[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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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↗
Association of left dominant coronary arterial system with congenital bicuspid aortic valve.
Clinical angiographic studies have documented an association of left dominance of the coronary arteries with aortic stenosis and congenital bicuspid aortic valve. The postmortem arteriograms of 973 autopsy patients were reviewed for pattern of coronary dominance and the hearts examined for the nature of any aortic valve disease. There were 673 hearts (70 percent) with a right dominant pattern, 198 (20 percent) with equal dominance and 102 (10 percent) with left dominance. Of 34 hearts with congenital bicuspid aortic valve, 10 (29 percent) had left dominance, a difference significant at the 0.005 level. Of 44 hearts with calcific aortic stenosis, an acquired valve lesion, 9 (20 percent) had left dominance. Rheumatic aortic valve disease (47 cases) and aortic regurgitation (27 cases) had no apparent relation to the coronary arterial pattern. The results confirm the association of left coronary arterial dominance with congenital bicuspid aortic valve. It is suggested that a left dominant coronary system may arise as a consequence of disproportionately decreased blood flow in the left heart chambers, one cause of which is aortic valve stenosis, during early cardiogenesis.
Adolescent↗
The natural history of a non-stenotic bicuspid aortic valve.
Forty-one patients in whom the diagnosis of a non-stenotic bicuspid aortic valve had been established by noninvasive techniques were followed up for a mean of 10.9 years. During this period, 2 patients required aortic valve replacement because of the development of calcific aortic valve stenosis at the ages of 52 and 64 and 5 others developed evidence of mild aortic valve stenosis. The appearance of calcium in a bicuspid aortic valve suggests the possibility of subsequent calcific aortic stenosis, and patients with this feature should be carefully followed up. Bacterial endocarditis on the aortic valve occurred in 3 patients, one of whom developed severe aortic regurgitation and subsequently died. Patients with a bicuspid aortic valve are at definite risk from bacterial endocarditis and should receive appropriate antibiotic prophylaxis. In 26/41 (63%) patients there was no clinical change during the follow-up period, including 7 of the patients over the age of 50.
Adolescent↗
Spontaneous rupture of bicuspid aortic valve. An unusual cause of aortic insufficiency.
This report documents the clinicopathologic correlation between pure aortic regurgitation and an exceptional form of congenitally bicuspid aortic valve. The patient was known for many years to have mild aortic insufficiency. His condition suddenly deteriorated, with signs of an aggravated aortic regurgitation. Infectious endocarditis was considered, but the diagnosis was never established. Surgery revealed an exceptional form of a bicuspid aortic valve in which the conjoined cusp had prolapsed, due to rupture of a fibrous strand which previously had anchored the free rim of the cusp to the inner wall of the aorta. There were no signs of infectious endocarditis. It is suggested that spontaneous rupture of the cord caused the sudden aggravation of aortic regurgitation.
Aortic Rupture↗
Familial congenital bicuspid aortic valve: secondary calcific aortic stenosis and aortic aneurysm.
A patient with an aneurysm of the ascending aorta and calcific stenosis of a congenital bicuspid aortic valve, whose brother also had a stenotic congenital bicuspid aortic valve, is described. Predominant aortic stenosis at cardiac catheterization and the presence of an aneurysm distal to and not including the aortic valvular ring made the initial diagnosis of Marfan's syndrome unlikely. Cystic medial necrosis present in the aneurysmal wall probably arose as a consequence of poststenotic dilation. Adequate noninvasive evaluation of the ascending aorta requires echocardiographic studies, as well as a chest x-ray film.
Adult↗
[Bicuspid aortic valve. Echocardiographic diagnostic potentials].
The echocardiographic potentialities in the diagnosis of bicuspid aortic valve are discussed. In the group of 32 patients examined with a clinic and murmurs suspected for that disease, the index of excentricity in five patients, was found to be respectively 1.6, 1.4, 1.6, 1.8, 1.5, accepted as a reliable index for the diagnosis of aortic bicuspidia. Excentricity index is equal to 1/2 of the aortic diameter, divided to the minimal distance from the diastolic echo of the closed aortic valve to the closer aortic wall. The control group consisted of 44 patients with tricuspid aortic valve, 17 of them being with aortic defect (isolated stenosis or combined aortic defect) and the rest 28 -- without valvular heart disease. Excentricity index for the control group was low -- 1.0--1.33 (average value 1.28). Conclusions are drawn that the echocardiographic method, from the group of the bloodless methods, is with the best diagnostic potentialities in the bicuspid valve and should be looked for in patients with expulsion murmur and click of aortic origin.
Adolescent↗
The bicuspid nature of the valves of the peripheral collecting lymphatic vessels of the dog.
A controversy presently exists about the structure of lymphatic valves, the bicuspid structure vs. the funnel-like architechture. This present study, along with a review of the literature was undertaken in an attempt to clarify this controversy. Twenty lymphatic valves from the collecting lymphatic channels draining the back paw region in four dogs were examined and several were photographed. The bicuspid nature and the three dimensional anatomy of these valves is clearly demonstrated.
Animals↗
Results of surgical exposure of impacted cuspids and bicuspids in relation to patients' somatic and dental maturation.
The aim of the study was to correlate the rate of postoperative eruption of impacted teeth after surgical exposure to the patient?S' SOMATIC AND DENTAL DEVELOPMENT. THIS RELATIONSHIP WAS ANALYZED IN 31 PATIENTW WITH IMPACTED CUSPIDS OR BICUSPIDS, 12 OF WHOM WERE OVER 25 AND 19 UNDER 21 YEARS OF AGE. Surgical uncovering of the impacted teeth was carried out and the degree of subsequent eruption was evaluated 4 months after the operation. On uncovering the crown was exposed as far as the cementoenamel juction and the surrounding bone was covered with mucous membrane. Dental age and growth rate were used as variables describing individual somatic and dental maturation. After surgical uncovering eruption took place in all but one tooth. Eruption was quicker and more complete in children who were still growing than in young adults. The eruption process was also more favorable in dentitions still under formation than in cases with completed dental development. Early intervention and total surgical uncovering of the crown are advocated in cases of impacted teeth.
Adolescent↗
Congenital aortic stenosis secondary to dysplasia of congenital bicuspid aortic valves without commissural fusion.
The hearts of nine children with clinical evidence of congenital valve stenosis and a congenital bicuspid aortic valve were reviewed. Aortic stenosis was diagnosed on the basis of cardiac catheterization data in four patients, operative findings in two and auscultatory findings in three. The patients were 1 month to 9 years old; six were male and three female. In each patient the two commissures of the valve were free to the aortic wall. The cusps were thickened, rolled and redundant; microscopic studies revealed that they consisted of immature loose connective tissue consistent with a dysplastic or incompletely differentiated valve. The valve orifice was obstructed by the dysplastic cusps, and dysplastic changes rather than the commissural fusion were responsible for the observed aortic stenosis. These valves do not appear amenable to valvotomy because obstruction is due to the abnormal valve tissue. These findings may explain the occasional poor results of valvotomy in infants and children with congenital aortic valve stenosis.
Aortic Valve↗