Integrated Clinical and Magnetic Resonance Imaging Assessments Late After Fontan Operation

Clair, Nicole St.; Powell, A.; Geva, Tal; Rathod, Rahul H.

doi:10.1016/j.jacc.2021.03.312

Cited by 31 publications

(18 citation statements)

References 24 publications

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“…However, other than in healthy controls, the rise in ventricular Ees was blunted which demonstrates reduced contractile reserve, a phenomenon that might be related to adverse intrinsic myocardial remodelling characterized by focal and diffuse fibrotic changes. Considering the prognostic relevance of strain measurements in single ventricle patients, 18 the application of CMR tissue tracking to quantify strain and strain rate during exercise would give further insights into myocardial performance. This technique, however, relies on adequate spatial and temporal resolution to ensure correct endocardial tracking but was not sufficient with the used RT sequence in our study.…”

Section: Discussionmentioning

confidence: 99%

Exercise-dependent changes in ventricular–arterial coupling and aortopulmonary collateral flow in Fontan patients: a real-time CMR study

Latus

Hofmann

Gummel

et al. 2022

European Heart Journal - Cardiovascular Imaging

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Aims Inefficient ventricular–arterial (V–A) coupling has been described in Fontan patients and may result in adverse haemodynamics. A varying amount of aortopulmonary collateral (APC) flow is also frequently present that increases volume load of the single ventricle. The aim of the study was to assess changes in V–A coupling and APC flow during exercise CMR. Methods and results Eighteen Fontan patients (age 24 ± 3 years) and 14 controls (age 23 ± 4 years) underwent exercise CMR using a cycle ergometer. Ventricular volumetry and flow measurements in the ascending aorta (AAO), inferior (IVC), and superior (SVC) vena cava were assessed using real-time sequences during stepwise increases in work load. Measures of systemic arterial elastance Ea, ventricular elastance Ees, and V–A coupling (Ea/Ees) were assessed. APC flow was quantified as AAO − (SVC + IVC). Ea remained unchanged during all levels of exercise in both groups (P = 0.39 and P = 0.11). Ees increased in both groups (P = 0.001 and P < 0.001) with exercise but was lower in the Fontan group (P = 0.04). V–A coupling was impaired in Fontan patients at baseline (P = 0.04). Despite improvement during exercise (P = 0.002) V–A coupling remained impaired compared with controls (P = 0.001). Absolute APC flow in Fontan patients did not change during exercise even at maximum work load (P = 0.98). Conclusions Inefficient V–A coupling was already present at rest in Fontan patients and aggravated during exercise due to a limited increase in ventricular contractility which demonstrates the importance of a limited functional reserve of the single ventricle. APC flow remained unchanged suggesting no further increase in volume load during exercise.

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Section: Discussionmentioning

confidence: 99%

Exercise-dependent changes in ventricular–arterial coupling and aortopulmonary collateral flow in Fontan patients: a real-time CMR study

Latus

Hofmann

Gummel

et al. 2022

European Heart Journal - Cardiovascular Imaging

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“…Longitudinal studies using transthoracic echocardiography have shown progressive ventricular dilation and dysfunction after the Fontan operation; however, the clinical importance of these changes are not well known [ 16 – 19 ]. Prior publications from our institution using cross-sectional cohorts that overlap with the current study have demonstrated relationship between ventricular dilation and dysfunction with transplant-free survival [ 10 , 12 ]. In the current study, over a median follow-up period of 10 years, 14% met the composite outcome and this study reaffirmed the association of this outcome with ventricular dilation and dysfunction.…”

Section: Discussionmentioning

confidence: 82%

“…Routine surveillance testing recommendations include echocardiograms every year and cardiovascular magnetic resonance imaging (CMR) every 2-3 years [4]. Prior cross-sectional studies have established ventricular dilation, dysfunction, and the presence of late gadolinium enhancement (LGE) on CMR as risk factors for adverse clinical outcomes [9][10][11][12].…”

Section: Introductionmentioning

confidence: 99%

Longitudinal changes in ventricular size and function are associated with death and transplantation late after the Fontan operation

