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PubMed2002

[Relation of aortic wall alterations and ascending aorta diameter in patients with bicuspid aortic valve].

Patients with a bicuspid aortic valve tend to develop local enlargement in the convexity of the ascending aorta. There is controversial discussion as to whether this is a dilatation caused by abnormal hemodynamic stress or an aneurysm due to a common developmental defect of the aortic valve and the aortic media. Systematic studies of aortic wall specimens from patients with different diameters of the ascending aorta have not been available until now. We investigated histologically (HE, E.v.G., alpha-Actin, Alcian blue/van Gieson) aortic wall tissue obtained from 107 patients (31 female, 76 male, mean age 60.9+/-12.8 years) with a bicuspid aortic valve. According to the preoperative measurement of the ascending aorta diameter, which was done by CT scan, angiography or echocardiography, the patients were divided into three groups [Group 1: ascending aorta diameter <3.8 cm; Group 2: diameter >/=3.8-4.9 cm and Group 3: diameter >/=5.0 cm]. We looked for histological signs of dilatation or more severe structural changes. All patients of group 1 showed normal findings in histological examination. In 23 of 43 patients in group 2 (65.1%), we found histological signs of dilatation. Histological signs of dilatation were present in all patients in group 3. We conclude that in patients with a bicuspid aortic valve histological signs of dilatation were more frequently found with increasing diameter of the ascending aorta. More severe histological changes, such as cystic media necrosis, were not present.

Age Factors↗
PubMed2006

Bundle branch reentrant tachycardia in a patient with a calcified bicuspid aortic valve and normal ventricular function.

We report the case of a bundle branch reentrant tachycardia (BBRT) in a 40-yearold patient with a calcified bicuspid aortic valve and normal left ventricular function. The ventricular tachycardia was eliminated by successful radiofrequency ablation of the right bundle branch. As the aortic valve annulus is in close proximity to the specialized conduction system, premature degeneration of a bicuspid aortic valve may involve the bundle of His and the proximal bundle branches by invading calcifications. We speculate that calcifications invading the proximal bundle branches from the bicuspid aortic valve may have created the substrate for the BBRT in this patient.

Adult↗
PubMed1978

Association of left dominant coronary arterial system with congenital bicuspid aortic valve.

Clinical angiographic studies have documented an association of left dominance of the coronary arteries with aortic stenosis and congenital bicuspid aortic valve. The postmortem arteriograms of 973 autopsy patients were reviewed for pattern of coronary dominance and the hearts examined for the nature of any aortic valve disease. There were 673 hearts (70 percent) with a right dominant pattern, 198 (20 percent) with equal dominance and 102 (10 percent) with left dominance. Of 34 hearts with congenital bicuspid aortic valve, 10 (29 percent) had left dominance, a difference significant at the 0.005 level. Of 44 hearts with calcific aortic stenosis, an acquired valve lesion, 9 (20 percent) had left dominance. Rheumatic aortic valve disease (47 cases) and aortic regurgitation (27 cases) had no apparent relation to the coronary arterial pattern. The results confirm the association of left coronary arterial dominance with congenital bicuspid aortic valve. It is suggested that a left dominant coronary system may arise as a consequence of disproportionately decreased blood flow in the left heart chambers, one cause of which is aortic valve stenosis, during early cardiogenesis.

Adolescent↗
PubMed1995

A bicuspid pulmonary valve is not a contraindication for the arterial switch operation.

There are no obvious criteria concerning the optimal repair for complete transposition with bicuspid pulmonary valve if neither the organic changes in the valve nor the pressure gradient between the left ventricle and the pulmonary trunk are severe. Instead of intraatrial switching or intraventricular rerouting in such circumstances, we have proceeded to the arterial switch procedure in 6 patients with an adequate diameter of the pulmonary valve (greater than 100% of the calculated normal aortic orifice). Postoperative catheterization (at approximately 8 months after the procedures) showed no pressure gradient between the left ventricle and the neoaorta except for a finding of 34 mm Hg difference in 1 patient who had undergone simultaneous subpulmonary myotomy. Echocardiography (7 years later in the longest follow-up) has shown no more than slight regurgitation across the bicuspid neoaortic valve, with no progressive increase of blood velocity across the valve. From these results in the middle term, we conclude that the arterial switch procedure remains an option of choice for patients with initially bicuspid pulmonary valve, providing there is no severe subpulmonary stenosis.

