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PubMed1984

Prevalence of aortic valve prolapse with bicuspid aortic valve and its relation to aortic regurgitation: a cross-sectional echocardiographic study.

Although aortic valve prolapse (AVP) has been suggested as a cause of aortic regurgitation (AR) in patients with bicuspid aortic valves, neither the frequency of AVP nor its relation to AR in this setting has been defined. To assess these relations, 64 patients with bicuspid aortic valves diagnosed by 2-dimensional echocardiography and 20 normal subjects, similarly distributed according to age and sex, were studied. The presence and degree of AVP were defined using 3 quantitative terms: aortic valve prolapse distance (AVPD), area (AVPA) and volume (AVPV). Each was corrected (c) for patient size with reference to the diameter of the aorta at the level of insertion of the valve cusps. In normal subjects, the AVPDc averaged 0.09 +/- 0.06 (range 0 to 0.16) and the AVPAc averaged 0.08 +/- 0.06 cm (range 0 to 0.15). In patients with bicuspid aortic valves, the AVPDc averaged 0.26 +/- 0.10 (range 0.11 to 0.59, p = 0.00005 vs normal subjects), whereas the AVPAc averaged 0.35 +/- 0.17 cm (range 0.05 to 0.90, p = 0.00005 vs normal subjects). When the AVPDc criteria were used, 81% of the bicuspid valves were abnormal; when the AVPAc criteria were used, 87% were abnormal. The degree of prolapse defined by the AVPVc, which considers both cusp area and degree of apical displacement, was significantly greater for patients with bicuspid aortic valve with clinical AR than for those without (p = 0.008). However, because of the overlap between groups, there was no point at which this measure uniquely separated patients with and without AR.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1994

Mucoid dysplasia of tricuspid and congenital bicuspid aortic valves in Syrian hamsters (Mesocricetus auratus).

A histological study was made of the aortic valves of 141 Syrian hamsters aged between 35 and 657 days, belonging to an inbred laboratory colony with a high incidence of congenital bicuspid aortic valves. A tricuspid aortic valve was found in 81 specimens, whereas the remaining 60 possessed a bicuspid aortic valve. In all bicuspid valves there were two aortic sinuses, a ventral and a dorsal, each supporting one cusp. Thirty-three (23.4%) of the 141 specimens showed mucoid dysplasia of the aortic valve. The defect was characterized by thickening of the valve cusps and disruption of the fibrosa layer accompanied by an increased amount of glycosaminoglycans. Ten (12.3%) of the 81 tricuspid aortic valves and 23 (38.3%) of the 60 bicuspid aortic valves were dysplastic. This difference was statistically significant (P < 0.001). The findings indicate that, in the Syrian hamster, the simultaneous occurrence of bicuspid aortic valve and aortic valve dysplasia is not a random event. However, the fact that these valve defects also occur independently suggests that there is no primary morphogenetic dependence between them, but that some other cause predisposes to their concurrence.

Animals↗
PubMedDate not supplied

Different hemodynamic stress of the ascending aorta wall in patients with bicuspid and tricuspid aortic valve.

It is unclear whether ascending aorta dilation in patients with bicuspid aortic valve is caused by abnormal hemodynamics or by a common developmental defect of the aortic valve and aortic wall. We performed an echocardiographic study to examine the differences in hemodynamic stress at the ascending aorta in patients with bicuspid and tricuspid aortic valve. We studied prospectively 58 consecutive patients referred for preoperative echocardiographic examination with aortic valve stenosis and either bicuspid or tricuspid valve and an ascending aortic diameter of </=4.5 cm. Echocardiographic examination was performed from the parasternal long-axis view using ALOKA SDD 5.500 (Aloka, Tokyo) with 3.5 MHz probe. With aortic wall tissue Doppler imaging we obtained wall motion velocity patterns from the anterolateral and posteromedial region of the ascending aorta. The tissue Doppler examination showed a significantly higher peak systolic wall velocity of the anterolateral region of the ascending aorta in patients with bicuspid aortic valve (12.2 +/- 4.3 cm/sec vs. 8.8 +/- 2.6 cm/sec, p = 0.047). We conclude that in patients with bicuspid aortic valve and aortic valve stenosis the anterolateral region of the ascending aorta is subject to greater hemodynamic stress than in patients with tricuspid aortic valve.

