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PubMed1994

Stenotic bicuspid aortic valve associated with a ventricular septal defect in an adult presenting with congestive heart failure: a rare observation.

This report is concerned with an adult presenting with stenotic bicuspid aortic valve associated with a ventricular septal defect (VSD). The association between aortic regurgitation (AR) and VSD has often been described, but that between a stenosed aortic valve and VSD has been rarely observed, although bicuspid aortic valves and ventricular septal defects are probably the two most common congenital heart defects. The development of congestive heart failure in the presented case was considered to be due to an increase in the left to right shunt through the VSD. This was attributable to a progressive elevation in left ventricular pressure as a result of the development, with age, of stenosis of the bicuspid aortic valve.

Aged↗
PubMed1987

Stenosed bicuspid aortic valves in twins.

Hereditary factors may have a part to play in the development of bicuspid aortic valve. This report concerns a pair of identical twins, probably monozygotic, who required aortic valve replacement as a result of aortic stenosis occurring in bicuspid aortic valves. This supports the contention that there may be a genetic influence in the development of bicuspid aortic valve, though environmental factors may be of greater importance.

Aortic Valve↗
PubMed2006

Sudden death in infancy due to bicuspid aortic valve.

Symptoms of bicuspid aortic valve usually occur in the age group of 50-70 years, but rarely, it can also lead to sudden unexpected death in infancy and early childhood. The autopsy of a 2-month-old baby boy, found dead in his cot, revealed the heart weight as 25 g, and the macroscopic examination showed the circumference of the aortic valve consisting of two leaflets as 8 mm. The thickness of the left ventricle, right ventricle, and septum was measured as 8, 7, and 10 mm, respectively. Microscopically, the heart revealed hypertrophic changes of myocytes. Subendocardial areas displayed necrosis of myocytes, and severe and diffuse ischemic changes characterized by loss of myofibers and vacuolization. Interstitial pneumonia was identified in the lungs. Death occurred as a result of a congenital bicuspid aortic valve obstructing the left ventricular outflow tract complicated by lung infection. As there are only a few reported cases in infancy, and congenital bicuspid aortic valve can lead to sudden unexpected death, this case is presented to the forensic community.

Aortic Valve↗
PubMed1978

The natural history of a non-stenotic bicuspid aortic valve.

Forty-one patients in whom the diagnosis of a non-stenotic bicuspid aortic valve had been established by noninvasive techniques were followed up for a mean of 10.9 years. During this period, 2 patients required aortic valve replacement because of the development of calcific aortic valve stenosis at the ages of 52 and 64 and 5 others developed evidence of mild aortic valve stenosis. The appearance of calcium in a bicuspid aortic valve suggests the possibility of subsequent calcific aortic stenosis, and patients with this feature should be carefully followed up. Bacterial endocarditis on the aortic valve occurred in 3 patients, one of whom developed severe aortic regurgitation and subsequently died. Patients with a bicuspid aortic valve are at definite risk from bacterial endocarditis and should receive appropriate antibiotic prophylaxis. In 26/41 (63%) patients there was no clinical change during the follow-up period, including 7 of the patients over the age of 50.

Adolescent↗
PubMed1982

Two dimensional echocardiographic assessment of aortic valve morphology: feasibility of bicuspid valve detection. Prospective study of 100 adult patients.

Two dimensional echocardiographic criteria for bicuspid aortic valve recognition have greater specificity than previously proposed M-mode echocardiographic criteria. The potential clinical use of the two dimensional technique is, however, limited by the technical inability to image adequately the aortic valve leaflets in many patients. One hundred consecutive adult patients undergoing two dimensional echocardiography were prospectively studied. Valve cusp number could not be determined because of dense calcification in eight patients. A bicuspid aortic valve was diagnosed in a single subject. A parasternal short axis view disclosed three commissures (diastolic "Y" configuration) in only 26 patients. Technically adequate parasternal short axis imaging was more likely in younger patients and in non-smokers. In patients not successfully imaged from the parasternal approach, an anteriorly tilted apical four chamber view showed a diastolic "Y" configuration in an additional eight cases. Considering the high prevalence in our population of incomplete two dimensional echocardiographic aortic valve leaflet imaging, angiographic and/or pathological studies must be performed to establish the correlation between these incomplete echocardiographic patterns and aortic valve anatomy if two dimensional echocardiography is to have widespread application in the diagnosis of the congenital bicuspid aortic valve.

