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Congenital ostial left main coronary artery stenosis associated with a bicuspid aortic valve in a young woman.

We describe a 41-year-old woman with no cardiac risk factors, typical exertional angina and an abnormal noninvasive stress test. Coronary angiography demonstrated an ambiguous left main coronary artery (LMCA) stenosis. Intravascular ultrasound (IVUS) demonstrated no atheroma, but the minimum lumen diameter and area of the ostial LMCA were significantly reduced. Transesophageal echocardiography showed normal left ventricular function with a bicuspid aortic valve. Two-vessel coronary artery bypass grafting was subsequently performed. To our knowledge, this is the first IVUS-documented case of a congenital left main coronary artery stenosis associated with a bicuspid aortic valve.

Adult↗

[Aortic coarctation is correlated with bicuspid aortic valve and intracranial aneurysm: a case report and review of the literature].

Aortic coarctation is often linked to bicuspid aortic valve and only rarely to congenital aneurysm of the circle of Willis. It is possible that a common pathogenetic substrate determines this association, but it has not yet been demonstrated. In this report we describe the case of a 32-year-old woman, affected by previous anamnestic functional cardiac murmur. She has suddenly presented a hemorrhagic stroke due to the breaking of the sylvian artery, angiographically demonstrated and surgically corrected. On the same occasion aortic angiography revealed aortic coarctation. Later, an echocardiographic exam demonstrated the coexistence of bicuspid aortic valve. Afterwards the patient underwent angioplasty in order to correct aortic coarctation. Any coexisting cardiac and vascular disease may influence survival in unoperated adults with aortic coarctation, which can even be lethal. In this case the simultaneous presence of these three malformations was particularly dangerous since the patient was not enough symptomatic and so an early diagnosis was not possible. Therefore, we strongly recommend to perform echocardiography in every young patient affected by hemorrhagic stroke and to take into consideration the possibility of performing cerebral angiography in all cases of aortic coarctation.

Adult↗

Configuration of the ascending aorta in patients with bicuspid and tricuspid aortic valve disease undergoing aortic valve replacement with or without reduction aortoplasty.

BACKGROUND AND AIM OF THE STUDY: Patients with bicuspid aortic valves (BAV) tend to develop dilatation of the ascending aorta. The study aim was to analyze differences in aortic root diameter and configuration in patients with bicuspid and tricuspid aortic valve disease. METHODS: A retrospective analysis was conducted of the angiographies of 461 patients allocated to four groups with: (i) BAV disease with (n = 179) and (ii) without (n = 78) dilatation of the ascending aorta; (iii) tricuspid aortic valve disease (TAV) and dilatation of the ascending aorta (n = 154); and (iv) coronary artery disease (CAD), TAV and normal diameter of the ascending aorta (n=50). Diameters and distances in the aortic root region were measured, and the ascending aorta configuration analyzed. RESULTS: The diameter of the ascending aorta in patients with BAV and dilatation was significantly larger than in those with TAV and dilatation (26.6 +/- 5.22 versus 24.4 +/- 3.74 mm/m2, p = 0.002). Distances between aortic valve level and point of maximum diameter of the ascending aorta at the outer and inner curve of the vessel in patients with BAV without dilatation were greater than those of the CAD group (31.1 +/- 5.27 versus 28.0 +/- 4.86 mm/m2, p = 0.002 and for the indexed values 21.6 +/- 4.05 versus 20.0 +/- 2.71 mm/m2, p = 0.011). All patients with BAV and enlargement of the ascending aorta showed asymmetric dilatation of the vessel. CONCLUSION: All patients with BAV had an abnormal configuration of the ascending aorta. In cases with enlargement of the ascending aorta exclusively, asymmetric dilatation at the convexity of the vessel occurred. Patients with BAV and normal ascending aorta diameter showed an elongation of this vessel segment.

Adult↗

[Characteristics of jets in adult bicuspid aortic valve by color Doppler imaging].