Aged↗
PubMed1978

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↗
PubMed1981

Coronary arterial anatomy in bicuspid aortic valve. Necropsy study of 100 hearts.

In a necropsy study, the conjoined cusps of 50 congenitally and 50 acquired bicuspid aortic valves most commonly involved the right and left aortic cusps. In hearts with congenitally bicuspid aortic valves, the left coronary ostium arose at or above the aortic sinotubular junction in 44 per cent, whereas the incidence for the left coronary ostium in the acquired group was 20 per cent and that for the right coronary ostium in both groups was less than 20 per cent. In hearts with congenitally bicuspid aortic valves, the incidence of left coronary dominance (26%) was higher than in normal hearts. In hearts with apparently acquired bicuspid aortic valves, this incidence was also higher than normal, possibly because of acquired fusion of atypical congenitally bicuspid valves in some cases. In both types of aortic valve disease, the length of the left main coronary artery was similar; this length, however, was significantly shorter in hearts with left coronary dominance than in those with right or shared dominance.

Adolescent↗
PubMedDate not supplied

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↗
PubMed1979

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↗
PubMed1982

Extreme distal migration of the mandibular second bicuspid. A variant of eruption.

A five-year study of 26,264 panoramic radiographs found distal positioning of the mandibular second bicuspid associated with a missing adjoining first molar in one of every 505 patients. Following early removal of the first molar, there is a 5% to 10% chance that the second bicuspid will migrate distally. It usually continues until it contacts the root of the second molar, and then rotates up into occlusion adjacent to the parallel with that tooth. Mesial drift seldom occurs in this circumstance; the resulting space between lower first and second bicuspids remains. Early conservative management can be very important in these cases, making periodic x-ray examination advisable whenever a molar has been lost and the adjoining bicuspid has not yet erupted.

Bicuspid↗
PubMed2005

[Valve repair surgery for incompetent bicuspid aortic valves].

BACKGROUND: Surgical valve repair is a good alternative for correction of incompetent bicuspid aortic valve. AIM: To report the early and late surgical, clinical and ecochardiographic results of surgical repair of incompetent bicuspid aortic valves. PATIENTS AND METHODS: Retrospective review of medical records of 18 patients aged 19 to 61 years, with incompetent bicuspid aortic valve in whom a valve repair was performed. Four patients had infectious endocarditis and 17 were in functional class I or II. Follow up ranged from 3 to 113 months after surgery. RESULTS: A triangular resection of the prolapsing larger cusp, which included the middle raphe, was performed in 17 cases; in 13 of these, a complementary subcommisural annuloplasty was performed. In the remaining case, with a perforation of the non-coronary cusp, a pericardial patch was implanted; this procedure was also performed in 2 other cases. In 3 cases large vegetations were removed. Postoperative transesophageal echocardiography showed no regurgitation in 11 patients (62%) and mild regurgitation in 7 (38%). There was no operative morbidity or mortality. There were no deaths during the follow-up period. In 3 patients (17%) the aortic valve was replaced with a mechanical prosthesis, 8 to 108 months after the first operation. Reoperation was not needed in 93%+/-6,4% at 1 year and 85%+/-9,5% at 5 years, these patients were all in functional class I at the end of the follow-up period. 60% had no aortic regurgitation, 20% had mild and 20% moderate aortic regurgitation on echocardiographic examination. A significant reduction of the diastolic diameter of the left ventricle was observed, but there were no significant changes in systolic diameter or shortening fraction. CONCLUSIONS: Surgical repair of incompetent bicuspid aortic valves has low operative morbidity and mortality and has a low risk of reoperation.

