Congenital quadricuspid aortic valve: report of nine surgical cases.

We report the clinicopathologic characteristics of the congenital quadricuspid aortic valve necessitating surgery. Among 616 patients for whom we performed an aortic valve operation over the past 20 years, nine patients (1.46%) (five men and four women, mean age 60 years) with quadricuspid aortic valve were encountered. All had aortic regurgitation (AR) except one with aortic stenosis and mild regurgitation (ASr). All were free of cardiac anomaly including that of the coronary arterial system. Macroscopically, severe calcification of the valve was seen in the one case of ASr. Fenestration of the cusp was seen in five cases of AR. Infective endocarditis was not seen. Histological study disclosed fibrous thickening and myxoid degeneration in the AR cases. In accordance with the Hurwitz and Roberts classification, four valves were type b (three equal-sized cusps and one smaller cusp), two valves were type a (four equal-sized cusps), two valves were type d (one large, two intermediate, and one small cusp), and one valve was type g (four unequal-sized cusps). Valve repair failed in one patient and was converted to valve replacement during the operation. All patients underwent successful aortic valve replacement (AVR).     

Classification of congenitally bicuspid aortic valve and its angiographic and surgical significance]

From January, 1978 to December, 1990, 85 patients with congenital bicuspid aortic valve underwent aortic valve replacements (AVR) with St. Jude Medical valve prosthesis. We classified congenital bicuspid aortic valve into four types. Type I (44.7%): Two cusps are situated right and left, a coronary artery arises from each related sinus of valsalva. Type II (22.4%): Type I + raphe in the right cusp. Type III (3.5%): one cusp is located anteriorly, the other posteriorly and both coronary arteries arise from anterior cusp. Type IV (29.4%): Type III + raphe in the anterior cusp. Regarding to preoperative diagnosis, aortic stenosis dominated in Type I (78.8%) and aortic regurgitation dominated in Type IV (72.0%). Implanted valve sizes were 22.2 +/- 1.8 (Type I), 23.4 +/- 1.6 (Type II) and 24.0 +/- 2.2 (Type IV). There was a significant difference between Type I and Type II, same as Type I and Type IV. Babb's method and outflow measurement method were utilized to predict the aortic annular size. However, both of them were not reliable for estimating the size of the aortic annulus in cases of aortic stenosis undergoing AVR with a 21 mm prosthesis.     

Pure aortic valve regurgitation due to congenital bicuspid valve--analysis of 7 cases and a report of 2 rare cases

The congenital bicuspid aortic valves usually become stenotic with severe calcification or regurgitant due to infective endocarditis (IE). However, pure aortic valve regurgitation without calcification or IE may be occurred. We report seven these cases out of 30 bicuspid valved patients who underwent aortic valve replacements. Pathological findings of these resected valves revealed neither calcium deposit nor findings of infection, but commonly showed myxoid degeneration. They were operated on at the mean age of 39, while those with calcified bicuspid aortic valves had an average age of 56. Two rare cases in whom an anomalous fibrous band on the larger cusp attached to the aortic wall were also reported.     

Ruptured sinus of valsalva aneurysm: early recurrence and fate of the aortic valve

BACKGROUND: We reviewed our experience with congenital ruptured sinus of Valsalva aneurysms (RSVA) to determine patterns of early recurrence and the fate of the aortic valve (AV). METHODS: Over a 28-year period, RSVA was identified in 34 patients, (mean age 31.6 years). Primary closure of the RSVA was performed in 10 patients, and a patch employed in 24. Aortic insufficiency was present in 24 patients. AV replacement (AVR) was performed in 5 patients; AV repair in 6. RESULTS: Follow-up of 9.2 +/- 8.3 years (6 months to 24 years) was complete in all but 2 patients. Five early fistula recurrences (in 4 patients) correlated with primary rather than patch closure (p < 0.03). Kaplan-Meier survival at 10 years is 90 +/- 7%. Freedom from reoperative AVR at 10 years is 83 +/- 9%. Late AVR was performed in 6 patients for progressive aortic insufficiency due to bicuspid valve (n = 3), cusp disease of affected sinus (n = 2), or aortic root dilatation (n = 2). CONCLUSIONS: Patch closure of the RSVA should be routinely employed. A bicuspid valve may be associated with the late need for AVR.     

