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PubMed2005

Planimetric assessment of anatomic valve area overestimates effective orifice area in bicuspid aortic stenosis.

BACKGROUND: Although the continuity equation remains the noninvasive standard, planimetry using transesophageal echocardiography is often used to assess valve area for patients with aortic stenosis (AS). Not uncommonly, however, anatomic valve area (AVAA) obtained by planimetry overestimates continuity-derived effective valve area (AVAE) in bicuspid AS. METHODS: Transthoracic Doppler and transesophageal echocardiography were performed to obtain AVAE and AVAA in 31 patients with bicuspid AS (age 61 +/- 11 years) and 22 patients with degenerative tricuspid AS (age 71 +/- 13 years). Aortic root and left ventricular outflow tract dimensions and the directional angle of the stenotic jet were assessed in all patients. Using these data, a computational fluid dynamics model was constructed to test the effect of these variables in determining the relationship between AVAE and AVAA. RESULTS: For patients with tricuspid AS, the correlation between AVAA (1.15 +/- 0.36 cm2) and AVAE (1.13 +/- 0.46 cm2) was excellent (r = 0.91, P < .001, Delta = 0.02 +/- 0.21 cm2). However, AVAA was significantly larger (1.19 +/- 0.35 cm2) than AVAE (0.89 +/- 0.29 cm2) in the bicuspid AS group (r = 0.71, P < .001, Delta = 0.29 +/- 0.25 cm2). Computer simulation demonstrated that the observed discrepancy related to jet eccentricity. CONCLUSION: For a given anatomic orifice, functional severity tends to be greater in bicuspid AS than in tricuspid AS. This appears to be primarily related to greater jet eccentricity and less pressure recovery.

Anatomy, Cross-Sectional↗
PubMed2004

Bicuspid aortic valve is heritable.

OBJECTIVES: Previous studies have established familial clustering of bicuspid aortic valve (BAV), presumably indicating genetic inheritance. Our objective was to statistically test whether the segregation pattern of BAV is consistent with genetic inheritance and to obtain an estimate of the size of the genetic effect (heritability). BACKGROUND: Bicuspid aortic valve occurs in 1% of the population, making it the most common cardiovascular malformation (CVM). Bicuspid aortic valve is frequently an antecedent to aortic valve stenosis or insufficiency and is often associated with other CVMs, including aortic root dilation. The genetic and developmental significance of these findings remains obscure. METHODS: In 50 probands with BAV, we obtained a three-generation family history and echocardiograms on first-degree relatives. Heritability (h2) of BAV and BAV and/or other CVMs were estimated using maximum-likelihood-based variance decomposition extended to dichotomous traits implemented in the computer package Sequential Oligogenic Linkage Analysis Routines (SOLAR, San Antonio, Texas). RESULTS: A total of 309 probands and relatives participated. Bicuspid aortic valve was identified in 74 individuals (prevalence = 24%). A total of 97 individuals had BAV and/or other CVM (prevalence = 31%), including aortic coarctation, ventricular or atrial septal defect, abnormal mitral valve, aortic root dilation, or hypoplastic left heart syndrome. The heritability (h2) of BAV and BAV and/or other CVMs were 89% and 75%, respectively. CONCLUSIONS: The high heritability of BAV suggests that in this study population BAV determination is almost entirely genetic. The heritability of BAV plus other cardiovascular anomalies suggests that valve malformation can be primary to defective valvulogenesis or secondary to other elements of cardiogenesis.

Adolescent↗
PubMed2004

Should the ascending aorta be replaced more frequently in patients with bicuspid aortic valve disease?

