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PubMed1998

[A case report of aortic valve replacement and ascending + hemi aortic arch replacement for congenital bicuspid aortic stenosis with poststenotic dilatation of ascending aorta].

The patient of 21-year-old female, who had undergone VSD closures at the age 5 and had been pointed out to have aortic valve stenosis at that time, was admitted to our hospital with complaints of palpitation and easy fatigability. Pressure gradient of 140 mmHg between left ventricle and ascending aorta, and poststenotic dilatation of max 55 mm in diameter from ascending aorta to hemi aortic arch was recognized by cardiac catheterization and aortogram. For fear of aortic dissection in late phase caused by dilatation of ascending aorta left over, graft replacement from ascending aorta to hemi aortic arch was carried out simultaneously adding to aortic valve replacement (AVR). We are of the opinion that not only AVR but also simultaneous graft replacement should be performed actively on the case with dilatation of ascending aorta of over 55 mm in diameter in order to prevent aortic dissection.

Adult↗
PubMed2002

Ultrasound evaluation of aortic valve anatomy in the fetus.

OBJECTIVE: To assess the feasibility of ultrasound identification of aortic valve anatomy in the fetus, with particular emphasis on the detection of bicuspid aortic valve. METHODS: This study was a prospective analysis of 21 fetuses with prenatally diagnosed congenital left heart obstructive lesions and 45 normal fetuses undergoing routine ultrasound evaluated at a tertiary referral center. These fetuses underwent detailed echocardiography, including the study of the aortic valve on a targeted short-axis view of the right ventricle. Necropsies or postnatal echocardiograms were available for confirmation of the diagnosis in all cases. RESULTS: Aortic cusps and commissures were satisfactorily visualized in 38/45 (84%) normal fetuses and in 18/21 (86%) fetuses with congenital heart disease. The aortic valve was correctly defined as bicuspid in one normal fetus and in six fetuses with congenital heart disease. In two fetuses with a positive family history, the bicuspid aortic valve was isolated. There was one incorrect diagnosis (a unicuspid unicommissural valve diagnosed prenatally as a bicuspid aortic valve in a fetus with severe aortic stenosis) and one false-positive diagnosis in a fetus diagnosed with a coarctation and a bicuspid aortic valve late in the third trimester of pregnancy and in which both anomalies were not confirmed at neonatal echocardiography. CONCLUSIONS: This study demonstrated that aortic valve anatomy can be satisfactorily assessed in fetuses with and without left heart obstructive lesions. We believe that a detailed search for a bicuspid aortic valve should be attempted in all patients referred for a positive family history of congenital heart disease, in general, and of left ventricle outflow tract obstruction or bicuspid aortic valve, in particular. In fact, the presence of an asymptomatic bicuspid aortic valve has been demonstrated to represent an important factor predisposing to the development of bacterial endocarditis and dissecting aortic aneurysm late in adult life. Therefore, an early detection of such an anomaly may contribute to ensure a longer symptom-free lifespan of individuals with the most common cardiac anomaly at birth.

Aortic Coarctation↗
PubMed2000

Coronary artery anomalies and aortic valve morphology in the Syrian hamster.

In the Syrian hamster, anomalies in the origin of the left coronary artery are significantly associated with the bicuspid condition of the aortic valve. In this species, bicuspid aortic valves are expressions of a trait, the variation of which takes the form of a phenotypic continuum, ranging from a tricuspid aortic valve with no commissural fusion to a bicuspid aortic valve with the aortic sinuses located in ventrodorsal orientation and devoid of any raphe. The intermediate stages of the continuum are represented by tricuspid aortic valves with a more or less extensive fusion of the ventral commissure and bicuspid aortic valves with a more or less developed raphe located in the ventral aortic sinus. The present study was designed to decide whether there is a gap between tricuspid and bicuspid aortic valves regarding the incidence of coronary artery anomalies, or whether this incidence varies according to the different tricuspid and bicuspid morphotypes of the continuum. The study was carried out in Syrian hamsters belonging to a single inbred family with a high incidence of tricuspid aortic valves with fusion of the ventral commissure, bicuspid aortic valves, and anomalies in the origin of the left coronary artery, i.e. single right coronary artery ostium in aorta, anomalous origin of the left coronary artery from the pulmonary artery, and anomalous origin of the left coronary artery from the dorsal aortic sinus. The specimens were examined by means of a stereomicroscope and, in several cases, scanning electron microscopy was also used. The relationships between anomalous coronary artery patterns and aortic valve morphologies were tested using a logistic regression model. The results obtained indicate that there is no discontinuity between tricuspid and bicuspid aortic valves regarding the incidence of coronary artery anomalies. The probability of occurrence of anomalous coronary artery patterns increases continuously according to the deviation degree of the aortic valve from its normal (tricuspid) design. The present findings suggest that in the Syrian hamster, the morphogenetic mechanisms involved in the formation of congenital anomalous aortic valves and anomalies in the origin of the left coronary artery, respectively, are strongly related from an aetiological viewpoint.

