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Review
. 2025 Jun;4(6 Pt 1):101758.
doi: 10.1016/j.jacadv.2025.101758. Epub 2025 May 14.

Diagnosis of Diastolic Dysfunction in Adults With Failing Fontan Circulation

Ibrahim M Khayyat  1 , Maria Victoria Ordonez  2 , Ariane Marelli  3 , Judith Therrien  4
Affiliations
Review

Diagnosis of Diastolic Dysfunction in Adults With Failing Fontan Circulation

Ibrahim M Khayyat et al. JACC Adv. 2025 Jun.

Abstract

Since the initial Fontan procedure introduced in 1968 for tricuspid atresia, significant advancements have expanded its application to various congenital cardiac anomalies where a biventricular circulation is unattainable. Despite improved survival rates, Fontan circulation tends to fail over time leading to late morbidity and mortality. Diastolic dysfunction is increasingly recognized as a significant contributor to circulatory insufficiency and failure in Fontan patients. This review aims to assess the current evidence for diagnosing diastolic dysfunction in adults with failing Fontan circulation, including biomarkers, echocardiography, cardiac magnetic resonance imaging, and catheterization. While advancements have been made in understanding diastolic dysfunction in single ventricles, challenges remain due to the unique anatomy and physiology of Fontan patients. Future research should focus on refining diagnostic parameters and exploring potential therapies tailored to the distinct needs of this population.

Keywords: diastolic dysfunction; failing Fontan circulation; heart failure with preserved ejection fraction.

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Conflict of interest statement

Funding support and author disclosures The authors have reported that they have no relationships relevant to the contents of this paper to disclose.

Figures

None
Graphical abstract
Figure 1
Figure 1
Fontan Complications AVV = atrioventricular valve; FALD = Fontan associated liver disease; HCC = hepatocellular carcinoma; PH = pulmonary hypertension; PHT = portal hypertension; SVOTO = single ventricle outflow tract obstruction; TED = thromboembolic disease.
Figure 2
Figure 2
Failing Fontan Phenotypes EDP = end-diastolic pressure; FFal = failing Fontan with abnormal lymphatics; FFpEF = failing Fontan with preserved ejection fraction; FFnHeart = failing Fontan with normal heart; FFrEF = failing Fontan with reduced ejection fraction; FP = Fontan pressure; PAWP = pulmonary artery wedge pressure; SV = single ventricle.
Figure 3
Figure 3
Failing Fontan Hemodynamics Scenarios and Its Correlation With Survival CO = cardiac output; other abbreviations as in Figure 2.
Figure 4
Figure 4
Clinical Presentation of Patients With Failing Fontan Circulation
Figure 5
Figure 5
Measurement of the Atrioventricular Systolic to Diastolic Duration Ratio From the Continuous Wave Doppler Signal The atrioventricular systolic to diastolic duration ratio is calculated from the continuous wave Doppler signal of the dominant atrioventricular valve. S:D = systolic to diastolic duration; TC-TO = tricuspid valve closure to tricuspid valve opening; TV = tricuspid valve.
Figure 6
Figure 6
Atrial Strain Measurements in Fontan Patients Using CMR Common atrial contour measurements on (A) 2-chamber view and (C) 4-chamber view. (B) Demonstrates atrial global longitudinal strain curve. (D) Demonstrates atrial strain rate curve. Atrial conduit strain was measured by calculating the difference between reservoir and pump strain. CMR = cardiac magnetic resonance; RV = right ventricle; other abbreviation as in Figure 2.
Figure 7
Figure 7
Proposed Algorithm to Diagnosing Diastolic Dysfunction in Adults With Failing Fontan Circulation BNP = brain natriuretic peptide; LVEDP = left ventricular end-diastolic pressure; LVEF = left ventricle ejection fraction; MMP = matrix metalloproteases; NTproBNP = N-terminal pro–B-type natriuretic peptide; TIMP = tissue inhibitor of metalloproteinase; urinary NAGL = urinary neutrophil gelatinase-associated lipocalin.
Central Illustration
Central Illustration
Diagnosis of Diastolic Dysfunction in Adults With Fontan Circulation Abbreviations as in Figures 2, 3, 6, and 7.
See this image and copyright information in PMC

References

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