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At least 289 records · Page 16Linked to original sources
PubMed1993

Amyloid, thrombosis, and acute myocardial infarction in association with a bicuspid aortic valve.

A 34 year old man presented with an inferior non-Q-wave myocardial infarction. Echocardiography showed a bicuspid aortic valve with aortic outflow obstruction. Left coronary cusp morphology was normal but the right coronary cusp was grossly distorted and replaced by a mobile echodense mass encroaching upon the aortic valve orifice. The aortic valve was replaced and pathological analysis of the excised valve showed primary amyloid infiltration of the right coronary cusp but a normal left coronary cusp. The mass adherent to the right coronary leaflet had the histological appearances of organised thrombus and this was assumed to be the source of coronary embolism. This is the first reported case of primary valvar amyloid presenting with clinical sequelae and it illustrates the need for careful clinical assessment in young patients presenting with acute ischaemic syndromes.

Adult↗
PubMed2006

Effect of G-force on bicuspid aortic valve in aviators.

BACKGROUND AND AIMS: Bicuspid aortic valve (BAV) is a common congenital cardiac malformation. The major complications are aortic stenosis (AS), aortic regurgitation (AR), infectious endocarditis and aortic dissection. This paper aims to assess the hemodynamic importance of incidentally-found BAV in military aviators and evaluate the effect of high G-force on disease progression. METHODS: Aviators with BAV were detected by reviewing all cardiac assessment records between 1987 and 2005. All aviators underwent annual flight surgeon examination. Echocardiography was performed as recommended by our cardiologists and flight surgeons. RESULTS: Eight newly diagnosed cases of BAV were found. All of the aviators continued active aviation throughout the study period. Repeat echocardiography demonstrated progressive widening of the aortic diameter in five of the eight aviators. No worsening of valve dysfunction was seen in those with mild aortic regurgitation at diagnosis. Left ventricular dimensions and function did not deteriorate. No new valve complications, including infectious endocarditis, were seen. The age at diagnosis strongly correlated with the root diameter change; both total (r = 0.74, p = 0.02) and annualized (r = 0.78, p = 0.02) change. Over a mean follow-up period of 12.1 years, no difference was seen in the progression of BAV in high-performance as compared to low-performance aviators. CONCLUSIONS: Exposure to G-force and anti-G maneuvers does not appear to worsen cardiac and valve function in aviators with BAV.

Adolescent↗
PubMed1994

Rare association of congenital bicuspid aortic valve, annuloaortic ectasia, and anomalous origin of left circumflex coronary artery.

A bicuspid aortic valve (BAV) is one of the most common congenital heart anomalies. Although each annuloaortic ectasia (AAE) and malformation of the left coronary artery has been described as a complication of BAV, no case associated with both abnormalities has been reported previously. We present here a very rare case of congenital BAV complicated by AAE and the anomalous origin of the left coronary artery.

Aorta, Thoracic↗
PubMed2003

Failure to prevent progressive dilation of ascending aorta by aortic valve replacement in patients with bicuspid aortic valve: comparison with tricuspid aortic valve.

BACKGROUND: Patients with bicuspid aortic valve (BAV) have been frequently complicated with ascending aortic dilation possibly because of hemodynamic burdens by aortic stenosis (AS) or regurgitation (AR) or congenital fragility of the aortic wall. METHODS AND RESULTS: To clarify if the aortic dilation could be prevented by aortic valve replacement (AVR) in BAV patients, we studied 13 BAV (8 AR dominant, 5 AS dominant) and 14 tricuspid aortic valve (TAV) patients (7 AR, 7 AS) by echocardiography before and after AVR (9.7+/-4.8 years). We also studied 18 BAV (11 AR, 7 AS) without AVR. Diameters of the sinuses of Valsalva, sinotubular junction and the proximal aorta were measured. The annual dilation rate was calculated by dividing changes of diameters during the follow-up period by the body surface area and the observation interval. We found that aortic dilation in BAV patients tended to be faster than that in TAV patients, although a significant difference was found only at the proximal aorta (0.18+/-0.08 versus -0.08+/-0.08 mm/(m2/year), P=0.03). BAV patients with and without AVR showed similar progressive dilation. AR dominant group showed tendency of more progressive dilation than AS dominant group in BAV, although it did not reach statistical significance. TAV patients did not show further aortic dilation after AVR. CONCLUSIONS: AVR could not prevent progressive aortic dilation in BAV. Since the aorta did not dilate in TAV, progressive aortic dilation in BAV seems mainly due to the fragility of the aortic wall rather than hemodynamic factors.

