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PubMed2000

Calcified bicuspid aortic valve mass prolapsing into the left main coronary artery.

We report a case of a mobile calcific mass on the aortic valve that prolapsed into the left main coronary artery of a 51-year-old man. This case and a review of the literature suggest that calcific embolization to coronary arteries is a rare but possibly underrecognized complication of calcified degenerative or bicuspid aortic valves. This potentially catastrophic complication of calcified aortic valves needs to be suspected and recognized in clinical practice.

Aortic Valve↗
PubMed2006

Fate of bicuspid aortic valves in patients undergoing aortic root repair or replacement for aortic root enlargement.

OBJECTIVE: To better understand the outcome of the bicuspid aortic valve (BAV) after valve-sparing root replacement, we evaluated BAV function after its preservation at the time of the ascending aorta (AA) repair. PATIENTS AND METHODS: From January 1, 1994, through March 31, 2002, BAVs were preserved during repair of AA aneurysms in 21 patients (mean +/- SD age, 45+/-12 years; 13 males). We reviewed the clinical and surgical records of these patients for baseline characteristics, operative procedures, and data at latest follow-up. RESULTS: The primary indication for operation was AA enlargement (median diameter, 55 mm; range, 43-65 mm) in 18 patients and severe aortic regurgitation (AR) in 3 patients. Preoperatively, the BAV exhibited mild or moderate stenosis in 5 patients (mean gradient, 25 mm Hg; range, 19-34 mm Hg), moderate AR in 2, and severe AR in 3. Aortic valve repair was performed in 11 patients. Seventeen patients had excision and graft replacement of the enlarged AA, and 4 patients had primary AA repair. There were no perioperative deaths. Moderate or severe AR was relieved in all patients early postoperatively and during follow-up (median, 2.5 years; maximum, 7.6 years). One patient required aortic valve replacement at 4.8 years for AR. An additional patient had recurrent, severe AR but was asymptomatic and has not undergone reoperation. CONCLUSION: Repair of diseased BAVs or preservation of functionally normal BAVs during surgery for AA aneurysms has a low perioperative risk, and early results are encouraging. Additional observation is necessary to determine whether this strategy has real advantages over early aortic valve replacement.

Adolescent↗
PubMedDate not supplied

[Antivitamin activity of thiaminase from freshwater bicuspid molluscs after parenteral administration of the enzyme to albino mice].

Thiaminase from bicuspid mollusc, after a single administration into mice (25 or 50 un, subcutaneously), caused a decrease in the activity of transketolase and alpha-ketoglutaric acid dehydrogenase in the animal tissues within 3 hrs. The maximal antivitaminous effect of thiaminase was manifested within 2-5 days and slightly decreased within 7 days (at a dose of 25 un). Amount of thiamin was decreased in mice liver tissue from 6.54+/-0.36 mcg/g to 4.84+/-0.23 mcg/g within the second day after the thiaminase administration. Transketolase and alpha-ketoglutarate dehydrogenase from liver and kidney were more sensitive to the antivitaminous effect of thiaminase. General condition of the animals was not altered by parenteral administration of thiaminase during a week. Thiaminase may be recommended for modelling of thiamin deficiency in animals maintained at conventional diets.

Animals↗
PubMed1999

Aortic medial changes associated with bicuspid aortic valve: myth or reality?

OBJECTIVE: To determine whether aortic medial changes are more severe in patients who require aortic valve replacement of congenitally bicuspid aortic valves (BAV) than in patients who require replacement of tricuspid aortic valves and acquired valvular disease (AVD). DESIGN: Aortas from autopsies of 14 patients with BAV and 25 with AVD were histologically assessed by two 'blinded' cardiovascular pathologists and analyzed independently with computer-aided morphometry. The aortic valves were examined for valvular fibrosis and calcification. SETTING: The patient population was from a tertiary-care facility. PATIENTS: Patients were selected by retrospective review of autopsy records for patient deaths after aortic valve replacement, over the period 1984 to 1995. RESULTS: There were no significant differences in age (P=0.89), sex (P=0.94), prevalence of systemic arterial hypertension (P=0.37), valvular degenerative changes (P=0.10 and P=1.0) or heart weights (P=0.60) between the two groups. Histological scores for aortic medial degenerative changes including elastic fragmentation, fibrosis and medionecrosis were not statistically different between the groups. However, morphometry demonstrated less elastic tissue in patients with BAV (P=0.003). CONCLUSION: Routine microscopy shows no significant difference in the degree of aortic medial degenerative changes between patients with BAV and AVD. However, morphometry shows less elastic tissue in the aortas of BAV patients. This may explain the anecdotal increase in aortic fragility and propensity for aortic dissection in these patients.

