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Review
. 2020 Jun 2;9(11):e015737.
doi: 10.1161/JAHA.119.015737. Epub 2020 May 16.

Atrioventricular Valve Regurgitation in Single Ventricle Heart Disease: A Common Problem Associated With Progressive Deterioration and Mortality

Stephanie Y Tseng  1 , Saira Siddiqui  1 , Michael V Di Maria  2 , Garick D Hill  1 , Adam M Lubert  1 , Shelby Kutty  3 , Alexander R Opotowsky  1   4 , Mathias Possner  5 , David L S Morales  1 , James A Quintessenza  1 , Tarek Alsaied  1
Affiliations
Review

Atrioventricular Valve Regurgitation in Single Ventricle Heart Disease: A Common Problem Associated With Progressive Deterioration and Mortality

Stephanie Y Tseng et al. J Am Heart Assoc. .

Abstract

The Fontan procedure has provided patients with single ventricle physiology extended survival into adulthood and in many cases has improved their quality of life. Atrioventricular valve regurgitation (AVVR) is common in single ventricle patients and is associated with increased risk of mortality. AVVR is more common in patients with a systemic tricuspid or common atrioventricular valve but is generally progressive irrespective of underlying valve morphology. AVVR can be attributable to diverse structural and functional abnormalities at multiple levels of the valvar apparatus, as well as ventricular dysfunction and dilation. Multiple imaging modalities including recent advances in 3-dimensional echocardiography and cross-sectional imaging have been used to further understand AVVR. Surgery to address AVVR must be tailored to the underlying mechanism and the timing of surgical repair should be chosen carefully. In this review, we discuss the etiologies, treatment options, surgical timing, and outcomes of valve repair or replacement for AVVR in patients with single ventricle congenital heart disease, with a focus on those with a Fontan circulation as AVVR is associated with increased risk for Fontan failure and mortality. In-depth understanding of the current literature will help guide clinicians in their approach and management of AVVR in this population.

Keywords: Fontan; atrioventricular valve regurgitation; single ventricle congenital heart disease.

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Figures

Figure 1
Figure 1. Atrioventricular valve regurgitation as a mechanism for multi‐organ system involvement in Fontan failure.
Figure 2
Figure 2. Atrioventricular valve regurgitation mechanisms.
Figure 3
Figure 3. Surgical repair techniques for atrioventricular valve regurgitation.
A, Tricuspid valve (TV), partial annuloplasty; (B) TV, commisuroplasty; (C) TV, DeVega annuloplasty; (D) TV, Kay annuloplasty; (E) TV, edge‐to‐edge repair; (F) Common atrioventricular valve, polytetrafluoroethylene bridge repair.
See this image and copyright information in PMC

References

    1. Gersony WM. Fontan operation after 3 decades: what we have learned. Circulation. 2008;117:13–15. - PubMed
    1. Pundi KN, Johnson JN, Dearani JA, Pundi KN, Li Z, Hinck CA, Dahl SH, Cannon BC, O'Leary PW, Driscoll DJ, et al. 40‐year follow‐up after the Fontan operation: long‐term outcomes of 1,052 patients. J Am Coll Cardiol. 2015;66:1700–1710. - PubMed
    1. Iyengar AJ, Winlaw DS, Galati JC, Wheaton GR, Gentles TL, Grigg LE, Justo RN, Radford DJ, Weintraub RG, Bullock A, et al. The extracardiac conduit Fontan procedure in Australia and New Zealand: hypoplastic left heart syndrome predicts worse early and late outcomes. Eur J Cardiothorac Surg. 2014;46:465–473. - PubMed
    1. Diller GP, Kempny A, Alonso‐Gonzalez R, Swan L, Uebing A, Li W, Babu‐Narayan S, Wort SJ, Dimopoulos K, Gatzoulis MA. Survival prospects and circumstances of death in contemporary adult congenital heart disease patients under follow‐up at a large tertiary centre. Circulation. 2015;132:2118–2125. - PubMed
    1. King G, Ayer J, Celermajer D, Zentner D, Justo R, Disney P, Zannino D, d'Udekem Y. Atrioventricular valve failure in Fontan palliation. J Am Coll Cardiol. 2019;73:810–822. - PubMed

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