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[An operated case of supravalvular aortic stenosis and aortic regurgitation and narrowed right coronary ostium].

A 38-year-old man was referred to our hospital because of a heart murmur and mild fever. Physical examination disclosed a systolic murmur of grade 3/6 at the apex and a diastolic murmur of grade 4/6 at the 2nd intercostal space of the left sternal border. The chest radiograph revealed mild cardiomegaly. The ECG showed left ventricular hypertrophy and ST segment depression in leads I, aVL and V4 to V6. Aortogram demonstrated supravalvular aortic stenosis (SAS) and aortic regurgitation. At the operation, we found the right coronary ostium was severely narrowed by the thickened right coronary cusp and the fibrous ring causing SAS. Aortic valve replacement and extended aortoplasty were performed. He had been doing well but suddenly died 11 months after the operation.

Adult↗

[New insight into the treatment of aortic stenosis].

Aortic valve stenosis (AS) is the most frequent heart disease after coronary artery disease and arterial hypertension and it is associated with a high incidence of adverse outcomes. Historically, calcific AS has been considered as "degenerative" because it was thought to be the result from aging and "wear and tear" of the aortic valve. However, this perception has changed over the years with the publication of several studies showing that the calcific AS shares many histological similarities with atherosclerosis, thus suggesting that this disease could eventually be modified by lifestyle or pharmacological therapies. Furthermore, recent data support the notion that AS is not an isolated disease uniquely limited to the valve. Indeed, AS is frequently associated with abnormalities of the systemic arterial system, and in particular with reduced arterial compliance, which may have important consequences on the pathophysiology and clinical outcome of this disease. Hence, the assessment of AS severity as well as its therapeutic management should be conducted with the use of a comprehensive evaluation that includes not only the aortic valve but also the systemic arterial system and the left ventricle, since these 3 entities are tightly intricated on both the pathophysiological and hemodynamic standpoints.

Aortic Valve Stenosis↗

Comparison of mitral valve dimensions in adults with valvular aortic stenosis, pure aortic regurgitation and hypertrophic cardiomyopathy.

To assess the effect of left ventricular dilatation on mitral valve size, this study compared the dimensions of the mitral valve in patients with aortic valve disease and hypertrophic cardiomyopathy (HC). A total of 216 valves, removed at operation or necropsy, was analyzed by quantitative morphometric methods from: (1) 2 patient groups with dilated left ventricular cavities (i.e., 17 patients with dilated valvular aortic stenosis [AS] and 31 with pure aortic regurgitation), (2) 2 patient groups without dilated left ventricular cavities (i.e., 29 patients with nondilated AS and 94 with HC), and (3) 45 control subjects without heart disease. Mean mitral leaflet areas in patients with AS with dilated left ventricular cavities (13.1 +/- 3.0 cm2) and aortic regurgitation (12.0 +/- 3.6 cm2) were significantly greater than in control subjects (8.7 +/- 2.0 cm2; p < 0.001). Mean mitral leaflet areas in patients with AS without dilated left ventricular cavities (9.8 +/- 2.0 cm2) were similar to those of normal valves (p = NS). However, mean mitral leaflet area in patients with HC, in whom left ventricular cavities were also nondilated, was significantly greater (12.8 +/- 3.7 cm2) than in those with nondilated AS and in normal subjects (p < 0.001). Therefore, increased mitral leaflet area: (1) is often present in patients with aortic valve disease with dilated left ventricles in whom it appears to be secondary to left ventricular chamber dilatation; and (2) cannot be attributed to left ventricular cavity dilatation in patients with HC, since their cavities were of normal or small size.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗

Supravalvular aortic stenosis in aortic dissection.

Supravalvular aortic stenosis is a rare complication of aortic dissection. We report on echocardiographic and magnetic resonance observations in 2 cases of aortic dissection with false lumen thrombosis of the ascending aorta and severe narrowing of the true lumen.

Aged↗

Timing of surgery in aortic stenosis.

Aortic valve stenosis (AVS) usually results from three distinct processes (degenerative-calcific, rheumatic, and congenital), with a final common pathway of significant aortic outflow tract obstruction. The stenotic lesion tends to progress slowly, but once symptoms develop clinical deterioration can ensue rapidly. Chest pain, dyspnea, and syncope are the most common symptoms of significant AVS. Detection of symptoms, subtle or obvious, is critical to the management of AVS because their presence portends a worse overall prognosis and is an indication for intervention. There are several special clinical scenarios that require added consideration, including individuals with concomitant coronary artery disease, the presence of a relatively small transvalvular pressure gradient in the setting of low cardiac output (so-called low-gradient AVS), and elderly with severe AVS. Surgical aortic valve replacement (AVR) is the mainstay treatment for relief of obstruction in patients with symptomatic AVS. Percutaneous balloon valvuloplasty is reserved for the small minority of patients who are not surgical candidates and is associated with a high restenosis rate. Percutaneous AVR is a new technology that is being tested in a few select centers on patients who are not operative candidates.