Aged↗
PubMed2000

[Aortic valve replacement for aortic valve stenosis due to congenital bicuspid aortic valve with abnormal positioning of coronary orifice, pseudotendon, and persistent left superior vena cava, report of a case].

A patient was a 65-year-old female who had a complaint of palpitation was diagnosed aortic valve stenosis due to congenital bicuspid aortic valve with pseudotendon by the echocardiographic examination. We suspected left single coronary artery by the aortography and the coronary artery angiography. Aortic valve replacement and resection of pseudotendon was performed with Carbomedics supra-annular aortic valve (21 A). During surgery, persistent left superior vena cava was detected. High-posterior take-off right coronary artery was casually detected at aortic closure. Ventricular fibrillation due to insufficient supply of cardioplegic solution at right coronary area frequently occurred after cardio-pulmonary bypass and percutaneous cardiopulmonary support was required. The patient was discharged 32 days after the operation. Preoperative and intraoperative evaluation was important in the case of aortic valvular disease.

Aged↗
PubMed2001

Aortic valve repair of congenital bicuspid aortic valve associated with aneurysm of the ascending aorta.

Two unusual cases of congenital bicuspid aortic valve associated with aneurysm of the ascending aorta are reported. One patient with a 7-cm ascending aortic dilatation and aortic regurgitation (AR) (II/IV), and another with a 6-cm ascending aorta and AR (III/IV), presented for treatment. Replacement of the ascending aorta and aortic valve repair were performed in both cases. Aortic valve repair included resection of the raphe, leaflet plication and subcommissural annuloplasty. Both patients had satisfactory results in the early postoperative period. Despite the promising outcomes after surgery in these patients, long-term changes in valve function and durability remain unknown. Additional close observation and monitoring are required before the procedure can be recommended as the standard of care.

Aged↗
PubMed2002

Bicuspid aortic valve: abnormal aortic elastic properties.

BACKGROUND AND AIMS OF THE STUDY: Bicuspid aortic valve (BAV) is frequently associated with clinically relevant abnormalities of the aorta, suggesting the existence of a common underlying developmental defect involving the aortic valve and wall of the ascending aorta. The study aim was to evaluate noninvasively the elastic properties of the aortic root in young males with BAV, to discover whether structural abnormalities of the aorta might be manifested by impairment in elasticity. METHODS: Forty-nine young male subjects with isolated BAV were consecutively detected during preenrollment military screening, and studied using transthoracic echocardiography. Data were compared with those obtained in 45 normal subjects, matched for gender and age. RESULTS: Patients and controls were comparable for body size, and systolic and diastolic blood pressures. BAVs were normally functioning in 18 patients (37%), and mildly regurgitant in 31 (63%). Measurements made by two-dimensional echocardiography showed that BAV patients had significantly larger aortic root dimensions at the annulus (2.4+/-0.2 versus 2.2+/-0.2 cm, p <0.001), at the sinus of Valsalva (3.3+/-0.4 versus 2.6+/-0.3 cm, p <0.001), at the sinotubular junction (2.9+/-0.3 versus 2.5+/-0.2 cm, p <0.001), and at the proximal ascending aorta (2.8+/-0.3 versus 2.5+/-0.2 cm, p <0.001). Measurements made using M-mode echocardiography at 3 cm from the annulus, showed the difference between systolic and diastolic diameters of the aortic root to be significantly smaller in patients than in controls (2.1+/-1.2 versus 3.0+/-1.1 mm, respectively, p <0.001). In patients and in controls, both aortic distensibility (2.7+/-1.5 versus 4.8+/-2.2 x 10(-6) cm2 dyne(-1), respectively, p <0.001) and aortic stiffness index (10.2+/-5.3 versus 5.03+/-1.97, respectively, p <0.001) were significantly different. CONCLUSION: Young male subjects with BAV and no or mild aortic regurgitation display large aortic size and abnormal elastic properties of the ascending aorta compared with controls. These findings confirm the notion that, in these patients, aortic root dilatation is a morphological correlate of intrinsic structural aortic abnormality.

