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Outcomes of uncomplicated aortic valve stenosis presenting in infants.

BACKGROUND: The management of uncomplicated aortic valve stenosis presenting with critical obstruction in infants continues to be associated with significant morbidity and mortality. However, not all infants have critical obstruction, and outcomes spanning the broader spectrum of disease severity are less well defined. METHODS: In a 12-year period, 55 infants (<3 months of age) were seen with aortic valve stenosis and with anatomy suitable for biventricular repair. Clinical, echocardiographic, angiographic, management, and outcome data were reviewed. RESULTS: Status at presentation (median age 6 days) included signs of congestive heart failure in 20 patients, cardiovascular collapse in 5 patients, and an asymptomatic heart murmur in 30 patients. The initial echocardiogram showed reduced left ventricular function in 26% of patients, with a mean peak instantaneous gradient of 69 +/- 30 mm Hg in patients with normal function. There were 5 deaths (9%), all in patients with poor ventricular function. The initial intervention was balloon valvotomy in 24 patients and surgical valvotomy in 20 patients, with 11 patients having no intervention to date. The freedom-from-intervention rate was 69% at age 1 week, 58% at 1 month, 36% at 3 months, and 28% at 1 year. Patients without cardiovascular collapse, normal left ventricular function, and gradients <60 mm Hg at presentation (n =1 9) had better survival and longer freedom from intervention than patients with poor ventricular function or gradients >or=60 mm Hg (n = 36, P =.0001). CONCLUSION: Most infants with aortic valve stenosis receive intervention, although this may be safely delayed in selected patients with lower initial gradients and good left ventricular function.

Aortic Valve↗

[Echocardiographic evaluation of left ventricular diastolic function in patients with aortic valve stenosis].

Impairment of left ventricular diastolic function in aortic valve stenosis occurs very early and precedes the impairment of systolic function. Aim was to examine left ventricular diastolic function and its association with severity of myocardial hypertrophy and clinical picture. The paper comprised 78 patients with isolated aortic valve stenosis in whom were performed ultrasonography and catheterization. No significant differences in parameters of diastolic filling were observed in patients with mild hypertrophy and preserved systolic function compared to healthy subjects. In patients with moderate myocardial hypertrophy, left ventricular filling was decreased in an early diastole (Emax 51 +/- 5 cm/s, Evti 6.4 +/- 1.1 cm) and increased in late diastole (Amax 88 +/- 11 cm/s, Avti 11.4 +/- 1.8 cm), while deceleration time was prolonged (DT 171 +/- 32 ms). Pulmonary vein flow was decreased during diastole (Dmax 33 +/- 5 cm/s, Dvti 7.6 +/- 2 cm). Pseudonormalization of flow through mitral valve was observed in patients with pronounced hypertrophy of left ventricular wall (mass > 180 g/m2): increase of the velocity during the phase of fast left ventricular filling (Emax 72 +/- 13 cm/s, Evti 9.8 +/- 2.1 cm), decrease during atrial contraction (Amax 31 +/- 6 cm/s, Avti 3.7 +/- 0.4 cm), reduction in deceleration time (DT 116 +/- 11 ms), while pulmonary vein flow velocity was increased during the early diastole (Dmax 64 +/- 17 cm/s, Dvti 10.7 +/- 2.2 cm). Likewise, significant correlation between clinical picture and type of blood flow through mitral valve was observed.

Adolescent↗

Bicuspid aortic valve stenosis complicated by descending aortic dissection mimicking coarctation of the aorta.

We report a rare case of bicuspid aortic stenosis complicated by an ascending aortic aneurysm and aortic dissection of DeBakey type IIIb. A 35-year-old woman was admitted to our hospital to examine her systolic murmur identified at birth. Severe aortic stenosis, dilatation of the ascending aorta, and the narrow color flow signal in the descending aorta were detected by transthoracic echocardiography. Initially, coarctation of the descending aorta was suspected, but aortic dissection, DeBakey type IIIb, was revealed by transesophageal echocardiography. Transesophageal echocardiography is indicated when only insufficient information is available on valve and aortic morphology in patients with bicuspid aortic valve.

Adult↗

[Fatal infectious endocarditis in a patient with annulo-aortic ectasia and predominant bicuspid valve aortic stenosis. Anatomoclinical case].

A 25 year old female with bicuspid aortic valve and aortic stenosis developed infectious endocarditis due to beta hemolytic streptococcus. Specific antibiotic therapy was not successful, the patient developed multiple embolic episodes and platelet dysfunction that prevented surgery. The patient died 5 weeks after admission. Necropsy showed aortic annulo-ectasia, cystic medial necrosis and localized dissection of the proximal aorta. There were multiple aortic valve vegetations and evidence of inflammatory myocardial involvement, coronary embolization and myocardial infarction. Aortic annulo-ectasia should be investigated in patients with aortic valve endocarditis and early surgery would be advised to prevent aortic dissection.

