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Canadian Journal of Cardiology

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

Published:September 05, 2019DOI:https://doi.org/10.1016/j.cjca.2019.08.038

      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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      References

        • Fontan F.
        • Baudet E.
        Surgical repair of tricuspid atresia.
        Thorax. 1971; 26: 240-248
        • Van Arsdell G.S.
        • McCrindle B.W.
        • Einarson K.D.
        • et al.
        Interventions associated with minimal Fontan mortality.
        Ann Thorac Surg. 2000; 70: 568-574
        • Downing T.E.
        • Allen K.Y.
        • Glatz A.C.
        • et al.
        Long-term survival after the Fontan operation: twenty years of experience at a single center.
        J Thorac Cardiovasc Surg. 2017; 154: 243-253.e242
        • Tomkiewicz-Pajak L.
        • Hoffman P.
        • Trojnarska O.
        • et al.
        Abnormalities in blood coagulation, fibrinolysis, and platelet activation in adult patients after the Fontan procedure.
        J Thorac Cardiovasc Surg. 2014; 147: 1284-1290
        • Egbe A.C.
        • Connolly H.M.
        • Niaz T.
        • et al.
        Prevalence and outcome of thrombotic and embolic complications in adults after Fontan operation.
        Am Heart J. 2017; 183: 10-17
        • Balling G.
        • Vogt M.
        • Kaemmerer H.
        • et al.
        Intracardiac thrombus formation after the Fontan operation.
        J Thorac Cardiovasc Surg. 2000; 119: 745-752
        • Coon P.D.
        • Rychik J.
        • Novello R.T.
        • et al.
        Thrombus formation after the Fontan operation.
        Ann Thorac Surg. 2001; 71: 1990-1994
        • Tsang W.
        • Johansson B.
        • Salehian O.
        • et al.
        Intracardiac thrombus in adults with the Fontan circulation.
        Cardiol Young. 2007; 17: 646-651
        • Sughimoto K.
        • Okauchi K.
        • Zannino D.
        • et al.
        Total cavopulmonary connection is superior to atriopulmonary connection Fontan in preventing thrombus formation: computer simulation of flow-related blood coagulation.
        Pediatr Cardiol. 2015; 36: 1436-1441
        • Grewal J.
        • Al Hussein M.
        • Feldstein J.
        • et al.
        Evaluation of silent thrombus after the Fontan operation.
        Congenit Heart Dis. 2013; 8: 40-47
        • Udink Ten Cate F.E.
        • Hannes T.
        • Germund I.
        • et al.
        Towards a proposal for a universal diagnostic definition of protein-losing enteropathy in Fontan patients: a systematic review.
        Heart. 2016; 102: 1115-1119
        • Elder R.W.
        • McCabe N.M.
        • Hebson C.
        • et al.
        Features of portal hypertension are associated with major adverse events in Fontan patients: the VAST study.
        Int J Cardiol. 2013; 168: 3764-3769
        • Yuan R.
        • Shuman W.P.
        • Earls J.P.
        • et al.
        Reduced iodine load at CT pulmonary angiography with dual energy monochromatic imaging: comparison with standard CT pulmonary angiography - a prospective randomized trial.
        Radiology. 2012; 262: 290-297
        • Raju R.
        • Thompson A.G.
        • Lee K.
        • et al.
        Reduced iodine load with CT coronary angiography using dual-energy imaging: A prospective randomized trial compared with standard coronary CT angiography.
        J Cardiovasc Comput Tomogr. 2014; 8: 282-288
        • Potter B.J.
        • Leong-Sit P.
        • Fernandes S.M.
        • et al.
        Effect of aspirin and warfarin therapy on thromboembolic events in patients with univentricular hearts and Fontan palliation.
        Int J Cardiol. 2013; 168: 3940-3943
        • Marrone C.
        • Galasso G.
        • Piccolo R.
        • et al.
        Antiplatelet versus anticoagulation therapy after extracardiac conduit Fontan: a systematic review and meta-analysis.
        Pediatr Cardiol. 2011; 32: 32-39
        • Khairy P.
        • Fernandes S.M.
        • Mayer Jr., J.E.
        • et al.
        Long-term survival, modes of death, and predictors of mortality in patients with Fontan surgery.
        Circulation. 2008; 117: 85-92
        • Man B.L.
        • Cheung Y.F.
        Plasma brain natriuretic peptide and systemic ventricular function in asymptomatic patients late after the Fontan procedure.
        Heart Vessels. 2007; 22: 398-403
        • Wilson T.G.
        • d'Udekem Y.
        • Winlaw D.S.
        • et al.
        Hepatic and renal end-organ damage in the Fontan circulation: a report from the Australian and New Zealand Fontan Registry.
        Int J Cardiol. 2018; 273: 100-107
        • Lee D.
        • Levin A.
        • Kiess M.
        • et al.
        Chronic kidney damage in the adult Fontan population.
        Int J Cardiol. 2018; 257: 62-66
        • Broda C.R.
        • Sriraman H.
        • Wadhwa D.
        • et al.
        Renal dysfunction is associated with higher central venous pressures in patients with Fontan circulation.
        Congenit Heart Dis. 2018; 13: 602-607

      Linked Article

      • Fontan Circuit Thrombus in Adults: Often Silent, Rarely Innocent
        Canadian Journal of CardiologyVol. 35Issue 12
        • Preview
          A 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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