Adult↗
PubMedDate not supplied

Congenitally bicuspid aortic valves in adults.

The congenitally bicuspid aortic valve occurs in more than 1% of the population. Although it may never cause difficulty and first be discovered at autopsy, more often it results in some hemodynamic abnormality. Its clinical manifestations are varied, and its early recognition is essential if we are to prevent the dreaded complication of infective endocarditis.

Adult↗
PubMed2003

Histopathological grading of ascending aortic aneurysm: comparison of patients with bicuspid versus tricuspid aortic valve.

BACKGROUND AND AIMS OF THE STUDY: Bicuspid aortic valve (BAV) is a common inherited condition that is often accompanied by ascending aortic aneurysm. A high level of histological wall abnormalities was reported to be present in non-dilated aortas of patients with BAV. In patients with tricuspid aortic valve, there appears to exist a direct relationship between the diameter of the ascending aorta and degree of histopathological aortic wall abnormalities. Whether this situation exists in patients with BAV has not yet been investigated. METHODS: Surgical and medical records of all patients undergoing surgery of the ascending aorta were reviewed. A total of 65 patients was identified in whom an aortic wall specimen was obtained intraoperatively. These specimens were systematically re-evaluated, and graded according to the severity of seven histopathological conditions: fibrosis, atherosclerosis, medionecrosis, cystic medial necrosis, smooth muscle cell orientation, elastic fiber fragmentation, and inflammation. RESULTS: BAVs were present in 26 patients (40%). Patients with BAV had significantly less aortic wall alterations than patients with tricuspid aortic valves (p < 0.001) in all variables examined. The severity of aortic wall abnormalities was significantly dependent on aortic diameter in patients with BAV as well as tricuspid aortic valve (p = 0.036 and 0.019), but dependent on age (p = 0.009) only in patients with tricuspid aortic valve. CONCLUSION: The study results provide evidence that ascending aortic aneurysm in patients with BAV differs clinically and histologically from that in patients with tricuspid aortic valve. Further studies are needed to elucidate the impact of inherited and acquired aortic wall abnormalities on the development of aneurysms.

Adult↗
PubMed2003

Complete situs inversus and bicuspid aortic valve stenosis.

Patients with congenital heart disease are increasingly surviving to adulthood and present interesting challenges during cardiac catheterization. Situs inversus with dextrocardia is a type of cardiac malposition most likely to exist with a structurally normal heart. We present the case of a patient with complete situs inversus and bicuspid aortic valve stenosis, a hitherto unreported association. Prototypical findings in this cardiac malposition are illustrated for proper recognition with emphasis on technical tips for left and right heart catheterization.

Aortic Valve Stenosis↗
PubMed2003

[Aortic valve replacement with a freestyle stentless valve using the modified subcoronary technique and hemiarch replacement for bicuspid aortic valve and ascending aortic aneurysma; report of a case].

An association between bicuspid aortic valve disease and ascending aortic aneurysma has long been recognized. Root replacement with a composite valve graft for such disease is a well-established technique. But it may involve serious technical difficulties, and may be a more time-consuming procedure than separate valve replacement and graft replacement. We performed an aortic valve replacement with Freestyle stentless valve using the modified subcoronary technique and hemiarch replacement for a 72-year-old man with severe aortic stenosis and ascending aortic aneurysma. Angiographic studies after surgery showed no residual aortic regurgitation (AR) and no deformity of aorta. This technique is an acceptable option for an aortic disease and ascending aneurysma in elderly patients.

Aged↗
PubMed1992

[Fatal infectious endocarditis in a patient with annulo-aortic ectasia and predominant bicuspid valve aortic stenosis. Anatomoclinical case].

A 25 year old female with bicuspid aortic valve and aortic stenosis developed infectious endocarditis due to beta hemolytic streptococcus. Specific antibiotic therapy was not successful, the patient developed multiple embolic episodes and platelet dysfunction that prevented surgery. The patient died 5 weeks after admission. Necropsy showed aortic annulo-ectasia, cystic medial necrosis and localized dissection of the proximal aorta. There were multiple aortic valve vegetations and evidence of inflammatory myocardial involvement, coronary embolization and myocardial infarction. Aortic annulo-ectasia should be investigated in patients with aortic valve endocarditis and early surgery would be advised to prevent aortic dissection.