Adult↗

[Percutaneous balloon valvuloplasty for congenital aortic valve stenosis].

We performed percutaneous balloon aortic valvuloplasty for 10 patients with congenital aortic valve stenosis aged from 2 to 17 years and a 54-day-old infant with critical stenosis. The retrograde single balloon technique was used for 6 patients including the infant; the retrograde double balloon technique was used for 3 patients; and both techniques for 2 patients. The valvuloplasty was effective for 10 patients except for the infant in terms of the peak systolic pressure gradient between the left ventricle and aorta (from 80.6 +/- 21.9 to 29.4 +/- 17.0 mmHg). Follow-up cardiac catheterizations one year after valvuloplasty in 3 patients and 3 years after valvuloplasty in one patient disclosed no re-stenosis. Aortic regurgitation newly developed in one patient and advanced Sellers' classification I in 3 patients, however, all of them were asymptomatic and did not progress further. In the infant with critical stenosis, sufficient dilatation could not be achieved and acute myocardial infarction mainly at the lateral wall of the left ventricle developed during the valvuloplasty. He died 3 days after the valvuloplasty. The double balloon technique was found to be superior to the single balloon technique with the latter being ineffective in 2 cases, because the fixation of the balloon at the annulus was very difficult. Double balloon technique has low risk of vascular trauma and is applicable to a large sized annulus, because it enables blood supply between the 2 balloons during the inflation period.

Adolescent↗

Coexisting right and left hypertrophic subvalvular stenosis and fixed left ventricular outflow obstruction due to aortic valve stenosis.

A case of fixed left ventricular outflow tract obstruction due to aortic valve stenosis coexisting with right- and left-sided subvalvular hypertrophic stenosis is documented with hemodynamic data, angiograms, echocardiograms and findings at surgery. Histologic examination of the septal muscle with light and electron microscopy revealed hypertrophy of the muscle but none of the characteristics of idiopathic hypertrophic subaortic stenosis. Septal hypertrophy with subvalvular obstruction can occur secondary to left ventricular pressure overload due to fixed left ventricular outflow tract obstruction and is not always the chance occurrence of two separate diseases.

Aged↗

Diagnosing aortic valve stenosis by parameter extraction of heart sound signals.

The objective of this study was to develop an automatic signal analysis system for heart sound diagnosis. This should support the general practitioner in discovering aortic valve stenoses at an early stage to avoid or decrease the number of surgical interventions. The applied analysis method is based on classification of heart sound signals utilising parameter extraction. From the wavelet decomposition of a representative heart cycle as well as from the Short Time Fourier Transform (STFT) and the Wavelet Transform (WT) spectra new time series were derived. In several segments, parameters were extracted and analysed. In addition, features of the Fast Fourier Transform (FFT) of the raw signal were examined. In this study, 206 patients were enrolled, 159 with no heart valve disease or any other heart valve disease but aortic valve stenosis and 47 suffering from aortic valve stenosis in a mild, moderate or severe stage. To separate the groups, a linear discriminant function analysis was applied leading to a reduced parameter set. The introduced two classification stage (CS) system for automatic detection of aortic valve stenoses achieves a high sensitivity of 100% for moderate and severe aortic valve stenosis and a sensitivity of 75% for mild aortic valve stenosis. A specificity of 93.7% for patients without aortic valve stenosis is provided. The developed method is robust, cost effective and easy to use, and could, therefore, be a suitable method to diagnose aortic valve stenosis by general practitioners.

Aortic Valve Stenosis↗

Results of operation for aortic valve stenosis in infants, children, and adolescents.

Surgical procedures for aortic valve stenosis may be considered either corrective or palliative. During a 22-year period from 1962 to 1984, 120 patients required operation for aortic valve stenosis. The operations done included 117 valvotomies and three initial valve replacements. Six patients, five infants and a 7-year-old girl, died at operation. The remaining 114 patients were followed up for 1 to 23 years (mean 8.7 years). Twenty-six patients (23%) required a second operation 1 to 15 years (mean 6.8 years) after initial valvotomy. Six patients (5%) required a third operation 3 months to 8 years (mean 4.4 years) after the second operation. Eighteen of the 26 patients (69%) having second operations required valve replacement. All third operations were valve replacements. No perioperative deaths occurred at the second and third operations. There were four sudden late deaths (3.5%). Eighty-four of the 114 patients (74%) followed up for 1 to 23 years (mean 7.7 years) have had a satisfactory result from initial valvotomy, being free of symptoms and major events (stroke, endocarditis, sudden death), and have not required reoperation. Fifty-nine percent of a subgroup of 22 patients followed up for a mean of 17.7 years have had a satisfactory result from initial valvotomy.

