A case of quadricuspid aortic valve with aortic regurgitation

A 67-year-old man with grade 3 aortic valve regurgitation was found to have a quadricuspid aortic valve. The aortic valve consisted of 1 large, 2 intermediate and 1 small sized cusp. An accessory cusp located between the right and noncoronary cusps, and shaped like a hammock which sling by the fibrous strings originating from the both commissures to the aortic wall. Aortic valve replacement was successfully performed with a 23 mm St. Jude Medical prosthetic valve, and the patient is asymptomatic five months post-operatively. Histological examination of the resected cusps showed fibrous thickening and no rheumatic valvulitis or infective endocarditis.     

Geometrical difference between an ascending aneurysm and a root aneurysm in valve-sparing operations

Objective: The aortic root geometry of the leaflet size and coaptation in an ascending aortic aneurysm, and in a root aneurysm, may predict the early and late outcomes from valve-sparing surgery.Methods: The aortic root was investigated using intraoperative endoscopy before and after valve-sparing root reconstruction. The definition of ‘root aneurysm’ was marked sinus dilatation proximal to the sinotubular junction. ‘Ascending aneurysm’ was defined as dominant dilatation distal from the sinotubular junction. Fifteen cases were examined and classified into two groups; Group A with an ascending aneurysm (four patints of Group R with a root aneurysm (eleven patients).Results: Cusp prolapse was seen in 10 (90.9%) patients of Group R, and in only one (25%) patient of Group A. The length of the free margin of all cusps in Group R was significantly longer than those in Group A. In Group R, the lengths of the free margin of an individual cusp were significantly different, indicating asymmetric cusps. In Group A, the length of the free margin were similar, indicating symmetric cusps. There was no significant difference in the degree of immediate postoperative aortic insufficiency, between the two groups. At the most recent follow-up, progressive aortic insufficiency was present in two patients of Group R.Conclusions: A root aneurysm had asymmetric, elongated and prolapsed aortic cusps, while an ascending aneurysm had symmetric cusps without prolapse. The mechanism of aortic insufficiency in a root aneurysm was more complex, suggesting a difficulty in achieving long-term valve competence. Such geometrical difference should be considered in the indication for the surgical technique of valve-sparing operations.     

Sub-mitral membranous curtain: A potential anatomical basis for congenital Sub-mitral aneurysms

Objective To seek an anatomic basis for congenital sub-mitral aneurysms. Materials and Methods 75 normal human autopsy heatts were incised between the right and left coronary cusps up to the apex. The membranous septum was trans illuminated from the right side. The mitral valve was lifted anteriorly to observe extension of any membranous tissue to the sub-mitral region. Findings In 17 out of 75 hearts, the tissue of the membranous septum extended beyond the nadir of the non-coronary cusp, beneath the aortic leaflet of the mitral valve, up to the postero-medial commissural line. In 28 hearts, this sub-mitral membranous extension continued beyond the postero-medial commissural line beneath the annulus of the mural leaflet, to a distance varying from 4mm to 21mm, with height between 0.5mm to 4mm. This extension of the membranous tissue beyond the nadir of the non coronary cusp, caudal to the aortic and mural leaflets of the mitral valve is preferably labeled sub-mitral curtain. The sub-mitral curtain was in continuity with the membranous, septum in 40 hearts. In 5 hearts, trans illumination was interrupted between membranous septum and sub-mitral curtain by varying extent of opaque area due to tissue of the central fibrous body, right fibrous trigone and ventricular musculature. Conclusions The membranous sub-mitral curtain of the mitral valve seen in 45 out of 75 hearts is a potentially weak area, through which congenital sub-mitral true aneurysms can occur.     

Quadricuspid aortic valve: case reports

Two cases of quadricuspid aortic valve with aortic regurgitation are reported. Case 1, a 66-year-old woman was operated on because of aortic regurgitation, and an aortic valve replacement with a bioprosthesis was performed. When the valve was exposed during the operation, it showed four cusps, three of which were of equal size and one smaller cusp which was interposed between the right and left coronary cusp. Case 2, a 46-year-old man was diagnosed, using echocardiography and aortography before surgery, as suffering from aortic regurgitation because of a quadricuspid aortic valve. During surgery, two larger cusps and two smaller cusps and a displacement of the right coronary artery ostium, (which was placed in a lower position and close to the commissure between the right coronary and the right posterior cusps) were found. The four cusps were excised and replaced by a tilting disc prosthesis. In both cases, the postoperative recovery was uneventful. Using the 24 cases from the literature and two of our own cases, the correlation between the size or the position of the accessory cusp and the occurrence of aortic regurgitation was analyzed. The larger the accessory cusp was, the higher the incidence of aortic regurgitation occurred (p less than 0.05).     