Animals↗
PubMed1975

Multiple extraction patterns in severe discrepancy cases.

Thirty-five cases have been collected from colleagues which illustrate that removal of additional maxillary teeth, following first bicuspid extractions, can allow the successful resolution of difficult discrepancy and anchorage cases. Charts 1 and 2 describe the amounts of space that might be expected by removal of additional upper bicuspids, upper first molars, and upper second molars. The findings on upper second molars are admittedly limited. Anchorage values as expressed by an efficiency percentage were approximately what would be expected from a study of anchorage values of the roots of teeth. The removal of upper second bicuspids has a better anchorage efficiency potential than the upper first molar, but this may be overcome somewhat by the greater size of the molar. Clear guidance cannot be given as to which teeth to remove in a specific case, but it is the observation of the author that for cases that are still in full Class II following four bicuspid space closure, upper second bicuspid removal would be more helpful from an anchorage perspective, whereas for cases that are in end-to-end molar relationship or require only a few millimeters to move into Class I, the upper first molar might be the tooth of choice. Also, the supper first molar removal allows for a more "normal" appearing arch assuming normal alignment and size of the upper second and third molars. The comparison with the nonextraction control group showed an enormous difference in the amount of incisor retraction that extractions provide when related to the maxilla. The nonextraction control group, though experiencing dramatic correction of Class II relationships, showed no incisor movement within the maxilla. Some problems which appeared in the sample were described. Removal of upper teeth in addition to the four first bicuspids can be a solution to an occasional anchorage, skeletal, growth or cooperation problem.

Adolescent↗
PubMedDate not supplied

Anomalous origin of the left coronary artery from the pulmonary trunk and its relationship with the morphology of the cardiac semilunar valves in Syrian hamsters.

The conditions of the aortic and pulmonary valves and the arrangement in the origin of the coronary arteries were studied in 247 Syrian hamsters belonging to a single family subjected to high endogamous pressure. Most specimens (n = 216) were examined using a stereomicroscope. The remaining 31 were studied histologically. In 110 specimens both cardiac semilunar valves were normal. The aortic valve was bicuspid and the pulmonary valve was normal in 79 animals, while a normal aortic valve and a bicuspid pulmonary valve occurred in 37. In the remaining 21 specimens both semilunar valves were bicuspid. In 34 cases, the left coronary artery originated from the pulmonary trunk. Statistical analyses indicate that there is no significant relationship between the bicuspid condition of the pulmonary valve and the fact that a coronary artery arises from the pulmonary trunk. On the other hand, they substantiate that the frequency of this coronary artery anomaly significantly increases when the aortic valve is bicuspid. The present findings agree with the hypothesis that abnormal migration of the neural crest cells may be responsible for the combined occurrence of bicuspid aortic valve and anomalous origin of the left coronary artery from the pulmonary trunk. In addition, they suggest that the neural crest cells involved in the formation of the pulmonary valve diverge from those migrating into the aortic valve and those imposing spatial order upon the development of the proximal coronary arteries.

Animals↗
PubMed1996

Demographic characteristics of patients undergoing aortic valve replacement for stenosis: relation to valve morphology.

