An Option for Concomitant Management of Moderate Marfan Root Aneurysm at the Time of Mitral Valve Repair:A Role for Personalized External Aortic Root Support

Two patients had mitral valve repair for severe regurgitation in the presence of a Marfan aortic root aneurysm. Concomitant personalized external aortic root support was used at the same operation to halt aneurysm progression and to correct mild aortic regurgitation

Quadricuspid aortic valve : a case report and review of the literature

Quadricuspid aortic valve (QAV) is a rare congenital anomaly that can present as aortic insufficiency later in life. We report a case of aortic regurgitation associated with a QAV, treated by aortic valve replacement. The patient presented with breathlessness, lethargy and peripheral oedema. Echocardiography and cardiac magnetic resonance revealed abnormal aortic valve morphology and coronary angiography was normal. The presence of a quadricuspid aortic valve was confirmed intraoperatively. This was excised and replaced with a bioprosthetic valve and the patient recovered well postoperatively. Importantly, the literature indicates that specific QAV morphology and associated structural abnormalities can lead to complications. Hence, early detection and diagnosis of QAV allows effective treatment. Aortic valve surgery is the definitive treatment strategy in patients with aortic valve regurgitation secondary to QAV. However, the long-term effects and complications of treatment of this condition remains largely unknown

Is there an association between early weight status and utility based health-related quality of life in young children?

Purpose Few studies focus on the health-related quality of life (HRQoL) of preschool children with overweight or obesity. This is relevant for evaluation of obesity prevention trials using a quality-adjusted life year (QALY) framework. This study examined the association between weight status in the preschool years and HRQoL at age 5 years, using a preference-based instrument. Methods HRQoL [based on parent proxy version of the Health Utilities Index Mark 3 (HUI3)] and weight status were measured in children born in Australia between 2007 and 2009. Children’s health status was scored across eight attributes of the HUI3—vision, hearing, speech, ambulation, dexterity, emotion, cognition and pain, and these were used to calculate a multi-attribute utility score. Ordinary least squares (OLS), Tobit and two-part regressions were used to model the association between weight status and multi-attribute utility. Results Of the 368 children for whom weight status and HUI3 data were available, around 40% had overweight/obesity. After adjusting for child’s sex, maternal education, marital status and household income, no significant association between weight status in the preschool years and multi-attribute utility scores at 5 years was found. Conclusions Alternative approaches for capturing the effects of weight status in the preschool years on preference-based HRQoL outcomes should be tested. The application of the QALY framework to economic evaluations of obesity-related interventions in young children should also consider longitudinal effects over the life-course

Personalised external aortic root support (PEARS) in Marfan syndrome: Analysis of 1-9 year outcomes by intention-to-treat in a cohort of the first 30 consecutive patients to receive a novel tissue and valve-conserving procedure, compared with the published results of aortic root replacement

Objective: Among people with Marfan syndrome who have a typical aortic root aneurysm, dissection is a characteristic cause of premature death. To pre-empt Type A dissection, composite root replacement with a mechanical valve became the standard of care in the 1980s and 1990s. This is being superseded by valvesparing aortic root replacement to avoid lifelong anticoagulation. In 2004, a total root and valve-sparing procedure, personalised external aortic support, was introduced. We report here results among the first 30 recipients. Methods: From cross-sectional digital images, the patient's own aorta is modelled by computer aided design and a replica is made in thermoplastic by rapid prototyping. On this, a personalised support of a macroporous polymer mesh is manufactured. The mesh is positioned around the aorta, closely applied from the aortoventricular junction to beyond the brachiocephalic artery. The operation is performed with a beating heart and usually without cardiopulmonary bypass. Results: Between 2004 and 2011, 30 patients, median age 28 years (IQR 20-44) had this operation and have been prospectively followed for 1.4-8.8 years by February 2013. During a total of 133 patient-years there were no deaths or cerebrovascular, aortic or valve-related events. These early outcomes are better than published results for the more radical extirpative root replacement operations. Conclusions: The aortic valve, the root architecture, and the blood/endothelia interface are conserved. The perioperative burden is less and there has been freedom from aortic and valvular events. A prospective comparative study is planned

