Abstract
Background
The impact of Fontan circuit thrombus is poorly understood. The objectives of this
study were to determine (1) the incidence of Fontan circuit thrombus and proportion
of silent thrombus; (2) any association between Fontan circuit thrombus and markers
of Fontan circulatory dysfunction; and (3) the association of Fontan circuit thrombus
with adverse cardiac outcomes.
Methods
We conducted a retrospective review of adult patients who underwent the Fontan procedure
(aged > 18 years) followed at St. Paul’s Hospital who underwent cardiac computed tomography
or magnetic resonance imaging assessment (n = 67). Fontan circulatory dysfunction
markers included clinical heart failure, N-terminal pro-brain natriuretic peptide,
ventricular dysfunction, atrioventricular valvular regurgitation, refractory arrhythmias,
declining exercise capacity, and hepatic/renal dysfunction. Adverse cardiac outcomes
were death, heart transplantation, or surgery for Fontan revision or atrioventricular
valve replacement.
Results
Fontan circuit thrombus was present in 15 of 67 patients (22%): 41% (7/17) classic/modified
Fontan and 16% (8/50) total cavopulmonary connection. Incidence was 36% among those
suspected to have Fontan circuit thrombus; 14% in those with no clinical/echocardiographic
suspicion; and clinically silent in 40% diagnosed with Fontan thrombus. The time from
Fontan surgery to Fontan circuit thrombus diagnosis was 22 ± 6 years in the classic/modified
group vs 14 ± 8 years in the total cavopulmonary connection group (P = 0.03. Fontan circuit thrombus was associated with adverse cardiac outcomes (27%
[4/15] vs 8% [4/52], P = 0.02), but there was no difference in Fontan circulatory dysfunction markers.
Conclusion
Given the incidence of Fontan circuit thrombus and association with adverse cardiac
outcomes, routine surveillance of the Fontan circuit should strongly be considered.
The identification of thrombus should lead to anticoagulation implementation/optimization,
along with screening/intervention for reversible Fontan circulatory issues in an attempt
to prevent adverse cardiac outcomes.
Résumé
Contexte
Les répercussions d’une thrombose du circuit de Fontan sont mal comprises. L’étude
visait à déterminer : 1) l’incidence de la thrombose du circuit de Fontan et la proportion
de thromboses silencieuses; 2) l’existence d’un lien entre la thrombose du circuit
de Fontan et les marqueurs d’une dysfonction circulatoire du circuit de Fontan; et
3) le lien entre la thrombose du circuit de Fontan et les issues cardiovasculaires
indésirables.
Méthodologie
Nous avons réalisé une revue rétrospective des cas des patients adultes (18 ans ou
plus) qui ont subi une intervention de Fontan à l’hôpital St. Paul et fait l’objet
d’un suivi comprenant un examen par tomodensitométrie cardiaque ou par imagerie par
résonance magnétique (n = 67). Les marqueurs de la dysfonction circulatoire du circuit
de Fontan comprenaient l’insuffisance cardiaque clinique, le propeptide natriurétique
de type B N-terminal, la dysfonction ventriculaire, la régurgitation valvulaire auriculoventriculaire,
l’arythmie réfractaire, le déclin de la capacité à l’effort et la dysfonction hépatique/rénale.
Les issues cardiovasculaires indésirables étaient le décès, la greffe cardiaque ou
une intervention chirurgicale visant à corriger le circuit de Fontan ou à remplacer
la valve auriculoventriculaire.
Résultats
Une thrombose du circuit de Fontan était présente chez 15 (22 %) des 67 patients :
7 (41 %) des 17 patients ayant un circuit de Fontan classique/modifié et 8 (16 %)
des 50 patients ayant une connexion cavopulmonaire totale. L’incidence s’établissait
à 36 % chez les patients chez qui une thrombose du circuit de Fontan était soupçonnée
et à 14 % chez ceux chez qui on ne soupçonnait aucune anomalie clinique/échographique;
la thrombose était silencieuse chez 40 % des patients ayant reçu un diagnostic de
thrombose du circuit de Fontan. L’intervalle entre l’intervention de Fontan et le
diagnostic de thrombose du circuit de Fontan était de 22 ± 6 ans chez les patients
ayant un circuit de Fontan classique/modifié et de 14 ± 8 ans chez les patients ayant
une connexion cavopulmonaire totale (p = 0,03). La thrombose du circuit de Fontan a été associée à des issues cardiovasculaires
indésirables (27 % [4/15] vs 8 % [4/52], p = 0,02), mais il n’y avait pas de différence entre les marqueurs de dysfonction circulatoire
du circuit de Fontan.
Conclusion
Compte tenu de l’incidence de la thrombose du circuit de Fontan et du lien établi
avec les issues cardiovasculaires indésirables, nous recommandons vivement une surveillance
de routine du circuit de Fontan. Si une thrombose est détectée, il convient d’instaurer
une anticoagulothérapie ou d’optimiser le traitement anticoagulant déjà prescrit et
de procéder à un dépistage ou, s’il y a lieu, à une intervention à l’égard des problèmes
circulatoires réversibles du circuit de Fontan afin de prévenir les issues cardiovasculaires
indésirables.
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Article info
Publication history
Published online: September 05, 2019
Accepted:
August 30,
2019
Received:
May 23,
2019
Footnotes
See page 1814 for disclosure information.
See editorial by Laflamme and Roche, pages 1631--1634 of this issue.
Identification
Copyright
© 2019 Canadian Cardiovascular Society. Published by Elsevier Inc. All rights reserved.
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Access this article on ScienceDirectLinked Article
- Fontan Circuit Thrombus in Adults: Often Silent, Rarely InnocentCanadian Journal of CardiologyVol. 35Issue 12
- PreviewA Fontan circulation is a surgically modified vasculature with pathways created to separate oxygenated and deoxygenated blood and to permit function with only a single ventricular pump. In effect, systemic venous blood reaches the lungs passively. Achieving a Fontan circulation can require several surgeries or interventional procedures and has become the usual approach for diagnoses such as tricuspid atresia, hypoplastic left heart syndrome, double inlet left ventricle, or an unbalanced atrioventricular septal defect.
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