Aorta↗
PubMed1994

Repair of insufficient bicuspid aortic valves.

A technique for the repair of bicuspid aortic valves that includes resection of the flail segment of the prolapsing leaflet, annuloplasty, and resection of the raphe, when present, has been reported. To assess the efficacy of this technique in the repair of insufficient bicuspid aortic valves, the results in 72 consecutive patients were assessed. The mean age of the patients was 39 +/- 11 years; 94% were male. Fifty-six patients (78%) underwent isolated aortic valve repair, 9 (12.5%) underwent aortic and mitral valve repair, and 7 (9.7%) had other associated procedures. All patients underwent leaflet resection, including 35 (48%) at the raphe. The mean aortic occlusion time was 39 +/- 12 minutes. There were no operative deaths. The severity of aortic insufficiency, as assessed by Doppler echocardiography (graded from 0 to 4) preoperatively and intraoperatively and at late follow-up, was 3.6 +/- 0.6, 0.4 +/- 0.4, and 0.9 +/- 0.8, respectively, with a p value of < 0.0001 for the latter two values versus the preoperative one. There have been no postoperative deaths. Patients did not receive anticoagulation treatment and there were no strokes or episodes of endocarditis. Six patients have required reoperation; 3 underwent repeat repair. The Kaplan-Meier freedom from aortic valve reoperation probabilities at 12 and 24 months were 94% and 89.5%, respectively. We conclude that valvuloplasty for insufficient bicuspid aortic valves is technically safe, is associated with a low incidence of recurrent insufficiency, and has been associated with no other valve-related complications.

Actuarial Analysis↗
PubMed2005

Feasibility of valve repair for regurgitant bicuspid aortic valves--an echocardiographic study.

BACKGROUND: There is increasing interest in the role of valve repair for patients with isolated severe aortic regurgitation. Those with bicuspid aortic valves are suggested as most suitable for repair. Morphologic features of these valves that suggest feasibility of repair are not well defined. METHODS: Perioperative echocardiograms on 132 consecutive patients (mean age 42 +/- 12 years; 94% male), with bicuspid valves and isolated aortic regurgitation undergoing surgery at our institution were reviewed. Seventy-five patients (57%) underwent successful valve repair. Repair was attempted but unsuccessful for another 8 patients (6 intraoperatively and 2 before discharge). RESULTS: Cusp prolapse was the most common primary mechanism of regurgitation (88 patients [67%]), with 81 patients having primarily eccentrically directed regurgitation. Echocardiographic examination of 72 (55%) had evidence of cusp thickening with 40 (30%) having cusp calcification. By multivariate analysis, an eccentric regurgitant jet direction (odds ratio = 14.3; 95% confidence interval [CI] = 3.4 to 59.6), lack of cusp thickening (odds ratio = 5.9 [1.7 to 20]), lack of cusp calcification (odds ratio = 4.2; [1.1 to 16.7]) and the absence of commissural thickening (odds ratio = 4.8 [1.3 to 16.7]) were independently associated with a greater likelihood of successful valve repair. Greater cusp thickening was the only factor associated with attempted but failed repair. CONCLUSIONS: Successful repair of regurgitant bicuspid aortic valves was more feasible for those patients with eccentric regurgitant jets, those without cusp or commissural thickening or cusp calcification. Recognition of these features may enhance patient selection and improve procedural outcomes with aortic valve repair.

Adult↗
PubMed2006

Tricuspidisation of the aortic valve with creation of a crown-like annulus is able to restore a normal valve function in bicuspid aortic valves.