Color flow mapping of 15 adults with bicuspid aortic valves confirmed angiographically and at surgery comprising 8 regurgitations and 7 stenoses was analysed, retrospectively in 12 cases. The object was to detect any special features of the jets of this congenital abnormality. The site of emergence of the jet at the aortic orifice and its direction in the left ventricle were studied in the long axis, short transaortic and left ventricular axes by sequential analysis. Two types of regurgitant jet were observed: eccentric anterior origin (5, Type I), eccentric posterior origin (3, Type II), extending towards the structure opposite to their origin in the left ventricular outflow tract. In cases of stenosis, the cross-sectional view of the jet had an almost transverse slit-like appearance extending from one side of the aortic orifice to the other or an anterior or posterior eccentric oval shape. The Type I cases and the slit-like anterior stenoses had fusion of the coronary cusps whilst the Type II and posterior slit-like stenoses had fusion of the right coronary and non-coronary cusps. This preliminary study suggests that bicuspid aortic valves are associated with jet characteristics related to the abnormal commissural axis which allows diagnosis and precision of the anatomic type in adults despite the presence of calcification.

Adult↗

Two dimensional and Doppler echocardiographic diagnosis of bicuspid pulmonary valve.

The diagnosis of bicuspid pulmonary valve is usually established postmortem, occurring in 7% of patients with pulmonary stenosis and is usually associated to tetralogy of Fallot or ventricular septal defect. The authors report the case of a 14-year-old girl with patent ductus arteriosus in whom 2-D echocardiography disclosed a pulmonary valve formed of two slightly thickened leaflets that opened with the shape of a dome in systole, and moved under the valve ring plane during diastole. A Doppler examination showed a 21 mmHg transvalvar gradient. These characteristic features suggest that 2-D and Doppler echocardiography are the techniques of choice in the diagnosis of bicuspid pulmonary valve.

Adolescent↗

[The bicuspid aortic valve].

Results are reported of an examination of 8 patients with congenital bicuspid aortal valves confirmed at autopsy. Clinical symptoms of this abnormality of development appear as a result of valvular dystrophy and addition of calcinosis with formation of aortal cardiac failure (most frequently stenosis of the aortic ostium). Aortal cardiac failure is very frequently interpreted as rheumatic or atherosclerotic. The author proposes criteria of during life diagnosis of congenital bicuspid aortal valve pathology.

Adult↗

[Surgery of aortic valvular disease with congenital bicuspid or quadricuspid aortic valve].

Twelve patients (age 18 to 69 years old) with surgical treatment for congenital bicuspid aortic valve and one (age 26 years old) for quadricuspid aortic valve were reported. These 13 patients occupied 12.3% of the total number of patients with aortic valve replacement during the same period. Among the patients with bicuspid aortic valve, 10 patients were male (83%) and 2 were female (17%). Seven patients exhibited aortic stenosis and 5 showed predominant aortic regurgitation. The mean age of patients with aortic stenosis was higher than that of patients with aortic regurgitation. (63.3 years vs 32.0 years old). Infective endocarditis occurred in 2 patients (17%). Three patients had pure aortic regurgitation without evidence of endocarditis. One 26-year-old female patient who had a quadricuspid aortic valve presented with aortic regurgitation associated with PDA. All patients underwent aortic valve replacement with no early and late deaths.

Adult↗

[Ruptured aneurysms of sinus of Valsalva with bicuspid aortic valve--a case report of an aged patient (72 years old)].

A 72-year-old man with ruptured aneurysms of the sinus of Valsalva and a bicuspid aortic valve was operated. Ruptured aneurysms were found in the noncoronary sinus region, and they were opened to the commissure between the anterior cusp and septal cusp of the tricuspid valve. The tissue around the aneurysm was very weak, so closure of the ruptured hole was carried out from the inside of both aorta and right atrium. Ruptured aneurysm of Valsalva is very rare in a aged patient. In this case, it is considered that the bicuspid aortic valve associated with malformations of the aortic cusp caused a weakness of the sinus wall, and this region gradually became aneurysmal.

Aged↗

[Double orifice mitral valve associated with bicuspid aortic valve].

A 63-year-old man with double orifice mitral valve (DOMV) and bicuspid aortic valve was reported. Preoperative echocardiography showed prolapse of the posterior leaflet and mitral regurgitation but was unable to show the existence of the duplication of the mitral valve. He underwent aortic and mitral valve replacement and did well after surgery. DOMV is a rare congenital malformation, and DOMV associated with bicuspid aortic valve is the first reported case in Japan.