Pure aortic regurgitation due to congenital bicuspid valve: a case report

A rare case of pure severe aortic regurgitation (AR) accompanied with congenital bicuspid aortic valve is presented. A 36-year-old female was admitted with rapid development of congestive heart failure. Examinations clarified severe AR with congenital bicuspid valve. Either calcification or vegetation on the valve was completely denied. At operation, both leaflets were apart from each other with a distance of 7 mm at the anterior commissura. A mechanical valve (Bj?rk-Shiley 23A) was sutured and she has been well. Pathological study of the resected leaflets revealed neither calcium deposit nor finding of infection but collagenous fibrous tissue with focal mucinous degeneration. A few cases of AR with bicuspid aortic valve have been reported in patients of annuloaortic ectasia. The partial annular ectasia was suspected as the cause of solitary aortic regurgitation with bicuspid aortic valve in our case.     

Management of aortic valve bypass surgery

The 3 leading causes of aortic stenosis (AS) in adults are calcific degeneration of a normal trileaflet aortic valve (AV), calcific degeneration of a congenital bicuspid AV, and rheumatic AS. Therapeutic options in patients with severe AS include aortic valve replacement (AVR), transcatheter aortic valve implantation (TAVI), or aortic valve bypass (AVB). An AVB involves the placement of a valved conduit between the apex of the left ventricle and the descending thoracic aorta. AVB serves as a useful alternative to treat severe AS in patients deemed high risk for conventional AVR (ie, porcelain aorta, previous cardiac surgery) or TAVI (ie, severe aorto-iliac disease, limited experience, lack of hybrid operating room). Advantages of on-pump AVB include the avoidance of aortic cannulation, cross-clamping, and cardioplegic cardiac arrest. The procedure can also be performed without cardiopulmonary bypass. In this article, the authors review the circulatory physiology, perioperative anesthetic management, the role of intraoperative transesophageal echocardiography, and surgical considerations of AVB surgery through 3 cases.     

Risk factors for requirement of permanent pacemaker implantation after aortic valve replacement

BACKGROUND: The aim of this study was to evaluate the frequency of requirement for permanent pacing and related risk factors after aortic valve replacement. METHODS: Among 465 patients operated between 1994 and 2004, 19(4.1%) patients with a mean age 49.9 +/- 17.2 years required the implantation of a permanent pacemaker. Eleven of them were female (57.9%). The main indication was aortic stenosis (89.5%). Severe annular calcification was documented in 78.9% of them, and the aortic valve was bicuspid in 57.9%. RESULTS: Risk factors for permanent pacing after aortic valve replacement (AVR) identified by univariate analysis were female sex, hypertension, preoperative ejection fraction, aortic stenosis, annular calcification, bicuspid aorta, presence of right bundle branch block (RBBB) or left bundle branch block (LBBB), prolonged aortic cross-clamp and perfusion times, and preoperative use of calcium channel blockers. Multivariate analysis showed that female sex (p = 0.01, OR; 5.21, 95% CI: 1.48-18.34), annular calcification (p < 0.001, OR; 0.05, 95% CI: 0.01-0.24), bicuspid aortic valve (p = 0.02, OR; 0.24, 95% CI: 0.07-0.84), presence of RBBB (p = 0.009, OR; 0.03, 95% CI: 0.003-0.44) or LBBB (p = 0.01, OR; 0.13, 95% CI: 0.02-0.69), hypertension (p = 0.03, OR; 0.22, 95%CI: 0.05-0.89), and total perfusion time (p = 0.002, OR; 1.05, 95% CI: 1.01-1.08) were associated risk factors. CONCLUSION: Irreversible atrioventricular block requiring a permanent pacemaker implantation is an uncommon complication after AVR. Risk factors are annular calcification, bicuspid aorta, female sex, presence of RBBB or LBBB, prolonged total perfusion time, and hypertension.     

