Abstract
Background
To describe long-term survival and cardiovascular events in adult patients with single
ventricle physiology (SVP) without Fontan palliation, focusing on predictors of mortality
and comparing groups according to their cardiovascular physiology.
Methods
Multicentre observational and retrospective study including adult patients with SVP
without Fontan palliation since their first adult clinic visit. The cohort was subdivided
into 3 groups: Eisenmenger, restricted pulmonary flow, and aortopulmonary shunt. Death
was considered as the main end point. Other clinical outcomes occurring during follow-up
were considered as secondary end points.
Results
A total of 146 patients, mean age 32.5 ± 11.1 years, were analysed. Over a mean follow-up
of 7.3 ± 4.1 years, 33 patients (22.6%) died. Survival was 86% and 74% at 5 and 10
years, respectively. Right ventricular morphology was not associated with higher mortality.
Four variables at baseline were related to a higher mortality: at least moderate atrioventricular
valve regurgitation, platelet count < 150 × 103/mm3, GFR < 60 mL/min/1.73 m2, and QRS > 120 ms). A total of 34.2% of patients were admitted to the hospital due
to heart failure, and 7.5% received a heart transplant. Other cardiovascular outcomes
were also frequent: atrial arrhythmias in 19.2%, stroke in 15.1%, and pacemaker/implantable
cardioverter-defibrillator in 6.2%/2.7%.
Conclusions
Adult patients with SVP who had not undergone Fontan exhibit a high mortality rate
and frequent major cardiovascular events. At least moderate atrioventricular valve
regurgitation, thrombocytopenia, renal dysfunction, and QRS duration > 120 ms at baseline
visit allow identification of a cohort of patients at higher risk of mortality.
Résumé
Objectif
Décrire la survie à long terme et les événements cardiovasculaires chez les patients
adultes présentant une physiologie à ventricule unique (PVU) sans intervention de
Fontan, en se concentrant sur les prédicteurs de mortalité et en comparant les groupes
en fonction de leur physiologie cardiovasculaire.
Méthodes
Étude observationnelle et rétrospective, multicentrique, incluant des patients adultes
atteints de PVU sans intervention de Fontan depuis leur première visite en clinique
adulte. La cohorte a été subdivisée en trois groupes : syndrome d'Eisenmenger, flux
pulmonaire restreint ou fenêtre aorto-pulmonaire. Le décès a été considéré comme le
critère principal d'évaluation. Les autres observations cliniques survenues au cours
du suivi ont été considérées comme des critères secondaires.
Résultats
Un total de 146 patients, d'un âge moyen de 32,5 ± 11,1 ans, a été considéré pour
l’analyse. Sur un suivi moyen de 7,3 ± 4,1 ans, 33 patients (22,6 %) sont décédés.
La survie était de 86 % et 74 % à 5 et 10 ans, respectivement. La morphologie du ventricule
droit n'était pas associée à une mortalité plus élevée. Quatre variables initiales
étaient liées à une mortalité plus élevée : régurgitation au moins modérée de la valve
auriculo-ventriculaire, numération plaquettaire < 150 × 103/mm3, DFG < 60 ml/min/1,73 m2 et QRS > 120 ms). Au total, 34,2 % des patients ont été admis à l'hôpital en raison
d'une insuffisance cardiaque, et 7,5 % ont reçu une transplantation cardiaque. D'autres
conséquences cardiovasculaires étaient également fréquentes : arythmies auriculaires
dans 19,2 % des cas, accident vasculaire cérébral dans 15,1 % des cas et stimulateur
cardiaque/ défibrillateur cardioverteur implantable dans 6,2 % / 2,7 % des cas.
Conclusions
Les patients adultes atteints de PVU qui n'ont pas subi d'intervention de Fontan présentent
un taux de mortalité élevé et des événements cardiovasculaires majeurs fréquents.
Une régurgitation au moins modérée de la valve auriculo-ventriculaire, une thrombocytopénie,
une dysfonction rénale et une durée du QRS > 120 ms lors de la visite initiale permettent
de distinguer une cohorte de patients présentant un risque de mortalité plus élevé.
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Article info
Publication history
Published online: June 08, 2021
Accepted:
June 2,
2021
Received:
December 9,
2020
Footnotes
See editorial by Massarella and Oechslin, pages 858–861 of this issue.
See page 1119 for disclosure information.
Identification
Copyright
© 2021 Canadian Cardiovascular Society. Published by Elsevier Inc. All rights reserved.
ScienceDirect
Access this article on ScienceDirectLinked Article
- The “Forgotten Ones”: The Natural and Unnatural History of Univentricular Physiology Without Fontan PalliationCanadian Journal of CardiologyVol. 38Issue 7
- PreviewUniventricular heart (UVH) refers to a subset of congenital cardiac defects characterised by 1 hypoplastic ventricle ill-equipped to handle 50% of combined ventricular output and thus rendering biventricular repair unfeasible. Univentricular physiology (UVP) includes those with a Fontan-type palliation and those without a Fontan-type palliation. Those without a Fontan palliation remain cyanotic due to an intracardiac shunt and can live into adulthood without cardiac intervention.1-3 A plethora of publications about risk factors for the long-term outcome in patients with a Fontan circulation contrasts with the paucity of data on adults with a univentricular circulation without a Fontan palliation (Table 1).
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