Key result
Combining biomarkers, echo, CMR, and cath makes diastolic dysfunction diagnosis feasible in failing Fontan circulation.
Why the study?
Diastolic dysfunction contributes to circulatory failure in Fontan patients, but challenges remain in diagnosing it due to unique single-ventricle anatomy and physiology.
This review highlights that diagnosing diastolic dysfunction in adults with failing Fontan circulation is feasible using a combination of noninvasive and invasive modalities, despite the challenges posed by single ventricle physiology.
Supports multimodal evaluation in failing Fontan; leaves open standardization and outcome validation.
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. • Diastolic dysfunction is known as a contributor to Fontan circulatory insufficiency or failure. • Diastolic dysfunction in Fontan patients leads to worse prognosis and worse long-term clinical outcomes during adulthood. • Diastolic dysfunction is usually occult in the setting of a Fontan circulation, masked by the underfilled state of the single ventricle. Therefore, its prevalence is still unclear due to the challenges that carry its diagnosis. • Diagnosing FFC due to diastolic dysfunction is feasible through a combination of noninvasive methods including biomarkers, echocardiography, and cardiac MRI, as well as invasive techniques such as cardiac catheterization.
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Khayyat et al. (2025) conducted a review in Failing Fontan circulation with diastolic dysfunction. Diagnostic methods (biomarkers, echocardiography, cardiac MRI, catheterization) was evaluated. Diagnosing diastolic dysfunction in adults with failing Fontan circulation is feasible using a combination of biomarkers, echocardiography, cardiac MRI, and cardiac catheterization.
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