Clinical Significance of the Bicuspid Aortic Valve
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Heart 2000;83:81–85 81 REVIEW Heart: first published as 10.1136/heart.83.1.81 on 1 January 2000. Downloaded from Clinical significance of the bicuspid aortic valve C Ward The association of bicuspid aortic valve with features of degenerative valve disease, the third aortic stenosis, aortic regurgitation, and infective major aetiological group: endocarditis has been known for almost 150 + Calcification increases with age and is years and with dissection of the aorta for 75 largely confined to the raphé (which does years.1 These complications are mentioned only not extend to the commissure) and base of briefly in cardiology textbooks3 and a standard the cusps, as it is in degenerative valves. It is work on medical insurance underwriting4 im- more diVuse in postinflammatory disease. plies that it is usually a benign lesion: when Calcification is conspicuous in virtually all diagnosed at routine medical examination and if adults with a severely stenosed bicuspid aor- the valve is functionally normal, insurance is tic valve but is uncommon in isolated aortic oVered at ordinary rates. The authors also state regurgitation.910 “If there is no stenosis by age 20 years (defined +Only in postinflammatory valves are cusp as a peak gradient of less than 40 mm Hg) and margins severely distorted and fused, result- only trivial insuYciency, there is an excellent ing in the valve often having a central tri- chance that significant haemodynamic change radiate orifice. will not develop before age 70.” However, on the + In virtually all cases, the aetiology of aortic basis of published reports by this age the major- stenosis can be determined by naked eye ity of patients will have died or developed serious examination of the valve.8 symptoms requiring surgical intervention. This + Where doubt exists, histological examina- review summarises the major studies that tion shows no valve tissue in the raphé of a provide information on the incidence, pathol- congenital bicuspid aortic valve, whereas ogy, and natural history of the bicuspid aortic postinflammatory valves show evidence of http://heart.bmj.com/ valve. Clinical diagnosis of this condition was previous valvitis.78These criteria have been unsatisfactory before the widespread use of consistently applied in subsequent patho- cross sectional echocardiography approximately logical studies, thus providing a high level of 15 years ago. Most of the information in this consistency to the identification of those paper therefore comes from large necropsy valves which are congenitally bicuspid. series and from reports on patients who have The orientation of the valve cusps, antero- undergone aortic valve replacement. It has to be posterior or left-right in approximately equal accepted that conclusions based on these numbers,57may have some bearing on compli- reports may be inaccurate, because of non- cation rates. The coronary arteries usually arise on September 29, 2021 by guest. Protected copyright. uniformity of diagnostic criteria and varying age in front of the anterior cusp5 which is the cusp range, source/selection of patients studied, and with the raphé. Stenosis usually develops in study objectives. bicuspid valves containing no redundant cusp A small percentage of malfunctioning con- tissue, and incompetence often in valves in genitally abnormal aortic valves are unicuspid which redundancy and prolapse are or unicommissural,5 but as most reports do not prominent.1 This had been confirmed by more distinguish this from bicuspid aortic valve the recent studies.9 Unicommissural aortic valves abbreviation “bicuspid aortic valve” will used are of two types, both of which are dome to include both types of valve unless stated shaped.10 One has no commissure, and the otherwise. other a single commissure which only partially divides the cusps. Most have some evidence of Anatomy and pathology two raphés, suggesting that they may have been Early pathology studies16 documented three tricuspid at an early stage of development. characteristics of bicuspid aortic valve: inequal- Many vascular abnormalities have been ity of cusp size, the presence of a central raphé associated with bicuspid aortic valve but only two are of common practical importance in North West Regional (ridge), usually in the centre of the larger of the Cardiac Centre, South two cusps, and smooth cusp margins even in adult cardiology. Manchester University diseased valves. Subsequently bicuspid aortic First, a bicuspid aortic valve occurs in NHS Trust, valve was diVerentiated from an initially tricus- 20–85% of cases of coarctation of the aorta.11 12 Southmoor Road, pid valve,7 two cusps of which had become The presence of an untreated, inadequately Manchester M23 9LT, fused as a result of rheumatic or other inflam- treated, or recurrent coarctation increases the UK C Ward matory processes. Other necropsy series have likelihood of developing aortic stenosis, aortic confirmed, elaborated, and refined