Impact of preoperative 64-row multislice computed tomography for congenital aortic stenosis; report of a case

A 63-year-old woman was diagnosed as having severe aortic stenosis (AS) with 98 mmHg peak pressure gradient detected by echocardiography. Since, preoperative enhanced 64-row multislice computed tomography (MSCT) showed bicuspid aortic valve with only 2 sinuses of Valsalva, congenital aortic stenosis was suspected. The left and right coronary arteries originated from respective sinus of Valsalva, and severely thickened cusps of aortic valve were detected clearly by preoperative 64-row MSCT. Aortic valve replacement with a 21 mm ATS mechanical bileaflet prosthesis was performed without aortic annulus enlargement. The postoperative course was uneventful and postoperative 64-row MSCT indicated good performance of the ATS valve. Preoperative 64-row MSCT could be useful to detect complex aortic valve disease in detail. Moreover. 64-row MSCT might be a reliable tool to evaluate valvular heart disease.     

Calcification resistance with aluminum-ethanol treated porcine aortic valve bioprostheses in juvenile sheep

BACKGROUND: Calcification of glutaraldehyde fixed bioprosthetic heart valve replacements frequently leads to the clinical failure of these devices. Previous research by our group has demonstrated that ethanol pretreatment prevents bioprosthetic cusp calcification, but not aortic wall calcification. We have also shown that aluminum chloride pretreatment prevents bioprosthetic aortic wall calcification. This study evaluated the combined use of aluminum and ethanol to prevent both bioprosthetic porcine aortic valve cusp and aortic wall calcification in rat subcutaneous implants, and the juvenile sheep mitral valve replacement model. METHODS: Glutaraldehyde fixed cusps and aortic wall samples were pretreated sequentially first with aluminum chloride (AlCl3) followed by ethanol pretreatment. These samples were then implanted subdermally in rats with explants at 21 and 63 days. Stent mounted bioprostheses were prepared either sequentially as previously described or differentially with AlCl3 exposure restricted to the aortic wall followed by ethanol pretreatment. Mitral valve replacements were carried out in juvenile sheep with elective retrievals at 90 days. RESULTS: Rat subdermal explants demonstrated that sequential exposure to AlCl3 and ethanol completely inhibited bioprosthetic cusp and aortic wall calcification compared with controls. However the sheep results were markedly different. The differential sheep explant group exhibited very low levels of cusp and wall calcium. The glutaraldehyde group exhibited little cusp calcification, but prominent aortic wall calcification. All sheep in the two groups previously described lived to term without evidence of valvular dysfunction. In contrast, animals in the sequential group exhibited increased levels of cusp calcification. None of the animals in this group survived to term. Pathologic analysis of the valves in the sequential group determined that valve failure was caused by calcification and stenosis of the aortic cusps. CONCLUSIONS: The results clearly demonstrate that a combination of aluminum and ethanol reduced aortic wall calcification and prevented cuspal calcification. Furthermore, this study demonstrates that exclusion of aluminum from the cusp eliminated the cuspal calcification seen when aluminum and ethanol treatments were administered in a sequential manner.     

Efficacy of the Chitosan Posttreatment in Calcification Prevention of the Glutaraldehyde-Treated Porcine Aortic Noncoronary Cusp Implanted in the Right Ventricular Outflow Tract in Dogs

Abstract: Calcific tissue failure results in poor performance of the bioprosthetic heart valve. Chitosan post-treatment has been shown to be effective in calcification prevention of the glutaraldehyde-treated bovine pericardium when implanted subdermally in rats for 12 weeks. The present study investigated the effectiveness of the chitosan posttreatment in prevention of calcification of the glutaraldehyde-treated porcine aortic noncoronary cusp 5 months after implantation in the right ventricular outflow tract (RVOT) in mongrel dogs. Either 0.625% glutaraldehyde-treated (Group 1, n = 6) or glutaralde-hyde-chitosan-treated (Group 2, n = 6) porcine aortic noncoronary cusp with the aortic wall was sewn to the RVOT. Gross histological observations showed moderate calcification of the glutaraldehyde-treated cusps, but no calcification was noticed in the glutaraldehyde-chitosan-treated grafts at 5 months. This was confirmed by results of quantitative analyses for calcium in half of each ex-planted cusp with aortic wall. The calcium content of the 0.625% glutaraldehyde-treated cusps (Ca, 40.6 ± 24.9 mg/g dry wt) was significantly (p < 0.01) higher than that of glutaraldehyde-chitosan-treated cusps (Ca, 1.3 ± 0.29 mg/g dry wt). These findings suggest that chitosan post-treatment is effective in complete calcium mitigation of the glutaraldehyde-treated porcine aortic noncoronary cusps implanted in the RVOT in dogs.     