OBJECTIVE: The optimal diameter at which replacement of the ascending aorta should be performed in patients with bicuspid aortic valve disease is not known. METHODS: We reviewed all patients with bicuspid aortic valves undergoing aortic valve replacement at our institution from 1979 through 1993 (n = 201). Patients undergoing concomitant replacement of the ascending aorta were excluded. RESULTS: Follow-up was obtained on 98% of patients and was 10.3 +/- 3.8 (mean +/- SD) years. The average patient age was 56 +/- 15 years, and 76% were male. The ascending aorta was normal (<4.0 cm) in 115 (57%) patients, mildly dilated (4.0-4.4 cm) in 64 (32%) patients, and moderately dilated (4.5-4.9 cm) in 22 (11%) patients. All patients with bicuspid aortic valves with marked dilation (>5.0 cm) underwent replacement of the ascending aorta and were therefore excluded. Fifteen-year survival was 67%. During follow-up, 44 patients required reoperation, predominantly for aortic valve prosthesis failure. Twenty-two patients had long-term complications related to the ascending aorta: 18 required an operative procedure to replace the ascending aorta (for aortic aneurysm), 1 had aortic dissection, and 3 experienced sudden cardiac death. Fifteen-year freedom from ascending aorta-related complications was 86%, 81%, and 43% in patients with an aortic diameter of less than 4.0 cm, 4.0 to 4.4 cm, and 4.5 to 4.9 cm, respectively ( P < .001). CONCLUSIONS: Patients undergoing operations for bicuspid aortic valve disease should be considered for concomitant replacement of the ascending aorta if the diameter is 4.5 cm or greater.

Aged↗
PubMed1977

Congenital bicuspid aortic valve after age 20.

The bicuspid aortic valve is recognized as a frequent cause of aortic stenosis in adults. Aortic stenosis has been reported to occur in as many as 72 percent of adults with a congenital bicuspid aortic valve, with peak incidence occurring in the 5th and 6th decades of life. Review of the clinical records of 152 patients aged 20 years and older found to have a bicuspid aortic valve at autopsy revealed aortic stenosis in only 28 percent. The incidence of aortic stenosis increased progressively with age; 46 percent of patients over age 50 years and 73 percent over age 70 years had some degree of stenosis. The stenotic valves were obstructed by nodular, calcareous masses but commissural fusion was present in only eight cases. The largest group of patients in the series (40 percent) died of infective endocarditis; 77 percent of these were under age 50 years. Primary aortic regurgitation without infective endocarditis was uncommon. Thirty-two percent of the patients in this series had an apparently normally functioning aortic valve, and this rate remained relatively constant with increasing age; 37 percent of patients over age 50 years and 27 percent over age 70 years had an apparently normal valve. The bicuspid aortic valve in patients over age 20 does not invariably become stenotic or insufficient.

Adult↗
PubMed2000

Remodeling of the aortic root and reconstruction of the bicuspid aortic valve.

BACKGROUND: Currently, isolated reconstruction of a regurgitant bicuspid aortic valve can be performed with adequate early results. Dilatation of the proximal aorta is known to be associated with this valve anomaly and may be partially responsible for the development of primary regurgitation or secondary failure of valve repair. We have used repair of the bicuspid valve with remodeling of the aortic root as an alternative to insertion of a composite graft. METHODS: Between October 1995 and May 1999, 16 patients (12 men, 4 women, aged 35 to 73 years) were seen with a regurgitant bicuspid aortic valve and dilatation of the proximal aorta of more than 50 mm. All patients underwent repair of the valve using either coapting sutures alone (n = 12) or in combination with triangular resection of a median raphe (n = 4). Using a Dacron graft, the aortic root was remodeled and the ascending aorta (n = 16) and proximal arch (n = 4) replaced. RESULTS: No patient died. The postoperative degree of aortic regurgitation was less than grade II in all patients. Valve function has remained stable in all patients between 2 and 43 months postoperatively. CONCLUSIONS: Reconstruction of the regurgitant bicuspid valve in the presence of proximal aortic dilatation is feasible with good results by combining the root remodeling technique with valve repair.

Adult↗
PubMed2002

Extending the usable size range of homografts in the pulmonary circulation: outcome of bicuspid homografts.

BACKGROUND: Small-sized homografts are often not available, making the use of surgically reduced cryopreserved homograft conduits appealing. METHODS: From January 1993 to January 2000, 21 large homografts were size-reduced by excising one leaflet and were implanted in the pulmonary circulation. Valve function was compared with 21 children-matched for weight, homograft size, and year of operation-who received a standard homograft. RESULTS: Implanted homograft size and patient weight were equivalent in both the bicuspid and standard groups. Median (range) in-hospital peak instantaneous echocardiographic gradient across the valve was 0 mm Hg (0 to 19) in the bicuspid group versus 0 mm Hg (0 to 17) in the standard group (p = 0.65). Median (range) in-hospital pulmonary insufficiency (scale of 0 to 4) was 2 (0 to 3) in the bicuspid group versus 1.5 (0 to 3) in the standard group (n = 10, p = 0.34). At a follow-up of 54+/-29 months there was no significant difference in conduit reinterventions between the groups. CONCLUSIONS: Surgical creation of a bicuspid valve in the pulmonary circulation results in a functionally equivalent conduit compared with standard homograft as measured by early and midterm valve function.

