Dr. Bernard David Prendergast,
A packed audience enjoyed an educational plenary session “The aortic valve and aorta – what the clinician needs to know” led by a world renowned faculty in the Porto room (zone 6) on Sunday evening.
Catherine Otto (Seattle, US) provided an overview of progress in the clinical assessment and management of patients with Marfan’s syndrome. Diagnosis often remains difficult with a constellation of clinical signs and overlapping syndromes but international classification (Ghent criteria) and improved understanding of the underlying genetic pathway (involving TGF-β mediated disruption of fibrillin) assist in assessment of both index cases and their families. β-blockers have hitherto been the mainstay of medical therapy (reducing aortic wall stress and delaying root dilatation) but recent clinical data have confirmed findings in mouse knockout models demonstrating the role of AT1 receptor blockade. Armed with programmes of regular echocardiographic surveillance, improved medical therapy and a low threshold for aortic root surgery (diameter > 5cms, or less if rapid rate of growth, coexistent aortic regurgitation, or family history of dissection/rupture), Marfan’s patients and their families can now anticipate longevity beyond 70 years of age.
Similar advances in our understanding of the underlying embryology and pathophysiology have improved the management of bicuspid aortic valve disease. Dr Jens Kaden (Mannheim, DE) outlined the frequency of this condition, which affects 0.5-2.0% of the general population and is associated with premature valve degeneration, calcification and bone deposition resulting in aortic stenosis. Underlying genetics are complex (involving a variety of pathways, notably NOTCH 1) and elastic lamellar degeneration affecting the aortic wall resembles changes seen in Marfan’s syndrome. Rate of progression can be estimated from baseline characteristics and overall prognosis is good, though radically altered by the onset of symptoms or clinical events. Guidelines emphasise initial assessment of the valve and ascending aorta using echocardiography or CMR, regular follow up and a low threshold for surgery.
The International Registry of Aortic Dissection (IRAD) provides invaluable insight into the current clinical characteristics and devastating nature of this condition. Dr Arturo Evangelista (Barcelona, ES), one of the principal investigators in this 11 nation study involving over 20 centres, described outcomes in 2345 patients enrolled to date. Mean age is 62 years with background hypertension in >70 % and previous cardiac surgery in 18%. Presenting ECG and chest X-ray are often normal (15% and 29%, respectively), though changes mimicking NSTEMI (18%) and STEMI (6%) frequently confuse assessment and treatment. Diagnosis is often delayed (particularly in females, the elderly, and those without presenting chest or back pain) and most often achieved with CT (61%) or TEE (33%). Despite the benefits of surgery for Type A dissection (except in those >80 years), improved medical management and availability of percutaneous treatment for Type B dissection, mortality remains high – 25% at 30 days – calling for improved diagnostic pathways and referral networks.
Aortic root surgery remains challenging and best results are obtained in the hands of expert, experienced surgeons. Dr A Nobre (Lisbon, PT) provided an overview of surgical technique, including the need for concomitant coronary implantation, coupled with results form his own personal series of 116 patients treated from 2005-2007. Mortality and long term outcomes equivalent to isolated aortic valve replacement indicate that root surgery should be offered more frequently (with lower threshold root diameters) at the time of valve surgery.
The aortic valve and the aorta: what the clinician needs to know
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