The Fontan circulation: who controls cardiac output?

Gewillig, Marc; Brown, Stephen C.; Eyskens, Bénédicte; Heying, Ruth; Gáname, Javier; Budts, Werner; Gerche, André La; Gorenflo, Matthias

doi:10.1510/icvts.2009.218594

Cited by 248 publications

(198 citation statements)

References 57 publications

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“…Thus, the Fontan patient is unable to increase cardiac output to the extent that a biventricular circulation can ( Figure 5). (16) There is therefore a built in limitation and fragility to the Fontan physiology, which becomes important when considering the late complications that patients may face.…”

Section: A B C Figure 2: the Fontan Operation And Its Modifi Cationsmentioning

confidence: 99%

Management of the Fontan circulation in the adult patient

Clift

2017

SAHJ

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Section: A B C Figure 2: the Fontan Operation And Its Modifi Cationsmentioning

confidence: 99%

Management of the Fontan circulation in the adult patient

Clift

2017

SAHJ

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“…Izrazito disfunkcionalan ventrikul, osobito ako je po anatomskoj građi desni, znači nevolju. Dijastolička je disfunkcija pogubna, ali ona može biti i posljedica nedostatnog punjenja 61 .…”

Section: Zatajivanje Srca U Posebnim Uvjetimaunclassified

“…A markedly dysfunctional ventricle, especially anatomically right one, is a calamity. Diastolic dysfunction is detrimental, but it may be due to limited preload itself 61 .…”

Section: Zatajivanje Srca U Posebnim Uvjetimamentioning

confidence: 99%

Heart Failure in Adults with Congenital Heart Disease: An Emerging Challenge.

Šmalcelj¹,

Strozzi²,

Malčić³

et al. 2016

Cardiol Croat.

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SAŽETAK: Rastuća populacija mladih bolesnika s kompleksnim prirođenim srčanim greškama (PSG) koji su odrasli zahvaljujući zahtjevnim kardiokirurškim operacijama u ranome djetinjstvu pojavljuje se kao zbunjujući izazov za suvremenu kardiologiju. To se odnosi ponajprije na bolesnike s: 1. operativno korigiranom Fallotovom tetralogijom i posljedičnom teškom insuficijencijom pulmonalne valvule; 2. jednom klijetkom i Fontanovim krvotokom; 3. kompletnom transpozicijom velikih arterija palijativno operiranom skretanjem krvi na atrijskoj razini; 4. kongenitalno korigiranom transpozicijom velikih arterija; 5. Eisenmengerovim sindromom. Zatajivanje desne klijetke zajednički je nazivnik za sva ta stanja, dok je u onom pod 3. i 4., često i pod 2., dekompenzirana desna klijetka ujedno i sistemna. Jako izobličena anatomija u kompleksnim PSG uzrokuje hemodinamiku zatajivanja srca (ZS) jako različitu od "uobičajene", ali vjerojatno sa sličnim sistemnim odgovorom. Vremenski tijek ZS-a uz PSG jako se razlikuje od uobičajenog. Pojavljuje se mnogo ranije, a jako je ovisan o kirurškim i perkutanim intervencijama. Kirurške operacije, često više palijativne nego korektivne, odgađaju ZS s nagovještajem skore smrti iz djetinjstva u ranu ili srednju odraslu dob, ali pravi je izazov kako ga odgoditi dalje nakon što se mogućnosti kirurških i perkutanih intervencija iscrpe. Zatajivanje srca uz PSG opire se standardnim konceptima farmakološkog liječenja. Učinkovitost antagonista reninsko-angiotenzinskoaldosteronskog sustava i beta-blokatora nije provjerena. Antiaritmički su lijekovi u jako izobličenim srcima uglavnom neučinkoviti, ali ablativne intervencije i elektrostimulacija koji popravljaju hemodinamiku mogu biti korisni. Napredni pulmonalni vazodilatatori preporodili su liječenje Eisenmegerova sindroma ranije smatranog neizlječivim. Raznolikost PSG-a priječi definiranje jedinstvenih koncepata liječenja. U individualiziranom kliničkom pristupu treba imati na umu specifična hemodinamska stanja, ali su očito potrebni i novi načini liječenja: farmakološki, intervencijski i kirurški.SUMMARY: A growing population of young heart failure (HF) patients with complex congenital heart disease, who have survived into adulthood owing to sophisticated cardiac surgery in infancy, emerges as a challenging quandary of contemporary cardiology. This new population primarily includes patients with: 1) repaired tetralogy of Fallot with consequent severe pulmonary valve regurgitation, 2) univentricular heart and Fontan circulation, 3) complete transposition of great arteries palliated by atrial switch surgery, 4) congenitally corrected transposition of great arteries, and 5) Eisenmenger syndrome. Right ventricular failure is a common denominator of all those entities, while in items 3 and 4, and often also in 2, the failing right ventricle is the systemic one. The grossly distorted anatomy of complex congenital heart disease, even if palliated by an early and often staged surgery, results in peculiar HF hemodynamics grossly aberrant from norm, but probably wi...

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“…Known strategies aiming to ''normalize'' these secondary changes are very unlikely to yield a good clinical result, and will cause great frustration to the clinician. Interpretation of the numerous abnormal findings thus requires a profound knowledge of the Fontan physiology [8].…”

Section: Fontan Physiology Pvr and Exercisementioning

confidence: 99%

“…At worst (red line), the output is severely reduced at rest and barely augments during exercise. Adapted from [8] (Color figure online) theoretical treatment is to avoid excessive volume overload, thereby avoiding dilation and overgrowth, and avoiding excessive unloading [9].…”

Section: Fontan Physiology Pvr and Exercisementioning

confidence: 99%

The Fontan Circulation: The Known, the Unknown and the Plausible

Louw

Gewillig

2013

Curr Pediatr Rep

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The Fontan circulation is used as palliative surgery in the functionally univentricular heart. Due to the advances made in prenatal diagnosis, cardiac surgery, technology, and monitoring in complex congenital heart disease, survival rates have improved significantly. For this group of patients, the physiology and pathology remain challenging and eluding, even more so in the long-term follow-up. In the last decade pulmonary vascular resistance has been shown to be of utmost importance regarding cardiac output both at rest and during exercise. The aim of this article is to review the most recently published data, namely new concepts and our current understanding regarding the physiology; performance during exercise; the value of imaging modalities; and chronic liver pathology in the Fontan circulation.

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The Fontan circulation: who controls cardiac output?

Cited by 248 publications

References 57 publications

Management of the Fontan circulation in the adult patient

Management of the Fontan circulation in the adult patient

Heart Failure in Adults with Congenital Heart Disease: An Emerging Challenge.

The Fontan Circulation: The Known, the Unknown and the Plausible

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