Cardiac Surgical Procedures↗
PubMed1999

Pulmonary autograft replacement of the bicuspid aortic valve: a successful surgical option for young adults.

A severely dysfunctioning congenitally bicuspid aortic valve may require surgical treatment within the fourth decade of life. Among conventional options, the pulmonary autograft (PA) offers many theoretical advantages particularly for young patients, including potential for growth, hemodynamic performance, no need for anticoagulants and freedom from endocarditis. However the operation is more complex and longer, may interfere with coronary and right ventricular anatomy and function and may expose the patient to the downside of two valves at risk. Aim of this retrospective study was to evaluate the mid-term results achieved with the PA performed in adolescents and young adults with a bicuspid aortic valve. Between July 94 and June 98, 26 patients, 22 males and four females, with a mean age of 24+/-10 years (range, 11 to 38), underwent bicuspid aortic valve replacement with a pulmonary autograft (stenosis 2-8%; insufficiency 13-50%; combined 11-42%). Eight patients (31%) were in NYHA FC I, 17 (65%) in II, and 1 (4%) in III. Mean preoperative ejection fraction was 67+/-7%. Three patients (11.5%) had a past medical history of endocarditis (healed in all) and in two the PA was a re-do procedure. The PA was inserted as a subcoronary implant in one case (4%) and utilized as a root in the remaining 25 (96%). The right ventricular outflow tract was reconstructed with a cryopreserved pulmonary homograft conduit in all cases. Mean cardiopulmonary bypass and aortic crossclamp times were 204+/-50 min (range, 174 to 300) and 157+/-35 min (range, 133 to 193) respectively. No early or late deaths had occurred at a mean follow-up of 22.5 months (range, 5 to 47.5). The first patient in the series (4%) was reexplored for bleeding and needed transfusions. The subsequent routine use medical and surgical strategies resulted in no further need for postoperative reexploration, and successful containment of total postoperative blood loss (<350 ml/m2BSA). 2-D Echo evaluation of neo-aortic valve competence at 6 months, revealed no evidence of aortic valve regurgitation in 17 (65%), trivial regurgitation in seven (27%), mild in one (4%) and mild-to-moderate in one (4%). The latter patient (subcoronary implant PA) required reoperation. At six months, the mean degree of regression of left ventricular mass compared to pre-operative data, was 36% (333+/-94 to 212+/-60 gr, p<0.05). All patients are asymptomatic, in NYHA FC I, and enjoy normal social interaction. In conclusion, PA root implantation can be offered as a low-risk alternative to conventional prosthetic aortic valve replacement to adolescents and young adults with a bicuspid aortic valve. The routine achievement of blood loss containment has minimized the risk of transfusion thus contributing to expand the indication in young patients. Continued patients evaluation particularly with regard to evidence of neo-aortic valve degeneration, root dilatation and homograft dysfunction in the long term is warranted.

Adolescent↗
PubMed1994

The congenital bicuspid aortic valve with post-inflammatory disease--a neglected pathological diagnosis of clinical relevance.

It is unusual to find surgically excised congenital bicuspid aortic valves with post-inflammatory disease. We have analysed retrospectively all surgically excised aortic valves over a 6-year period for this particular diseased state in relation to relevant clinical data. There were 181 congenital bicuspid aortic valves, and the characteristic gross pathology of the tricuspid aortic valve with post-inflammatory disease served as reference. Twelve of the 181 congenital bicuspid aortic valves were identified as post-inflammatory (6.6%); one with clinically established rheumatic disease. The mean age at operation of these 12 patients was significantly lower compared to 'other' aortic valves. The study indicates that post-inflammatory involvement of a congenital bicuspid aortic valve can be identified, which is of clinical relevance since these patients have a significantly younger age at operation.

