[Case report on bilateral absence of upper bicuspid of primary and permanent dentition correlated with upper permanent bicuspid].
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Adult↗
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Bicuspid aortic and pulmonary valves in the Syrian hamster.
We studied the conditions of the aortic and pulmonary valves of 153 Syrian hamsters belonging to a single family subjected to high endogamous pressure. Semilunar valves of 143 specimens were examined using a stereomicroscope, and in 5 of these cases a histologic study was also performed. The remaining 10 specimens were studied by means of scanning electron microscopy. In 77 specimens both semilunar valves were normal. The aortic valve was bicuspid and the pulmonary valve was normal in 46 animals, while a normal aortic valve and a bicuspid pulmonary valve occurred in 24. In the remaining 6 specimens both semilunar valves were bicuspid. All bicuspid semilunar valves detected herein showed the same morphotype, namely with the leaflets oriented ventrodorsally. The incidence of these anomalous valves did not significantly differ between sexes. Results of a chi 2-test substantiate that conditions of both outflow tract semilunar valves are independent traits. Thus, concurrence of a bicuspid aortic valve and a bicuspid pulmonary valve in an individual is a random event. The present findings support the assumption that bicuspid aortic and pulmonary valves have different morphogenetic origin, although factors producing a bicuspid aortic valve and those leading to a bicuspid pulmonary valve are not mutually exclusive.
Animals↗
Aortic valve regurgitation and the congenitally bicuspid aortic valve: a clinico-pathological correlation.
OBJECTIVE: To investigate the morphology of congenitally bicuspid aortic valves causing pure valve regurgitation. DESIGN: A case series collected over five years. SETTING: An academic hospital. PATIENTS AND METHODS: One hundred and forty eight excised congenitally bicuspid aortic valves. The morphological findings were correlated with sex, age, clinical history, and data on haemodynamic function before operation. Pure valve regurgitation was defined as grade 3-4/4 with a gradient less than 30 mm Hg. Aortic root dilatation was evaluated angiographically or echocardiographically or both. RESULTS: Three types were recognised: valves that were purely bicuspid (23%), bicuspid valves with a raphe (34%), and valves with an additional indentation of the free edge of the conjoined cusp (43%). In 14 cases pure valve regurgitation was present. Dilatation of the aortic root was present in 47 cases. The relative risk for regurgitation when the aortic root was dilated (compared with no dilatation) was 3.99. The relative risk for valve regurgitation when there was indentation of the conjoined cusp (compared with no indentation) was 4.95. The mean age at operation in patients with pure regurgitation was 56 years, which is significantly younger (p = 0.0008) than that of patients with a congenitally bicuspid valve with combined valve stenosis and regurgitation (64.7 years). CONCLUSIONS: Congenitally bicuspid aortic valves with a central indentation of the free edge of the conjoined cusp seem particularly likely to develop pure aortic valve regurgitation.
Adult↗
Echocardiographic assessment of bicuspid aortic valves. Angiographic and pathological correlates.
Aortic root echocardiograms were recorded from 89 patients whose aortic valves had also been adequately defined by selective angiography or viewed surgically or at autopsy. The eccentricity index (E.I.) of the aortic leaflets was measured at the onset of diastole and an E.I. of 1.3 or greater was taken as abnormal. Of 31 patients with isolated nonobstruced or mildly obstructed bicuspid aortic valves (7 viewed previously at valvotomy and 24 diagnosed radiologically) 23 (74%) had an abnormal E.I. Varying eccentricity occurred in some of these patients. Central leaflet echoes (E.I. of 1.0 to 1.25) were present in the other eight patients. All 14 patients with nonobstructed tricuspid aortic valves had central echoes. Additional multilayered diastolic echoes were found in patients with bicuspid aortic valves as well as in two patients with abnormal tricuspid aortic valves. The valves of 13 patients with aortic stenosis or incompetence were viewed surgically and the E.I. was abnormal in all patients with a bicuspid aortic valve in this group. Aortic leaflet echo findings were not diagnostically helpful in ten patients with tetralogy of Fallot, one of whom had a normal E.I. with a surgically confirmed bicuspid aortic valve. Of 21 patients with VSD only one had a bicuspid aortic valve but six had an abnormal E.I. This false positive sign was related to a high membranous VSD, sometimes with aortic valve prolapse. It is concluded that an E.I. of greater than or equal to 1.3 in the absence of an associated VSD is diagnostic of a bicuspid aortic valve and can be expected to be found in approximately three-quarters of subjects with this abnormality.
Adolescent↗
Association of aortic dilation with regurgitant, stenotic and functionally normal bicuspid aortic valves.
