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 59 publications

(49 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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“…Although abnormal pre-operative RV diastolic profiles were associated with difficult separation from CPB (41), the independent (20). Patients with single ventricle palliation are predisposed to diastolic ventricular dysfunction although the pathophysiology remains poorly understood and early detection is difficult (42). Diastolic and systolic dysfunction in the single ventricle physiology are thought to be closely intertwined (42).…”

Section: Rv Diastolic Dysfunctionmentioning

confidence: 99%

Peri-operative right ventricular dysfunction—the anesthesiologist’s view

Navaratnam¹,

DiNardo²

2020

Cardiovasc Diagn Ther

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Successful pre-operative assessment of RV function requires a multi-modal approach with trending of clinical symptoms and signs, hematological, hemodynamic, and imaging parameters (2). Clinical profileRight heart failure (RHF) as strictly defined is " a clinical syndrome due to an alteration of structure and/or function of the right heart circulatory system that leads to sub-optimal delivery of blood flow (high or low) to the pulmonary circulation and/or elevated venous pressures at rest or with exercise" (3). Consequently, while RV systolic dysfunction is an important cause of RHF, RHF can be present in the absence of RV systolic dysfunction due to volume or pressure overload lesions or RV diastolic dysfunction.Assessment of recent clinical changes is important in assessing disease progression. Symptoms and signs of Editorial Commentary on Right Ventricular Dysfunction

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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 59 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

Peri-operative right ventricular dysfunction—the anesthesiologist’s view

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