Journal Article↗

RETRACTED: Right ventricular function in patients with aortic stenosis undergoing aortic valve replacement.

The effects of aortic stenosis (AS) on right ventricular function during cardiac surgery are not fully understood. Forty patients undergoing aortic valve replacement with either a systolic transvalvular gradient of less than 100 mm Hg (82.1 +/- 5.5 mm Hg; group 1, n = 20) or greater than 120 mm Hg (131.1 +/- 6.9 mm Hg, group 2, n = 20) were investigated with regard to right ventricular function in the perioperative period. Right ventricular ejection fraction (RVEF), right ventricular end-systolic volume (RVESV), and right ventricular end-diastolic volume (RVEDV) were measured by means of the thermodilution technique. Before cardiopulmonary bypass (CPB), RVEF was significantly lower in group 2 patients (34% +/- 6%) than in group 1 (45% +/- 5%). After CPB, RVEF increased significantly in group 2 (28% +/- 4% to 49% +/- 5%), and no further differences were noted between the groups. In the patients with a higher systolic transvalvular gradient, RVEDV and RVESV were lower at the start of surgery, but increased after opening the pericardium. Cardiac index was also lower in these patients. Pericardiotomy resulted in a decrease in right ventricular end-systolic pressure (RVESP) only in the patients of group 2. In these patients more epinephrine was necessary to maintain stable hemodynamics during the post-bypass period. It is concluded that patients with AS are at risk of reduced right ventricular function when the systolic transvalvular pressure gradient is more than 120 mmHg. Knowledge of the complex interaction between the two sides of the heart may enable anesthesiologists to optimize management during the perioperative period.

Aged↗

Is valve replacement indicated in asymptomatic patients with aortic stenosis or aortic regurgitation?

With rare exceptions, aortic valve replacement should be performed in all symptomatic patients with hemodynamically significant aortic stenosis; however, the asymptomatic patient requires a difficult risk-benefit analysis. In most asymptomatic patients the risks of aortic valve replacement outweigh the risks of conservative therapy and careful follow-up. Symptomatic patients with chronic aortic regurgitation should undergo aortic valve replacement. Asymptomatic patients with normal left ventricular function are not surgical candidates, but aortic valve replacement should be performed in most patients with reliable evidence for left ventricle dysfunction, even if symptoms are not yet present.

Aortic Valve Insufficiency↗

Sudden death in supravalvular aortic stenosis: fusion of a coronary leaflet to the sinus ridge, dysplasia and stenosis of aortic and pulmonic valves.

Supravalvular aortic stenosis (SVAS) is an uncommon congenital cardiac anomaly. We report sudden death, occurring during exercise, of a child who had SVAS with fusion of the right coronary aortic leaflet to the supravalvular aortic ridge, resulting in a closed sinus of Valsalva except for a few pinpoint fenestrations in the dysplastic leaflet. In addition, both aortic and pulmonic valves were dysplastic and stenotic. We postulate that near total isolation of the right coronary artery ostium from the aortic lumen compromised the blood supply to the hypertrophied ventricles. We emphasize the importance of other cardiac anomalies associated with SVAS as well as the development of coronary insufficiency.

Abnormalities, Multiple↗

Progressive diffuse aortic stenosis after localized supravalvular aortic stenosis repair.

A 6-year-old boy presented with diffuse stenosis of the aortic arch. He had undergone patch augmentation for localized supravalvular aortic stenosis at 2 years of age. Before the initial operation, the aortic arch and descending aorta were noticed to be hypoplastic with a diameter of 60 to 73% of that of the aortic annulus, without a pressure gradient. 4 years later, progressive diffuse stenosis of the aortic arch with a diameter of 42% of that of the aortic annulus was found. Reoperation, consisting of patch augmentation of the aortic arch, was carried out. A pressure gradient of 40 mmHg distal to the repair was newly recognized, again. This is a rare case of supravalvular aortic stenosis, which required reoperation because of the progression of diffuse stenosis of the distal aorta.

Aorta, Thoracic↗

Outcomes after the Norwood operation in neonates with critical aortic stenosis or aortic valve atresia.

