Visualization of the intracavitary blood flow in systemic ventricles of Fontan patients by contrast echocardiography using particle image velocimetry

<p>Abstract</p> <p>Background</p> <p>Flow patterns in univentricular hearts may have clinical value. Therefore, it is our objective to asses and characterize vortex flow patterns with Fontan circulation in comparison with healthy controls.</p> <p>Methods</p> <p>Twenty-three patients (8 Fontan and 15 normal patients) underwent echocardiography with intravenous contrast agent (Sonovue^®) administration. Dedicated software was used to perform particle image velocimetry (PIV) and to visualize intracavitary flow in the systemic ventricles of the patients. Vortex parameters including vortex depth, length, width, and sphericity index were measured. Vortex pulsatility parameters including relative strength, vortex relative strength, and vortex pulsation correlation were also measured.</p> <p>Results</p> <p>The data from this study show that it is feasible to perform particle velocimetry in Fontan patients. Vortex length (VL) was significantly lower (0.51 ± 0.09 vs 0.65 ± 0.12, <it>P </it>= 0.010) and vortex width (VW) (0.32 ± 0.06 vs 0.27 ± 0.04, <it>p </it>= 0.014), vortex pulsation correlation (VPC) (0.26 ± 0.25 vs -0.22 ± 0.87, <it>p </it>= 0.05) were significantly higher in Fontan patients. Sphericity index (SI) (1.66 ± 0.48 vs 2.42 ± 0.62, <it>p </it>= 0.005), relative strength (RS) (0.77 ± 0.33 vs 1.90 ± 0.47, <it>p </it>= 0.0001), vortex relative strength (VRS) (0.18 ± 0.13 vs 0.43 ± 0.14, <it>p </it>= 0.0001) were significantly lower in the Fontan patients group.</p> <p>Conclusions</p> <p>PIV using contrast echocardiography is feasible in Fontan patients. Fontan patients had aberrant flow patterns as compared to normal hearts in terms of position, shape and sphericity of the main vortices. The vortex from the Fontan group was consistently shorter, wider and rounder than in controls. Whether vortex characteristics are related with clinical outcome is subject to further investigation.</p

Atrial Septal Defect Type Secundum and Eisenmenger Syndrome

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FUELing the Search for Medical Therapies in Late Fontan Failure

The Fontan operation has become the final step in the palliation of many patients with single ventricle disease1. Although outcome has undoubtedly improved over the years, late failure is common and notoriously difficult to treat2, 3. In the face of increasing numbers of young failing Fontan patients with heart transplant as the only, high risk exit strategy, the quest for medical therapies to prevent decline and/or treat Fontan failure has reached a new level of urgency. In this issue of Circulation , Goldberg et al. report on their experience with udenafil, a long-acting phosphodiesterase type 5 inhibitor, in 400 adolescents with a Fontan circulation4. The authors have to be congratulated for completing a long awaited large prospective, multicenter, placebo-controlled trial, which showed a small, non-significant improvement in peak oxygen consumption (primary aim) and multiple significant but limited improvements at the ventilatory anaerobic threshold (secondary aims). Although essentially a nearly negative study, and with the clinical relevance of the changes observed still uncertain, this work nevertheless provides invaluable information for clinical practice and further research.status: publishe

The peculiar challenges of breathing and exercising with a Fontan circulation

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Providing a framework of principles for conceptualising the Fontan circulation

The Fontan operation remains the final palliation for thousands of patients with complex congenital heart disease. By creating a Fontan circuit, control of cardiac output and congestion is wrested away from the ventricle and new haemodynamic forces take control. Understanding how to control the flow in the Fontan circuit will enable clinicians to improve patient management and possibly prevent future complications. CONCLUSION: This review proposes a framework of principles to conceptualise the functionality and limitations of a Fontan circulation.status: publishe

Does physical functioning as assessed by SF-36 predict peak oxygen uptake in patients with atrial septal defect type secundum?

Background: Patients with congenital heart disease overestimate their exercise capacity as measured by cardiopulmonary exercise testing because of long-term adaptation. Most patients with atrial septal defect (ASD) type secundum present with New York Heart Association (NYHA) classification I or II, making it difficult to estimate actual exercise capacity. Therefore, a perceived health status questionnaire (SF-36) may be more sensitive to detect physical and mental limitations in ASD patients. This study investigated the correlation between perceived health status and actual exercise capacity in open and closed ASDs. Methods: At the outpatient clinic of adult congenital heart disease, 33 patients (20 female, median age 32.8 years, range 18-74 years) with ASD type secundum were selected to be included in the study. Twelve patients had open ASD (4 female, median age 28.7 years, range 18-74 years), 21 patients (16 female, median age 33.2 years, range 19 - 70 years) had repaired ASD. Age and gender matched controls from a database of normals were selected for each subgroup. All of the patients completed a perceived health status questionnaire. Afterwards, a symptom-limited, incremental cardiopulmonary exercise test using an upright cycle ergometer was performed. Maximal exercise capacity was assessed by peak oxygen consumption (vO2max). Results: Both patients with open (vO2max 25,5 versus 36,7 mL/min.kg, P = 0.019) and repaired ASD (vO2max 26,1 versus 33,6 mL/min.kg, P = 0,004) showed limitations at exercise when compared to age and gender-matched controls. Only physical functioning (r = 0.795, P = 0.003) and role limitation (r = 0.777, P = 0.005) showed significant correlation with vO2max in patients with open ASD. Physical functioning correlated less with v02max in patients with repaired ASD (r = 0.552, P = 0.012), and did not reach statistical significance after adjustment for multiple testing. Conclusions: Both patients with open and repaired ASD type secundum presented with impaired exercise capacity. Peak oxygen consumption correlated with self-reported physical functioning in patients with open ASD. SF-36 was sensitive to detect reductions in physical functioning in patients with open ASD.status: publishe