To determine whether aortic root dilation associated with a bicuspid aortic valve occurs independently of valvular hemodynamic abnormality, aortic root dimensions were measured by two-dimensional echocardiography in 83 adults with a functionally normal (n = 19), mildly regurgitant (n = 26), severely regurgitant (n = 27) or stenotic (n = 11) bicuspid aortic valve and compared with findings in normal subjects matched for age and gender. Aortic root measurements were made at four levels: anulus, sinuses of Valsalva, supraaortic ridge and proximal ascending aorta. Seventy-one percent of patients with a bicuspid aortic valve were men. When compared with control subjects, all hemodynamic subgroups showed a significantly larger aortic root size at three levels: sinuses of Valsalva, supraaortic ridge and proximal ascending aorta (p less than 0.05 to p less than 0.001). The prevalence of aortic root enlargement among all hemodynamic subgroups ranged from 9% to 59% at the level of the anulus, 36% to 78% at the sinuses, 47% to 79% at the supraaortic ridge and 50% to 64% in the ascending aorta. Thus, there is a high prevalence of aortic root enlargement in patients with a bicuspid aortic valve that occurs irrespective of altered hemodynamics or age. These findings support the hypothesis that bicuspid aortic valve and aortic root dilation may reflect a common developmental defect.
Adult↗
The diagnosis of a non-stenotic bicuspid aortic valve.
Follow-up of a group of subjects in whom an aortic ejection sound was the only abnormal finding revealed a bicuspid aortic valve at necropsy or operation in 6 cases. High speed echophonocardiographic studies in 15 subjects with aortic stenosis and known to have bicuspid valves, showed the ejection sound to be exactly synchronous with final halting of the opening aortic valve cusps. Echocardiographic visualisation of the aortic valve from the apex, looking up the left ventricular outflow tract, showed valve echoes during systole indicating abnormal cusp configuration. This proved a more sensitive indicator of a bicuspid aortic valve than the finding of an eccentric aortic valve closure line. These findings were used to evaluate 37 subjects with the auscultatory finding of an isolated aortic ejection sound and the diagnosis of a non-stenotic bicuspid aortic valve was confirmed in 30. The usual cause of misdiagnosis was a sound associated with late tricuspid valve closure. The auscultatory finding of an aortic ejection sound, in the absence of other signs of aortic stenosis, indicates a non-stenotic, bicuspid aortic valve. Confirmation of aortic valvar origin of the sound can be provided by a simultaneous echophonocardiogram.
Aortic Valve↗
Anomalous coronary artery origin and bicuspid aortic valve.
A patient presenting with signs and symptoms suggesting myocardial ischemia was found to have anomalous origin of his right coronary artery from the left aortic sinus of valsalva associated with a bicuspid aortic valve. Abnormal left ventricular function with anaerobic myocardial metabolism was documented in the absence of significant coronary artery disease or aortic obstruction. The possible relationship of these findings to the coronary artery anomaly and bicuspid aortic valve is considered. The necessity of identification of the origin of the coronary arteries is re-emphasized in the evaluation of patients with bicuspid aortic valves coming to cardiac catheterization. Anomalous origin of a coronary artery is a rare occurrence. The association of this anomaly with a bicuspid aortic valve has not been emphasized and the possible clinical consequences of this combination of malformations have not been described. We recently evaluated a patient with anomalous origin of right coronary artery from the left coronary ostium and a bicuspid aortic valve. A description of these findings follows.
Angiography↗
Two-dimensional echocardiographic features of bicuspid aortic valve.
The two-dimensional cross-sectional echocardiographic diagnosis of bicuspid aortic valves is described and compared with results of M-mode echocardiograms. Aortic valve anatomy was determined in 19 selected patients by angiography, and confirmed in five by direct surgical visualization. Using an eccentricity index (EI) of 1.3 or greater as diagnostic of bicuspid aortic valve, M-mode correctly identified anatomy in 14 of 19 valves (74 percent), although EI varied in several patients. For two-dimensional diagnosis of bicuspid aortic valve, short axis cross section was preferred, and criteria included number of cusps seen in real time motion, irregularity of folding of cusp margins, and location of commissural insertions. Two-dimensional echocardiography correctly identified anatomy in 18 of 19 valves (95 percent). Long axis cross section disclosed valvular doming in all 8 patients in whom doming was observed angiographically, correlating with hemodynamic findings. Two-dimensional echocardiography aids in the detection of bicuspid aortic valve in a suspected population, can give an estimate of valve gradients, and explains variability in M-mode findings. As such, two-dimensional echocardiography is a valuable tool in the noninvasive diagnosis of the bicuspid aortic valve.
Adolescent↗
Congenital bicuspid aortic valve after age 20.
