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PubMed1978

Assessment of atrioventricular conduction in aortic valve disease.

To determine the frequency of atrioventricular conduction disturbances in aortic valve disease, 26 consecutive patients (age 54 +/- 2 years) with symptomatic aortic valve disease were studied by His bundle electrocardiography at the time of cardiac catheterisation and were compared with a group of patients who underwent cardiac catheterisation and were found to have coronary artery or mitral valve disease but no aortic valve disease. Patients with aortic valve disease had significantly longer PR, AH, and HV intervals than cardiac patients not having this abnormality. Patients with aortic stenosis had prolonged HV, 52 +/- 6 vs +/- 42 +/- 2 ms (P = 0.06), whereas patients with chronic aortic regurgitation had prolonged PR, 245 +/- 27 vs 163 +/- 5 ms (P less than 0.001), and prolonged AH, 178 +/- 30 vs 102 +/- ms (P less than 0.001). Patients with combined lesions had significant prolongation of PR, AH, and HV intervals. Three patients with acute aortic regurgitation caused by endocarditis had normal atrioventricular conduction. Though the presence of valvular calcification did not significantly alter the pattern of atrioventricular conduction in these patients, those with calcified aortic valves had longer HV (P less than 0.005) than the control group. In addition, ventricular dysfunction or coronary artery disease did not affect the pattern of atrioventricular conduction in these patients. Thus, atrioventricular conduction disturbances are common in symptomatic aortic valve disease. With aortic stenosis the site of delay occurs more frequently below the His deflection, whereas in aortic regurgitation it is more frequent above the His deflection.

Adult↗
PubMed2004

Is it time for medical therapy for aortic valve disease?

Calcific aortic stenosis is the most common indication for surgical valve replacement. Currently there are no medical therapies approved for the treatment of this disease. This review will summarize the clinical and experimental studies published over the past 5 years that indicate that medical therapy may be an option for this patient population.

Aged↗
PubMed1983

Left ventricular response to isometric exercise in aortic valve diseases and its value in the optimal timing of aortic valve replacement.

In order to evaluate the left ventricular response to isometric exercise in different types of aortic valve disease, isometric exercise tests were performed during cardiac catheterization in 14 patients with pure aortic stenosis, 20 with combined aortic stenosis and regurgitation, and 18 with pure aortic regurgitation. Patients with angina pectoris in whom coronary angiography had not been performed were excluded. Thirty-seven patients were recatheterized 12 months after aortic valve replacement, and the ventricular response to exercise was re-evaluated. Preoperatively, the ejection fraction did not change significantly during exercise in patients with aortic stenosis, tended to decrease in patients with combined valve lesion, and decreased significantly in patients with aortic regurgitation (p less than 0.001). In the three patients whose ejection fraction during preoperative exercise decreased to below 0.40, it remained below 0.50 after successful aortic valve replacement. It appears possible to reveal left ventricular dysfunction in many patients with aortic regurgitation and in some with combined aortic valve disease by means of isometric exercise. The severely depressed ventricular dysfunction during exercise does not appear to correct totally after surgery.

Adult↗
PubMed2006

Molecular genetics of aortic valve disease.

PURPOSE OF REVIEW: Bicuspid aortic valve is the most common type of cardiac malformation and predisposes to aortic valve calcification, which is the third leading cause of heart disease in adults. These seemingly temporally disparate diseases have been described as having genetic influences but limited data exist on the precise genetic causes in humans. Several advances in the molecular genetics of aortic valve formation and calcification have recently been identified and are described here. RECENT FINDINGS: A large family with autosomal-dominant aortic valve disease consisting of bicuspid aortic valve and aortic valve calcification was studied using genome-wide linkage analysis. This led to the identification of a nonsense mutation in NOTCH1 in affected individuals. This finding was supported by the discovery of a NOTCH1 frameshift mutation in an unrelated family with similar aortic valve disease, suggesting that NOTCH1 haploinsufficiency was a genetic cause of aortic valve malformations and calcification. The NOTCH signaling pathway was linked to a molecular pathway for aortic valve calcification, as NOTCH1 was found to repress activation of Runx2 - a transcription factor critical for osteoblast cell fate that is up-regulated in calcified human aortic valves. SUMMARY: The recent genetic and biochemical findings provide novel insights into the molecular and genetic basis for aortic valve dysmorphogenesis and calcification. Future studies focusing on the identification of additional disease-causing and susceptibility genes will aid in the development of prevention strategies. Ultimately, it will be the careful dissection of these molecular pathways that will hopefully lead to novel therapeutic options.

