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PubMedDate not supplied

Reduced left ventricular size and endocardial fibroelastosis as correlates of mortality in newborns and young infants with severe aortic valve stenosis.

30 patients with severe aortic valve stenosis presented in severe congestive heart failure within the first 2 months of life. In 25 of them, left ventricular volume and contractility were assessed; five of them had a left ventricle of normal size, in 11 left ventricular size was diminished, and in nine patients it was enlarged. Eleven of the infants had extensive endocardial fibroelastosis (EFE) evidenced angiographically by myocardial sinusoids in ten of them and established at autopsy in six. The presence of EFE correlated with the size of the left ventricle; eight of 11 with a small left ventricle, two of five with a normal-sized left ventricle, and one of nine with an enlarged left ventricle displayed EFE. The severe depression of left ventricular function associated with EFE was documented by left ventricular volume determinations on exclusion of the myocardial sinusoids. Of 30 patients, 12 (including eight of 26 who underwent surgery) did not survive. Mortality, severity, and early onset of symptoms were associated mainly with small size of the left ventricle and with the severe left ventricular dysfunction associated with EFE.

Aortic Valve Stenosis↗
PubMed2003

[Preoperative magnetic resonance imaging evaluation of the aortic annular diameter in aortic valve stenosis].

Accurate preoperative knowledge of the aortic annular diameter is a great value in determining the appropriate procedure for aortic valve stenosis. We have successfully used magnetic resonance imaging (MRI) to evaluate the size of the aortic annular diameter preoperatively. Between October 1997 and October 1998, 5 aortic valve replacements were performed in patients with aortic valve stenosis. MRI was performed preoperatively in all cases. All images were gated to the R-wave of the electrocardiogram. Images representing 5 mm slices were acquired in the plane which is parallel to the line through the center points of the aortic root and left ventricle outflow tract. The maximum diameter of the aortic annulus (D) was measured at the point just below the attachment of the aortic valve leaflets. This diameter was compared to the maximum diameter of the prosthetic valve sizer (D 1) which could be easily inserted into the aortic annulus intraoperatively, and to the diameter of the prosthetic valve (D 2) that ultimately was implanted. Twenty-five minutes were required to scan each patient. There were no complications related to scanning. Mean values were D = 23.4 mm, D 1 = 22.3 mm and D 2 = 21.4 mm. There was a significant correlation between D and D 1, and D 2 was approximately 2 mm smaller than D. MRI evaluation is an accurate, noninvasive method for determining the aortic annular diameter preoperatively. We highly recommend this method for measuring the aortic annular diameter in case of aortic valve stenosis as part of the preoperative evaluation.

Aorta↗
PubMed1993

Incidence and prognosis of congenital aortic valve stenosis in Liverpool (1960-1990).

OBJECTIVE: To determine the incidence and prognosis of congenital aortic valve stenosis in the five Health Districts of Liverpool that make up the Merseyside area. DESIGN: The records of the Liverpool Congenital Malformations Registry and the Royal Liverpool Children's Hospital identified 239 patients (155 male, 84 female) born with aortic valve stenosis between 1960 and 1990. Patients were traced to assess the severity of stenosis at follow up. Information on the severity at presentation and all subsequent events was obtained. RESULTS: Congenital aortic valve stenosis occurred in 5.7% of patients with congenital heart disease born in the Merseyside area. The median age at presentation was 16 months (range 0-20 years). Stenosis was mild at presentation in 145 patients, moderate in 33, severe in one and critical in 21 and 39 had a bicuspid valve without stenosis. Additional cardiac lesions were significantly more common in children presenting under one year of age and in those with critical stenosis. The median duration of follow up was 9.2 years (range 1-28 years) and seven patients were lost to follow up. 81 operations were performed in 60 patients. The reoperation rate was 28.3% after a median duration of 8.7 years (range 2.5-18 years). 15% of patients who presented with mild stenosis subsequently required operation compared with 67% of those with moderate stenosis. There were no sudden unexpected deaths and no deaths after aortic valvotomy, except in those presenting with critical stenosis. Mortality was 16.7% but patients presenting with critical aortic stenosis had a much worse prognosis. Actuarial and hazard analysis showed that the survival and absence of serious events (aortic valve surgery or balloon dilatation, endocarditis, or death) were significantly better in patients who presented with mild aortic stenosis than in those who presented with moderate aortic stenosis. 75% of patients presenting with mild stenosis had not progressed to moderate stenosis after 10 years of follow up. CONCLUSIONS: Congenital aortic valve stenosis may be progressive even when it is mild at presentation. Patients presenting with mild stenosis, however, have a significantly better prognosis than those presenting with moderate stenosis. An accurate clinical and echocardiographic assessment of the severity of aortic valve stenosis at presentation provides a good guide to prognosis into early adult life.