Adolescent↗

Transcarotid balloon valvuloplasty with continuous transesophageal echocardiographic guidance for neonatal critical aortic valve stenosis: an alternative to surgical palliation.

Neonatal critical aortic valve stenosis is a life-threatening malformation if untreated. Before the late 1980s, the preferred treatment was surgical valvotomy; however, operative mortality was high. Early reports of transcatheter balloon dilation were encouraging, although femoral artery damage and aortic valve insufficiency were procedural limitations. With new balloon catheter technology, transumbilical, transvenous, and transcarotid approaches have been advocated, although a comparison with recent surgical results has not been performed. We compared all neonates who presented to our institution since 1985 with the diagnosis of critical aortic stenosis. Ten patients underwent surgical transventricular valvotomy and 13 patients underwent balloon valvuloplasty via a right carotid cutdown with continuous transesophageal echocardiographic guidance. Prior to intervention, all patients had either left ventricular dysfunction, an aortic valve gradient > 100 mmHg, significant mitral valve insufficiency, and/or ductal dependent systemic blood flow. All patients had successful relief of aortic valve obstruction with normalization of left ventricular function and successful discontinuation of prostaglandin E1. Use of continuous transesophageal echocardiographic guidance resulted in fluoroscopic exposure of only 12 +/- 8 minutes. At the latest follow-up, a similar proportion of patients has required additional aortic valve procedures (38% vs 25%) and overall mortality (20% vs 15%) is similar. In the transcarotid group, 9 of 13 patients (69%) have a normal appearing right carotid artery by Duplex imaging, and no neurologic events have been reported. Balloon aortic valvuloplasty via a right transcarotid approach is safe, simplifies crossing the valve, and is effective for the initial palliation of neonatal critical aortic stenosis. The use of transesophageal echocardiographic guidance reduces fluoroscopy exposure, enables accurate assessment of hemodynamics without catheter manipulation or angiography, and avoids femoral artery injury.

Aortic Valve Stenosis↗

[Severe aortic valve stenosis and coronary disease in the elderly. Epidemiology and hemodynamic features].

BACKGROUND: Aortic valve replacement is an effective and safe intervention in the elderly, but to-day the prevalence of coronary artery disease in the elderly has still to be clarified. Aim of this paper is to analyze in a retrospective study the epidemiology and the hemodynamic relation of the association of severe aortic valve stenosis with coronary artery disease in patients over 75 years. METHODS: In a retrospective study of 12.000 cardiac catheterization procedures, patients with severe aortic valve stenosis were selected: the patients over 75 years (30 patients) were screened for presence/absence of coronary lesion forming two groups: correlations with anatomic and hemodynamic variables were made. RESULTS: 36.6% (11 patients) of the over-75 had significative coronary lesions; the coronary arteries involved were the anterior descendent coronary artery and the right coronary artery; no significative differences were found as to risk factors between the two groups except hypertension; the greater number of calcifications and mitral and aortic regurgitation was found in patients with coronary disease; the values of ejection fraction and cardiac index were significantly smaller in patients with coronary disease. CONCLUSIONS: In patients over 75 the severe aortic valve stenosis is frequently associated with coronary disease and the association is greater than in younger persons; some particular features confirm the elderly patients as a class at particular risk in which coronary angiography and combined bypass graft and valve replacement are of primary importance for the outcomes.

Aged↗

Prevalence of coronary artery disease in patients with isolated aortic valve stenosis.

The prevalence of significant coronary artery disease (reduction in luminal diameter by more than 50%) among 88 consecutive patients with aortic stenosis requiring aortic valve replacement at Hammersmith Hospital was examined. Twenty two (34%) patients had significant coronary disease. Nineteen of 42 (45%) patients with typical angina had coronary disease; three of 20 (15%) patients with atypical chest pain had coronary disease, while none of 26 patients free of chest pain had significant coronary disease. Risk factors for coronary disease were equally distributed among patients with and without significant luminal obstruction. Because of the small, but definite, hazard of coronary arteriography and in the interest of cost containment it is suggested that patients with aortic stenosis who are free of chest pain do not require routine coronary arteriography. This applies particularly to patients requiring urgent aortic valve replacement.

Adult↗

Carotid artery approach for balloon dilation of aortic valve stenosis in the neonate: a preliminary report.