Adult↗
PubMed1978

Congenitally bicuspid aortic valves. Clinicogenetic study of 41 families.

The families of 41 patients with surgically proved isolated bicuspid aortic valves were examined. There were 275 first degree relatives of whom 220 were living, and 188 (85.5%) of these were examined. Seven first degree relatives were found to have aortic valve disease, and in a further 11 there was 'doubtful' evidence of bicuspid aortic valves. In 6 families there was more than 1 affected member and in an additional 7 families there was 1 or more 'doubtful' first degree relative. The minimum family incidence was therefore 14.6 per cent, or 31.7 per cent if 'doubtful' cases were included. The inheritance is most probably multifactorial, but occasionally the condition may occur as an autosomal dominant. The difficulties of diagnosing bicuspid aortic valves before the development of obstruction of left ventricular outflow were encountered and are discussed. The association of a bicuspid aortic valve with asymmetric septal hypertrophy, hypertrophic cardiomyopathy, and Marfan's syndrome was also noted.

Aortic Valve↗
PubMed1978

Left coronary artery anatomy in patients with bicuspid aortic valves.

The anatomy of the proximal left coronary artery in 33 adult patients with bicuspid aortic valves was compared with that in 33 adult patients with aortic valve disease of other aetiologies and with that in 50 adult control patients with no valve or congenital heart disease. Patients with bicuspid aortic valves had a higher incidence of immediate bifurcation of the left main coronary artery, of left main coronary length less than 10 mm, and of left coronary artery dominance. The mean length of the left main coronary artery was significantly less in the patients with bicuspid aortic valves. These variations from the usual coronary artery anatomy may be part of the developmental abnormalities responsible for bicuspid aortic valves, and require evaluation and consideration when considering angiography and valve replacement in patients with aortic stenosis.

Anthropometry↗
PubMed2004

Influence of structural geometry on the severity of bicuspid aortic stenosis.

Doppler-derived gradients may overestimate total pressure loss in degenerative and prosthetic aortic valve stenosis (AS) due to unaccounted pressure recovery distal to the orifice. However, in congenitally bicuspid valves, jet eccentricity may result in a higher anatomic-to-effective orifice contraction ratio, resulting in an increased pressure loss at the valve and a reduced pressure recovery distal to the orifice leading to greater functional severity. The objective of our study was to determine the impact of local geometry on the total versus Doppler-derived pressure loss and therefore the assessed severity of the stenosis in bicuspid valves. On the basis of clinically obtained measurements, two- and three-dimensional computer simulations were created with various local geometries by altering the diameters of the left ventricular outflow tract (LVOT; 1.8-3.0 cm), orifice diameter (OD; 0.8-1.6 cm), and aortic root diameter (AR; 3.0-5.4 cm). Jet eccentricity was altered in the models from 0 to 25 degrees. Simulations were performed under steady-flow conditions. Axisymmetric simulations indicate that the overall differences in pressure recovery were minor for variations in LVOT diameter (<3%). However, both OD and AR had a significant impact on pressure recovery (6-20%), with greatest recovery being the larger OD and the smaller recovery being the AR. In addition, three-dimensional data illustrate a greater pressure loss for eccentric jets with the same orifice area, thus increasing functional severity. In conclusion, jet eccentricity results in greater pressure loss in bicuspid valve AS due to reduced effective orifice area. Functional severity may also be enhanced by larger aortic roots, commonly occurring in these patients, leading to reduced pressure recovery. Thus, for the same anatomic orifice area, functional severity is greater in bicuspid than in degenerative tricuspid AS.

Adult↗
PubMed1975

Reversal of dominance of the coronary arterial system in isolated aortic stenosis and bicuspid aortic valve.

The dominance of the coronary arterial system was determined angiographically in 75 adult patients with isolated aortic stenosis with or without insufficiency, 25 adult patients with pure aortic insufficiency, 51 adult patients with combined aortic stenosis and mitral valve disease, and 44 children with bicuspid aortic valves. There was an unusually high incidence of left dominance in adults with isolated aortic stenosis (36%) and in children with bicuspid aortic valves (56.8%). These findings suggest the existence of a variant developmental complex consisting of a bicuspid aortic valve and reversal of dominance of the coronary arterial system. Furthermore, it adds support to the concept that progressive stenosis of a bicuspid aortic valve is frequently the etiology of isolated aortic stenosis in adults.