Actuarial Analysis↗
PubMedDate not supplied

Inflammatory regulation of extracellular matrix remodeling in calcific aortic valve stenosis.

BACKGROUND: Calcific aortic stenosis (AS), the most frequent heart valve disorder in developed countries, leads to the calcification and fibrous thickening of the valve. While several studies have addressed the process of valvular calcification, the molecular pathomechanisms of the extensive matrix remodeling remain unclear. Because inflammation is present in stenotic valves, we hypothesized that the proinflammatory cytokine tumor necrosis factor alpha (TNFalpha) might influence cell proliferation and regulate the expression and activation of matrix metalloproteinases (MMPs)--enzymes that are thought to be involved in calcific AS. METHODS: Immunohistochemistry for leukocytes, TNFalpha, MMP-1, and the endogenous MMP inhibitor tissue inhibitor of metalloproteinase (TIMP)-1 was performed on human stenotic (n = 19) and control (n = 8) valves. Primary cultures of human aortic valve myofibroblasts were incubated with and without TNFalpha, and cell proliferation was assessed. The expression and activation of MMP-1 were detected by Western blotting and a specific MMP-1 activity assay. RESULTS: Control valves showed scattered macrophages and low expression of TNFalpha, MMP-1, and TIMP-1. In stenotic valves, leukocyte infiltration and a strong, colocalized expression of TNFalpha and MMP-1 were present, while TIMP-1 remained unchanged. Double-label immunofluorescence localized TNFalpha mainly to macrophages. In cultured human aortic valve myofibroblasts, TNFalpha stimulated proliferation and induced a time-dependent increase in MMP-1 expression and activation, while TIMP-1 remained unchanged. CONCLUSION: The results indicate that matrix remodeling in calcific AS involves the expression and activation of MMPs. Activated leukocytes, by the secretion of TNFalpha, may stimulate valvular myofibroblasts to proliferate and express MMPs, thus regulating actively the matrix remodeling in calcific AS.

Aortic Valve Stenosis↗
PubMed1988

The natural history of aortic valve stenosis.

Despite different aetiologies, acquired aortic stenosis is a self-maintaining, slowly progressive process with good long-term prognosis. In 142 patients with mild stenosis, there was clinical progression within 10 years of the initial diagnosis in only 12% of patients. Twenty-five years after the diagnosis had been established, the severity of aortic stenosis was clinically unchanged in 38%, while 25% of patients had moderate stenosis and 35% had undergone valve replacement. Progression of moderate aortic stenosis was more rapid: the average time interval between the manifestation of moderate aortic stenosis and surgery was 13.4 years. Age at the onset of initial symptoms was related to aetiology: 39 +/- 18 years with rheumatic aortic stenoses, 48 +/- 6 years in patients with bicuspid valves who had no history of rheumatic fever, infective endocarditis or myocarditis, and 66 +/- 12 years in degenerative, calcific stenoses of tricuspid aortic valves. Patients with haemodynamically severe stenosis who had refused the recommended operation (n = 55) had an overall poor prognosis: mean survival averaged 23 +/- 5 months and the five-year probability of survival was 18 +/- 7%. All these patients died within 12 years of observation. Mean survival after the occurrence of angina pectoris was 45 +/- 13 months, after syncope 27 +/- 15 months, and after first occurrence of left heart failure 11 +/- 10 months.

Adolescent↗
PubMed1986

Retrograde transfemoral catheterization of the left ventricle in children with aortic valve stenosis.

In 19 consecutive children with aortic valve stenosis, the left ventricle was entered retrogradely with a Gensini catheter guided by a tip-deflector guidewire which could produce any desirable degree of curvature at its tip. In all patients, the aortic valve was retrogradely traversed for measurement of the pressure gradient (mean gradient = 53 mmHg, mean valve area = 0.36 cm2), and left ventriculography. No complications were encountered. This technique is safe, simple, and effective in retrograde catheterization of the left ventricle in children with aortic stenosis.