Aortic regurgitation caused by rupture of the abnormal fibrous band between the aortic valve and aortic wall

This report documents the sudden onset of aortic regurgitation (AR) by an exceptional cause. A 68-year-old woman suddenly experienced general fatigue, and AR was diagnosed. One year later, we performed aortic valve replacement. At surgery, three aortic cusps with a larger noncoronary cusp had prolapsed along with a free-floating fibrous band that had previously anchored the cusp to the aortic wall. Its rupture had induced the sudden onset of AR. There was no sign of infectious endocarditis. We performed successful aortic valve replacement.     

Surgical treatment of valvular heart disease associated with Beh?et's disease

We experienced two patients of valvular heart disease associated with Beh?et's disease. First patient presented aortic regurgitation (AR) due to perforation of left coronary cusp of the aortic valve and underwent valve replacement (AVR). Second patient showed AR due to shortening of the aortic valve along with mitral regurgitation caused by dilatation of the annulus, and received AVR as well as mitral annuloplasty by Kay's method. Each patient has been doing well for 5 and 2 years after operation, respectively. Patients with Beh?et's disease often have perivalvular leakage after valve replacement. We treated our patients as follows to prevent this serious postoperative complication. 1. The operation was performed when the inflammatory signs and findings subsided with enough adrenal steroids. 2. At operation, the prosthetic valve of one size smaller than the size considered suitable was selected for valve replacement. 3. The adrenal steroids have been continuously administrated and adjusted according to the values of CRP and ESR as well as the cutaneous and mucous manifestations of this disease.     

A case of aortic bicuspid valve with thrombus formation without severe stenosis and calcification

We encountered a patient with a history of juvenile cerebral infarction with an unknown cause in whom a mass adhering to the aortic valve (AV) surface was observed on echocardiography performed upon the development of heart failure. Mild AV stenosis (AS) with moderate regurgitation was noted, and valve repair was applied. It was found during surgery that the AV was a bicuspid valve (BAV) without calcification, and the mass was an organized thrombus. Thrombus formation on the AV with severe AS in BAV has been reported, but the organic lesion in the AV was mild in this patient.     

Stenosis of the bicuspid aortic valve with systemic lupus erythematosus

We herein present a rare case of severe aortic valve stenosis with a bicuspid valve in a patient with systemic lupus erythematosus. The symptoms resulted from aortic valve stenosis, such as chest pain, dyspnea and syncope, which subsided after the insertion of an intra-aortic balloon pump. Thereafter, a calcified bicuspid aortic valve was successfully replaced with a mechanical valve. The pathological findings of the resected valve included irregular fibrotic thickening and marked calcification without any vegetation or thrombus formation. The efficacy of an intra-aortic balloon pump for the relief of symptoms associated with severe aortic valve stenosis indicates its usefulness for such critically ill patients prior to undergoing valvular surgery.     

The influence of valve physiology on outcome following aortic valvotomy for congenital bicuspid valve in children: 30-year results from a single institution.

OBJECTIVE: Aortic valvotomy is widely used for the treatment of congenital aortic stenosis in children. We sought to evaluate whether the predominant post-valvotomy physiology, aortic insufficiency (AI) or aortic stenosis (AS) independently affected patient outcome. METHODS: From 1972-2002, 57 children with congenital aortic stenosis underwent valvotomy. We divided age-matched patients with residual lesions based on their predominant pathology into three groups: Group I (n=14), patients with moderate AI; Group II (n=14), patients with moderate AS, and Group III (n=14), patients with combined AI and AS. Fifteen patients with severe AI or mild residual lesions following valvotomy were excluded from analysis. RESULTS: mean freedom from aortic valve replacement (AVR) was 11.2+/-1.7 years in Group I and 21.5+/-3.9 years in Group II, P=0.05. AVR was required in 11 patients (79%) in Group I vs. only 5 (36%) in Group II, P=0.05. Group III was intermediate, with 9 (64%) requiring AVR. At the time of AVR, patients with aortic stenosis had significantly higher fractional shortening % than those with insufficiency or combined lesions, (Group 1: 38.2+/-7.9 vs. Group II: 46.3+/-5.5 vs. Group III: 39.2+/-3.7, P=0.007). Patients in Group II also had less severely dilated ventricles (mm) than those in the other groups, (Group 1: 50.2+/-12.5 vs. Group II: 39.5+/-8.3 vs. Group III: 49.0+/-8.1, P=0.030). CONCLUSIONS: patients with predominant AI following valvotomy are more likely to need AVR sooner than those with residual stenosis without AI. Therefore, cautious use of repeat valvotomy using maneuvers to avoid AI (small balloons), may prolong freedom from aortic valve replacement in those patients with significant residual AS.     

