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The Ross procedure, where a pulmonary autograft (neoaorta) replaces the aortic valve, has excellent long-term outcomes in patients with congenital aortic valve disease. However, there are reports of neoaortic dilatation and dissection. An increasing number of women are wishing to become pregnant following the Ross procedure, but little is known about the occurrence and risks of neoaortic dilatation and complications in pregnancy. We investigated neoaorta function and outcomes in pregnancy following the Ross procedure.This retrospective study investigated women post-Ross procedure at a tertiary ACHD unit between 1997 and 2021. Imaging evaluated neoaortic root dimensions and regurgitation pre-, and post- pregnancy, compared with matched non-pregnant controls. Primary endpoints were change in neoaortic dimensions, degree of regurgitation and adverse maternal outcomes.Nineteen pregnancies in 12 women were included. The mean change in neoaortic root diameter post-pregnancy was 1.8 mm (SD 3.4) (p = 0.017). There was no significant change in neoaortic dimensions in matched controls during follow-up. There were no cases of dissection, arrhythmia, acute coronary syndrome, or maternal mortality. Three deliveries were pre-term, including one emergency Caesarean section due to maternal cardiac decompensation, requiring aortic root replacement post-partum but there were no neonatal deaths.Pregnancy following the Ross procedure is associated with neoaortic dilatation, and pregnancy is generally well tolerated. Although adverse maternal outcomes are uncommon, there are still rare cases of cardiac complications in and around the time of pregnancy. These findings emphasise the need for accessible pre-pregnancy counselling, risk stratification and careful surveillance through pregnancy by specialist cardio-obstetric multi-disciplinary teams.

Background: Pulmonary vasodilator therapy in Fontan patients can improve exercise tolerance. We aimed to assess the potential for non-invasive testing of acute vasodilator response using four-dimensional (D) flow MRI during oxygen inhalation. Materials and Methods: Six patients with well-functioning Fontan circulations were prospectively recruited and underwent cardiac MRI. Ventricular anatomical imaging and 4D Flow MRI were acquired at baseline and during inhalation of oxygen. Data were compared with six age-matched healthy volunteers with 4D Flow MRI scans acquired at baseline. Results: All six patients tolerated the MRI scan well. The dominant ventricle had a left ventricular morphology in all cases. On 4D Flow MRI assessment, two patients (Patients 2 and 6) showed improved cardiac filling with improved preload during oxygen administration, increased mitral inflow, increased maximum E-wave kinetic energy, and decreased systolic peak kinetic energy. Patient 1 showed improved preload only. Patient 5 showed no change, and patient 3 had equivocal results. Patient 4, however, showed a decrease in preload and cardiac filling/function with oxygen. Discussion: Using oxygen as a pulmonary vasodilator to assess increased pulmonary venous return as a marker for positive acute vasodilator response would provide pre-treatment assessment in a more physiological state – the awake patient. This proof-of-concept study showed that it is well tolerated and has shown changes in some stable patients with a Fontan circulation.

Background The inverse association between estimated glomerular filtration rate (eGFR) and cardiovascular risk is well recognised but not fully explained. Kidney donation is known to reduce renal function by approximately 30% and allows the prospective study of the cardiovascular effects of a reduced eGFR in healthy subjects without confounding comorbidities. We report five year results in a group of kidney donors and healthy controls who underwent extensive cardiovascular assessment. Methods A 5 year longitudinal, parallel group, blinded end-point study of living kidney donors (n=50) and healthy controls (n=45). Participants underwent office and 24-hr ambulatory blood pressure measures, multi-parametric cardiac magnetic resonance imaging (MRI) (3 Tesla), measurement of arterial stiffness (SphygmoCor), carotid intima-media thickness and blood and urine analysis at baseline, one year and 5 years from donation. Results The eGFR in donors at baseline was 95 ± 15ml/min/1.73m2, 65 ± 13ml/min/1.73m2 at one year and 67 ± 14ml/min/1.73m2 at 5 years. Left ventricular (LV) mass was 112g at baseline in both groups and was not different at 5 years (113 ± 31 vs. 115 ± 30, p=0.707). There was also no difference in LV volumes, LV geometry, LV function, T1 times or extracellular volume (ECV) on MRI. Office and ambulatory blood pressures did not differ from controls at any time point. Pulse wave velocity was higher in donors at one year but not significantly different by 5 years. At 5 years, there was no significant differences in the prevalence of a detectable troponin or mean plasma NT pro-BNP. Conclusion The stable reduction in eGFR to levels of 60-70 ml/min/1.73m2 after live kidney donation does not lead to deleterious changes in cardiovascular structure and function or biomarkers of cardiovascular disease at 5 years. Factors associated with kidney disease other than an isolated fall in eGFR are likely to explain the increased cardiovascular risk in patients with chronic kidney disease. Figure A and B: Longitudinal change in LV mass and PWV before and after donation in donors and controls. Black solid lines are means with confidence intervals for donors and dashed lines represent controls. Black squares indicate study visits. A; Left ventricular mass (g) and B; Adjusted pulse wave velocity (m/s) (adjusted for mean arterial pressure and heart rate). Conflict of Interest None