Adolescent↗
PubMed1993

Progression of aortic valve dysfunction in 51 adult patients with congenital bicuspid aortic valve: assessment and follow up by Doppler echocardiography.

OBJECTIVE: To assess the pattern and progression of aortic valve dysfunction by serial Doppler echocardiographic examinations in ambulatory adult patients with congenital bicuspid aortic valve. DESIGN AND SETTING: Retrospective analysis of patients referred for Doppler echocardiography over a four year period. SUBJECTS: Fifty one adult patients with echocardiographic diagnosis of congenital bicuspid aortic valve had serial Doppler echocardiographic studies at least six months apart. There were 40 men and 11 women with a mean age of 36 years. MAIN OUTCOME MEASURE: Doppler echocardiographic values of aortic valve dysfunction. Cardiac events including endocarditis and aortic valve replacement were also evaluated. RESULT: Coarctation was present in five patients. 31 (61%) patients had a functionally normal bicuspid aortic valve defined as a mean gradient < 25 mm Hg and mild regurgitation. Significant aortic regurgitation was present in 15 patients (moderate in 12 and severe in three). Three patients had isolated aortic stenosis and two patients had combined aortic valve dysfunction. At a median follow up of 21 months (range six to 46 months), six patients had aortic valve surgery (one for aortic stenosis, three for aortic regurgitation, and two for endocarditis). Only 22 patients (43%) continued to have a functionally normal aortic valve. CONCLUSION: In this cohort of fairly young patients, aortic regurgitation is more common than aortic stenosis. Progression of aortic valve dysfunction occurs in patients with pre-existing valve dysfunction and even in those with normal aortic valve function at the initial echocardiographic examination.

Adult↗
PubMed2005

Frequency by decades of unicuspid, bicuspid, and tricuspid aortic valves in adults having isolated aortic valve replacement for aortic stenosis, with or without associated aortic regurgitation.

BACKGROUND: Aortic valve stenosis (with or without aortic regurgitation and without associated mitral stenosis) in adults in the Western world has been considered in recent years to most commonly be the result of degenerative or atherosclerotic disease. METHODS AND RESULTS: We examined operatively excised, stenotic aortic valves from 932 patients aged 26 to 91 years (mean+/-SD, 70+/-12), and none had associated mitral valve replacement or evidence of mitral stenosis: A total of 504 (54%) had congenitally malformed valves (unicuspid in 46 [unicommissural in 42; acommissural in 4] and bicuspid in 458); 417 (45%) had tricuspid valves (either absent or minimal commissural fusion); and 11 (1%) had valves of undetermined type. It is likely that the latter 11 valves also had been congenitally malformed. Of the 584 men, 343 (59%) had either a unicuspid or a bicuspid valve; of the 348 women, 161 (46%) had either a unicuspid or a bicuspid aortic valve. CONCLUSIONS: The data from this large study of adults having isolated aortic valve replacement for aortic stenosis (with or without associated aortic regurgitation) and without associated mitral stenosis or mitral valve replacement strongly suggest that an underlying congenitally malformed valve, at least in men, is more common than a tricuspid aortic valve.

Adult↗
PubMed2006

Repair of bicuspid aortic valves in patients with aortic regurgitation.

BACKGROUND: Bicuspid aortic valve regurgitation can be caused by a defect in the valve itself or by dysfunction of one or more components of the aortic root complex. A successful repair thus requires correction of all aspects of the problem simultaneously. We review our experience addressing both the valve and the aortic root when correcting bicuspid valve regurgitation. METHODS AND RESULTS: Between 1996 and 2004, we treated 68 patients for aortic regurgitation. Thirty patients had isolated aortic regurgitation, and 38 had an associated ascending aortic aneurysm. All patients were treated using a standardized and integrated surgical technique, which included resection of the median raphe or leaflet plication, subcommissural annuloplasty, reinforcement of the leaflet free edge, and sinotubular junction plication. In the 38 patients with proximal aortic dilatation, reimplantation or remodeling of the aortic root was performed. Immediate postoperative echocardiography showed grade < or = 1 aortic regurgitation in all patients. Three patients nonetheless needed an early re-operation because of recurrent regurgitation. No hospital mortality was observed. At a mean follow-up of 34 months after surgery, all patients were in New York Heart Association (NYHA) class 1 or 2. Two patients needed a re-operation (23 and 92 months, respectively). Echocardiographic follow-up showed no progression of the regurgitation in 58 surviving patients. Four patients progressed to grade 2 regurgitation. CONCLUSIONS: Our data indicate that regurgitant bicuspid aortic valves, whether alone or in association with a proximal aortic dilatation, can be repaired successfully provided that both the valve and the aortic root problems are treated simultaneously.