Adult↗
PubMed2003

Aortic valve repair versus replacement in bicuspid aortic valve disease.

BACKGROUND AND AIM OF THE STUDY: The study aim was to compare the results of aortic valve repair and replacement with biological valves in adult patients with aortic insufficiency (AI) caused by congenital bicuspid aortic valve (BAV) METHODS: Forty-four patients who had aortic valve repair were matched for age and left ventricular function to 44 patients who had aortic valve replacement (AVR) with biological valves. Patients were followed annually using echocardiography. The mean follow up was 2.6 +/- 2.1 years for the repair group, and 3.5 +/- 2.1 years for the replacement group. Follow up was complete. RESULTS: There was no operative or late death in either group. Early postoperative echocardiography showed trace or no AI in 35 patients and mild AI in nine who had repair, and trace or no AI in 38 patients and mild AI in five who had AVR. The mean peak systolic gradient was 16.2 +/- 7.6 mmHg for repair and 13.2 +/- 7.2 mmHg for AVR. Four patients who had valve repair and two who had AVR, needed repeat aortic valve surgery because of progressive AI or endocarditis. Freedom from reoperation at five years was 91 +/- 5% for repair and 94 +/- 6% for replacement (p = 0.2), while freedom from moderate or severe AI at five years was 79 +/- 8% for repair and 94 +/- 6% for replacement (p = 0.024). The peak systolic gradient at follow up was 11.7 +/- 6.8 mmHg after repair and 13.3 +/- 9.6 mmHg after AVR (p = 0.4). There were no thromboembolic complications in either group. CONCLUSION: Repair of BAV is feasible in certain patients with AI, but the hemodynamics and clinical outcomes do not appear to be superior to AVR with biological valves during the first five years of follow up.

Adult↗
PubMed2004

[Surgery for aortic valve disease with congenital bicuspid aortic valve].

From January 1998 to December 2002, a total of 120 patients underwent aortic valve surgery at our institution. Of these, 26 patients (22%) had congenital bicuspid aortic valve (BAV). Main valvular lesion of BAV was aortic valve stenosis (AS) in 17 and regurgitation (AR) in 9. There was no significant difference in the aortic annular size between BAV and non-BAV cases measured by echocardiography preoperatively [22.8 +/- 2.0 mm versus 22.5 +/- 2.2 mm in AS (NS), and 25.4 +/- 2.4 mm versus 23.4 +/- 2.5 mm in AR (NS)]. At operation, however, supraannular type prosthetic valve was selected more frequently in BAV than in non-BAV because of the difficulty of implanting the proper size prosthetic valve in annular position in BAV [10/25 (40%) in BAV versus 18/91 (20%) in non-BAV (p < 0.05)]. Moreover, ascending aortic dimension in BAV cases was significantly larger than in non-BAV cases [43.7 +/- 7.3 mm versus 39.6 +/- 7.3 mm (p<0.05)]. These features were more prominent in non-raphe BAV cases. These results suggested that operative procedure including prosthetic valve selection needs careful consideration in BAV especially no-raphe patients.

Aged↗
PubMed1992

[Bicuspid aortic valve with sudden onset of aortic insufficiency due to rare causes: report of two cases].

This report describes two cases of aortic valve replacement which was warranted by the suddenly developed aortic regurgitation due to a congenitally bicuspid aortic valve. Aortic insufficiency was caused by the rupture of the strand which lifted a larger cusp to the aortic wall in one, and by the perforation of valvular aneurysm in another. Reviewing the literature these types of aortic insufficiency are rarely reported.

Acute Disease↗
PubMed2005

Results of valve preservation and repair for bicuspid aortic valve insufficiency.