Ghelani

Lü

Sleeper

et al. 2022

Journal of Cardiovascular Magnetic Resonance

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Background Cross-sectional studies have reported that ventricular dilation and dysfunction are associated with adverse clinical outcome in Fontan patients; however, longitudinal changes and their relationship with outcome are not known. Methods This was a single-center retrospective analysis of Fontan patients with at least 2 cardiovascular magnetic resonance (CMR) scans without intervening interventions. Serial measures of end-diastolic volume index (EDVI), end-systolic volume index (ESVI), ejection fraction (EF), indexed mass (massi), mass-to-volume ratio, and end-systolic wall stress (ESWS) were used to estimate within-patient change over time. Changes were compared for those with and without a composite outcome (death, heart transplant, or transplant listing) as well as between patients with left (LV) and right ventricular (RV) dominance. Results Data from 156 patients were analyzed with a mean age at 1st CMR of 17.8 ± 9.6 years. 490 CMRs were included with median of 3 CMRs/patient (range 2–9). On regression analysis with mixed effects models, volumes and ESWS increased, while mass, mass-to-volume ratio, and EF decreased over time. With a median follow-up of 10.2 years, 14% met the composite outcome. Those with the composite outcome had a greater increase in EDVI compared to those without (4.7 vs. 0.8 ml/BSA1.3/year). Compared with LV dominance, RV dominance was associated with a greater increase in ESVI (1.4 vs. 0.5 ml/BSA1.3/year), a greater decrease in EF (− 0.61%/year vs. − 0.24%/year), and a higher rate of the composite outcome (21% vs. 8%). Conclusions Ventricles in the Fontan circulation exhibit a steady decline in performance with an increase in EDVI, ESVI, and ESWS, and decrease in EF, mass index, and mass-to-volume ratio. Those with death or need for heart transplantation have a faster increase in EDVI. Patients with rapid increase in EDVI (> 5 ml/BSA1.3/year) may be at a higher risk of adverse outcomes and may benefit from closer surveillance. RV dominance is associated with worse clinical outcomes and remodeling compared to LV dominance.

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“…In a large study of patients with a Fontan circulation who underwent cardiac MRI, lower end-diastolic volume was the strongest predictor of transplant-free survival. For patients with ventricular dilation (end diastolic volume ≥156 ml/BSA), worse global circumferential strain was particularly predictive of death or transplant ( 41 ). On multivariable analysis, end-diastolic volume, end-systolic volume, global circumferential strain, and ventriculoarterial coupling ratio by MRI have been shown to be independently predictive of death or transplant.…”

Section: Modes Of Fontan Circulatory Failurementioning

confidence: 99%

Management of circulatory failure after Fontan surgery

2022

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With improvement in survival after Fontan surgery resulting in an increasing number of older survivors, there are more patients with a Fontan circulation experiencing circulatory failure each year. Fontan circulatory failure may have a number of underlying etiologies. Once Fontan failure manifests, prognosis is poor, with patient freedom from death or transplant at 10 years of only about 40%. Medical treatments used include traditional heart failure medications such as renin-angiotensin-aldosterone system blockers and beta-blockers, diuretics for symptomatic management, antiarrhythmics for rhythm control, and phosphodiesterase-5 inhibitors to decrease PVR and improve preload. These oral medical therapies are typically not very effective and have little data demonstrating benefit; if there are no surgical or catheter-based interventions to improve the Fontan circulation, patients with severe symptoms often require inotropic medications or mechanical circulatory support. Mechanical circulatory support benefits patients with ventricular dysfunction but may not be as useful in patients with other forms of Fontan failure. Transplant remains the definitive treatment for circulatory failure after Fontan, but patients with a Fontan circulation face many challenges both before and after transplant. There remains significant room and urgent need for improvement in the management and outcomes of patients with circulatory failure after Fontan surgery.

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Integrated Clinical and Magnetic Resonance Imaging Assessments Late After Fontan Operation

Cited by 31 publications

References 24 publications

Exercise-dependent changes in ventricular–arterial coupling and aortopulmonary collateral flow in Fontan patients: a real-time CMR study

Exercise-dependent changes in ventricular–arterial coupling and aortopulmonary collateral flow in Fontan patients: a real-time CMR study

Longitudinal changes in ventricular size and function are associated with death and transplantation late after the Fontan operation

Management of circulatory failure after Fontan surgery

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