OBJECTIVE: To evaluate the early results of a new method to repair malfunctioning bicuspid aortic valves by creating a tricuspid valve with a crown-like (i.e. anatomic) annulus. MATERIAL AND METHODS: Twelve patients (ages from 10 to 27 years) with chronic regurgitation (and flow-dependent stenosis) of a bicuspid aortic valve underwent repair with the principle of creating a tricuspid valve and a crown-like annulus. The fused leaflets were trimmed and reinserted underneath the existing aortic annulus to create one new native cusp. The third leaflet was fashioned out of a xenopericard patch and was inserted underneath the existing annulus as well to restore the crown-like anatomy of a normal aortic annulus. A tricuspid aortic valve with a morphologically normal annulus was thus created, which resulted in improved coaptation of the leaflets. The repair was immediately assessed by transesophageal echocardiography (TEE) with the heart loaded at 50%. In two patients, a second run helped fine-tune the repair. Median cross-clamping time was 82 min. Follow-up ranged from 3 to 46 months (median 13 months). RESULTS: No significant complication occurred. The function of the aortic valve was excellent with trivial or mild regurgitation in 11 patients and moderate regurgitation in 1 patient. There was no stenosis across the valve. The repair remained stable over time. Remodelling of the left ventricle occurred as expected. CONCLUSIONS: Aortic valve repair is feasible in some dysfunctioning bicuspid aortic valves. Tricuspidisation of the valve can result in excellent systolic and diastolic functions. The creation of a crown-like annulus results in improved coaptation of the cusps and could lead to more reliable outcome. Although long-term results are needed, this anatomic correction seems to be a good alternative to valvular replacement in certain sub-groups of patients.

Adolescent↗
PubMed2005

Formation of cartilage in congenital bicuspid aortic valves of Syrian hamsters (mesocricetus auratus).

The formation, topographical location and calcification of cartilage in congenital bicuspid aortic valves of 235 Syrian hamsters aged 0--173 days were studied by histological, histochemical and immunohistochemical techniques. In all animals the aortic valve was bicuspid; it had two leaflets, ventral and dorsal, each supported by its own aortic sinus. In 141 valves, a more or less developed raphe was located in the ventral sinus. The remaining 94 valves were devoid of any raphe. The first sign of valvular chondrogenesis was the presence of small groups of cells embedded in a type II collagen-positive extracellular matrix. These cellular groups, which appeared as early as 2 days after birth, became converted into hyaline cartilage or fibrocartilage. A considerable proportion (67%) of the valvular cartilages developed within the first 6 weeks of life. The cartilaginous tissue was capable of forming anywhere along the attachments of the valve leaflets to their supporting sinuses. However, statistical analyses substantiated the observation that the bases of the sinuses and raphes were the valvular regions particularly prone to the development of cartilage. At these sites, the cartilage was usually hyaline and often became calcified. The findings were consistent with the assumption that intense mechanical stimulation plays an important role in the formation of the valvular cartilage. Moreover, these findings supplied new evidence that in the cardiac semilunar valves of Syrian hamsters, cartilage formation does not involve the aggregation of large numbers of cells before their differentiation into chondrocytes. The valvular hyaline cartilages appear to act as competent pivots, resisting mechanical tensions generated during the cardiac cycle. Deposition of calcium in the matrix can be regarded as a reinforcement process of the cartilaginous tissue. Finally, it is hypothesized that the formation of cartilage in the aortic valves of hamsters prevents dystrophic calcification of the valve, a pathological change that causes aortic stenosis in man, especially in patients with a bicuspid aortic valve.

Age Factors↗
PubMed2000

Reconstruction of the pulmonary valve and outflow tract with bicuspid prosthesis in tetralogy of Fallot.

