study of TCPC hemodynamics to improve patient quality of life.…”

Section: Introductionmentioning

confidence: 99%

Using a Novel In Vitro Fontan Model and Condition-Specific Real-Time MRI Data to Examine Hemodynamic Effects of Respiration and Exercise

et al. 2017

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Several studies exist modeling the Fontan connection to understand its hemodynamic ties to patient outcomes (Chopski in: Experimental and Computational Assessment of Mechanical Circulatory Assistance of a Patient-Specific Fontan Vessel Configuration. Dissertation, 2013; Khiabani et al. in J Biomech 45:2376-2381, 2012; Taylor and Figueroa in Annu Rev Biomed 11:109-134, 2009; Vukicevic et al. in ASAIO J 59:253-260, 2013). The most patient-accurate of these studies include flexible, patient-specific total cavopulmonary connections. This study improves Fontan hemodynamic modeling by validating Fontan model flexibility against a patient-specific bulk compliance value, and employing real-time phase contrast magnetic resonance flow data. The improved model was employed to acquire velocity field information under breath-held, free-breathing, and exercise conditions to investigate the effect of these conditions on clinically important Fontan hemodynamic metrics including power loss and viscous dissipation rate. The velocity data, obtained by stereoscopic particle image velocimetry, was visualized for qualitative three-dimensional flow field comparisons between the conditions. Key hemodynamic metrics were calculated from the velocity data and used to quantitatively compare the flow conditions. The data shows a multi-factorial and extremely patient-specific nature to Fontan hemodynamics.

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Fontan Circulation

Cited by 10 publications

References 24 publications

The use of Macitentan in Fontan circulation: a case report

The use of Macitentan in Fontan circulation: a case report

The efficacy and safety of pulmonary vasodilators in patients with Fontan circulation: a meta‐analysis of randomized controlled trials

Using a Novel In Vitro Fontan Model and Condition-Specific Real-Time MRI Data to Examine Hemodynamic Effects of Respiration and Exercise

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