Balloon valvuloplasty in neonates with severe aortic valve stenosis is limited by difficulties in catheter manipulation around the arch and across the valve and by the risk of femoral artery complications. A right common carotid artery cutdown was utilized for balloon aortic valvuloplasty in five neonates 1 to 20 days of age, weighing 3.1 to 3.9 kg. Standard balloon valvuloplasty was performed through a 6F sheath inserted in the right carotid artery. The arteriotomy was repaired at the end of the procedure. Mean left ventricular systolic pressure was reduced from 142 to 97 mm Hg, with a decrease in mean peak systolic pressure gradient from 76 to 33 mm Hg. Only one patient developed mild aortic regurgitation. One patient with a hypoplastic left ventricle died, and one patient required open valvotomy. All four survivors have a normal carotid pulse and no neurologic sequelae. Two of these patients required repeat balloon dilation to treat residual aortic valve stenosis at 8 and 10 months of age, respectively. Balloon valvuloplasty using a carotid artery approach is feasible and was safe in five neonates with severe aortic valve stenosis.

Aortic Valve↗

Neoangiogenesis, T-lymphocyte infiltration, and heat shock protein-60 are biological hallmarks of an immunomediated inflammatory process in end-stage calcified aortic valve stenosis.

OBJECTIVES: We investigated the main biomolecular features in the evolution of aortic stenosis, focusing on advanced lesions. BACKGROUND: "Degenerative" aortic valve stenosis shares risk factors and inflammatory similarities with atherosclerosis. METHODS: We compared nonrheumatic stenotic aortic valves from 26 patients undergoing surgical valve replacement (group A) and 14 surgical control patients (group B). We performed semiquantitative histological and immunohistochemical analyses on valve leaflets to measure inflammation, sclerosis, calcium, neoangiogenesis, and intercellular adhesion molecule-1 (ICAM-1) and vascular cell adhesion molecule-1 (VCAM-1) expression. We assessed heat shock protein 60 (hsp60) gene expression as an index of cellular stress and C-reactive protein, erythrocyte sedimentation rate, and fibrinogen as systemic inflammatory markers. RESULTS: In group A valves, we found a prevalence of calcium nodules surrounded by activated inflammatory infiltrates, neovessels, and abundant ICAM-1, VCAM-1, and hsp60 gene expression. Specimens from group B were negative for all of these markers, except 2 of 14 positivity for hsp60. The presence of active inflammatory infiltrates correlated with an abundance of thin neovessels (p < 0.01) and hsp60 gene expression (p = 0.01), whereas neoangiogenesis correlated with inflammation (p = 0.04), calcium (p = 0.01), and hsp60 gene expression (p = 0.04). CONCLUSIONS: "Degenerative" aortic valve stenosis appears to be a chronic inflammatory process associated with atherosclerotic risk factors. The coexistence of neoangiogenesis, T-lymphocyte infiltration, adhesion molecules, and hsp60 gene expression indicates an active immunomediated process in the final phases of the disease.

Aged↗

Decreased platelet function in aortic valve stenosis: high shear platelet activation then inactivation.

OBJECTIVE: To elucidate the mechanism of the bleeding tendency observed in patients with aortic valve stenosis. DESIGN: A prospective study of high and low shear platelet function tests in vitro in normal controls compared with that in patients with severe aortic valve stenosis with a mean (SD) systolic gradient by Doppler of 75 (18) mm Hg before and at least 4 months after aortic valve replacement. SETTING: District general hospital. RESULTS: The patients showed reduced retention in the high shear platelet function tests. (a) Platelet retention in the filter test was 53.6 (12.6)% in patients with aortic valve stenosis and 84.8 (9.6)% in the controls (P < 0.001). (b) Retention in the glass bead column test was 49.8 (19.2) in the patients and 87.4 (8.7) in the controls (P < 0.001). (c) The standard bleeding time was longer in the patients (P < 0.06). Results of the high shear tests (a, b, and c) after aortic valve replacement were within the normal range. The platelet count was low but within the normal range before surgery and increased postoperatively (P < 0.01). There were no differences in the results of standard clotting tests, plasma and intraplatelet von Willebrand's factor, or in 15 platelet aggregation tests using five agonists between patients with aortic valve stenosis and controls. CONCLUSIONS: The high shear haemodynamics of aortic valve stenosis modify platelet function in vivo predisposing to a bleeding tendency. This abnormality of platelet function is detectable only in vitro using high shear tests. The abnormal function is reversed by aortic valve replacement. High shear forces in vitro activate and then inactivate platelets. By the same mechanisms aortic valve stenosis seems to lead to high shear damage in vivo, resulting in a clinically important bleeding tendency in some patients.

Aged↗