Adolescent↗
PubMed1993

Spontaneous calcific embolization to the supraclinoid internal carotid artery from a regurgitant bicuspid aortic valve.

Calcific brain embolization leading to stroke is rarely recognized. We report a case of spontaneous embolization to the right cerebral circulation from a severely regurgitant, calcified bicuspid valve. Bicuspid aortic valves constitute the most common congenital heart abnormality, and have a tendency to become stenosed, regurgitant, calcified, or infected. The presence of heavy calcification in a noninfected bicuspid valve may lead to dislodgement of calcific embolic material which is not necessarily heralded by acute valvular rupture. Accurate localization of the calcific embolus to the right supraclinoid internal carotid artery was provided by spiral CT imaging in this case.

Adult↗
PubMed2004

Reconstructed bicuspid aortic valve after 10 years: clinical and echocardiographic follow-up.

BACKGROUND: Sixteen patients (mean age, 30.9 +/- 12.9 years; range, 9-79 years) with incompetent bicuspid aortic valves underwent valve-sparing correction between 1992 and 1995. METHODS: All patients underwent triangular resection of the enlarged leaflet. In addition, 13 patients underwent commissuroplasty. In 3 patients leaflet perforations were corrected with glutaraldehyde-fixed autologous pericardial patch. All patients underwent annual echocardiographic and clinical examinations at our institution. RESULTS: During follow-up 1 patient died of heart failure, and 3 patients underwent reoperations because of valve incompetence or dilatation of the sinus. Two patients underwent reoperation perioperatively. At long-term follow-up (mean, 10.06 +/- 1.01 years) the remaining 10 patients were in New York Heart Association class I. No patient was receiving anticoagulation, and no thromboembolic, bleeding, or endocarditis events were observed in 107.2 cumulative patientyears of follow-up. Mean grade of regurgitation was 0.7 +/- 0.5; mean aortic flow velocity was 2.29 +/- 0.47 m/s. Optimal valvular function led to normal ventricular diameters (left ventricular end systolic diameter, 39.2 +/- 4.3 mm; left ventricular end diastolic diameter, 56.2 +/- 5.9 mm) and normal ventricular function (fractional shortening, 31.5% +/- 0.1%). The dimensions of the aortic root were stable from 1-year to late followup (mean aortic annulus, 27.1 +/- 6.8 mm; sinus of Valsalva, 33.0 +/- 7.1 mm; sinotubular junction, 34.1 +/- 7.7 mm; ascending aorta, 31.6 +/- 7.4 mm). CONCLUSION: In contrast to early follow-up results of 5 reoperations, clinical and echocardiographic results were excellent for 10 patients who had undergone reconstruction of bicuspid incompetent aortic valves, and the patients were in stable condition after 10 years. However, the mode of early failure is unknown. Reconstruction of bicuspid valves is possible in selected patients.

Adolescent↗
PubMed1982

Superiority of two-dimensional echocardiography in the diagnosis of congenitally bicuspid aortic valve.

This study compares the role of M-mode and two-dimensional echocardiography in the evaluation of the nonstenotic, congenitally bicuspid aortic valve. Ten asymptomatic healthy subjects (age 20-35 years) with a systolic ejection click and short systolic murmur were evaluated. M-mode echocardiograms were entirely normal. The aortic root, diastolic closure line, eccentricity index of the aortic valve, and left ventricular size and function were normal in every subject. The two-dimensional parasternal long axis view of the aortic root was also normal in all patients with bicuspid aortic valves; there were no abnormalities of systolic excursion of the aortic cusps and doming of the aortic valve was absent. Images obtained in the two-dimensional parasternal short axis view of the aortic root at the level of the aortic valve revealed only two cusps, anterior and posterior, in all patients. The classic closure pattern of the normal tricuspid aortic valve forming a "Y" was absent in every subject. Instead, a horizontal closure line along the "X" axis was demonstrated in all patients; changing the angulation of the transducer in various positions did not alter this pattern. All subjects had increased reflectance of echoes along the line of diastolic closure, probably resulting from redundancy of the aortic cusp tissue. We conclude that because of the inability of the M-mode echocardiographic technique to accurately diagnose this condition in the study group, two-dimensional echocardiography should be the technique of choice in the evaluation of patients with a suspected bicuspid aortic valve. Recognition of this condition is important in view of its susceptibility to subacute bacterial endocarditis, aortic stenosis, and regurgitation.