Adult↗
PubMed1978

Dissecting aortic aneurysm associated with congenital bicuspid aortic valve.

Among 119 cases of fatal dissecting aneurysm of the aorta, exclusive of those iatrogenically caused or associated with arachnodactyly or aortic stenosis, there were observed 11 cases of congenital bicuspid aortic valve (9%). The ages ranged from 17 to 69 years, five of the patients being 29 years old or younger. Among the latter, three had coarctation of the aorta and one had Turner's syndrome without coarctation. In one of the older patients, aortic insufficiency was present. Hypertension was either established or inferred from cardiac weight in 73% of the cases. In each case, cystic medial necrosis of the aorta was present. Prolapse of valves other than the aortic was observed in 45% of the cases with bicuspid aortic valve. Compared to an estimated incidence of bicuspid aortic valve of about 1 to 2% in the population, the high incidence among subjects with dissecting aneurysm suggests a causative relationship between bicuspid aortic valve and aortic dissecting aneurysm.

Adolescent↗
PubMed2001

Time course of aortic valve function and root dimensions after subcoronary ross procedure for bicuspid versus tricuspid aortic valve disease.

BACKGROUND: The freestanding aortic root, which is the currently preferred operative technique for pulmonary autografts, is reported to dilate and potentially promote aortic insufficiency, which has led to a controversial debate on the appropriate surgical technique, especially for congenital bicuspid aortic valve disease. Desirable data on the time course of valve function and root dimensions for the alternative subcoronary technique comparing bicuspid and tricuspid aortic valve disease are scarce. METHODS AND RESULTS: Echocardiographic examinations of 31 patients with congenital bicuspid aortic valve disease (group A; age 50.5+/-11.0 years) and 51 patients with acquired tricuspid aortic valve disease (group B; age 48.1+/-15.7 years) who were operated on between June 1994 and August 1998 were performed twice postoperatively. At first and second follow-up, respectively, maximum (mean) pressure gradients were 6.0+/-2.0 (3.6+/-1.0) and 5.1+/-2.1 (2.9+/-1.1) mm Hg in group A and 6.5+/-3.5 (3.9+/-1.9) and 5.0+/-1.7 (2.9+/-1.0) mm Hg in group B (P>0.05 between groups). In group A, grade 0 aortic insufficiency at first and second follow-up occurred in 8 and 7 patients, respectively, grade 0-I in 12 and 9 patients, grade I in 9 and 11 patients, grade I-II in 1 and 0 patients, and grade II in 1 and 4 patients; in group B, grade 0 aortic insufficiency occurred in 16 and 18 patients, grade 0-I in 16 and 8 patients, grade I in 17 and 21 patients, grade I-II in 0 and 1 patient, and grade II in 0 and 1 patient (P>0.05). Aortic insufficiency decreased in 10 patients (17%). However, there was an overall tendency for aortic insufficiency to increase over time (n=23, 38%), although it remained subclinical. Aortic root dimensions did not differ between groups and were constant during follow-up. CONCLUSIONS: This study provides some evidence that the function of the subcoronary pulmonary autograft in bicuspid aortic valve disease is excellent, with stable root dimensions, and is not different from that of tricuspid aortic valves at least up to 5.5 years postoperatively, which suggests the subcoronary technique should be reconsidered.

Adult↗
PubMed1982

M-mode echocardiographic failure to consistently identify the equally bicuspid aortic valve: correlation with orifice-view aortography.

The M-mode echocardiogram is considered a satisfactory diagnostic tool for the determination of the presence of a bicuspid aortic valve. M-mode echocardiographic failure to determine this condition is occasionally encountered and has been suggested to be due to the anatomic configuration wherein the two aortic valve leaflets are nearly equal in size. By employing a specific angiographic study (orifice-view aortography), it has been shown that accurate identification of aortic valve anatomy is possible. Ten patients having orifice-view aortographic findings of an aortic valve having two essentially equally sized leaflets have been studied both by this technique and M-mode echocardiography. Five of these had M-mode echographic evidence of a bicuspid aortic valve. However, 3 exhibited no criteria diagnostic of a bicuspid valve and 2 had only suggestive criteria. The results confirm that M-mode echocardiography alone is insufficient to determine reproducibly the presence of an aortic valvular malformation with or without stenosis when the anatomy is that of an equally bicuspid aortic valve.

