Left Atrial Geometry and Phasic Function Determined by Cardiac Magnetic Resonance Are Independent Predictors for Outcome in Non-Ischaemic Dilated Cardiomyopathy.

Left atrial (LA) geometry and phasic functions are frequently impaired in non-ischaemic dilated cardiomyopathy (NIDCM). Cardiac magnetic resonance (CMR) can accurately measure LA function and geometry parameters. We sought to investigate their prognostic role in patients with NIDCM. We prospectively examined 212 patients with NIDCM (49 ± 14.2-year-old; 73.5% males) and 106 healthy controls. LA volumes, phasic functions, geometry, and fibrosis were determined using CMR. A composite outcome (cardiac death, ventricular tachyarrhythmias, heart failure hospitalization) was ascertained over a median of 26 months. LA phasic functions, sphericity index (LASI) and late gadolinium enhancement (LA-LGE) were considerably impaired in the diseased group (p < 0.001) and significantly correlated with impaired LV function parameters (p < 0.0001). After multivariate analysis, LA volumes, LASI, LA total strain (LA-εt) and LA-LGE were associated with increased risk of composite outcome (p < 0.001). Kaplan-Meier analysis showed significantly higher risk of composite endpoint for LA volumes (all p < 0.01), LASI > 0.725 (p < 0.003), and LA-εt < 30% (p < 0.0001). Stepwise Cox proportional-hazards models demonstrated a considerable incremental predictive value which resulted by adding LASI to LA-εt (Chi-square = 10.2, p < 0.001), and afterwards LA-LGE (Chi-Square = 15.8; p < 0.0001). NIDCM patients with defective LA volumes, LASI, LA-LGE and LA-εt had a higher risk for an outcome. LA-εt, LASI and LA-LGE provided independent incremental predictive value for outcome.

Clinical and cost-effectiveness of the Ross procedure versus conventional aortic valve replacement in young adults.

Objectives: In young and middle-aged adults, there are three current options for aortic valve replacement (AVR), namely mechanical AVR (mechAVR), tissue AVR (biological AVR) and the Ross operation, with no clear guidance on the best option. We aim to compare the clinical effectiveness and cost-effectiveness of the Ross procedure with conventional AVR in young and middle-aged adults. Methods: This is a systematic literature review and meta-analysis of AVR options. Markov multistate model was adopted to compare cost-effectiveness. Lifetime costs, quality-adjusted life years (QALYs), net monetary benefit (NMB), population expected value of perfect information (EVPI) and expected value of partial perfect information were estimated. Results: We identified 48 cohorts with a total number of 12 975 patients (mean age 44.5 years, mean follow-up 7.1 years). Mortality, bleeding and thromboembolic events over the follow-up period were lowest after the Ross operation, compared with mechAVR and biological AVR (p<0.001). Aortic reoperation rates were lower after Ross compared with biological AVR, but slightly higher when compared with mechAVR (p<0.001). At a willingness-to-pay threshold of £20effective. At a willingness-to-pay threshold of £20, 000 per QALY000 per QALY, the Ross procedure is more cost-effective compared the Ross procedure is more cost-effective compared withwith conventional AVR, with a lifetime incremental NMB of £60 conventional AVR, with a lifetime incremental NMB of £60 952 (952 (££3030 236236 to to ££7979 464). Incremental costs were £12464). Incremental costs were £12 323 (323 (££61086108 to to ££1515 972) and incremental QALYs 3.66 (1.81972) and incremental QALYs 3.66 (1.81 to to 4.76). The population EVPI indicates that a trial costing up to £2.03 million could be cost 4.76). The population EVPI indicates that a trial costing up to £2.03 million could be cost--effective.At a willingness-to-pay threshold of £20 000 per QALY, the Ross procedure is more cost-effective compared with conventional AVR, with a lifetime incremental NMB of £60 952 (£30 236 to £79 464). Incremental costs were £12 323 (£6108 to £15 972) and incremental QALYs 3.66 (1.81 to 4.76). The population EVPI indicates that a trial costing up to £2.03 million could be cost-effective. Conclusions: In young and middle-aged adults with aortic valve disease, the Ross procedure may confer greater quality of life and be more cost-effective than conventional AVR. A high-quality randomised trial could be warranted and cost-effective.

Surgery for Young Adults With Aortic Valve Disease not Amenable to Repair.

Aortic valve replacement is the gold standard for the management of patients with severe aortic stenosis or mixed pathology that is not amenable to repair according to currently available guidelines. Such a simplified approach may be suitable for many patients, but it is far from ideal for young adults considering emerging evidence demonstrating that conventional valve replacement in this cohort of patients is associated with inferior long-term survival when compared to the general population. Moreover; the utilisation of mechanical and bioprosthetic valves can significantly impact on quality and is linked to increased rates of morbidities. Other available options such as stentless valve, homografts, valve reconstruction and Ross operation can be an appealing alternative to conventional valve replacement. Young patients should be fully informed about all the options available - shared decision making is now part of modern informed consent. This can be achieved when referring physicians have a better understanding of the short and long term outcomes associated with every intervention, in terms of survival and quality of life. This review presents up to date evidence for available surgical options for young adults with aortic stenosis and mixed disease not amenable to repair.

Aortic root and hemiarch replacement in a patient with Loeys-Dietz and hypoplastic left heart syndromes.

A 15-year-old patient with hypoplastic left heart syndrome underwent 3-stage palliation by the age of 3 years. He was later diagnosed with Loeys-Dietz syndrome. On follow-up imaging, the neoaorta was dilated at 50 mm in diameter. He underwent aortic root replacement with a composite valve conduit and hemiarch replacement, using a boat-shaped Dacron graft. The uncertainty of how univentricular circulation would tolerate long bypass time steered us away from a total arch replacement. His postoperative recovery was uneventful. Imaging postoperatively demonstrated stable dilatation of the aorta distal to the hemiarch replacement. Considering the risk of reintervention, we elected to monitor the distal aorta. The combination of Loeys-Dietz syndrome and hypoplastic left heart syndrome presents unique challenges, rarely reported in the literature.