BACKGROUND: Right ventricular outflow tract and pulmonary valve implant in patients with tetralogy of Fallot may be required to avoid late postoperative right ventricular impairment. The use of porcine bicuspid prosthesis might be a technical alternative, although comprehensive studies on the long-term use of these prostheses are not available. METHODS: Sixty-three patients (aged 5 months to 34 years; mean, 6 years) with tetralogy of Fallot and pulmonary hypoplasia underwent surgical repair and enlargement of the right ventricular outflow using preserved porcine pulmonary bicuspid prostheses. Fifty-two patients (82.5%) were followed and underwent clinical evaluation and serial Doppler echocardiography. The first 15 patients (29.4%), with ages ranging from 5 to 16 years (mean, 8.2 years) and postoperative follow-up of 48 to 87 months (mean, 65.1 months) underwent hemodynamic and cineangiographic evaluations. RESULTS: There were 11 deaths (17.4%) in the early postoperative period. Of the 52 surviving patients (82.5%), 51 (80.9%) were followed for 1 to 87 months (mean, 42.0 months). Four patients (7.6%) had additional treatment. Of the 15 patients (29.1%) undergoing hemodynamic evaluation, 9 (60%), had mild valvular pulmonary insufficiency and 6 (40%) had moderate insufficiency. Only the right ventricle-to-pulmonary artery pressure gradients and the right ventricular ejection fraction showed statistically significant differences between groups. Right ventricular dimension, although increased in all patients, did not show statistically significant differences. CONCLUSIONS: Right ventricular outflow tract and pulmonary valve repair in patients with tetralogy of Fallot using a bicuspid porcine pulmonary prosthesis is a simple, reliable procedure with good results in postoperative medium term follow-up.

Adolescent↗
PubMed2002

Reduction aortoplasty for dilatation of the ascending aorta in patients with bicuspid aortic valve.

BACKGROUND: Individuals with bicuspid aortic valve tend to develop a dilatation of the ascending aorta. It is controversial whether the dilated ascending aorta should be replaced with a tube graft or whether the diameter of the aorta should be reduced by reduction aortoplasty. Furthermore, it is unclear whether an external prosthetic support of the reduction aortoplasty is necessary. The aim of this study is to analyze the results of reduction aortoplasty with and without external prosthetic support. METHODS: Between 1985 and 1999, a total of 115 patients with bicuspid aortic valve and dilatation of the ascending aorta underwent reduction aortoplasty in combination with other types of open-heart procedure at our institution. The diameter of the ascending aorta was measured before and early after surgery and then later between 12 and 144 months (mean 40 months) postoperatively using echocardiography and computed tomography. RESULTS: The reduction aortoplasty decreased the internal diameter of the aorta from 48.7+/-5.1 mm preoperatively to 36.9+/-3.6 mm early after surgery (p = 0.0001). During follow-up, there was no increase of the aortic diameter either in patients with external prosthetic support or in 97 of 106 patients without external prosthetic support. The diameter increased only in 9 (8.5%) of 106 patients without external aortic support by 4 to 8 mm. In patients with postoperative diameter increase, the aortic diameter after operation had been higher than in patients without a postoperative increase of the aortic diameter (41.4+/-3.1 mm vs 36.6+/-3.4 mm; p < 0.0001). CONCLUSIONS: Reduction aortoplasty showed good long-term results in patients with bicuspid aortic valve and dilatation of the ascending aorta. Redilation of the aorta occurred only in patients with a suboptimal diameter reduction.

Adolescent↗
PubMed1983

Echocardiography reveals a high incidence of bicuspid aortic valve in Turner syndrome.

The most common cardiac defect in Turner syndrome has been described previously as coarctation of the aorta. We have evaluated 35 consecutive patients with Turner syndrome by clinical examination and by M-mode and two-dimensional echocardiography. Twelve patients (34%) had isolated, nonstenotic bicuspid aortic valve. A high correlation (82%) existed between the presence of a systolic ejection click and echocardiographic evidence of a bicuspid aortic valve. These data indicate that bicuspid aortic valve may be the most common cardiac anomaly in Turner syndrome.

Adolescent↗
PubMed1998

Thoracic aortic aneurysm associated with congenital bicuspid aortic valve.