Abnormalities, Multiple↗

Evaluation of long-term results of bicuspidalization annuloplasty for functional tricuspid regurgitation. A seventeen-year experience with 133 consecutive patients.

Between 1968 and 1985, 133 consecutive patients underwent bicuspidalization annuloplasty for moderate to severe functional tricuspid regurgitation associated with mitral or combined mitral and aortic valve disease. Over this period, the incidence of tricuspid valve replacement was only 2.3% (3/136 patients). There were 18 early deaths (13.5%) in the entire series--three (5.0%) of 60 patients in the last 5 years of the study--and 10 late deaths (8.7%). Actuarial survival rate for the entire series, excluding early deaths, was 91.0% +/- 3.0% at 10 and 17 years. There were seven reoperations (6.1%) on the tricuspid valve, needed because of residual or recurrent mitral valve lesions after the initial operation. Actuarial rates of freedom from reoperation on the tricuspid valve were 93.6% +/- 3.0% (10 years) and 69.7% +/- 16% (17 years) for the entire series: 78% +/- 10% (15 years) for the open mitral commissurotomy plus tricuspid annuloplasty group (44 patients); 90% +/- 9.0% (15 years) for the mitral plus tricuspid annuloplasty group (10); 75.2% +/- 22% (17 years) for the mitral replacement plus tricuspid annuloplasty group (58); and 92.6% +/- 7.0% (16 years) for the combined aortic and mitral valve surgery plus tricuspid annuloplasty group (21). Ninety-eight percent of the survivors were in New York Heart Association class I or II postoperatively. Of 21 randomly selected patients investigated by pulsed Doppler echocardiography, 14 (67%) had no regurgitation or grade 1/4 tricuspid regurgitation and the remaining seven (33%) had grade 2/4 regurgitation postoperatively. Our experiences suggest that bicuspidalization annuloplasty can be a reliable method in the vast majority of patients with functional tricuspid regurgitation.

Actuarial Analysis↗

[Bicuspid aortic valve. Echocardiographic diagnostic potentials].

The echocardiographic potentialities in the diagnosis of bicuspid aortic valve are discussed. In the group of 32 patients examined with a clinic and murmurs suspected for that disease, the index of excentricity in five patients, was found to be respectively 1.6, 1.4, 1.6, 1.8, 1.5, accepted as a reliable index for the diagnosis of aortic bicuspidia. Excentricity index is equal to 1/2 of the aortic diameter, divided to the minimal distance from the diastolic echo of the closed aortic valve to the closer aortic wall. The control group consisted of 44 patients with tricuspid aortic valve, 17 of them being with aortic defect (isolated stenosis or combined aortic defect) and the rest 28 -- without valvular heart disease. Excentricity index for the control group was low -- 1.0--1.33 (average value 1.28). Conclusions are drawn that the echocardiographic method, from the group of the bloodless methods, is with the best diagnostic potentialities in the bicuspid valve and should be looked for in patients with expulsion murmur and click of aortic origin.

Adolescent↗

[Rheumatic cardiopathy in childhood. Epidemiological aspects, and differential diagnostic problems as compared with congenital bicuspid aortic valve and mitral valve prolapse].

A decrease in severity of rheumatic fever, in the number of recurrences of the disease, a relative decrease in prevalence of rheumatic heart disease compared to other forms of heart disease are well documented or suggested by the data collected in the last decades. The reasons of the decline are uncertain. Prevalence surveys in more recent years indicate that, while at one time almost all valvular heart disease was considered to be rheumatic in origin, current data suggest that a significant proportion of damaged valves may be attributed to congenital defects or to acquired disease of other etiologies. Bicuspid aortic valve and mitral valve prolapse deserve special mention. The main clinical and echocardiographic features of bicuspid aortic valve and mitral valve prolapse are discussed.

Adolescent↗

Bicuspid evolution of the arterial and venous atrioventricular valves.