Adult↗
PubMed2003

Abnormal extracellular matrix protein transport associated with increased apoptosis of vascular smooth muscle cells in marfan syndrome and bicuspid aortic valve thoracic aortic aneurysm.

BACKGROUND: Marfan syndrome (MS) is a genetic disorder caused by a mutation in the fibrillin gene FBN1. Bicuspid aortic valve (BAV) is a congenital heart malformation of unknown cause. Both conditions are associated with ascending aortic aneurysm and premature death. This study examined the relationship among the secretion of extracellular matrix proteins fibrillin, fibronectin, tenascin, and vascular smooth muscle cell (VSMC) apoptosis. The role of matrix metalloproteinase (MMP)-2 in VSMC apoptosis was studied in MS aneurysm. METHODS AND RESULTS: Aneurysm tissue was obtained from patients undergoing surgery (MS: 4 M, 1 F, age 27-45 years; BAV: 3 M, 2 F, age 28-65 years). Normal aorta from subjects with nonaneurysm disease was also collected (4 M, 1 F, age 23-93 years). MS and BAV aneurysm histology showed areas of cystic medial necrosis (CMN) without inflammatory infiltrate. Immunohistochemical study of cultured MS and BAV VSMC showed intracellular accumulation and reduction of extracellular distribution of fibrillin, fibronectin, and tenascin. Western blot showed no increase in expression of fibrillin, fibronectin, or tenascin in MS or BAV VSMC and increased expression of MMP-2 in MS VSMCs. There was 4-fold increase in loss of cultured VSMC incubated in serum-free medium for 24 hours in both MS (27+/-8%) and BAV (32+/-14%) compared with control (7+/-5%). CONCLUSIONS: In MS and BAV there is alteration in both the amount and quality of secreted proteins and an increased degree of VSMC apoptosis. Up-regulation of MMP-2 might play a role in VSMC apoptosis in MS VSMC. The findings suggest the presence of a fundamental cellular abnormality in BAV thoracic aorta, possibly of genetic origin.

Adult↗
PubMed1995

Familial aorto-cervicocephalic arterial dissections and congenitally bicuspid aortic valve.

BACKGROUND: A primary arteriopathy is often implicated in the etiology of spontaneous cervicocephalic arterial dissections, but its exact nature usually remains unknown. We describe the familial occurrence of spontaneous arterial dissections and congenitally bicuspid aortic valve (BAV) and propose a common developmental defect in these families. SUMMARY OF REPORT: In the first family, a 63-year-old man suffered an extracranial internal carotid artery (ICA) dissection, and his 43-year-old cousin with BAV suffered an intracranial vertebral artery (VA) dissection. Two other family members had pathologically proven BAV. In the second family, a 31-year-old woman suffered bilateral extracranial ICA and VA dissections. Her father, at age 46, suffered an aortic dissection associated with cystic medial necrosis and BAV. Her paternal uncle died from an aortic dissection at age 59. In the third family, a 39-year-old woman suffered extracranial ICA and VA dissections, and her brother died at age 48 from an aortic dissection associated with a BAV. CONCLUSIONS: The familial occurrence of spontaneous arterial dissections and BAV suggests a common developmental defect. The aortic valvular cusps and the arterial media of the aortic arch and its branches are derived from neural crest cells, suggesting that a neural crest defect may be the underlying abnormality in these families.

Adult↗
PubMed2001

Left ventricular septal aneurysm in association with bicuspid aortic valve--a case report.

Diverticula of the left ventricle are rare cardiac anomalies. Most cases arise from the apex of the left ventricle and are usually found in children. Only a few cases have been documented in adults. The authors report a case of a 38-year-old woman who presented with dyspnea and chest pain. She was found to have a septal left ventricular diverticulum associated with bicuspid aortic valve, aortic stenosis, and aortic regurgitation. The aortic valve was replaced with the resection of the diverticulum. Pathologic examination confirmed the diagnosis of fibrous diverticulum.

Adult↗
PubMed2001

Anomalous coronary artery origin associated with bicuspid aortic valve in a patient with rheumatic mitral stenosis: a case report.

A rare case of an anomalous left coronary artery arising from the right sinus of Valsalva associated with bicuspid aortic valve is presented. This case is unique because these congenital anomalies were associated with rheumatic mitral stenosis. This anomalous coronary origin was found at catheterization before balloon mitral valvuloplasty. The clinical significance of this finding is discussed.