Adult↗
PubMed2005

Bicuspid aortic valve disease: recent insights in pathophysiology and treatment.

Bicuspid aortic valve is a common congenital cardiac malformation with a broad spectrum of clinical outcomes. Bicuspid aortic valve may go undetected throughout an individual's lifetime or, alternatively, they may have devastating clinical consequences, resulting in death. Both clinicians and medical scientists have taken a renewed interest in the development, pathophysiology and treatment options for this subtle but often substantial clinical entity. Evidence is mounting to suggest that an underlying disease of the aorta is inherited with bicuspid aortic valve, although considerable controversy surrounds this theory. Novel molecular mechanisms underlying the valve and vascular pathologies, as well as new surgical therapies for these patients have been proposed in the past 10 years.

Aortic Valve↗
PubMed1999

[Stentless aortic root bioprosthesis (freestyle) to patients of bicuspid aortic valve with abnormal positioning of the coronary ostia].

Stentless aortic root bioprosthesis (Freestyle) was implanted to two patients of bicuspid aortic valve stenosis with anatomically abnormal positioning of the coronary ostia. In a patient of LR type bicuspid valve, the left coronary artery was located at 180 degrees against the right coronary ostium. To match the Valsalva sinus of the patient with bioprosthesis, the left half of the native annulus, 23 mm in the diameter, was plicated corresponding to the one third of the Freestyle inflow, 21 mm in the diameter. In the other patient of AP type bicuspid valve, both coronary ostia were closely positioned at 90 degrees. To keep both ostia in the sinus of bioprosthesis, careful trimming and suturing were required in the narrow part of both ostia. Their postoperative courses were uneventful and no regurgitation has been observed in either case.

Aged↗
PubMed2002

The ross procedure for replacement of the bicuspid aortic valve: mid-term results from 55 consecutive cases.

BACKGROUND AND AIM OF THE STUDY: A congenitally dysfunctioning bicuspid aortic valve may require surgical treatment in children and young adults. This retrospective study evaluated mid-term clinical results obtained with the Ross procedure in this patient group. METHODS: Between July 1994 and December 2000, 55 patients (48 males, seven females; mean age 27+/-10 years; range: 7-49 years) underwent replacement of a diseased bicuspid aortic valve (stenosis in six cases (11%); insufficiency in 36 (65%); mixed lesion in 13 (24%)) with a pulmonary autograft. Mean NYHA functional class was 1.7. Five patients (9%) had healed endocarditis and six (11%) had previous cardiac surgery. The autograft was inserted as a subcoronary implant in two cases (4%), as a root in 40 (73%), and as a cylinder in 13 (23%). The right ventricular outflow tract was reconstructed with a cryopreserved pulmonary homograft in all cases. Mean cardiopulmonary bypass and aortic cross-clamp times were 207+/-34 min and 162+/-18 min, respectively. RESULTS: No early or late deaths had occurred at a mean follow up of 31+/-19 months. Two patients (4%) were re-explored for bleeding. Four patients (7%) experienced intraoperative coronary complications which resolved without sequelae. Two-dimensional echocardiographic evaluation of neoaortic valve competence at six months revealed no evidence of aortic valve regurgitation in 46 patients (84%), trivial regurgitation in seven (13%), mild regurgitation in one patient (2%), and moderate regurgitation in one patient. The latter patient (subcoronary implant) required reoperation. At six months, the degree of regression of left ventricular mass compared (versus preoperative) was 34+/-13% (p <0.05). Three patients (5%) showed mild dilatation (>4 cm) of the neoaortic root after two years follow up. All patients are currently asymptomatic, in NYHA class I, and enjoy a normal social lifestyle. CONCLUSION: The Ross procedure may be offered as a low-risk alternative in adolescents and young adults with a bicuspid aortic valve. Although the inclusion cylinder technique might help to prevent root dilatation, continued patient evaluation with regard to root sizing, evidence of neoaortic valve degeneration and homograft dysfunction is required in the long term.

Adolescent↗
PubMed2003

Midterm results of conservative repair of the incompetent bicuspid aortic valve.