Adolescent↗
PubMed2006

Clinical characteristics of bicuspid aortic valves in surgical patients.

Of 413 patients undergoing surgery for aortic valve disease, 42 were confirmed to have congenital bicuspid aortic valve. There were 24 males and 18 females, with a mean age of 54.9 +/- 14.6 years. The etiology of the surgical indications in patients with bicuspid valves were degeneration, rheumatic disease, and infectious endocarditis. Thirty-two patients had aortic stenosis and 10 had aortic regurgitation. Infective endocarditis was seen mainly in patients < or =50 years of age at the time of surgery, while degenerative aortic stenosis occurred more often in older patients. The annular diameter measured during surgery was significantly narrower in the 42 patients with bicuspid valves compared to 371 patients with tricuspid valves who underwent aortic valve replacement during the same period. It is important to prevent infectious endocarditis in younger patients diagnosed with bicuspid aortic valve, whereas careful attention should be paid to decalcification during surgery in older patients because the vast majority suffer from degenerative aortic stenosis.

Adolescent↗
PubMed1982

Recognition of bicuspid aortic valve by plain film calcification.

Differentiation of bicuspid aortic valve from other causes of calcific aortic stenosis is not possible by echocardiography or, in many cases, by aortography. This report describes newly recognized patterns of calcification on plain films that are diagnostic for a bicuspid aortic valve. These are based on identification of the calcified raphe and/or the calcified conjoint leaflet. In 120 patients who underwent surgical repair of calcific aortic stenosis, 40 were found to have bicuspid valve. Examination of the plain films retrospectively allowed a correct recognition of 26 (65%) of these valves. In contrast, only 10 (25%) could be recognized by aortography. Patterns of calcification on plain films represent an important tool for detection of calcified cogenital bicuspid aortic valve.

Aortic Valve↗
PubMed1999

Congenitally bicuspid aortic valves: a surgical pathology study of 542 cases (1991 through 1996) and a literature review of 2,715 additional cases.

OBJECTIVE: To describe a clinicopathologic study of a large group of congenitally bicuspid aortic valves surgically excised at a single institution. MATERIAL AND METHODS: The medical charts and bicuspid valves from patients undergoing aortic valve replacement at Mayo Clinic Rochester between 1991 and 1996 were retrospectively reviewed. RESULTS: The age of the 542 patients ranged from 1 to 86 years (mean, 61), and 372 (69%) were men. Among these, 409 (75%) had pure aortic stenosis (AS), 73 (13%) had pure aortic insufficiency (regurgitation) (AI), 53 (10%) had combined AS and AI, and 7 (1%) had normal function. The mean age was higher for those with AS than AI (65 versus 46 years; P < 0.001), whereas the male-to-female ratio was higher for AI than AS (17.3:1 versus 1.7:1; P < 0.001). The two cusps were not equal in size in 95%, and a raphe was present in 76% (67% typical, 9% atypical). Raphal position was described in 315 and was between the right and left cusps in 270 (86%). Raphal absence occurred more often in valves with equal-sized cusps than unequal (33% versus 14%; P = 0.005). Moderate to severe calcification affected valves with AS more frequently than AI (99% versus 41%; P < 0.001). In contrast, annular dilatation was associated with AI more than AS (48% versus 11%; P < 0.001). Acquired commissural fusion involved valves with combined AS and AI more often than the other functional states (31% versus 14%; P = 0.002). Sixteen patients (age range, 18 to 78 years; 13 men) had infective endocarditis (6 active, 10 healed), including 10 with AI (9 men), 3 with AS plus AI, 2 with AS, and 1 with normal function but embolization. CONCLUSION: Functionally, the most common fate of congenitally bicuspid aortic valves was calcific stenosis with or without regurgitation (85%). Because approximately 4 million US citizens have bicuspid valves and because valve replacement is currently the only treatment of symptomatic AS, this disorder will continue to affect health-care costs.

Adolescent↗