Diastolic Heart Failure in Patients With the Fontan Circulation

Budts, Werner; Ravekes, William; Danford, David A.; Kutty, Shelby

doi:10.1001/jamacardio.2019.5459

Cited by 49 publications

(44 citation statements)

References 36 publications

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“…Diastolic dysfunction is an under-recognised aetiology of Fontan circulatory failure. 3 ECV is therefore a promising non-invasive measure of adverse cardiac remodelling in Fontan patients.…”

Section: Discussionmentioning

confidence: 99%

“… 1 2 Diastolic dysfunction is increasingly recognised as a clinical or subclinical driver of Fontan circulatory insufficiency or even Fontan failure. 3 In the absence of validated non-invasive measures, direct invasive ventricular and Fontan circuit pressure measurement, with or without manoeuvres to unmask occult circulatory dysfunction, is the gold standard to diagnose diastolic dysfunction. 4 Fibrosis is a common final pathway in chronic myocardial disease, including in diastolic heart failure.…”

Section: Introductionmentioning

confidence: 99%

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Myocardial fibrosis, diastolic dysfunction and elevated liver stiffness in the Fontan circulation

et al. 2020

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IntroductionSingle ventricle diastolic dysfunction and hepatic fibrosis are frequently observed in patients with a Fontan circulation. The relationship between adverse haemodynamics and end-organ fibrosis has not been investigated in adolescents and young adults with Fontan circulation.MethodsProspective observational study of Fontan patients who had a cardiac catheterisation. Cardiac MRI with T1 mapping was obtained to measure extracellular volume (ECV), a marker of myocardial fibrosis. Hepatic magnetic resonance elastography was performed to assess liver shear stiffness. Serum biomarkers of fibrosis including matrix metalloproteinases (MMPs) and tissue inhibitor of metalloproteinases (TIMPs) were measured. Very high ECV was defined as >30% and elevated serum biomarkers as >75th percentile for each biomarker.Results25 Fontan patients (52% female) with mean age of 16.3±6.8 years were included. Mean ECV was 28%±5%. There was a significant correlation between ECV and systemic ventricular end-diastolic pressure (r=0.42, p=0.03) and between ECV and liver stiffness (r=0.45, p=0.05). Patients with elevated ECV demonstrated elevations in MMPs and TIMPs. Similarly, patients with elevated MMPs and TIMPs had greater liver stiffness compared with patients with normal levels of these biomarkers.ConclusionsIn Fontan patients, cardiac magnetic resonance evidence of myocardial fibrosis is associated with diastolic dysfunction, increased liver stiffness and elevated circulating biomarkers of fibrosis. These findings suggest the presence of a profibrotic milieu, with end-organ implications, in some patients with Fontan circulation.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Myocardial fibrosis, diastolic dysfunction and elevated liver stiffness in the Fontan circulation

et al. 2020

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“…25,31,37,39,40 The physiology behind diastolic dysfunction in the Fontan circulation is complex and research is ongoing to better understand all the contributing factors. 41 In many ways, it may mirror what is seen in the chronically underfilled left ventricle of mitral stenosis, which in turn becomes fibrotic with impaired diastolic properties. 42 Thus, with faster heart rates, there is less filling, and therefore the chronotropic incompetence observed in Fontan patients paradoxically may help augment ventricular preload during exertion.…”

Section: Structural Intervention On the Pulmonary Arteriesmentioning

confidence: 99%

The pulmonary vascular bed in patients with functionally univentricular physiology and a Fontan circulation

et al. 2021

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Fontan palliation represents one of the most remarkable surgical advances in the management of individuals born with functionally univentricular physiology. The operation secures adult survival for all but a few with unfavourable anatomy and/or physiology. Inherent to the physiology is passive transpulmonary blood flow, which produces a vulnerability to adequate filling of the systemic ventricle at rest and during exertion. Similarly, the upstream effects of passive flow in the lungs are venous congestion and venous hypertension, especially marked during physical activity. The pulmonary vascular bed has emerged as a defining character on the stage of Fontan circulatory behaviour and clinical outcomes. Its pharmacologic regulation and anatomic rehabilitation therefore seem important strategic therapeutic targets. This review seeks to delineate the important aspects of pulmonary artery development and maturation in functionally univentricular physiology patients, pulmonary artery biology, pulmonary vascular reserve with exercise, and pulmonary artery morphologic and pharmacologic rehabilitation.

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“…The guidelines differentiate between heart failure with reduced ejection fraction (HFREF) and heart failure with preserved ejection fraction (HFPEF), also known as diastolic dysfunction. The presence of HFPEF and its role in ACHD patients is not well investigated, although in specific cohorts such as Fontan patients some research is becoming available [30]. Diagnosis of heart failure in ACHD should consist of a comprehensive evaluation of signs and symptoms, diagnostic testing, and medical history, as advised by the 2016 ESC Guidelines for the diagnosis and treatment of acute and chronic heart failure.…”

Section: Diagnosis Of Heart Failure In Achdmentioning

confidence: 99%

Heart failure in congenital heart disease: management options and clinical challenges

Leusveld

Kauling

Geenen

et al. 2020

Expert Review of Cardiovascular Therapy

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Introduction: The population of adults with congenital heart disease (ACHD) is rapidly expanding and one of the major complications is heart failure. Timely diagnosis and treatment are crucial, but strong evidence for effectiveness of heart failure treatment in ACHD is currently lacking. Components of the medical history, physical examination, and further diagnostic tests including ECG, echocardiography, cardiac magnetic resonance imaging, exercise testing, and biomarkers can identify patients at risk for early mortality or heart failure. Areas covered: Although the number of studies guiding evidence-based treatment are expanding, many clinical questions have not been completely answered yet. Therefore, in this review we provide an overview of current available insights in epidemiology, diagnosis, risk stratification, and treatment options in ACHD patients, including non-medical therapies and advanced care planning. Expert opinion: We strongly advocate expanding current use of biomarkers in the diagnostic process and timely initiation of discussing advanced treatment options and advanced care planning with patients and their loved ones. More research in multi-center collaborations is needed to study all aspects of care of adult congenital heart disease patients.

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Diastolic Heart Failure in Patients With the Fontan Circulation

Abstract: Drs Budts and Kutty had full access to all of the data in the study and take responsibility for the integrity of the data and the accuracy of the data analysis. Concept and design: Budts, Kutty.

Cited by 49 publications

References 36 publications

Myocardial fibrosis, diastolic dysfunction and elevated liver stiffness in the Fontan circulation

Myocardial fibrosis, diastolic dysfunction and elevated liver stiffness in the Fontan circulation

The pulmonary vascular bed in patients with functionally univentricular physiology and a Fontan circulation

Heart failure in congenital heart disease: management options and clinical challenges

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