Adult↗
PubMed2005

[Coronary arteriography in severe aortic valve disease].

Three hundred and twelve patients with severe aortic valve disease underwent a pre-operative haemodynamic study including coronary arteriography either as routine (age more than 50 years) or because of chest pains, previous myocardial infarction or for patients with risk factors of coronary atherosclerosis. Significant coronary artery disease was present in 9% of all cases. Coronary artery disease was more frequent in patients with angina and in patients with previous myocardial infarction but none of these factors was specific. We conclude that except young patients < 40 years old, with no chest pain and no coronary risk factors, coronary arteriography is recommended in patients with severe aortic valve disease before aortic valve replacement.

Adult↗
PubMed2005

Mutations in NOTCH1 cause aortic valve disease.

Calcification of the aortic valve is the third leading cause of heart disease in adults. The incidence increases with age, and it is often associated with a bicuspid aortic valve present in 1-2% of the population. Despite the frequency, neither the mechanisms of valve calcification nor the developmental origin of a two, rather than three, leaflet aortic valve is known. Here, we show that mutations in the signalling and transcriptional regulator NOTCH1 cause a spectrum of developmental aortic valve anomalies and severe valve calcification in non-syndromic autosomal-dominant human pedigrees. Consistent with the valve calcification phenotype, Notch1 transcripts were most abundant in the developing aortic valve of mice, and Notch1 repressed the activity of Runx2, a central transcriptional regulator of osteoblast cell fate. The hairy-related family of transcriptional repressors (Hrt), which are activated by Notch1 signalling, physically interacted with Runx2 and repressed Runx2 transcriptional activity independent of histone deacetylase activity. These results suggest that NOTCH1 mutations cause an early developmental defect in the aortic valve and a later de-repression of calcium deposition that causes progressive aortic valve disease.

Adult↗
PubMed1976

Echocardiographic assessment of left ventricular function in aortic valve disease.

Echocardiography was used to study left ventricular size and contraction in 128 patients with isolated aortic valve disease -45 patients with aortic stenosis, 25 with mixed aortic valve disease and 58 with aortic regurgitation. Left ventricular measurements included the end-diastolic internal dimension (LVIDd), mural thickness (PWTd), an index of circumferential myocardial contraction-fractional shortening (FS=[(LVIDd-LVIDs)/LVIDd] X 100)--and stroke volume (LVSV). In the absence of left ventricular failure, measurements in aortic stenosis were characteristic of pressure overload with normal LVIDd and FS and an increase in PWTd related to the severity of stenosis; in aortic regurgitation, there was volume overload with increases in LVIDd and PWTd which were related to the severity of regurgitation, while FS was slightly reduced. In mixed aortic valve disease there was evidence of both pressure and volume overload. When left ventricular failure was associated with aortic stenosis, mixed aortic valve disease and chronic aortic regurgitation, FS was usually reduced. By contrast, in a recent patient with acute severe aortic regurgitation, FS was normal despite left ventricular failure, suggesting pump rather than myocardial failure.

Aortic Valve Insufficiency↗
PubMed1987

Spontaneous course of aortic valve disease.