AIM: Repair of the bicuspid aortic valve is not generally considered the treatment of choice. Our success with this procedure leads us to report our immediate and mid-term RESULTS METHODS: From August 1993 to December 2000, 19 patients with aortic regurgitation due to congenital aortic valve underwent aortic valve repair (17 men and 2 women with a mean age of 42+/-17 years; range, 16 to 70 years). The mean preoperative aortic regurgitation grade was 3.1+/-0.8 on a scale of 1 to 4. Mean preoperative New York Heart Association functional class was 1.9+/-0.8. Fourteen patients had pure aortic regurgitation, 2 also had infectious endocarditis, 1 had angina pectoris, and 2 had an ascending aortic aneurysm. RESULTS: There was 1 hospital death (5.2%), and 1 patient required re-operation due to recurrent infectious endocarditis. Mean aortic regurgitation grade at discharge was 1.1+/-0.9, and functional class was 1.1+/-0.2. All patients were followed for a mean duration of 40+/-23 months (range, 0.5 to 84 months). There was 1 late death, and two patients required aortic valve replacement. The 5-year survival rate was 90+/-7%. The 1- and 5-year re-operation-free rates were 87+/-12% and 76+/-23%. CONCLUSIONS: Bicuspid aortic valve repair is a safe procedure with good early postoperative RESULTS: However midterm results are not satisfactory. Re-operation is a promising alternative and progress aortic regurgitation were complications. Bicuspid aortic valve repair to valve replacement that requires additional study to individualize treatment.

Adolescent↗
PubMed1992

[A case of ascending aortic dissection after aortic valve replacement in congenital bicuspid aortic valve].

Congenital bicuspid aortic valve is a risk factor of aortic dissection, but the case is rare in Japan. Several reports described ascending aortic aneurysm after aortic valve replacement. In these reports, most of aneurysms were false aneurysm, but the cases of ascending aortic dissection were rare. In this case, dissecting aneurysm of the ascending aorta occurred 4 years after aortic valve replacement, which was performed with mechanical prosthesis because of infective endocarditis, and it was repaired successfully by the modified Cabrol's method. This case was congenital bicuspid aortic valve, and had already been complicated with moderate aortic dilatation in the ascending aorta. In patients of congenital bicuspid aortic valve with aortic dilatation, consideration of complete replacement of the ascending aorta with aortic valve replacement is important.

Adult↗
PubMed2003

Progressive dilation of the diameter of the aortic root in adults with a bicuspid aortic valve.

BACKGROUND: To determine the rate of progression of dilation of the aortic root in adults with a bicuspid aortic valve. METHODS: We reviewed retrospectively the transthoracic echocardiograms of 50 adults with a bicuspid aortic valve. Each patient had had at least two examinations made 12 months apart. Measurements were taken at four levels: at the basal attachment of the leaflets of the valve within the left ventricular outflow tract, at the widest point of the sinuses of Valsalva, at the sinutubular junction, and in the ascending aorta 1 cm beyond the sinutubular junction. RESULTS: Progressive dilation occurred at all levels, ranging from 0.3 mm/yr at the basal attachment within the left ventricular outflow tract to 1.0 mm/yr, 1 cm beyond sinutubular junction. These rates of dilation were greater than the reported rate of 0.8 mm per decade in the normal population. The rate of dilation found in the ascending aorta 1 cm beyond the sinutubular junction was significantly greater than at the other sites (p = 0.005). The 21 patients with baseline measurements greater than 34 mm had a significantly higher rate of progression (p = 0.007). Sex, age, and the degree of valvar obstruction or regurgitation did not significantly influence the rate of progression of dilation. CONCLUSION: There is a significantly higher rate of dilation of the aortic root in adults with a bicuspid aortic valve when compared to the normal population. Periodic evaluation of the ascending aorta is essential in these patients, even after replacement of the aortic valve. Other imaging modalities should be considered if the region beyond the sinutubular junction is not well visualized by transthoracic echocardiography.

Adult↗
PubMed2004

Isolated bicuspid pulmonary valve: an unusual finding.

Whilst the pulmonary valve is a tricuspid valve, very few reports exist of bicuspid pulmonary valves, the majority of which are associated with congenital heart disease. Isolated bicuspid valves not associated with congenital abnormalities are even more rare. Herein, the case is described of a 65-year-old man who died from a stroke, and in whom a bicuspid pulmonary valve was found during post-mortem dissection for tissue donation.

Autopsy↗