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Cardiac involvement in Takayasu's disease is well documented. This is often the result of severe hypertension. However, severe clinical manifestations of aortic regurgitation and coronary insufficiency are much less common. The authors report a case in which post-infarction angina and severe left ventricular failure led to a double valve replacement and an aorto-right coronary bypass graft procedure. The diagnosis of Takayasu's disease was suspected before surgery and was confirmed by histological examination.

Rheumatoid valvular heart disease and aortic valve replacement for a rheumatoid lesion have been previously reported in the literature. The authors report the first case of emergency surgery for acute aortic regurgitation due to necrosis and rupture of a rheumatoid granuloma: the anatomopathological lesions observed were patholognomic.

This cooperative study recensed 89 cases of patients operated for aortic regurgitation in whom the dystrophic process was confirmed on anatomical (thin, translucent valves without symphysis, multilation or sclerosis) and histological criteria (mucinous infiltration). They included 81 men (91%) and the average age was 52 +/- 14 years. The valvular degeneration was part of a generalised dystrophy of elastic tissue in 8 patients (6 "formes frustes" of Marfan's syndrome, 2 Lobstein's syndrome). The patients were divided into 2 groups according to the diameter of the ascending aorta measured by echocardiography and/or aortography. In Group 1 (n = 40), the aorta was not dilated (diameter less than 40 mm) whereas in Group 2 (n = 49), the diameter of the ascending aorta was dilated (40-55 mm) but not aneurysmal (loss of parallelism of the aortic walls). The two groups were comparable before surgery with respect to age, sex, functional class, degree of left ventricular dilatation, left ventricular ejection fraction and presence of associated coronary disease). There were no differences in the operative parameters but aortic parietal biopsy (n = 35) revealed clear signs of cystic medianecrosis more often in Group 2 than in Group 1 (14/25 versus 1/10, p less than 0.05). No operative procedure was performed on the ascending aorta during aortic valve replacement. One operative death occurred in each group. After an average follow-up of 4 years, there was a higher mortality in Group 2: the actuarial 7 year survival rate being 74% in Group 1 and 54% in Group 2. This was explained by a higher incidence of ascending aortic complications. Only 1 patient in Group 1 developed an aneurysm of the ascending aorta requiring reoperation compared to 14 patients in Group 2, 8 of whom were reoperated (p less than 0.01). These results suggest that non-aneurysmal dilatation of the ascending aorta in patients with dystrophic aortic regurgitation is a poor prognostic factor because of the high incidence of secondary aortic parietal complications (aneurysm, dissection).

Although dystrophic aortic regurgitation is considered to be a rare condition, if aortic regurgitation due to cystic media-necrosis which usually presents with annulo-aortic ectasia and regurgitation due to dystrophic aortic valves are included, it becomes a relatively common cause of aortic regurgitation. In the authors' experience of 313 patients operated for pure chronic aortic regurgitation, approximately 30% had dystrophic lesions and this was the second most common cause of aortic regurgitation after acute rheumatic fever. The clinical presentation is variable: excluding annulo-aortic ectasia, the other features of dystrophic aortic regurgitation are less well known. Eighty-nine cases without aneurysm of the ascending thoracic aorta were recensed and analysed in a French Cooperative study. They were divided into two groups with respect to the diameter of the ascending aorta measured by echocardiography. The incidence of late postoperative complications of the ascending aorta was higher in patients with a dilated aorta. The diagnosis of dystrophic aortic regurgitation is easy in patients with an aneurysm of the ascending aorta: in other cases, transoesophageal echocardiography is very useful for evaluating the valvular lesions. Surgical treatment of pure dystrophic aortic regurgitation with an aneurysm of the ascending aorta is well established but the best management of aortic regurgitation associated with only mildly dilated aorta is debatable.

The authors studies 154 cases of degenerative aortic regurgitation which presented macroscopically with atrophic changes of the valve and sometimes with hypertrophy and calcification. Histologically, the essential abnormality was the finding of mucopolysaccharide deposits dissociating the corpus spongiosa from the corpus fibrosa. Depending on the importance of these lesions, three degrees of severity can be defined, the most extensive (84% of our patient population) appearing to be typical of the disease. In addition, mild mitral valve prolapse (5%) and medial necrosis of the aortic wall (80% of patients undergoing aortic biopsy) were observed. These morphological features are on the whole quite different to those of other aortic valve pathologies (rheumatic, endocarditis). However, the border line with other pathologies with a similar anatomopathological substratum is less clearly defined: genetic abnormalities (Marfan's syndrome, Lobstein's disease, etc...) or age-related degenerative disease. The pathogenesis is not clearly understood but could be related to regional disturbances in collagen metabolism with collagenolysis predominating.