OBJECTIVE: This study was undertaken to determine the demographic, anatomic, institutional, and surgical risk factors associated with outcomes after the Norwood operation. METHODS: A total of 710 of 985 neonates with critical aortic stenosis or atresia enrolled in a prospective 29-institution study between 1994 and 2000 underwent the Norwood operation. Admission echocardiograms were independently reviewed for 64% of neonates. Competing risks analyses were constructed for outcomes after Norwood operation and after cavopulmonary shunt. Incremental risk factors for outcome events were sought. RESULTS: Overall survivals after the Norwood operation were 72%, 60%, and 54% at 1 month, 1 year, and 5 years, respectively. According to competing risks analysis, 97% of neonates reached a subsequent transition state by 18 months after Norwood operation, consisting of death (37%), cavopulmonary shunt (58%), or other state (2%, cardiac transplantation, biventricular repair, or Fontan operation). Risk factors for death occurring before subsequent transition included patient-specific variables (lower birth weight, smaller ascending aorta, older age at Norwood operation), institutional variables (institutions enrolling < or =10 neonates, two institutions enrolling >/=40 neonates), and procedural variables (shunt originating from aorta, longer circulatory arrest time, and management of the ascending aorta). Of neonates undergoing cavopulmonary shunt, 91% had reached a subsequent transition state by 6 years after cavopulmonary shunt, consisting of Fontan operation (79%), death (9%), or cardiac transplantation (3%). Risk factors for death occurring before subsequent transition included younger age at cavopulmonary shunt and need for right atrioventricular valve repair. CONCLUSIONS: Competing risks analysis defines the prevalence of the various outcomes after Norwood operation and predicts improved outcomes with successful modification of controllable risk factors.

Aorta↗

Comparison of frequency of new coronary events in older persons with mild, moderate, and severe valvular aortic stenosis with those without aortic stenosis.

Independent risk factors for new coronary events were prior myocardial infarction, valvular aortic stenosis, male gender, and increasing age in patients with aortic stenosis. In older persons with moderate or severe valvular aortic stenosis, congestive heart failure, syncope, or angina pectoris was present in 101 of 114 persons (89%) with new coronary events and in 1 of 22 persons (5%) without new coronary events (p <0.0001).

Aged↗

Reversibility of pulmonary artery hypertension in aortic stenosis after aortic valve replacement.

Fifty-two surgical patients with isolated aortic valve stenosis were studied preoperatively and postoperatively to determine the incidence of pulmonary hypertension and its response to surgical intervention. Pulmonary artery systolic hypertension was classified as absent (group 1, less than 30 mm Hg), mild (group 2, 30 to 39 mm Hg), moderate (group 3, 40 to 59 mm Hg), and severe (group 4, greater than 60 mm Hg). Thirty-seven of our patients (71%) had preoperative pulmonary hypertension. There was a positive correlation between left ventricular end-diastolic pressure and both systolic and diastolic pulmonary artery pressures preoperatively (p less than 0.001). After operation we found a decrease in mean systolic pulmonary pressure in group 4, from 85.8 +/- 23 mm Hg to 41.2 +/- 10.4 mm Hg (a 52% decrease, p less than 0.001), and in group 3, from 48.9 +/- 5.9 mm Hg to 32.1 +/- 7.1 mm Hg (a 34% decrease, p less than 0.001). A significant decrease in the mean diastolic pressure was found only in group 4, in which the pressure decreased from 33.7 +/- 8.7 mm Hg to 26.0 +/- 7.6 mm Hg (p less than 0.05). The operative mortality was 1.9%. Our data indicate that pulmonary artery hypertension in aortic stenosis is common, is related to end-diastolic pressure, and can be expected to improve in the early postoperative period.

Aged↗

Familial congenital bicuspid aortic valve: secondary calcific aortic stenosis and aortic aneurysm.

A patient with an aneurysm of the ascending aorta and calcific stenosis of a congenital bicuspid aortic valve, whose brother also had a stenotic congenital bicuspid aortic valve, is described. Predominant aortic stenosis at cardiac catheterization and the presence of an aneurysm distal to and not including the aortic valvular ring made the initial diagnosis of Marfan's syndrome unlikely. Cystic medial necrosis present in the aneurysmal wall probably arose as a consequence of poststenotic dilation. Adequate noninvasive evaluation of the ascending aorta requires echocardiographic studies, as well as a chest x-ray film.

Adult↗

Sinus of valsalva aneurysm with congenital aortic stenosis and aortic coarctation.

A 52-year-old female was diagnosed with the extremely rare combination of sinus of Valsalva aneurysm, congenital unicuspid aortic valve stenosis, and coarctation of the aorta. She was successfully treated in a two-stage operation. The first operation consisted of a bypass graft to correct the coarctation. The second, performed 31 days later, included aortic valve replacement, patch closure of the aneurysm, and translocation of the right coronary artery.

Abnormalities, Multiple↗