Adult↗
PubMed2005

Echocardiographic and hemodynamic characteristics of reconstructed bicuspid aortic valves at rest and exercise.

Repair of diseased bicuspid aortic valves has gained increasing interest as an alternative to conventional valve replacement. Hemodynamic data at exercise have not been reported before. The aim of this study was to investigate the clinical and echocardiographic status of patients after bicuspid aortic valve repair at rest and exercise. Between 03/94 and 09/02 a reconstruction of an incompetent bicuspid aortic valve was performed in 25 patients (mean age 35+/-12.1 years, group A, mean insufficiency 2.8 preoperatively). Patients were investigated clinically and echocardiographically after 2.1+/-2.4 (0.1-8.9) years at rest and exercise and compared to 20 controls (group B). Clinical followup was complete. There were no deaths, reoperations, thromboembolic or bleeding complications. At last examination 21 patients were in NYHA class I, n=4 in NYHA class II and mean aortic valve insufficiency (AI) was 1.0 with one patient having an AI>II degrees. Maximum and mean pressure gradient (dPmax/mean) across the aortic valve at rest were 14+/-5.5/7+/-2.6 mmHg for patients of group A and 7+/-2.5/3.6+/-1.1 mmHg in group B. Mean AVA at rest was 2.6+/-0.8 (group A) vs 2.9+/-0.6 cm(2) (group B, p=0.025), valvular resistance 13.4+/-4.8 (group A) vs 13.6+/-2.9 dyn x s x cm(-5) (group B, p>0.05). All individuals were stressed up to 100 W (dPmax/mean 21+/-6.8/11+/-3.6, group A vs 11+/-2.9/6+/-1.3 mmHg, group B). 56% of group A and 85% of group B could be stressed up to 175 W with dPmax/mean 24.5+/-8.3/12+/-4.2 and 16+/-3.6/8+/-1.4 mmHg, respectively (p<0. 01). Heart rate and blood pressure behavior were comparable. Left ventricular mass regression (preoperatively 369.3+/-76.4 vs 277.3+/-80.7 g at last examination, p<0.01) was significant in group A but did not reach normal values (group B, 227.8+/-71.1; p<0.01). Bicuspid aortic valve reconstruction reduces left ventricular volume load significantly. Although residual mild subclinical obstruction and incompetence were observed, the behavior of hemodynamics at exercise was comparable to controls. The clinical relevance of these findings in long term follow-up has to be evaluated.

Adolescent↗
PubMed2000

[Reconstruction of the bicuspid aortic valve].

Reconstruction of a regurgitant bicuspid aortic valve is a new alternative to aortic valve replacement. With concomitant aortic root dilatation adequate reconstruction is feasible by valve-sparing aortic replacement. Between 10/95 and 02/00, 30 patients underwent reconstruction of a regurgitant bicuspid aortic valve. Additional aortic replacement was performed in 23 cases. Valve reconstruction was performed by plication of the prolapsing leaflet. No patient died peri- or postoperatively. Freedom from aortic valve regurgitation > or = II as well as freedom from reoperation were 100% after 48 months. Reconstruction of a regurgitant bicuspid aortic valve is feasible with encouraging mid-term results. With concomitant dilatation of the ascending aorta, a combination of aortic replacement and valve reconstruction can achieve stable results even in bicuspid valve anatomy.

Adult↗
PubMed2006

Successful pregnancy achieved by assisted reproductive techniques in a woman with severe congenital bicuspid aortic valve stenosis.