The fate of patients with aortic valve disease of varying degrees of severity and the relationship between symptoms and haemodynamic status have been studied in 190 adults undergoing cardiac catheterization during the last two decades. During the follow-up period, 41 patients died and 86 underwent aortic valve replacement; these two events were the endpoints for the calculation of 'event-free' cumulative survival. First-year survival in haemodynamically severe disease was 60% in aortic stenosis and 96% in aortic regurgitation; in moderate and mild disease (in the absence of coronary artery disease) first-year survival was 100% in both groups. After 10 years, 9% of those with haemodynamically severe aortic stenosis and 17% of those with severe regurgitation were event-free, in contrast to 35% and 22%, respectively, of those with moderate changes and 85% and 75%, respectively, of those with mild abnormalities. In the presence of haemodynamically severe disease, 66% of the patients with stenosis and 14% of those with regurgitation were severely symptomatic (history of heart failure, syncope or New York Heart Association class III and IV); 23% of patients with moderate stenosis and 14% with moderate regurgitation were also severely symptomatic. Only 40% of those with disease that was severe both haemodynamically and symptomatically with either stenosis or regurgitation survived the first two years; only 12% in the stenosis group and none in the regurgitation group were event-free at 5 years. Patients with haemodynamically severe aortic stenosis who had few or no symptoms had a 100% survival at 2 years; the comparable figure for the aortic regurgitation group was 94%; 75% of the patients in the stenosis group and 65% in the regurgitation group were event-free at 5 years. In the moderate or mild stenosis and regurgitation groups there was no mortality within the first 2 years in the absence of coronary artery disease, regardless of symptomatic status. Haemodynamically and symptomatically severe aortic stenosis and regurgitation have a very poor prognosis and require immediate valve surgery. Asymptomatic and mildly symptomatic patients with haemodynamically severe aortic stenosis are at low risk and surgical treatment can be postponed until marked symptoms appear without a significant risk of sudden death. In severe aortic regurgitation, the decision for surgery should depend not only on symptoms but should be considered in patients with few or no symptoms because of risk of sudden death. In the absence of coronary artery disease, moderate aortic valve disease does not require valve operation for prognostic reasons.

Adolescent↗
PubMed1965

Recommended criteria for cardiac catheterization in patients with aortic valve disease.

Criteria for selection of patients with aortic valve disease for cardiac catheterization are described, based on a study of 81 cases. Children with aortic stenosis warrant catheterization at the time when the clinical diagnosis is made, but in adults this examination may be deferred until symptoms appear or left ventricular hypertrophy is recognized. In patients with pure aortic insufficiency catheterization may be deferred until symptoms appear. When severe stenosis and insufficiency co-exist, the valve is usually heavily calcified. Thirty-seven per cent of patients with aortic valve disease have co-existing mitral lesions and these patients are usually women, are fibrillating and, as a rule, have atrial enlargement in contrast to those with aortic valve disease only. On rare occasions, patients with mitral valve disease have clinically silent but angiographically demonstrable aortic insufficiency; therefore, aortography should precede open-heart correction of a mitral lesion so as to detect minor degrees of aortic insufficiency.

Adolescent↗
PubMed2002

Mechanical stress as cause of aortic valve disease. Presentation of a new aortic root prosthesis.

We present an overview of studies on the aortic valve and propose that mechanical stress is a main causative factor in the degenerative valvular disease. In the normal aortic valve, the leaflets have a smooth surface, free of wrinkles and creases, throughout the opening process. This smooth leaflet surface during motion is achieved by the "pull and release" movement of the commissures, which occurs because of the compliance of the aortic root. When the aortic root is stiffened, either by artificial means or by the loss of elasticity due to aging, the leaflet dynamics change significantly. The leaflets develop a significant number of creases and wrinkles during the opening process. In the bileaflet valve, the leaflets develop similar creasing and wrinkling during the opening process. This happens mainly due to the less-than-ideal design of the bileaflet valve and in spite of the compliant aortic root. When the aortic valve is spared using a noncompliant tube graft, a similar phenomenon of leaflet creasing occurs. Because the creasing produces high stresses from bending and buckling, it is damaging to the leaflet tissue and can lead to degenerative and calcific valvular disease. Based on these observations a new aortic root prosthesis with compliant sinuses has been designed for the valve sparing operation.