BACKGROUND AND AIM OF THE STUDY: There is increasing interest in aortic valve-sparing and repair techniques for the treatment of aortic insufficiency (AI) and/or root aneurysm. The results of bicuspid aortic valve (BAV) repair at the authors' institution were evaluated. An attempt was made to assess the mode of failure and to identify surgical methods that provide durable repair results. METHODS: Aortic valve repair for BAV was performed in 71 patients (62 men, nine women; mean age 41.5 +/- 13.2 years) between 1993 and 2005. Repair techniques included cusp free margin plication (n = 47), subcommissural annuloplasty (n = 27), free margin reinforcement (n = 10), and cusp triangular resection (n = 7). Thirteen patients had remodeling of one or both aortic sinuses, and 16 had reimplantation of the aortic valve. The ascending aorta and arch were replaced in 29 and five patients, respectively. Concomitant mitral and coronary bypass surgeries were performed in 10 and three patients, respectively. RESULTS: There were no operative deaths. Early postoperative echocardiography revealed no or trace AI in 54 patients and mild AI in 17. There was only one late death which was non-cardiac-related. Eight-year freedom from endocarditis, thromboembolism and anticoagulation-related hemorrhage was 90%, 100% and 100%, respectively. Eight-year freedom from AI grade > or =3+ (moderate) and aortic valve replacement were 44% and 82%, respectively. At the latest follow up, 89% of patients were in NYHA functional class I. Patients who underwent aortic valve-sparing procedures had more stable valve function than those who had cusp repair and subcommissural plication. CONCLUSION: BAV repair is a safe procedure with good early functional results. However, recurrent AI remains a problem at five to eight years of follow up. Since dilation of the aortic root is a common cause of AI and a common feature of patients with BAV, aortic valve-sparing reimplantation operations should provide better long-term outcomes.

Adult↗
PubMed2006

Spatial patterns of matrix protein expression in dilated ascending aorta with aortic regurgitation: congenital bicuspid valve versus Marfan's syndrome.

BACKGROUND AND AIM OF THE STUDY: Aortic wall stress has been shown to increase locally at the convex aspect of the ascending tract when axial root motion is increased, as occurs in aortic valve regurgitation. The study aim was to assess the expression of extracellular matrix (ECM) proteins involved in stress-induced vascular remodeling in the convexity and the concavity of dilated ascending aortas with aortic valve regurgitation. METHODS: Aortic wall specimens, harvested at the convexity and concavity of eight dilated ascending aortas with severe aortic valve regurgitation underwent morphometry, Western blot, RT-PCR and confocal immunohistochemistry. Five patients (group A) had congenital bicuspid aortic valve (BAV), and three (group B) had Marfan's syndrome. Specimens from the aorta of three multi-organ donors served as controls. RESULTS: At morphometry, medial degeneration was more severe in the convexity than in the concavity, especially in group A. Western blot, RT-PCR and immunohistochemistry disclosed an asymmetric pattern in the expression of some ECM proteins (laminin, tenascin, fibronectin). Fibronectin was increased in the convexity of both groups compared to controls at Western blot. Immunohistochemistry confirmed this pattern only in BAV. Higher levels of tenascin were found in the convexity in group A. The laminin content was greater in the concavity than in the convexity of both groups, but in group B the type of laminin was different, with the beta2 chain particularly expressed, and almost absent in non-Marfan patients. Type I and type III collagens were more markedly reduced in the convexity than in the concavity in BAV. In group B, type I collagen was decreased and type III increased, but without any significant difference between the two aspects of the aorta. CONCLUSION: A tissue remodeling response to valve disease-related wall stress may underlie aortic dilatation with BAV regurgitation. Although morphometry showed similar changes in Marfan aortas, molecular investigations differentiated this condition, qualitatively, from BAV.

Adult↗
PubMed2006

[Hemiarch replacement and concomitant valve repair for proximal aortic aneurysm accompanying bicuspid aortic valve with mild regurgitation].

A 64-year-old man who had proximal aortic aneurysm and bicuspid aortic valve with mild regurgitation underwent hemiarch replacement and concomitant valve repair under selective cerebral perfusion and deep hypothermic circulatory arrest. Valve repair technique consisted of leaflet plication according to Schaefers and subcommissural annular plication. No homologous transfusion was required, and the patient was extubated 5.5 hours after surgery. The repaired valve showed trivial regurgitation and no stenosis, and remained stable 22 months after surgery. Schaefers' technique of aortic leaflet plication is simple and reproducible, and is therefore recommendable for such cases.

Aortic Aneurysm, Thoracic↗