Adolescent↗
PubMed2001

Aortic valve replacement combined with endoventricular circulatory patch plasty (Dor operation) in a patient with aortic valve stenosis and severe ischemic cardiomyopathy.

A 58-year-old woman with ischemic cardiomyopathy and aortic valve stenosis, underwent aortic valve replacement and simultaneous endoventricular circulatory patch plasty (Dor operation). She underwent coronary artery bypass grafting for severe triple vessel disease 10 years ago. Recently she started to show severe congestive heart failure. Aortic valve stenosis with pressure gradient of 85-mmHg was also found. Coronary bypasses were all patent, but the left ventricle (LV) was severely dilated (LVDd/Ds=71/61 mm) and the ischemic cardiomyopathy was considered as the cause. She successfully underwent aortic valve replacement and endoventricular circulatory patch plasty. The initial postoperative course was complicated with intractable ventricular arrhythmia, but subsequent course was smooth and the patient was discharged with improved symptoms (NYHA Class II). Postoperative catheterization showed decreased left ventricular volume and improved contractility. This case implies the role of LV remodeling procedure in the ischemic cardiomyopathy combined with aortic valve lesion

Aortic Valve Stenosis↗
PubMed1975

Simultaneous operative correction of aortic valve stenosis and idiopathic hypertrophic subaortic stenosis.

Idiopathic hypertrophic subaortic stenosis (IHSS) may coexist with aortic valve stenosis (AVS). This combination of lesions is uncommon and may not be identified by the usual preoperative clinical evaluation and laboratory studies including cardiac catheterization. Surgical relief of AVS without concomitant septal myectomy to relieve unrecognized underlying IHSS may be followed by a low cardiac output, evidence of residual outflow tract obstruction, and death. Echocardiography (ECHO) will accurately identify IHSS and should be performed in all patients suspected of having AVS. This report describes the first three patients in whom the preoperative diagnosis of both AVS and IHSS was made and who subsequently had both lesions corrected at the same operation. All patients survived operation. The outflow tract gradient was abolished in two patients and significantly reduced in the third. All patients with this combination of lesions should be offered operation for the same indication as isolated aortic valve stenosis. The operation should include a septal myectomy as well as relief of the aortic valve stenosis.

Aortic Valve Stenosis↗
PubMed1987

Pulmonary atresia and aortic valve stenosis.

A case is described of pulmonary atresia with ventricular septal defect and severe aortic valve stenosis. The aortic valve gradient measured at the time of insertion of an aortic to pulmonary arterial shunt was only 5 mm Hg and aortic valvotomy was deferred. Post-operative low output cardiac failure resulted in the death of the patient. The severity of the aortic stenosis was confirmed by post-mortem examination. We suggest consideration of aortic valvotomy in similar cases as the intraoperative pressure gradient may be unreliable.

Abnormalities, Multiple↗
PubMed1984

Angiographic estimation of poststenotic jet kinetics in aortic valve stenosis.

The poststenotic jet phenomenon in aortic valve stenosis observed during angiography was studied by a kinetic model. The model allowed to estimate the linear momentum and the kinetic pressure of the jet, which were found to correlate to the poststenotic width of the ascending aorta indicating that the degree of poststenotic widening may be due to the pressure release from the jet.

Adult↗
PubMed2005

Quantification of aortic valve area and left ventricular muscle mass in healthy subjects and patients with symptomatic aortic valve stenosis by MRI.