Aging↗
PubMed1988

Aortic valve disease and the ST segment/heart rate relationship: a longitudinal study before and after aortic valve replacement.

The ST segment/heart rate relationship or maximal ST/HR slope has been validated as an index of myocardial ischemia in selected populations of patients with angina pectoris. The present study involved patients selected as having aortic valve disease unaccompanied by angiographic coronary artery narrowing. In each of seven patients, so far examined, a slope value and ST segment depression of greater than 1 mm were obtained which, according to previous experience, indicated myocardial ischemia equivalent to coronary heart disease. After aortic valve replacement, there was a significant reduction in heart size as assessed using the cardiothoracic ratio, and the amplitude of QRS complex on the electrocardiogram. The slope was abolished (two patients) or markedly reduced (five patients), and the decrease in the seven patients was statistically significant. ST segment depression could be obtained in one patient. This study has shown the occurrence of maximal ST/HR slope in patients without large coronary artery disease who have aortic valve disease and cardiac enlargement.

Aortic Valve↗
PubMed1998

Repair of nonsevere rheumatic aortic valve disease during other valvular procedures: is it safe?

OBJECTIVE: To investigate the long-term performance of aortic valve repair, we analyzed the results obtained in a 22-year period in patients who underwent repair of nonsevere rheumatic aortic valve disease during other valvular procedures. METHODS: Fifty-three patients (mean 40 +/- 11.6 years of age) with predominant rheumatic mitral valve disease had concomitant aortic valve disease in association with serious tricuspid valve disease in 25 of them. Preoperatively, aortic valve disease was considered moderate in 47.2% of the patients and mild in 52.8%. All patients underwent reparative techniques of the aortic valve (free edge unrolling, 44; subcommissural annuloplasty, 40; commissurotomy, 36) at the time of mitral or mitrotricuspid valve surgery. The completeness of follow-up during the closing interval was 100%, with a mean follow-up of 18.8 years (range 8 to 22.5 years). RESULTS: Hospital mortality rate was 7.5%. Of 49 surviving patients, 26 (53.1%) died during late follow-up. The actuarial survival curve including hospital mortality was 35.4% +/- 8.7% at 22 years. For patients who underwent mitral and aortic valve surgery, the actuarial survival curve at 22 years was 32.3% +/- 13%, whereas for patients who had a triple-valve operation the survival was 37.0% +/- 10.1% (p = 0.07). Twenty-five patients underwent an aortic prosthetic valve replacement. Actuarial free from aortic structural deterioration and valve-related complications at 22 years was 25.3% +/- 9.3% and 12.7% +/- 4.8%, respectively. CONCLUSIONS: Long-term functional results of reparative procedures of nonsevere aortic valve disease in patients with predominant rheumatic mitral valve disease have been inadequate at 22 years of follow-up. According to these data, conservative operations for rheumatic aortic valve disease do not seem appropriate.

Adolescent↗
PubMed2004

[Aortic root homograft in the surgical treatment of aortic valve disease with dilated ascending aorta].