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.     

Repair of aortic valve prolapse: experience with 44 patients.

OBJECTIVES: In regurgitant tricuspid aortic valves, cusp prolapse may be isolated or associated with dilatation of the proximal aorta. Newly appearing cusp prolapse can also appear after an aortic valve sparing operation (AVSO) and be responsible for residual aortic regurgitation. In this report, we describe our experience in repairing prolapsing aortic cusps in 44 patients with aortic regurgitation. METHODS: Between 1996 and 2003, 260 patients had aortic valve repair or valve sparing procedures in our department. All patients had peri-operative TEE. Prolapse of one or more of the aortic cusps was identified by TEE and confirmed by careful surgical inspection before and after valve sparing surgery. Forty-four patients with cusp prolapse were identified. Fifteen had an isolated prolapse, with a normal root (group I), 18 had cusp prolapse associated with dilatation of the proximal aorta (group IIa), and 11 had a newly appearing prolapse after AVSO (group IIb). Correction of the prolapsing cusp was achieved by either free edge plication, triangular resection or resuspension with PTFE. This procedure was associated with an aortic annuloplasty in group I, and with AVSO in groups II and III. RESULTS: Post-operative TEE showed AR trivial or grade I regurgitation. At a mean of 23 months follow-up, one patient with recurrent regurgitation required an aortic valve replacement with a homograft. All remaining patients were in NYHA class I or II. Echocardiography confirmed the durability of the valve repair. CONCLUSIONS: Among the common causes of aortic regurgitation, isolated cusp prolapse is frequent and is amenable to surgical repair with excellent mid-term results. In particular, in patents who are potential candidates for AVSO, identification and correction of an associated prolapse, either pre-existing or secondary to the AVSO procedure, may further extend the indications for this technique, increase its success rates and improve its long-term outcome.     

Geometrical difference between an ascending aneurysm and a root aneurysm in valve-sparing operations.

OBJECTIVE: The aortic root geometry of the leaflet size and coaptation in an ascending aortic aneurysm, and in a root aneurysm, may predict the early and late outcomes from valve-sparing surgery. METHODS: The aortic root was investigated using intraoperative endoscopy before and after valve-sparing root reconstruction. The definition of 'root aneurysm' was marked sinus dilatation proximal to the sinotubular junction. 'Ascending aneurysm' was defined as dominant dilatation distal from the sinotubular junction. Fifteen cases were examined and classified into two groups; Group A with an ascending aneurysm (four patients), and Group R with a root aneurysm (eleven patients). RESULTS: Cusp prolapse was seen in 10 (90.9%) patients of Group R, and in only one (25%) patient of Group A. The length of the free margin of all cusps in Group R was significantly longer than those in Group A. In Group R, the lengths of the free margin of an individual cusp were significantly different, indicating asymmetric cusps. In Group A, the length of the free margin were similar, indicating symmetric cusps. There was no significant difference in the degree of immediate postoperative aortic insufficiency, between the two groups. At the most recent follow-up, progressive aortic insufficiency was present in two patients of Group R. CONCLUSIONS: A root aneurysm had asymmetric, elongated and prolapsed aortic cusps, while an ascending aneurysm had symmetric cusps without prolapse. The mechanism of aortic insufficiency in a root aneurysm was more complex, suggesting a difficulty in achieving long-term valve competence. Such geometrical difference should be considered in the indication for the surgical technique of valve-sparing operations.     

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).     

Outcomes of Reimplantation of the Aortic Valve in Patients With Aortic Cusp Fenestration

BackgroundAortic cusp fenestrations are common in patients with aortic root aneurysm, and their management during aortic valve repair remains controversial. We believe that fenestrations in the area of the commissures may rupture after reimplantation of the aortic valve because this operation increases the mechanical stress on the cusps. For this reason we have reinforced the free margin of the aortic cusp with fenestration with fine Gore-Tex sutures (WL Gore). This study examines the outcomes of reimplantation of the aortic valve in patients who had cusp fenestration reinforced with Gore-Tex sutures.MethodsA review of all patients who had reimplantation of the aortic valve for aortic root aneurysm disclosed 111 patients who had at least 1 cusp fenestration reinforced with a double layer of a fine Gore-Tex suture. The outcomes of these patients were examined and compared with a group of patients without fenestration using propensity score analysis. All patients were followed prospectively with images of the heart.ResultsThe median follow-up was 8.3 years. Overall the cumulative incidence of aortic valve reintervention at 15 years was 4.8% and the cumulative incidence of aortic insufficiency of moderate or severe degree was 9.2%. Comparison of outcomes of patients with and without fenestrations showed similar results up to 15 years of follow-up.ConclusionsReinforcement of the free margins of cusps with fenestrations using Gore-Tex sutures is safe and does not seem to adversely affect the durability of reimplantation of the aortic valve.     