The bicuspid aortic valve is recognized as a frequent cause of aortic stenosis in adults. Aortic stenosis has been reported to occur in as many as 72 percent of adults with a congenital bicuspid aortic valve, with peak incidence occurring in the 5th and 6th decades of life. Review of the clinical records of 152 patients aged 20 years and older found to have a bicuspid aortic valve at autopsy revealed aortic stenosis in only 28 percent. The incidence of aortic stenosis increased progressively with age; 46 percent of patients over age 50 years and 73 percent over age 70 years had some degree of stenosis. The stenotic valves were obstructed by nodular, calcareous masses but commissural fusion was present in only eight cases. The largest group of patients in the series (40 percent) died of infective endocarditis; 77 percent of these were under age 50 years. Primary aortic regurgitation without infective endocarditis was uncommon. Thirty-two percent of the patients in this series had an apparently normally functioning aortic valve, and this rate remained relatively constant with increasing age; 37 percent of patients over age 50 years and 27 percent over age 70 years had an apparently normal valve. The bicuspid aortic valve in patients over age 20 does not invariably become stenotic or insufficient.
Adult↗
Congenitally bicuspid aortic valves. Clinicogenetic study of 41 families.
The families of 41 patients with surgically proved isolated bicuspid aortic valves were examined. There were 275 first degree relatives of whom 220 were living, and 188 (85.5%) of these were examined. Seven first degree relatives were found to have aortic valve disease, and in a further 11 there was 'doubtful' evidence of bicuspid aortic valves. In 6 families there was more than 1 affected member and in an additional 7 families there was 1 or more 'doubtful' first degree relative. The minimum family incidence was therefore 14.6 per cent, or 31.7 per cent if 'doubtful' cases were included. The inheritance is most probably multifactorial, but occasionally the condition may occur as an autosomal dominant. The difficulties of diagnosing bicuspid aortic valves before the development of obstruction of left ventricular outflow were encountered and are discussed. The association of a bicuspid aortic valve with asymmetric septal hypertrophy, hypertrophic cardiomyopathy, and Marfan's syndrome was also noted.
Aortic Valve↗
Left coronary artery anatomy in patients with bicuspid aortic valves.
The anatomy of the proximal left coronary artery in 33 adult patients with bicuspid aortic valves was compared with that in 33 adult patients with aortic valve disease of other aetiologies and with that in 50 adult control patients with no valve or congenital heart disease. Patients with bicuspid aortic valves had a higher incidence of immediate bifurcation of the left main coronary artery, of left main coronary length less than 10 mm, and of left coronary artery dominance. The mean length of the left main coronary artery was significantly less in the patients with bicuspid aortic valves. These variations from the usual coronary artery anatomy may be part of the developmental abnormalities responsible for bicuspid aortic valves, and require evaluation and consideration when considering angiography and valve replacement in patients with aortic stenosis.
Anthropometry↗
Reversal of dominance of the coronary arterial system in isolated aortic stenosis and bicuspid aortic valve.
The dominance of the coronary arterial system was determined angiographically in 75 adult patients with isolated aortic stenosis with or without insufficiency, 25 adult patients with pure aortic insufficiency, 51 adult patients with combined aortic stenosis and mitral valve disease, and 44 children with bicuspid aortic valves. There was an unusually high incidence of left dominance in adults with isolated aortic stenosis (36%) and in children with bicuspid aortic valves (56.8%). These findings suggest the existence of a variant developmental complex consisting of a bicuspid aortic valve and reversal of dominance of the coronary arterial system. Furthermore, it adds support to the concept that progressive stenosis of a bicuspid aortic valve is frequently the etiology of isolated aortic stenosis in adults.
Adult↗
Dissecting aortic aneurysm associated with congenital bicuspid aortic valve.
Among 119 cases of fatal dissecting aneurysm of the aorta, exclusive of those iatrogenically caused or associated with arachnodactyly or aortic stenosis, there were observed 11 cases of congenital bicuspid aortic valve (9%). The ages ranged from 17 to 69 years, five of the patients being 29 years old or younger. Among the latter, three had coarctation of the aorta and one had Turner's syndrome without coarctation. In one of the older patients, aortic insufficiency was present. Hypertension was either established or inferred from cardiac weight in 73% of the cases. In each case, cystic medial necrosis of the aorta was present. Prolapse of valves other than the aortic was observed in 45% of the cases with bicuspid aortic valve. Compared to an estimated incidence of bicuspid aortic valve of about 1 to 2% in the population, the high incidence among subjects with dissecting aneurysm suggests a causative relationship between bicuspid aortic valve and aortic dissecting aneurysm.
Adolescent↗
[Radiological study of congenital mono and bicuspid aortic valves (author's transl)].
Bicuspid aortic valves are much more common than monocuspid valves and diagnosed by A. P. aortography and, above all, lateral views. One may distinguish the true bicuspid valves which include two Valsalva sinuses, often asymmetrical, and bicuspid valves whith a supplementary raphe, the commonest type, in which on angiography, one may distinguish three Valsalva sinuses, one of which is larger than the two others. These cases of bicuspid aortic valve may be associated with other malformations of the aorta or may occur alone. In the latter case, the main risks are aortic valvular stenosis or aortic incompetence.