Congenital bicuspid aortic valve is a relatively rare malformation. It is reported that the presence of this anomaly predisposes the patient to development of true aortic aneurysms or dissecting aortic aneurysms. Between 1981 and August 1997, 25 patients with an aneurysm of the thoracic aorta associated with congenital bicuspid aortic valve underwent surgical treatment at the authors' institution. There were 20 males and five females. The age of the patients ranged from 27 to 74 years (mean 53 years). There were 18 patients with true ascending aortic aneurysms (of which 10 presented with annulo-aortic ectasia) and seven with dissecting aortic aneurysms (four with DeBakey type I dissection, two with type II and one with type IIIb). These 25 patients constituted 2.6% (25/973) of all cases of surgical operations for aneurysms in the thoracic aorta. Aortic valve dysfunction was noted in 20 patients. The authors performed a valved conduit operation in nine patients, aortic valve replacement and wrapping of the ascending aorta in six, graft replacement of the ascending aorta in five, graft replacement of the ascending aorta and aortic arch in four, and graft replacement of the descending aorta in one. No hospital deaths occurred in the authors' patients. Pathological examination of surgical specimens of the aortic wall showed cystic medial necrosis in 11 patients and mucoid degeneration in nine. In patients with congenital bicuspid aortic valve, attention should be paid to aneurysmal dilatation and aortic dissection as complications in addition to valve dysfunction.

Adult↗
PubMed2002

Creating a bicuspid valve from the aortic wall: a new surgical approach on aortic valve disease (in vitro study).

We described a new technique (called 'bicuspidization'), which is performed by using autogenous material, without replacement of the aortic valves for the surgical treatment of aortic stenosis and/or insufficiency and tested it in in vitro sheep model. Different stress conditions were simulated by applying three different flow patterns (hemodynamic challenge tests) successively by using a centrifugal pump. It was demonstrated that the competency of the new bicuspid valves was excellent (zero insufficiency). There was a 10-11 mm Hg-increase on trans-valvular gradient comparing the normal hearts. The autogenous bicuspid valve has not blocked the way of the coronary flow in the closed position.

Animals↗
PubMed1999

Intermediate-term durability of bicuspid aortic valve repair for prolapsing leaflet.

OBJECTIVE: To determine the durability of repair of a bicuspid aortic valve with leaflet prolapse, and to identify factors associated with repair failure. METHODS: From November 1988 to January 1997, 94 patients with a bicuspid aortic valve and regurgitation from leaflet prolapse had aortic valve repair. In 66 patients, the repair employed triangular resection of the prolapsing leaflet. The remainder underwent mid-leaflet plication of the prolapsing leaflet. Mean age was 38 +/- 10 years and 93% were male. Median follow-up was 5.5 years (range 0.2-9 years). Factors associated with aortic valve competence and durability were identified by multivariable logistic and hazard function analyses. RESULTS: Early valve competence was more difficult to achieve in patients with large, poor functioning ventricles (P = 0.02). Aortic valve reoperation was necessary in 12 patients that included three re-repairs and nine aortic valve replacements. Freedom from reoperation was 95, 87 and 84% at 1, 5 and 7 years, respectively. The instantaneous risk of reoperation was highest immediately after operation, and fell rapidly to approximately 2% per year and less after 2 years. The only risk factor identified was the presence of residual aortic regurgitation (trace to mild in 35 cases) on immediate intraoperative post-repair transesophageal echocardiography. Late aortic regurgitation did not progress detectably across time (P = 0.3). There were no deaths, early or late. CONCLUSION: Bicuspid aortic valve repair for prolapsing leaflet is a safe procedure with good intermediate-term outcome. However, any residual aortic regurgitation jeopardizes repair durability and initial repair achievement is more difficult in patients with dilated, poor functioning ventricles.

Adolescent↗
PubMed2004

An obstructive bicuspid aortic valve in the setting of tetralogy of Fallot with pulmonary atresia: a rare combination.