Severely calcified bicuspid aortic valve stenosis after valve-sparing root replacement: A word of caution

A 58-year-old man was admitted for reoperation for severe aortic stenosis in a previously preserved bicuspid aortic valve (BAV). He had undergone valve-sparing root replacement (VSSR) for dilated aortic root 6 years ago. Transesophageal echocardiography following VSSR showed good valve function with no aortic incompetence. However, the BAV became stenotic causing shortness of breath. At reoperation, the preserved BAV was noted to be fibrotic and calcified and had a fixed rigid small orifice. It was replaced with a biological valve plus root enlargement. Macroscopic finding showed thickening of the cusps and nodular calcification. Microscopic examination revealed severe nodular calcification.     

Aortic valve replacement for aortic regurgitation and stenosis, in patients with severe left ventricular dysfunction.

OBJECTIVE: Aortic valve replacement for aortic valve stenosis (AS) and regurgitation (AR) in patients with severe left ventricular (LV) dysfunction contains an increased risk. Few data are available on the outcome of such patients. METHODS: Fifty-five consecutive patients with severe LV dysfunction (ejection fraction, EF; <30%) and aortic valve replacement for AS (n=35) or AR (n=20) were investigated between 1994 and 2001. EF was 25+/-5%, mean transvalvular gradient 26+/-6mmHg (AS), aortic valve area 0.66+/-0.18cm(2) (AS), cardiac index (CI) 2.4+/-0.9l/min/m(2), enddiastolic LV diameter (LVEDD) 64+/-8mm and endsystolic LV diameters (LVESD) was 55+/-3mm. Ninety percent of patients were in New York Heart Association (NYHA) functional class III/IV at admission to the hospital. Concomitant coronary artery bypass grafts (CABG) were performed in 14 patients. Follow-up examinations including chest X-ray, echocardiography, exercise testing, were performed among survivors. RESULTS: The survival rates for AS were: 1-year 76%, 2-year 68.8%, 5-year 64.2%; for AR: 1-year 94.4%, 2-year 86.5%, 5-year 74.2%. NYHA functional class improved from 90% in class III/IV to 45 (AR group) and 24% (AS group) at follow-up (P<0.02). The LVEDD decreased to 54+/-8mm after 1 year. The EF improved to 38+/-4 (AR group) and 40+/-5% (AS group) at follow-up. CONCLUSIONS: Despite severe LV dysfunction, increased 1-year mortality especially in the AS group, aortic valve replacement was associated with improved functional status, symptoms and EF in both groups and in most patients. We, therefore, conclude that aortic valve replacement in patients with severe LV dysfunction can be performed with acceptable risk.     

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.     

Two decades of experience with root remodeling and valve repair for bicuspid aortic valves

Objective

Bicuspid aortic valve anatomy is associated with ascending aortic aneurysm in approximately 50% of individuals and may lead to severe aortic regurgitation with aortic dilatation. Both entities may be treated by valve repair and root remodeling. The objective was to review the cumulative experience of 20 years.

Quadricuspid aortic valve associated with aortic stenosis and regurgitation

A 75-year-old man with moderate aortic stenosis and regurgitation admitted due to heart failure underwent uneventful aortic valve replacement with a Carpentier-Edwards pericardial bioprosthesis valve. A quadricuspid aortic valve discovered incidentally during surgery consisted of 4 of different sizes and a supernumerary cusp between the right and noncoronary cusps. No coronary abnormality was involved. Resected cusps showed fibrotic thickening with calcification and no sign of previous inflammatory disease. Although quadricuspid aortic valve is a very rare anomaly, its potential for severe valve failure in adulthood should not be neglected.