MRI allows visualization and planimetry of the aortic valve orifice and accurate determination of left ventricular muscle mass, which are important parameters in aortic stenosis. In contrast to invasive methods, MRI planimetry of the aortic valve area (AVA) is flow independent. AVA is usually indexed to body surface area. Left ventricular muscle mass is dependent on weight and height in healthy individuals. We studied AVA, left ventricular muscle mass (LMM) and ejection fraction (EF) in 100 healthy individuals and in patients with symptomatic aortic valve stenosis (AS). All were examined by MRI (1.5 Tesla Siemens Sonate) and the AVA was visualized in segmented 2D flash sequences and planimetry of the performed AVA was manually. The aortic valve area in healthy individuals was 3.9+/-0.7 cm(2), and the LMM was 99+/-27 g. In a correlation analysis, the strongest correlation of AVA was to height (r=0.75, p<0.001) and for LMM to weight (r=0.64, p<0.001). In a multiple regression analysis, the expected AVA for healthy subjects can be predicted using body height: AVA=-2.64+0.04 x(height in cm) -0.47 x w (w=0 for man, w=1 for female).In patients with aortic valve stenosis, AVA was 1.0+/-0.35 cm(2), in correlation to cath lab r=0.72, and LMM was 172+/-56 g. We compared the AS patients results with the data of the healthy subjects, where the reduction of the AVA was 28+/-10% of the expected normal value, while LMM was 42% higher in patients with AS. There was no correlation to height, weight or BSA in patients with AS. With cardiac MRI, planimetry of AVA for normal subjects and patients with AS offered a simple, fast and non-invasive method to quantify AVA. In addition LMM and EF could be determined. The strong correlation between height and AVA documented in normal subjects offered the opportunity to integrate this relation between expected valve area and definitive orifice in determining the disease of the aortic valve for the individual patient. With diagnostic MRI in patients with AS, invasive measurements of the systolic transvalvular gradient does not seem to be necessary.

Aged↗
PubMed2006

Low-gradient aortic valve stenosis: value and limitations of dobutamine stress testing.

Aortic valve stenosis has already reached endemic proportions in Western countries. As the prognosis of low-flow aortic valve stenosis under medical treatment is dismal, surgery is recommended in most patients. Preoperative dobutamine stress testing may help to assess surgical risk, but there is no strong scientific evidence to deny surgery based exclusively on the results of this test. The problems associated with clinical decision making in this condition are reviewed.

Aortic Valve Stenosis↗
PubMed2006

Catheter management of aortic valve stenosis in neonates and children.

Therapy for severe aortic valve stenosis in infants and children has shifted from the operating suite to the catheterization laboratory and even to the bedside as a direct result of improved catheter technology, evolving techniques, and comparable results to conventional surgical intervention. This review summarizes the brief history pertaining to the various techniques and outcomes of transcatheter balloon valvuloplasty in infants and children with critical or severe aortic valve stenosis.

Adolescent↗
PubMedDate not supplied

[Aortic valve stenosis].

There is a long latent period in the clinical course of aortic valve stenosis in adults. Symptoms usually occur in the case of critical stenosis, when aortic orifice area is under 0.7 cm2 (0.4 cm2/m2). In this study 78 patients with critical aortic valve stenosis were investigated. The first manifestation of the disease was dyspnea (78.2%), angina (52.5%), less often a syncope (34.6%), while 17.9% of patients were asymptomatic. Left ventricular systolic function was preserved in 77% of patients, while left ventricular dyastolic dysfunction occurred in almost all the patients. In 42 patients (53.4%) aortic valve replacement was performed. Left ventricular systolic function improved in 88.1% of patients postoperativelly, as well as in patients with preoperatively preserved or poor systolic function. Recovery was fast particularly during the first 6 postoperative months. After the surgery the improvement of the left ventricular dyastolic function was slower then systolic, particularly in patients with extreme hypertrophy of myocardium in whom the process of recovery might last several years.

Adolescent↗
PubMed1993

The progression of mild congenital aortic valve stenosis from childhood into adult life.

UNLABELLED: We studied 187 patients who presented with mild congenital aortic valve stenosis or a bicuspid aortic valve without stenosis at presentation; 63% were males. Information on all clinical events was obtained, and patients were traced to assess current clinical status. RESULTS: The median age at presentation was 2 years (range, 0-15). Additional cardiac lesions occurred in 51 patients, more commonly in patients presenting under 1 year of age (P < 0.0001). The median duration of follow-up was 10 years (range, 1-28); seven patients were lost to follow-up. Thirty-two patients progressed to require intervention (28 surgical, five balloon valvuloplasty) at a median age of 10.5 years. No patient who presented with a bicuspid aortic valve required intervention. Two patients developed endocarditis. There were eight deaths; four after surgery for aortic stenosis and four due to other cardiac lesions. There were no sudden deaths. Actuarial and hazard analysis showed that progression beyond mild stenosis was closely related to duration of follow-up. CONCLUSIONS: Congenital aortic valve stenosis is most frequently mild at presentation. Progression is related to duration of follow-up. Fewer than 20% of patients are likely to still have mild stenosis after 30 years. Follow-up into adult life is essential.

Actuarial Analysis↗
PubMed2003

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↗