OBJECTIVE: To determine the relative importance of the different causes of isolated aortic valve stenosis in a surgical series, and to relate these to patient characteristics including the rate of insertion of bypass grafts for coronary artery disease. DESIGN: Survey of the clinical and pathological data on patients undergoing aortic valve replacement for isolated stenosis. SETTING: Tertiary care cardiothoracic surgical unit. PATIENTS AND METHODS: 465 adult patients undergoing aortic valve replacement representing a consecutive series in one surgical unit. Retrospective review of patients records and classification of cause of aortic stenosis based on pathological examination of excised valve cusps. RESULTS: 63.7% patients had calcific bicuspid valves, 26.9% tricuspid calcific valves, and 5.4% rheumatic, 2.6% mixed pathology and 1.5% unicommissural valves. The ratio of males to females for bicuspid valves was 1.85:1 and for tricuspid calcific valves 0.76:1. The mean age of patients with bicuspid valves was 64.9 years compared with 73.4 years for those with tricuspid valves. Some 22.3% of patients with bicuspid valves and 44.8% of those with tricuspid valves had sufficient coronary artery disease to necessitate insertion of coronary bypass grafts. The differential rate of insertion of coronary bypass grafts was independent of age. CONCLUSIONS: Bicuspid calcified aortic valves are the predominant cause of isolated aortic valve stenosis followed by tricuspid calcified aortic valves. The sex and age distribution of bicuspid and tricuspid calcific aortic valve stenosis is different. The higher rate of insertion of vascular grafts in tricuspid calcific aortic valves may indicate that risk factors for atherosclerosis enhance cusp calcification in these patients.

Adult↗
PubMed1988

Anatomically isolated aortic valve disease. Morphologic study of 100 cases at autopsy.

Anatomically isolated aortic valve disease accounted for 1.1% of all and 5.2% of cardiac autopsies over a 20 year period. Among a total of 100 symptomatic cases, 52% had congenitally bicuspid, 43% had tricuspid and 5% had congenitally unicuspid valves. Nineteen percent had undergone fibrous sclerosis, including 1% unicuspid, 3% tricuspid and 15% bicuspid valves. Thirty nine percent had been affected by infective endocarditis, including 20 bicuspid and 19 tricuspid valves. The remaining 42 showed fibrocalcification, including 4 unicuspid, 17 bicuspid and 21 tricuspid valves. The nature of the valvular disease showed a correlation with the age of the patient. Infective endocarditis, fibrosis and calcific disease occurred in an ascending age pattern, at average ages of around 30 years, mid to late thirties and mid to late forties, respectively. The lesions occurred much earlier on the bicuspid than on the tricuspid valves, except for infective endocarditis. The bicuspid deformity was not found to make the aortic valve more prone to infection, nor did infection occur earlier on it than on the tricuspid valve. Four of the 100 cases, all tricuspid, were considered to be of rheumatic origin, the reasons for which are discussed. Certain well established associated cardiac lesions were identified.

Age Factors↗
PubMed1990

Variations in atrioventricular valve innervation in four species of mammals.

In this series of studies, the innervation patterns of whole-mount preparations of bicuspid and tricuspid valves were studied by light microscopy in the mouse, rat, guinea pig, and opossum. The acetylcholinesterase-positive networks of nerve fibers showed many similarities in the basic patterns of valve innervation in all of the species studied, but several interspecies variations were observed. The basal zone of the valve adjacent to the fibromuscular atrioventricular ring displayed the most dense plexus of nerves, with acetylcholinesterase-positive fibers being seen across the width of the valve. In the intermediate zone of the valve, less dense plexuses of nerve fibers were found; and these were more numerous in the cuspal areas and less numerous in the intervening commissural areas. In the distal portions of the valve, nerve networks arborized extensively, with some of their nerve fibers extending toward the chordae tendineae and the free edges of the valve cusps. Only in the guinea pig and opossum did these fibers reach the free margin of the valve cusp, where they either ended directly as free nerve endings or lay parallel to the free edge of the cusp, often running between adjacent chordae tendineae. Although the patterns of innervation were similar in both bicuspid and tricuspid valves, the innervation density of the bicuspid valve was greater than that of the tricuspid valve for each species examined. A distinguishing feature of guinea pig and opossum tricuspid valves was that their chordae tendineae were relatively more prominent and more densely innervated than the bicuspid chordae tendineae. Free nerve endings with no light microscopic evidence of specialization were present throughout the bicuspid and tricuspid valves of all species studied. Some nerve endings in the opossum showed evidence of specialization, with brush-like arborizations leading to presumed free terminals seen chiefly in the distal zone of the valve cusps. Although some general tendencies were apparent, we have demonstrated that interspecies heterogeneity exists in the terminal networks of the atrioventricular valves of mouse, rat, guinea pig, and opossum.

Animals↗
PubMed1994

Downsizing of valve allografts for use as right heart conduits.