INTRODUCTION AND OBJECTIVES: Patients with aortic valve disease and a dilated ascending aorta are usually treated with a composite graft comprising a valve and conduit. We review here the results of treatment with an aortic root homograft as a valid alternative. PATIENTS AND METHOD: Twenty-two consecutive patients with a mean age of 64.8 (8.8) years were studied. Mean ascending aorta dilation was 54.55 mm, aortic valve insufficiency was present in 16 patients, and a combined lesion was present in 6. In all cases a cryopreserved aortic root homograft was used to replace the aortic valve and ascending aorta. In 9 cases a Dacron conduit was used beyond the sinotubular junction to restore continuity between the homograft and the native aorta. RESULTS: All patients survived surgery. One patient had postoperative systemic inflammatory response syndrome and one patient was re-explored for excessive bleeding. Mean duration of follow-up was 12.1 months (range 2-36 months). No patient was given anticoagulants, and one had an early transient cerebrovascular accident followed by complete recovery. At one month postsurgery the left ventricular systolic (P<.001) and diastolic (P<.009) diameters had decreased significantly on echocardiography, and these decreases persisted throughout follow-up. The caliber of the ascending aorta was normal in all patients (< or =30 mm) except one. CONCLUSIONS: Aortic root homografts are a valid alternative in the treatment of aortic valve disease with ascending aorta dilation. The main advantages of this therapy are that permanent anticoagulation is not needed, and that left ventricular dimensions recover rapidly.

Adult↗
PubMed1984

Left ventricular function and myocardial structure in aortic valve disease before and after surgery.

47 patients with aortic valve disease were studied by left ventricular micromanometry and cineangiography before and 18 months after successful valve replacement. There were 27 patients (17 with aortic stenosis, ten with aortic insufficiency) with moderate hypertrophy (angiographic mass less than 180 g/m2) and 20 patients (ten with aortic stenosis, ten with aortic insufficiency) with severe hypertrophy (angiographic mass greater than or equal to 180 g/m2). In the patients with moderate hypertrophy ejection fraction was slightly although not significantly smaller than in 14 controls. Peak systolic circumferential wall stress was, however, significantly increased. In the patients with severe hypertrophy ejection fraction was significantly decreased although afterload was similar to that in the patients with moderate hypertrophy. This indicated a depressed contractile state in the patients with mass exceeding 180 g/m2. After surgery peak systolic wall stress returned to normal levels in both groups. Among the patients with severe preoperative hypertrophy only the patients with aortic stenosis showed a normal ejection fraction after surgery, whereas in the patients with aortic insufficiency ejection fraction improved but remained depressed compared to the controls. In a second study, comparison of left ventricular function with myocardial structure (endomyocardial biopsies) was carried out in 30 patients with aortic valve disease (group 1: 18 with aortic stenosis, group 2: twelve with aortic insufficiency). Both groups were restudied 18.8 and 17.4 months, respectively, following successful aortic valve replacement. Preoperative biplane ejection fraction (60 and 57%), angiographic mass (183 and 186 g/m2), muscle fibre diameter (31.1 and 30.8 mu), interstitial fibrosis (17.1 and 17.4%) and left ventricular fibrous content (31.0 and 32.2 g/m2) were similar in the two groups.(ABSTRACT TRUNCATED AT 250 WORDS)

Aortic Stenosis, Subvalvular↗
PubMed2000

Aortic valve disease and left dominant coronary system a significant association.

OBJECTIVES: To study the dominance of coronary artery distribution in patients with aortic valve disease. MATERIAL AND METHODS: The prevalence of left dominant coronary artery system in patients with aortic valve disease was compared with patients without aortic valve disease undergoing coronary angiography. Group 1 consisted of 237 patients with symptomatic aortic valve disease and Group 2 consisted of 241 consecutive patients without aortic valve disease undergoing cardiac catheterisation. RESULTS: Forty two patients in Group 1 and 20 patients in Group 2 (p < 0.01) showed a left dominant pattern of supply. Fifteen patients in Group 1 and eight patients in Group 2 showed a co-dominant pattern of supply (p = NS). Among patients in Group 1, there was no significant difference in the increased prevalence of left dominant system between patients with congenital or acquired aortic valve disease or between the different categories of aortic valve lesions. CONCLUSION: Patients with aortic valve disease show a statistically significant higher prevalence of left dominant pattern of blood supply. This higher prevalence of left dominance is seen in all categories of aortic valve lesions, namely, predominant aortic stenosis, predominant aortic regurgitation and in combined aortic stenotic and regurgitant lesions.

Adolescent↗