Aortic Valve↗
PubMed2004

Cusp extension technique for bicuspid aortic valve in Turner-like stigmata.

A 19-year-old girl with bicuspid aortic valve and Turner-like stigmata underwent a successful repair with a cusp extension technique using fresh autologous pericardium for aortic insufficiency. She also had a simultaneous artificial graft replacement for ascending aortic dilatation. Aortic valve function showed no significant stenosis and slight insufficiency 2 years postoperatively. This technique is recommended to avoid a Ross or Ross/Konno procedure because of its ready availability, simplicity and excellent midterm results.

Adult↗
PubMed1981

TIA, stroke, and the bicuspid aortic valve.

Congenital bicuspid aortic valve, one of the most common congenital heart abnormalities, may become infected, may calcify, and may cause progressive stenosis or progressive insufficiency. While no previous reports suggested cerebral emboli in the absence of valvular infection, we studied four such cases. In all, cerebral angiography was normal and no extracardiac source of emboli was demonstrated. Microthrombus formation and valvular thickening with incompetence could eventuate in embolization.

Adult↗
PubMed2004

Transient left ventricular apical ballooning in a patient with bicuspid aortic valve created a left ventricular thrombus leading to acute renal infarction.

A 44-year-old woman had tako-tsubo-like ventricular dysfunction with chest pain and ST segment elevation on the ECG. Echocardiography revealed a bicuspid aortic valve with moderate to severe aortic regurgitation. She developed mild heart failure during the clinical course, but the medication (furosemide, enalapril, and asprin) had to be stopped because of skin eruptions. Four weeks after ceasing the antiplatelet agent, she was re-admitted with acute renal infarction. Enhanced chest computed tomography revealed a filling defect in the left ventricle and echocardiography showed a high echogenic mass in the left ventricular apical wall. These findings strongly suggested that the renal infarction was caused by an embolism derived from a left ventricular thrombus that formed during the clinical course of the transient left ventricular apical ballooning. Anticoagulation therapy with urokinase and warfarin successfully lysed the thrombus. Left ventricular thrombus should be considered a complication of transient left ventricular apical ballooning, especially in patients with organic heart disease.

Acute Disease↗
PubMed2005

Unilateral renal hypoplasia associated with bicuspid aortic valve.

A 20-year-old man with congestive heart failure (CHF) and hypertension (HT) was admitted to hospital. Ultrasonic echocardiography showed that he had aortic stenosis caused by a bicuspid aortic valve. The plasma renin concentration was slightly elevated, and enhanced magnetic resonance imaging and renography revealed a hypoplastic kidney that had almost lost its normal function. It is postulated that the increased afterload and preload of the left ventricle induced by both of these abnormalities contributed to the onset of CHF and HT. Pharmacological therapy alone failed to control the CHF and HT, but surgical removal of the hypoplastic kidney was effective in reducing the plasma renin concentration and treating the CHF and HT.

Abnormalities, Multiple↗
PubMed2006

Aortic dilatation in patients with bicuspid aortic valve.

The association of a bicuspid aortic valve (BAV) with abnormalities of the proximal thoracic aorta, including dilatation, aneurysm and dissection, has been previously described, leading to the hypothesis of a common underlying developmental defect involving the aortic valve and the aortic wall. Consequently, any patient with BAV should receive a careful assessment not only of the valve function, but also of the aortic root and the ascending aorta. Dilatation of the proximal thoracic aorta is a common finding in patients with BAV and is believed to be related to aortic rupture and dissection. Because progressive dilatation can occur, careful long-term surveillance of the aortic dimensions is required. Prophylactic surgical repair of the dilated aorta should be recommended more aggressively for patients with BAV than for those with a tricuspid aortic valve. However, the optimal timing of aortic surgery in BAV patients remains uncertain because of the limited data available on the natural history of asymptomatic aortic dilatation.

Aorta, Thoracic↗
PubMed1991

Anomalous origin of the right coronary artery associated with congenital bicuspid aortic valve.

Anomalous origin of the right coronary artery is an infrequent anomaly. A case with anomalous origin of the right coronary artery from the ascending aorta above the left sinus of Valsalva is described. This patient was also found to have a bicuspid aortic valve. The aortic valve was markedly calcified and stenotic. The aortic valve was replaced with a St. Jude Medical prosthesis.

Aortic Valve↗