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The Fontan circulation describes the circulatory state resulting from an operation in congenital heart disease where systemic venous return is directed to the lungs without an intervening active pumping chamber. As survival increases, so too does recognition of the potential health challenges. This document aims to allow clinicians, people with a Fontan circulation, and their families to benefit from consensus agreement about management of the person with a Fontan circulation. The document was crafted with input from a multidisciplinary group of health care providers as well as individuals with a Fontan circulation and families. It is hoped that the shared common vision of long-term wellbeing will continue to drive improvements in care and quality of life in this patient population and eventually translate into improved survival. KEYPOINTS.

Background Quantification of right ventricular (RV) volumes is challenging owing to variable reproducibility and is especially so in congenital heart disease. Cardiac magnetic resonance (CMR) has the ability to more comprehensively survey the entire right ventricle and is currently considered the gold standard. Aims We aimed to determine the inter-observer reproducibility of CMR-derived RV volumes generated by two independent and experienced (SCMR Level III) observers in Tetralogy of Fallot (ToF) patients with varying degrees of RV dilatation. Methods We performed a retrospective analysis of 120 consecutive patients with repaired ToF who underwent CMR. Two blinded observers calculated RV volumes in each oblique short axis slice independently. Bland-Altman analysis and inter-observer correlation coefficients (ICC) were assessed. Results The coefficients of variation for RV parameters were: 2.9%, 8% and 3.4% for right ventricular end diastolic volume (RVEDV), right ventricular end systolic volume (RVESV) and right ventricular ejection fraction (RVEF) respectively. For RVEDV the interobserver correlation was 0.992 demonstrating excellent volumetric correlation between observers. The mean difference between the observers for right ventricular end diastolic volume index (RVEDVi) was 2.5ml/m 2 (95% limits of agreement −7.3 to 12.2ml/m 2 ). For patients with mild-moderate RV dilatation (RVEDVi 2 ) the mean difference of RVEDVi was 1.8ml/m 2 (95% limits of agreement −5.7 to 9.3ml/m 2 ). For patients with severe RV dilatation (RVEDVi≥150ml/m 2 ) the mean difference was −3.4ml/m 2 (95% limits of agreement −8.6 to 15.4ml/m 2 ). Conclusions In patients with repaired ToF and variable degrees of RV dilatation, CMR assessment of RV volumes and function has high inter-observer reproducibility. This allows for optimal timing of pulmonary valve replacement, based on progression of RV dilatation over time.

Background The prevalence of thrombosis after the Fontan procedure depends upon the surgical technique used and the method of detection employed. Current investigations for thrombosis lack sensitivity and specificity or, in the paediatric population, require a general anaesthetic. We undertook a study to examine the feasibility of using magnetic resonance imaging (MRI) to detect thrombosis within the conduit, cardiac chambers and pulmonary arteries after the extracardiac conduit modification of the Fontan procedure. Methods Of the 50 children who had undergone this procedure at our institution between 1997 and 2002, 26 were eligible for, and 13 underwent, MRI study. The mean age was 10.2 years (range 8.2–16.8 years, median 9.5 years) and the average time from operation was 63 months (range 29–79 months, median 68 months). The mean age at Fontan operation was 4.9 years (range 2.1–10.5 years). Ten were on low dose aspirin, two were on warfarin and one was not anti-coagulated. In all cases, satisfactory imaging of the venous pathways and pulmonary arteries was obtained and there were no thrombi detected. Conclusions We conclude that MRI is a potentially useful tool for the detection of thrombus in patients who have undergone the Fontan operation.

Obesity is a risk factor for atrial fibrillation (AF) but the mechanisms underlying this association are unclear. We aimed to assess whether body mass index (BMI) is an independent determinant of left atrial size, in subjects in sinus rhythm.Subjects were consecutive ambulatory patients aged/=18 years who underwent outpatient transthoracic echocardiography at a major metropolitan teaching hospital in Sydney, Australia. At the time of examination, age, sex, height and weight were measured. Left atrial (LA) area was measured on ultrasound by planimetry. Left ventricular (LV) function and LV posterior wall thickness were measured by M-mode.Of 4859 consecutive subjects who underwent outpatient echocardiography at our institution over a three-year period, we analysed echocardiographic data from 2534 aged/=18 years with confirmed sinus rhythm, normal LV contractility and no evidence of significant aortic or mitral valve disease. In these subjects (age 47+/-16.6 years, BMI 27.1+/-6.1, 53% male), BMI was a significant predictor of LA size (p0.001), independent of the significant influences of LV end-diastolic volume and LV posterior wall thickness. Average LA size was 18.5+/-4.0 cm(2) in those with normal BMI, 20.7+/-4.5 cm(2) in the overweight and 22.3+/-4.1cm(2) in obese subjects (p for trend0.001).Obesity is associated with increased left atrial size in subjects undergoing clinically indicated echocardiography, independent of the effects of left ventricular size and posterior wall thickness. This may contribute, at least in part, to the rising incidence of atrial fibrillation in the community.