Aortic Coarctation↗
[Echocardiographic assessment of aortic regurgitation and aortic root dilatation in bicuspid aortic valve].
Aortic regurgitation (AR) and aortic root dilatation in 29 consecutive patients with bicuspid aortic valves but without aortic root disease (20 males, and 9 females: aged 27-85 years) were studied using two-dimensional echocardiography. The normal ranges of aortic root dimensions were calculated from values of 185 normal subjects, as 95% confidence intervals. AR was observed in 17 patients by color flow mapping. In 12 of the 17 AR patients, no significant lesion of the aortic cusp was detected by two-dimensional echocardiography. These 12 AR patients were compared with 12 patients without AR. Increase in dimension of the aortic root was relatively frequent in the 12 AR patients at the aortic annulus (AA) (67 vs 17%, p < 0.05), and at the sinus of Valsalva (A1) (67 vs 17%, p < 0.05). At the ascending aorta 5 mm distal to the sinus of Valsalva (A2), the difference was not significant (58 vs 17%, p < 0.09). The 12 bicuspid AR patients without significant lesions of the aortic cusp were compared with 41 AR patients with normal tricuspid aortic valves. The frequencies of cases with increased aortic root dimension were 67 vs 46% (ns) at the AA, 67 vs 22% (p < 0.05) at A1 and 58 vs 5% at A2 (p < 0.01). Thus, aortic annular dilatation was thought to be the cause of AR in bicuspid and tricuspid aortic valves without significant lesions of the aortic cusps, and generalized dilatation of the aortic root was more frequent in bicuspid AR patients than in tricuspid AR patients.(ABSTRACT TRUNCATED AT 250 WORDS)
Adult↗
[Congenital bicuspid aortic valve: stenotic type and insufficient type].
Two-hundred and eighty-one patients underwent surgical treatment of the aortic valves during a 10-year period of 1981 to 1991, 32 of whom (11%) had bicuspid aortic valve. Bicuspid aortic valve is well known to cause calcified aortic stenosis, however, some of these cases develop pure aortic insufficiency of unknown etiology. In our studies of 32 patients with bicuspid aortic valve, 28 patients had aortic stenosis, 2 were aortic insufficiency and 2 were infective endocarditis. Pathogenesis of aortic insufficiency in patients with bicuspid aortic valves was discussed and compared with that of aortic stenosis.
Adult↗
The neural crest as a possible pathogenetic factor in coarctation of the aorta and bicuspid aortic valve.
Patients (n = 109) operated on for coarctation of the aorta were analyzed for occurrence of associated cardiac and noncardiac anomalies. Attention was also paid to the prevalence of cardiac anomalies in the relatives of these patients. Of the patients with coarctation of the aorta, 57 (52%) had a bicuspid aortic valve. Forty-three (39%) of the 109 patients had one or more noncardiac anomalies. In 29 (27%) patients the noncardiac anomaly involved the head/neck structures. Noncardiac anomalies were much more prevalent in patients with coarctation and bicuspid aortic valve, especially anomalies involving the head/neck structures: 44% compared to 8% of patients with a normal aortic valve. Congenital cardiac malformations were present in relatives in the first or second degree of 18% of the patients. Bicuspid aortic valve was more prevalent in patients with an affected relative (75%) than in patients with unaffected relatives (47%). Recent studies showed that the neural crest plays an important role in the development of cardiac and a variety of noncardiac structures. The cardiac structures derived from the neural crest involve the outflow tract of the heart and the aortic arch system. Maldevelopment of neural crest cells could therefore be responsible for the combined occurrence of outflow tract (e.g., bicuspid aortic valve), aortic arch (e.g., coarctation), and noncardiac anomalies. This study supports the concept that some anomalies of the aortic arch system, including aortic coarctation, are cardiovascular manifestations of a spectrum of anomalies involving the head and neck region that may be due to a genetic-environmental disorder of the neural crest.
Aortic Coarctation↗
[Congenital bicuspid aortic valve associated with aberrant coronary artery origin: a case report].
A 61-year-old man with congenital bicuspid aortic valve associated with aberrant coronary artery origin is presented. His aortic valve was severely stenotic with marked calcification. At the aortic valve replacement, it was found that the valve was markedly calcified and the bicuspid valve and both coronary ostia were very close to each other in a posteriorly positioned sinus of Valsalva. There have been a few reports concerning the combination of the bicuspid aortic valve and aberrant coronary artery origin, and it is well known that the bicuspid aortic valve more frequently accompanies short left main trunk or left coronary arterial dominance than a normal aortic valve. Some genetic mechanisms surrounding the coronary artery, aortic valve and aorta are suggested to occur with such anomalies.
Aortic Valve↗