In recent years, there has been a worsening shortage of small and intermediate-sized aortic and pulmonary allografts for use as right ventricular-to-pulmonary artery conduits in infants and children. However, there is a surfeit of large pulmonary and aortic allografts from adult donors. The feasibility of reducing a large allograft to a more useful size was examined using human pulmonary and aortic allografts. Eleven pulmonary allografts (11 to 26 mm in diameter) and nine aortic allografts (5 to 27 mm in diameter) were studied. Valve competence before downsizing was tested with a column of saline to a static pressure equivalent to the normal pulmonary pressure (20 mm Hg). Regurgitant flow was measured for 15 minutes. One complete cusp of the valve was excised, together with a longitudinal strip of the arterial wall. A bicuspid valved conduit was created by suturing the allograft longitudinally. The diameter of the bicuspid valve was measured with a dilator. A nomogram was constructed that predicts the size of the bicuspid allograft based on the size of the original allograft. The competence of the bicuspid allograft was tested and the regurgitant flow was compared with that of the original tricuspid allograft. The transvalvular systolic pressure gradient was measured with the bicuspid allograft placed in a pulsatile extracorporeal perfusion circuit at a flow rate of 1 L/min and a mean pressure of 20.5 +/- 2.6 mm Hg.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗
PubMed1995

Anomalous origin of the left coronary artery from the dorsal aortic sinus and its relationship with aortic valve morphology in Syrian hamsters.

The condition of the aortic valve and the origin of the coronary arteries were examined in 2413 Syrian hamsters aged between 1 and 823 days, belonging to nine inbred laboratory families. The specimens were studied with either a stereomicroscope, a corrosion-cast technique, or histological techniques (light microscopy). The aortic valve was tricuspid in 1823 (75.5%) cases, and bicuspid in the remaining 590 (24.5%). In all bicuspid aortic valves there were two aortic sinuses, a ventral and a dorsal, each supporting one cusp. The left coronary artery arose from the dorsal aortic sinus in 71 (2.9%) hamsters, eight of which died unexpectedly between ages 1 and 622 days. In 29 (40.8%) of the 71 cases, the aortic valve was tricuspid and the right coronary artery originated from the right aortic sinus. In the remaining 42 (59.2%) cases, the aortic valve was bicuspid and the right coronary artery arose from the right side of the ventral aortic sinus. Results of a chi 2 contingency test demonstrated that the frequency of left coronary artery arising from the dorsal aortic sinus significantly increased when the aortic valve was bicuspid. This fact, together with previously reported data on coronary artery anomalies in the Syrian hamster, suggests that the left coronary artery arising from the dorsal aortic sinus may be an expression of a single morphogenetic defect which is expressed as bicuspid aortic valve in some cases, anomalous origin of the left coronary artery in others, or in the simultaneous occurrence of these two cardiac abnormalities.

Animals↗
PubMed1991

Aortic valve morphology influences regurgitant volume in aortic regurgitation: in vitro evaluation.

STUDY OBJECTIVE: According to the Gorlin hydraulic orifice equation, aortic regurgitation volume can be determined by the regurgitant orifice cross sectional area, diastolic filling period, mean pressure gradient between the aorta and left ventricle, and a constant relating the coefficients of contraction (Cc) and velocity (Cv). This study was performed to determine whether variation in aortic valve morphology affects regurgitant flow volume, Cc and Cv. DESIGN: Four aortic valve templates, modelled after circular, rheumatic, degenerative, and bicuspid lesions, were constructed with equal orifice cross sectional areas in two sizes, 0.2 and 0.7 cm2. These valves were studied in vitro in a flow model of aortic regurgitation, wherein aortic pressure was regulated by varying the height of a column of fluid. Flow, pressure, and velocity were measured, and the coefficient Cc and Cv were calculated from standard equations. MEASUREMENTS AND MAIN RESULTS: Regurgitant volume was assessed at diastolic filling periods of 0.5 and 1.0 s and averaged 15% greater for bicuspid and degenerative as compared to circular or rheumatic valve shapes (p = 0.0001). This difference was accentuated at the shorter diastolic filling time and higher pressure gradient, such that bicuspid lesions allowed 29% more regurgitant flow across the 0.2 cm2 orifice at fluid height of 120 cm over 0.5 s. This difference in regurgitant volume between valve shapes was due to an increased Cc for the bicuspid and degenerative valve shapes, suggesting that they are more efficient orifices than rheumatic or circular valve shapes. CONCLUSIONS: Aortic valve morphology influences regurgitant volume in aortic regurgitation. Specifically, degenerative and bicuspid orifice shapes have a higher contraction coefficient and allow more regurgitant flow than rheumatic or circular orifices at a given driving pressure and diastolic filling time.

Aortic Valve↗