Standards for heart valve surgery in a ‘heart valve centre of excellence’

Surgical centres of excellence should include multidisciplinary teams with specialist expertise in imaging, clinical assessment and surgery for patients with heart valve disease. There should be structured training programmes for the staff involved in the periprocedural care of the patient and these should be overseen by national or international professional societies. Good results are usually associated with high individual and centre volumes, but this relationship is complex. Results of surgery should be published by centre and should include rates of residual regurgitation for mitral repairs and reoperation rates matched to the preoperative pathology and risk

Long-term safety of drug-eluting and bare-metal stents: Evidence from a comprehensive network meta-analysis

Abstract Background Previous meta-analyses have investigated the relative safety and efficacy profiles of different types of drug-eluting stents (DES) and bare-metal stents (BMS); however, most prior trials in these meta-analyses reported follow-up to only 1 year, and as such, the relative long-term safety and efficacy of these devices are unknown. Many recent studies have now reported extended follow-up data. Objectives This study sought to investigate the long-term safety and efficacy of durable polymer-based DES, bioabsorbable polymer-based biolimus-eluting stents (BES), and BMS by means of network meta-analysis. Methods Randomized controlled trials comparing DES to each other or to BMS were searched through MEDLINE, EMBASE, and Cochrane databases and proceedings of international meetings. Information on study design, inclusion and exclusion criteria, sample characteristics, and clinical outcomes was extracted. Results Fifty-one trials that included a total of 52,158 randomized patients with follow-up duration ≥3 years were analyzed. At a median follow-up of 3.8 years, cobalt-chromium everolimus-eluting stents (EES) were associated with lower rates of mortality, definite stent thrombosis (ST), and myocardial infarction than BMS, paclitaxel-eluting stents (PES), and sirolimus-eluting stents (SES) and less ST than BES. Phosphorylcholine-based zotarolimus-eluting stents had lower rates of definite ST than SES and lower rates of myocardial infarction than BMS and PES. The late rates of target-vessel revascularization were reduced with all DES compared with BMS, with cobalt-chromium EES, platinum chromium-EES, SES, and BES also having lower target-vessel revascularization rates than PES. Conclusions After a median follow-up of 3.8 years, all DES demonstrated superior efficacy compared with BMS. Among DES, second-generation devices have substantially improved long-term safety and efficacy outcomes compared with first-generation device

Cardiac Energetics in Patients With Aortic Stenosis and Preserved Versus Reduced Ejection Fraction.