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.     

Endoscopic observation following aortic cusp plication

In valve-sparing operations for aortic root aneurysms, the dilated aortic root is replaced by a vascular graft. However, cusp disorders remain in some cases. We observed closed cusps endoscopically, and an improvement in the cusp coaptation was seen following plication. One of our cases of aortic valve plication is reported here. A rigid thoracoscope and cannula for cardioplegia delivery were inserted from the distal end of the aortic graft, and the graft was inflated with cardioplegic solution, providing an endoscopic view of the closed aortic cusps. We believe that endoscopic observation of closing cusps in this way may help surgeons to learn and master the techniques used in cusp repair more rapidly.     

Significance of raphe in congenitally bicuspid aortic valve

The decrease of rheumatic aortic regurgitation (AR) is observed due to the relative increase of non-rheumatic aortic valvular diseases since 1980. Among 240 patients who had undergone aortic valve replacement (AVR) including combined valvular diseases up to March 1989, the congenitally bicuspid aortic valve was responsible for AR and stenosis (AS) in 33 patients (13.8%) and were divided according to the presence of a raphe. In the raphe (+) group (n = 15), infective endocarditis (IE) (n = 5), prolapse of the aortic valve, mainly non-coronary cusp (n = 5), and thickening with contraction of cusp (n = 4) were the cause of AR. Calcification of the cusp was seen in 2 older (greater than 59 yrs) patients. In the raphe (-) group (n = 18), IE (n = 2), contraction of cusps (n = 2) in the relatively younger (less than 48 yrs) were the cause of AR. Rest of the patients exhibited severe AS due to the calcification of cusps except a case who showed IE with AR in the calcified cusp. Although not generally recognized, the bicuspid valve with a raphe, less tendency to deposit calcium, is an important cause of pure AR severe enough to warrant AVR. The bicuspid valve without raphe, as already recognized, prones to develop severe calcification and AS in later life.     

A case of minimal invasive aortic valve replacement for quadricuspid aortic valve with aortic regurgitation]

Congenital quadricuspid aortic valve is rare, which may be a cause of sever aortic regurgitation. We report a case of a 55-year-old man who had severe aortic regurgitation with congenital quadricuspid aortic valve. Preoperative aortography showed severe aortic regurgitation. Preoperative trans-esophageal echocardiography revealed abnormal quadricuspid aortic valve. We performed minimal invasive aortic valve replacement with SJM # 21 mm HP prosthetic valve through the limited upper sternotomy. Skin incision was 9 cm. Aortic cross-clamping time was 92 min, cardiopulmonary bypass time was 108 min. At the operation, a quadricuspid valve with three equal cusps and one small cusp was noted. The postoperative course was excellent and he discharged 8 days after the operation.     

Active infective endocarditis due to methicillin-resistant Staphylococcus aureus in the acute phase of infectious mononucleosis

A 26-year-old male was treated for acute hepatitis due to Epstein-Barr virus and infectious mononucleosis in our hospital. At 2 weeks after admission, there was relapse with high fever. A blood culture detected methicillin-resistant Staphylococcus aureus. A two-dimensional echocardiogram revealed severe aortic regurgitation and vegetation on the left coronary cusp of the aortic valve. The diagnosis was active infective endocarditis due to methicillin-resistant Staphylococcus aureus in the acute phase of infectious mononucleosis. Following preoperative administration of vancomycin, the aortic valve was replaced with a Carbomedics prosthetic valve. The aortic valve was bicuspid, and the right cusp and non-coronary cusp were conjoined. As the focus of infection was localized to the left coronary cusp, the infected tissue was fully removed with resection of all the cusps. Although fever persisted long after the operation, the blood culture became negative for methicillin-resistant Staphylococcus aureus, and repeated echocardiograms including transesophageal echocardiogram showed no prosthetic valve infection. Vancomycin was administered until the C-reactive protein became negative at 45 days after the operation.     

