Clinical Importance of Fontan Circuit Thrombus in the Adult Population: Significant Association With Increased Risk of Cardiovascular Events

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 as...

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Bibliographic Details
Published in:Canadian journal of cardiology 2019-12, Vol.35 (12), p.1807-1814
Main Authors: Sathananthan, Gnalini, Johal, Niall, Verma, Tony, Sandhu, Sherry, Chakrabarti, Shantabanu, Riahi, Mounir, Human, Derek, Leipsic, Jonathon, Grewal, Jasmine
Format: Article
Language:English
Subjects:
Adolescent
Adult
British Columbia
Cause of Death
Cohort Studies
Echocardiography - methods
Female
Fontan Procedure - adverse effects
Fontan Procedure - methods
Heart Defects, Congenital - diagnostic imaging
Heart Defects, Congenital - surgery
Humans
Kaplan-Meier Estimate
Magnetic Resonance Imaging, Cine - methods
Male
Middle Aged
Prognosis
Pulmonary Embolism - diagnostic imaging
Pulmonary Embolism - etiology
Pulmonary Embolism - mortality
Pulmonary Embolism - therapy
Retrospective Studies
Risk Assessment
Survival Analysis
Thromboembolism - diagnostic imaging
Thromboembolism - etiology
Thromboembolism - mortality
Thromboembolism - therapy
Ventricular Dysfunction - diagnostic imaging
Ventricular Dysfunction - etiology
Ventricular Dysfunction - mortality
Young Adult
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Summary: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. 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. 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. 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. 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 cardiovasculaire
ISSN:0828-282X
1916-7075
DOI:10.1016/j.cjca.2019.08.038