these regurgitation, or dissection of the aorta (see Accepted 4 August 1999 characteristics,58 and have also described the below). Bicuspid aortic valve has also been 82 Ward reported in 27% of 52 cases of interrupted aor- is from this age group. In the light of these fig- tic arch,13 suggesting a common developmental ures the statement that “this is a very common Heart: first published as 10.1136/heart.83.1.81 on 1 January 2000. Downloaded from pathogenesis for congenital abnormalities of defect, being found once in every 100 to 200 the aorta and aortic valve. autopsies”7 is reasonable and provides a valid Second, 90% of patients with a normal (tri- basis for the generally accepted figure of an cuspid) aortic valve have right coronary artery overall incidence of 1–2%. dominance, that is the right coronary artery Because of case selection, some of the figures supplies the crux of the heart, and the left main quoted will have overestimated the incidence of stem averages 10 mm in length. With a complications but they are the only relevant bicuspid aortic valve, left dominance is more published data. They indicate that, at a common (between 29% and 56.8%14 15) and in conservative estimate, one third and possibly 90% of such cases the left main stem is less the majority of patients with bicuspid aortic than 5 mm in length.16 Failure to recognise valve will develop serious complications. these associations may result in inadequate myocardial preservation at the time of aortic Specific complications valve replacement and an increased risk of AORTIC STENOSIS perioperative myocardial infarction.16 Four reports define the clinical and pathologi- cal characteristics of bicuspid aortic valve Clinical diagnosis stenosis, three based on necropsy data5822and A clinical diagnosis of bicuspid aortic valve one on surgical specimens.23 There is broad based purely on the presence of an aortic ejec- agreement that approximately 50% of adults tion click, with or without an ejection systolic with severe aortic stenosis have a bicuspid aor- murmur, lacks predictive accuracy as it may be tic valve. Severely stenosed bicuspid aortic heard in tricuspid aortic valve stenosis, or in valves are very rigid because of fibrosis and aortic dilatation from any cause, and is absent heavy calcification, but are not narrowed.82023 when the valve cusps are rigid.17 18 The reliabil- Both calcification and fibrosis (and thus ity of diagnosis has been significantly improved deteriorating valve function) are age related.23 by the introduction of cross sectional and In a clinical series a third of initially asympto- Doppler echocardiography—a specificity of matic patients with bicuspid aortic valve 96%, a sensitivity of 78%, and a predictive deteriorated during a mean period of 10.9 accuracy 93% have been reported.19 Diagnosis years.24 Echocardiographic studies25 26 have is based on the demonstration of two cusps and shown that sclerosis of the valve begins in the two commissures during short axis visualisa- second decade, and calcification is increasingly tion. Supportive features include cusp redun- prominent from the fourth decade onwards; dancy and eccentric valve closure, and a single the average aortic valve gradient increases con- coaption line between the cusps during dias- currently by 18 mm Hg per decade. Stenosis tole. However, the usually bicuspid nature of a progressed more rapidly (27 mm Hg per http://heart.bmj.com/ valve may be obscured by severe fibrosis or cal- decade) if the cusps were asymmetrical in size cification and false negative results may be and in the anteroposterior location.26 In a sec- produced by a prominent raphé (which can ond report27 of 31 patients with an initially give the appearance of a third coaption line); a functionally normal valve (mean gradient < 25 false positive diagnosis may occur when one mm Hg), four had an undefined increase in coaption line of a tricuspid valve is unclear. gradient and three required aortic valve surgery during a median follow up time of 21 months. The incidence of bicuspid aortic valve High serum low density lipoprotein choles- on September 29, 2021 by guest. Protected copyright. and complications terol, high serum lipoprotein (a), and smoking Four necropsy series56820 provide the only are independent risk factors for aortic stenosis basis we have for estimating the incidence of and presumably contribute to the age related bicuspid aortic valve in the adult population, as deterioration.28 diagnosis by auscultation is insuYciently accu- Bicuspid aortic valve is the commonest aeti- rate and no relevant echocardiographic study ology of aortic stenosis between the ages of 60 has been published. The incidence of normally and 75 years (59% of cases); it was also the functioning bicuspid aortic valves is 0.6% to cause in 40% of those aged under 60 years and 0.9%.5621 This is probably the minority of 32% of those aged more than 75 years.8 The bicuspid aortic valves, as only 15–28%56 are relation between age and bicuspid aortic valve reported as normal in necropsy series.