Cardiac remodeling, after a myocardial insult, often causes progression to heart failure. The relationship between alterations in left ventricular blood flow, including kinetic energy (KE), and remodeling is uncertain. We hypothesized that increasing derangements in left ventricular blood flow would relate to (1) conventional cardiac remodeling markers, (2) increased levels of biochemical remodeling markers, (3) altered cardiac energetics, and (4) worsening patient symptoms and functional capacity. Methods Thirty-four dilated cardiomyopathy patients, 30 ischemic cardiomyopathy patients, and 36 controls underwent magnetic resonance including 4-dimensional flow, BNP (brain-type natriuretic peptide) measurement, functional capacity assessment (6-minute walk test), and symptom quantification. A subgroup of dilated cardiomyopathy and control subjects underwent cardiac energetic assessment. Left ventricular flow was separated into 4 components: direct flow, retained inflow, delayed ejection flow, and residual volume. Average KE throughout the cardiac cycle was calculated.Patients had reduced direct flow proportion and direct-flow average KE compared with controls ( P0.0001). The residual volume proportion and residual volume average KE were increased in patients ( P0.0001). Importantly, in a multiple linear regression model to predict the patient's 6-minute walk test, the independent predictors were age (β=-0.3015; P=0.019) and direct-flow average KE (β=0.280, P=0.035; RThis study demonstrates an independent predictive relationship between the direct-flow average KE and a prognostic measure of functional capacity. Intracardiac 4-dimensional flow parameters are novel biomarkers in heart failure and may provide additive value in monitoring new therapies and predicting prognosis.

Background The neo-aorta produced by the pulmonary autograft following the Ross procedure has excellent long-term outcomes, with an increasing number of women now undergoing pregnancy following Ross. Cardiac Magnetic Resonance (CMR) has been used to evaluate the risk of progression of neo-aortic dilatation and dissection that may arise as a result of the additional cardiovascular risks posed during pregnancy. Although there have been reported cases of dilatation and dissection, there is little research. Aims To evaluate the extent of neo-aortic dilatation, and associated aortic regurgitation, during pregnancy following the Ross procedure. Methods A retrospective cohort of women were identified who had undergone a Ross procedure after 1985, who in January 2019 were aged >16, in an adult congenital cardiology and joint cardiac-obstetric centre. CMR data was used to evaluate neo-aortic root dimensions and aortic regurgitation extent pre-, during and post- pregnancy. A control group of case-matched patients who had undergone the Ross procedure, but not pregnancy, was used for comparison. Results In all, 8 women carried a total of 15 pregnancies to term. In only one pregnancy did the mother experience serious cardiac decompensation, necessitating early delivery and associated with subaortic stenosis, mild aortic root dilatation and increasing aortic regurgitation. Four women (50%) experienced an element of aortic root dilatation during pregnancy, with an average increase between them of 0.525 cm (range +0.4 to+0.7). In no case did this lead to progressive dilatation or dissection. When compared to the control group, pregnancy was found to carry an increased risk of neo-aortic dilatation (Mean aortic root change for group:+0.26 cm (SD 0.30) pregnancy vs. +0.16 cm (SD 0.27) control). There was a high rate of initial mild aortic regurgitation in both groups. In three women their extent of regurgitation increased post-pregnancy, correlated with increased parity and aortic dilatation. In the control group only two women experienced increased regurgitation, only one of which was associated with neo-aortic dilatation. Conclusions Pregnancy appears generally well tolerated by women following the Ross Procedure, although there is an increased risk of neo-aortic dilatation and corresponding increase in aortic regurgitation compared to the control group.