Atrioventricular valves were studied in pisces (shark), amphibians (frog), reptiles (turtle, snake and crocodile) and aves (duck, chicken and quail). Dog shark, Tiger shark and Hammerhead shark have a contractile common atrioventricular orifice with anterior and posterior bridging leaflets. Angel shark resembles the frog in having a contractile oval common atrioventricular orifice guarded by two lateral (right and left) and two central bridging (anterior and posterior) leaflets. In turtles and snakes the interatrial septation is complete. The common oval atrioventricular orifice is divided into two D-shaped orifices by the lower edge of the interatrial septum. Each orifice is guarded by a flap valve hanging down from the interatrial septum with rudimentary chordae tendinae, functionally corresponding to the septal leaflet of the human venous atrioventricular valve (VAVV) and the aortic leaflet of the human arterial atrioventricular valve (AAVV). There is no curved mural leaflet on either side related to curved contractile segments of the annuli. In crocodiles, the interventricular septation is complete. The VAVV is bicuspid with a prominent septal and diminutive mural component with no chordopapillary support. The AAVV is bicuspid with direct attachment of the fused commissural lines between the two leaflets to the ventricular musculature. In the aves, with four chambered hearts the VAVV has a curved mussular mural leaflet with a primitive muscular chorda and no septal leaflet. The AAVV resembles closely the human mitral valve. This study lends support to the view that VAVV and AAVV in man are similar in design and function with a D-shaped annulus and a skirt of leaflet tissue divisible into two functional units. The relatively straight part of the annuli suspend the straight leaflets; septal leafleft of the VAVV and aortic leaflet of the AAV. The curved annular components suspend the curved mural leaflets in both valves. Since this segment is contractile, the related mural leaflets of both valves need to have slits which split them into varying number of scallops to enable them to adapt themselves to the reduced systolic orifice and open widely during diastole. These scallops are best numbered as subsegments of the curved mural leaflet and not considered as separate leaflets. The curved leaflet of the VAVV needs to negotiate the acute border of the heart. Hence a major slit is usually present at this region which artificially divides it into an 'anterior' and 'posterior' portion resulting in assumption of a 'tricuspid' configuration.(ABSTRACT TRUNCATED AT 400 WORDS)

Animals↗

[Congenital bicuspid aortic valve with regurgitation--a rare case showing a fibrous band between the conjoined cusp and the ascending aorta].

It is quite unusual that the congenital bicuspid aortic valve shows aortic valve regurgitation except postinflammatory event. In a case of aortic regurgitation with bicuspid valve, a fibrous band was recognized between the conjoined cusp and the ascending aorta at the operation. Since a degenerative change alone was discerned histologically in the resected specimen, this abnormal band was supposed to be an embryogenic remnant of the aortic valve. Six cases were reported previously and all of them were male, and five were Japanese.

Aorta↗

[Surgical treatment of traumatic rupture of the bicuspid aortic valve: case report].

A 64-year-old female was admitted to our hospital with the complaints of chest oppression and dyspnea, 3 months after the traffic accident. She was operated on with the diagnosis of aortic regurgitation and bicuspid aortic valve. The tear of aortic valve was detected in the anterior cusp, and the size of the tear was 12 mm. The aortic valve replacement was performed and her postoperative course was uneventful. In the pathological study of the cusps, the tear was originated from trauma without inflammatory changes. This is the first report of traumatic rupture of the bicuspid aortic valve in Japan.

Aged↗

[Exercise tolerance in children with primary bicuspid valve prolapse syndrome].

Exercise tolerance in children with the primary bicuspid valve prolapse syndrome has been evaluated with ergometric test. Additionally, an effect of environmental factors on exercise tolerance has been assessed in children from the urban and rural areas. A good tolerance of submaximal loads has been shown in both groups. However, the obtained results have been significantly more favourable in children from rural areas. The authors have concluded that limitation of exercise in children with bicuspid valve prolapse is not necessary during an intensive growth.

Adolescent↗

[A case of aortic bicuspid valvular endocarditis with congenital left ventricular diverticulum].

A 29-year-old man had a prolonged fever and painful Osler's nodes on his right foot. The aortic valve was bicuspid with vegetation mass and the left ventricular diverticulum was additionally present connecting with the left ventricular outflow tract. An operation was performed after intravenous administration of antibiotics for 3 weeks. The aortic bicuspid valve and the vegetation were removed and replaced by an artificial valve (SJM-HP 19 mm). The left ventricular diverticulum was resected. The echocardiographic findings correlated well with the intraoperative observation.

Adult↗