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.     

Early valvular obliteration of cryopreserved aortic valve allograft

This report describes a 60-year-old male patient who developed early valvular obliteration of a cryopreserved aortic valve allograft with associated severe valvular leakage. The patient had previously undergone two operations for aortic valve insufficiency resulting from infective endocarditis, and prosthetic valve endocarditis: aortic valve replacement with a mechanical prosthesis was done 4 years ago, and two years later aortic root replacement with a cryopreserved allograft was performed. Perforation through the non-coronary cusp of the aortic allograft was found, and valve replacement was achieved using a mechanical prosthesis. The intraoperative findings, histological, immunological, and bacteriological studies of the resected cusps demonstrated negative for infection and rejection, therefore, the valvular perforation might have been caused by an injury or degeneration during management of the homologous graft. The patient showed neither aortic regurgitation on echocardiography nor recurrence of endocarditis 10 months after surgery.     

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.     

Pathology of the formalin-treated heterograft porcine aortic valve in the mitral position

The mitral valve was replaced by a pig aortic valve in 33 patients at Groote Schuur Hospital. Eleven of the failed heterograft aortic valves were examined at intervals of from 2 to 32 months after insertion. Fourteen control pig aortic valves were also examined. Electron microscopy was performed on two of the failed heterograft valves and three control pig valves. Failure of the heterograft was due to stretching and deformation of the cusps with resultant valvular incompetence. Stretching of the cusp was a result of reduction in the amount of its collagen content. The elastic tissue appeared little altered. A microscopic layer of fibrin thrombus was present on the surface of 8 of the 11 valves. Only 2 of the 11 valves showed invasion of the graft by immunologically competent cells. No valve showed any sign of infection or calcification. The denatured collagen of the heterograft has a low antigenicity and also, infortunately, a limited durability.     

Aortic regurgitation caused by prolapsed noncoronary cusp associated with dissecting aortic aneurysm treated by placing a prosthetic graft onto the anulus: report of a case

A 70-year-old man with severe aortic regurgitation (AR) associated with dissecting aortic aneurysm underwent a radical operation. AR was thought to be due to dilated sinotubular junction and prolapsed noncoronary cusp caused by dissecting flap extended into the sinus of Valsalva. At operation, the noncoronary cusp was slightly prolapsed into the left ventricle, but all cusps were seemed to be thin and pliable. An isolated "tongue shaped" graft was placed onto the anulus of the noncoronary cusp, and a 26 mm Woven Dacron graft was used to replace the ascending aorta. Postoperative angiogram showed mild AR and improved left ventricular (LV) function. This procedure was effective to repair AR caused by prolapsed noncoronary cusp without elongation or thickening of the valve.     

No-React Detoxification Process: A Superior Anticalcification Method for Bioprostheses

Background. Glutaraldehyde pretreatment of bioprosthetic heart valves is the major pathogenic factor in their calcific degeneration. This comparative study investigates the merit of the No-React aldehyde detoxification process as an alternative modifier of xenograft tissues.

Methods. Glutaraldehyde- and No-React-pretreated porcine aortic valve cusps were implanted subcutaneously in 6-week-old rats (n = 20). At 3, 6, and 14 weeks, randomly selected animals were sacrificed and the explants underwent mineral and morphologic analyses. Glutaraldehyde- and No-React-treated bovine pericardium and porcine aortic valve cusp were incubated in fibroblast cell culture plates. Cell viability was observed under reversed microscope at 6, 24, 48, and 96 hours. Erythrosin B dye exclusion test was used to validate percent cell death.

Results. Pretreatment with No-React significantly inhibited calcification of aortic cusp subcutaneous implants throughout the 14-week period (mean tissue Ca²⁺ content = 1.3 ± 0.7 μg/mg at 14 weeks.) Glutaraldehyde-treated cusps underwent protracted calcification (Ca²⁺ content = 190.6 ± 89.5 μg/mg; p < 0.01). Morphologic findings correlated with mineral analyses. One-hundred percent of fibroblast cells survived in the presence of No-React-treated tissue, with a growth pattern indistinguishable from control cell culture (ie, in the presence of no tissue). The cells incubated with glutaraldehyde-treated tissue showed signs of nonviability by 6 hours, with 100% cell death by 48 hours. Dye exclusion tests validated these findings.

Conclusions. The No-React detoxification process completely abolishes the cytotoxicity of the xenograft tissue and inhibits calcific degeneration.