BACKGROUND: Why some but not all patients with severe aortic stenosis (SevAS) develop otherwise unexplained reduced systolic function is unclear. We investigate the hypothesis that reduced creatine kinase (CK) capacity and flux is associated with this transition. METHODS: We recruited 102 participants to 5 groups: moderate aortic stenosis (ModAS) (n=13), SevAS, left ventricular (LV) ejection fraction ≥55% (SevAS-preserved ejection fraction, n=37), SevAS, LV ejection fraction 0.99). Accompanying the fall in CK flux, total CK and citrate synthase activities and the absolute activities of mitochondrial-type CK and CK-MM isoforms were also lower (P<0.02, all analyses). Median mitochondria-sarcomere diffusion distances correlated well with CK total activity (r=0.86, P=0.003). CONCLUSIONS: Total CK capacity is reduced in SevAS, with median values lowest in those with systolic failure, consistent with reduced energy supply reserve. Despite this, in vivo magnetic resonance spectroscopy measures of resting CK flux suggest that ATP delivery is reduced earlier, at the moderate AS stage, where LV function remains preserved. These findings show that significant energetic impairment is already established in moderate AS and suggest that a fall in CK flux is not by itself a necessary cause of transition to systolic failure. However, because ATP demands increase with AS severity, this could increase susceptibility to systolic failure. As such, targeting CK capacity and flux may be a therapeutic strategy to prevent and treat systolic failure in AS.This study was principally funded by a British Heart Foundation Clinical Training Research Fellowship FS/15/80/31803 (to Dr Peterzan) with support from a British Heart Foundation Program Grant (RG/18/12/34040). Drs Neubauer and Rider acknowledge support from British Heart Foundation Center of Research Excellence. Dr Neubauer acknowledges support from the National Institute of Health Research Oxford Biomedical Research Center. Dr Rodgers receives funding from the Wellcome Trust and the Royal Society (grant no. 098436/Z/12/B) and supported by the National Institute of Health Research Cambridge Biomedical Research Center. Dr Rider is funded by the British Heart Foundation FS/16/70/32157. Dr Miller was supported by a Novo Nordisk Postdoctoral Fellowship run in conjunction with the University of Oxford. The Biotechnology and Biological Sciences Research Council provided Advanced Life Sciences Research Technology Initiative 13 funding for serial block-face scanning electron microscopy through grant BB/C014122/1 (to Prof Chris Hawes, Oxford Brookes University)

Localized rest and stress human cardiac creatine kinase reaction kinetics at 3 T.

Changes in the kinetics of the creatine kinase (CK) shuttle are sensitive markers of cardiac energetics but are typically measured at rest and in the prone position. This study aims to measure CK kinetics during pharmacological stress at 3 T, with measurement in the supine position. A shorter "stressed saturation transfer" (StreST) extension to the triple repetition time saturation transfer (TRiST) method is proposed. We assess scanning in a supine position and validate the MR measurement against biopsy assay of CK activity. We report normal ranges of stress CK forward rate (kfCK ) for healthy volunteers and obese patients. TRiST measures kfCK in 40 min at 3 T. StreST extends the previously developed TRiST to also make a further kfCK measurement during <20 min of dobutamine stress. We test our TRiST implementation in skeletal muscle and myocardium in both prone and supine positions. We evaluate StreST in the myocardium of six healthy volunteers and 34 obese subjects. We validated MR-measured kfCK against biopsy assays of CK activity. TRiST kfCK values matched literature values in skeletal muscle (kfCK  = 0.25 ± 0.03 s-1 vs 0.27 ± 0.03 s-1 ) and myocardium when measured in the prone position (0.32 ± 0.15 s-1 ), but a significant difference was found for TRiST kfCK measured supine (0.24 ± 0.12 s-1 ). This difference was because of different respiratory- and cardiac-motion-induced B0 changes in the two positions. Using supine TRiST, cardiac kfCK values for normal-weight subjects were 0.15 ± 0.09 s-1 at rest and 0.17 ± 0.15 s-1 during stress. For obese subjects, kfCK was 0.16 ± 0.07 s-1 at rest and 0.17 ± 0.10 s-1 during stress. Rest myocardial kfCK and CK activity from LV biopsies of the same subjects correlated (R = 0.43, p = 0.03). We present an independent implementation of TRiST on the Siemens platform using a commercially available coil. Our extended StreST protocol enables cardiac kfCK to be measured during dobutamine-induced stress in the supine position.Funded by: a Sir Henry Dale Fellowship from the Wellcome Trust and the Royal Society [098436/Z/12/B] to CTR, the BHF Centre of Research Excellence (OJR), a BHF clinical research training fellowship [FS/15/80/31803] to MAP, a BHF fellowship [FS/14/54/30946] to JJR, an NIHR OBRC fellowship to BR, a BHF programme grant [RG/13/8/30266] to CAL and SN, and a DPhil studentship from the Medical Research Council to WTC. We acknowledge support from the Oxford NIHR Biomedical Research Centre

Potential of pre-contrast T1 mapping as a marker of interstitial fibrosis in severe aortic stenosis

International audiencen.