OBJECTIVES: To report a twin pregnancy achieved by assisted reproductive techniques in a woman with severe congenital bicuspid aortic valve stenosis. METHODS: Intracytoplasmic sperm injection and embryo transfer was performed in a 38-year-old woman with a complaint of infertility for 8 years due to male factor and who previously had the diagnosis of severe congenital bicuspid aortic stenosis. Clinical and echocardiography follow-up during pregnancy was done. RESULTS: The woman had severe congenital aortic stenosis with a valve area of 0.4 cm(2) and a peak gradient of 57 mmHg. In the first trial, twin pregnancy was achieved. She had an uneventful course of pregnancy in terms of cardiac functions. She underwent caesarean section at 36 weeks of gestation and had a healthy female and a male newborns. CONCLUSIONS: A woman with a severe congenital bicuspid aortic valve may get pregnant and deliver healthy newborns with intensive prenatal care and follow-up. The severe congenital bicuspid aortic valve stenosis may not be considered an absolute contraindication for assisted reproductive techniques and pregnancy.

Adult↗
PubMed1981

Congenitally bicuspid aortic valve causing severe, pure aortic regurgitation without superimposed infective endocarditis. Analysis of 13 patients requiring aortic valve replacement.

Although stenosis and infective endocarditis are commonly appreciated complications of the congenitally bicuspid aortic valve, pure severe aortic regurgitation complicating this congenital malformation, unassociated with either stenosis or infection, is not well recognized. Among 189 patients who had aortic valve replacement at the National Heart, Lung, and Blood Institute because of isolated pure aortic regurgitation, the congenitally bicuspid aortic valve, never the site of infective endocarditis, was responsible for the aortic regurgitation in 13 (7 percent). This report describes certain clinical and morphologic findings in 13 men, aged 26 to 65 years (mean 43), who required aortic valve replacement because of severe aortic regurgitation secondary to a noninfected, nonstenotic congenitally bicuspid aortic valve. Although not generally recognized, the noninfected congenitally bicuspid aortic valve is an important cause of pure aortic regurgitation severe enough to warrant aortic valve replacement.

Adult↗
PubMed1983

Accuracy of 2-dimensional echocardiographic diagnosis of congenitally bicuspid aortic valve: echocardiographic-anatomic correlation in 115 patients.

The preoperative 2-dimensional (2-D) echocardiograms of all patients less than 50 years of age in whom the aortic valve had been directly inspected by the surgeon or the pathologist or both were reviewed. From June 1977 to June 1981, 283 patients aged less than or equal to 50 years had aortic valve surgery at the Mayo Clinic: 115 (aged 1 to 50 years [mean 32]) had 2-D examinations preoperatively. The echocardiograms were reviewed blindly, and the aortic valve structure was categorized as bicuspid, tricuspid, or indeterminate. On the basis of combined surgical and pathologic inspection, 50 aortic valves were congenitally bicuspid, 60 were tricuspid, 4 were unicommissural, and 1 was quadricuspid. By 2-D echocardiography, the number of cusps was indeterminate in 29 patients (25%). When these patients were excluded, the sensitivity, specificity, and diagnostic accuracy of 2-D echocardiography for bicuspid aortic valve were 78,96, and 93%, respectively. Thus, with adequate 2-D images, echocardiography is a sensitive and highly specific technique for the diagnosis of bicuspid aortic valve.

Adolescent↗
PubMed1990

Contrasting histoarchitecture of calcified leaflets from stenotic bicuspid versus stenotic tricuspid aortic valves.

Preliminary findings from clinical trials of percutaneous balloon aortic valvuloplasty and intraoperative debridement of calcific deposits in patients with aortic stenosis have suggested that calcified, congenitally bicuspid aortic valves may be less amenable to these techniques than are calcified tricuspid aortic valves. Accordingly, we evaluated the histoarchitecture of calcific deposits in 30 operatively excised aortic valves. Light microscopic sections taken through the calcified aortic valve leaflets disclosed two principal types of histoarchitecture. In 11 aortic valves nodular calcific deposits were superimposed on an underlying fibrotic aortic valve leaflet (type A); in 17 valves calcific deposits were diffusely distributed throughout the body (spongiosa) of the aortic valve leaflets (type B). Two aortic valves could not be classified histologically. These histologic subtypes were not randomly distributed with regard to gross valvular morphology. All 14 bicuspid valves (100%) were type B; in contrast, 11 (69%) of 16 tricuspid aortic valves were type A, and only 3 (19%) of 16 tricuspid valves were type B (p less than 0.01). Both valves with nonclassifiable histologic features were tricuspid on the basis of gross examination. Thus, the histoarchitectural distribution of calcific deposits is different for bicuspid than for tricuspid stenotic aortic valves. The more diffuse distribution of calcium throughout the body of calcified bicuspid aortic valve leaflets may render these valves less amenable to operative and percutaneous valvuloplasty than are calcified tricuspid aortic valve leaflets on which calcific deposits are typically superimposed in nodular form.