As far as we are aware, a bicuspid aortic valve has not previously been reported in the setting of tetralogy of Fallot and pulmonary atresia. We describe this association in a newborn who presented with a murmur and cyanosis. Echocardiography showed tetralogy of Fallot with pulmonary atresia, and also a moderately stenotic bicuspid aortic valve. The patient underwent open-heart surgery guided by transesophageal echocardiography. Postoperatively, there was only mild obstruction across both outflow tracts. We have also reviewed the pertinent data from our Jesse E. Edwards Registry of Cardiovascular Disease to establish the incidence of bicuspid aortic valve in the setting of obstruction of the right ventricular outflow tract, finding the association in no patients with pulmonary atresia and tetralogy of Fallot, in 0.7% of those with tetralogy of Fallot and pulmonary stenosis, but in 6.6% of those with pulmonary atresia and intact ventricular septum.

Angiography↗
PubMed1985

Pseudocoarctation of the aortic arch associated with bicuspid aortic valve lesion: is it a surgical entity?

Three patients with bicuspid aortic valve lesion associated with pseudocoarctation of the aortic arch are presented. At operation, 2 had a bicuspid calcified stenotic aortic valve, and one a bicuspid non-calcified incompetent aortic valve. All required replacement of the diseased aortic valve. The pseudocoarctation was not corrected because it caused no hemodynamic abnormality.

Adult↗
PubMed2001

Unusual echocardiographic views of bicuspid and tricuspid pulmonic valves.

The pulmonary artery is typically seen on transthoracic echocardiography in its longitudinal axis. Therefore, short axis views of the pulmonic valve leaflets are not generally obtained, and the distinction between tricuspid and bicuspid pulmonic valves is difficult or impossible. Bicuspid pulmonic valve is one cause of pulmonic stenosis, which is especially common in tetralogy of Fallot. Presented here are 2 patients in whom the orientation of the pulmonary artery was unusual, and the pulmonic valve was seen en face. The first patient had tetralogy of Fallot and a bicuspid pulmonic valve. The severe obstruction to right ventricular outflow was infundibular. The second patient had severe stenosis of a tricuspid pulmonic valve, which was treated with balloon valvuloplasty. These unusual views of the pulmonic valve leaflets were obtained because of anterior displacement of the pulmonary artery, and precise anatomic delineation of the problem in each case was possible with transthoracic echocardiography.

Adult↗
PubMed2002

Ross operation for bicuspid aortic valve disease in adults: is it a valid surgical option?

OBJECTIVE: The validity of the Ross operation as freestanding root replacement in adult patients with bicuspid aortic valve disease has lately been questioned. We have analyzed retrospectively our results in 23 adult patients (19 males) operated for bicuspid aortic valve disease ad modum "Ross" employing a freestanding root replacement technique. DESIGN: In 9 patients the dominant aortic valve lesion was stenotic (aortic stenosis group) and in the remaining 14 patients it was aortic insufficiency (aortic insufficiency group). The fate of the pulmonary autograft in the two groups was studied. The intraoperatively measured aortic and pulmonary annuli diameters from the two groups were compared with those from a population of normal looking aortic and pulmonary valves matched for body surface area. RESULTS: The aortic insufficiency group needed significant reduction of the aortic annulus diameter to conform to the size of the pulmonary autograft. The pulmonary autograft annuli in this group were significantly larger in diameter than the ones in the aortic stenosis group. The mean pulmonary annulus diameter in the aortic stenosis group was, on the other hand, significantly smaller when compared with that in the normal matched population. After a mean follow-up period of about 19 months, the aortic insufficiency group showed significant dilatation of the neo-aortic sinuses. Between the two groups, the remaining echocardiographic variables remained either stable or improved at follow-up. CONCLUSION: Pre-existing larger diameters of the aortic and pulmonary annuli in the aortic insufficiency group combined with the significantly increased left ventricular end-diastolic diameters, may predispose these patients to significant dilatation of the unsupported aortic sinuses after a Ross operation. This dilatation does not, however, lead to increase in the autograft valve insufficiency at short-term follow-up if the aortic annulus and the distal ascending aorta are tailored to the size of the pulmonary autograft. Ross operation, employing freestanding aortic root replacement technique, may therefore be recommended in adult patients with bicuspid aortic valve disease with excellent short-term results.

Adolescent↗