Aged↗
PubMed2005

Initial experience with a bicuspid polytetrafluoroethylene pulmonary valve in 41 children and adults: a new option for right ventricular outflow tract reconstruction.

BACKGROUND: As the population of children with repaired congenital heart disease ages, an increasing number of patients will benefit from pulmonary valve insertion. We report our initial experience in 41 consecutive patients treated with placement of a surgically created polytetrafluoroethylene bicuspid pulmonary valve. METHODS: A bicuspid pulmonary valve with orifice size greater than 24 mm is created with polytetrafluoroethylene and sutured into the right ventricular outflow tract. To obviate the need for reoperation in growing children, this technique is limited to older children and adults. Polytetrafluoroethylene bicuspid pulmonary valves were placed in 41 patients (age: range, 5.0 to 64.7 years, median = 15.7 years; weight: range, 14.2 to 99.0 kilograms, median, 52.0 kg). All patients had pulmonary insufficiency, pulmonary stenosis, or both, after previous intervention for tetralogy of Fallot (27), pulmonary stenosis (11), pulmonary atresia with intact ventricular septum (2), or double outlet right ventricle (1). RESULTS: All patients left the operating theater with transesophageal echocardiography documenting no pulmonic stenosis and zero to trace pulmonic insufficiency. Median hospital length of stay was 5 days (range, 3 to 15 days; mean, 5.8 days). Follow-up including echocardiography ranged from 0.2 to 3.1 year (median follow-up, 1.5 years) and revealed significant improvement in New York Heart Association Classification, pulmonary insufficiency, and right ventricular end diastolic dimension. CONCLUSIONS: Polytetrafluoroethylene bicuspid pulmonary valve reconstruction of the right ventricular outflow tract is a safe, effective, and durable technique for the short term. Appropriate oversizing minimizes outflow tract obstruction while maximizing competence. Long-term follow-up is necessary to determine the true value of this technique.

Adolescent↗
PubMed2006

Pulmonary artery aneurysm with ascending aortic aneurysm concomitant with bilateral bicuspid semilunar valves.

Aneurysm of both the pulmonary trunk and the ascending aorta concomitant with bilateral bicuspid valves is very rare. The reason for the formation of aneurysm with bicuspid semilunar valve is still inconclusive. Surgical repair was performed successfully (ie, aortic valve replacement, graft replacement for the ascending aorta, and plication of the pulmonary artery). Pathology of the pulmonary artery wall did not demonstrate cystic medial necrosis. The hemodynamic turbulence by the bilateral bicuspid valve may cause the formation of aneurysms even at low pressure. This case demonstrates an explanation for aneurysm with the bicuspid valve.

Aneurysm↗
PubMedDate not supplied

Calcific bicuspid aortic valve disease in a patient with Cornelia de Lange syndrome: linking altered Notch signaling to aortic valve disease.

We describe a patient with Cornelia de Lange syndrome (CdLS) and a severely calcified bicuspid aortic valve. Cornelia de Lange syndrome is characterized by altered Notch signaling, and recent studies have provided a link between Notch signaling and heart valve development and calcific bicuspid aortic valve disease. In this case report, we propose that altered Notch signaling in CdLS may be causally linked to the calcific bicuspid aortic valve disease in these patients. Patients with CdLS should undergo routine echocardiographic examination for possible congenital cardiac defects including bicuspid aortic valve.

Adult↗