Dystrophic aortic insufficiencies represent at present time an important etiology of operated on pure and voluminous aortic regurgitations (n = 95, i.e. 28.1% of cases followed by our working team). The valvular dysplasic lesions by which they are characterized were associated in 2 out of 3 cases with parietal lesions of the ascending aorta: annulo-ectasic disease (n = 42) or important non-aneurysmal dilatation of the ascending aorta (n = 20). However, these valvular dysplasias may also appear isolated in an aorta morphologically normal at peroperative examination (n = 33). Among the 42 patients suffering from, annulo-ectasic disease (group 1) the type of surgical correction varied with the period of operation. Valvular replacement isolated (n = 3, group 1 a) or associated with aortic supracoronary surgery (n = 17, group 1b) were the procedures before 1978. Since then, 22 patients (group 1c) had a total replacement of the ascending aorta were subjected to valvular interventions (19 replacements, 1 valvuloplasty). When performed, parietal biopsy of the aorta showed in 11 out of 15 cases signs of cystic medio-necrosis. After a delay of 53 +/- 40 months, 11 out of 20 patients of group 1a and 1b died, 5 of them from the evolution of lesions at the ascending aorta (3 ruptures and 2 dissections); one patient was reoperated 12 years after the placing of a supracoronary tube, because of the development of a voluminous aneurysm of the sinuses of Valvalva which initially were only simply dilated.(ABSTRACT TRUNCATED AT 250 WORDS)

We report a case of spontaneous incomplete rupture of the first segment of the ascending aorta presenting as aortic incompetence and acute tamponade confirmed by preoperative angiography in a 57 year old hypertensive woman. This patient underwent emergency conservative surgery with good results at 8 months' follow-up. This rare pathology occurs in the same terrain as dissection of the aorta. The diagnosis should be suspected not only when chest pain and/or aortic incompetence are associated or not with acute tamponade, contrasting with a normal electrocardiogram, but also in atypical presentations which necessitate angiography in multiple incidences in order not to miss the diagnostic signs which are often invisible in the standard projections. When there are no complications, this condition may pass undiagnosed. However, in most cases, it leads to acute tamponade due to intrapericardial rupture or to an aortic aneurysm or aortic incompetence. The latter complications are usually associated with severe regurgitation requiring surgical correction, which in some cases may be conservative.

The natural history of an incomplete spontaneous rupture of the ascending aorta over a 4-year period is reported. The initial presentation was subacute pericarditis which regressed spontaneously. Aortic regurgitation developed on the 100th day of the disease and was perfectly well tolerated haemodynamically for 4 years. This favourable course was interrupted by type I aortic dissection. The post-mortem examination provided an explanation for the peculiar development and course of the aortic regurgitation: it was due not to the usual prolapse of the cups but to displacement of the left posterior commissure by the retractile fibrosis arising from the lower lip of the spontaneous rupture.

A patient with disseminated lupus erythematosus developed mitral and aortic valve disease, requiring double valve replacement. The ultra-structure of the lesions of the endo- and myocardium are described, and their significance discussed.

The author report the cases of three women with aortic regurgitation associated with aneurysmal dilatation of the ascending thoracic aorta. Aortic valve replacement was carried out in the 3 cases, with resection of the aortic aneurysm in 2 cases. Histological examination of the aortic wall in all 3 cases showed non-specific aortitis in the adventitia and media, appearances comparable to those described in Takayasu's disease. The incidence of aortic regurgitation in Takayasu's disease is about 10%. These 3 cases are compared with 30 other cases with histological confirmation in the medical literature, only 9 of which have undergone aortic valve replacement. The mechanism of the aortic regurgitation is analysed: dilatation of the aortic ring, inflammation of the valve cusps, commissural dysjunction or dilatation of the ascending aorta involving the line of commissural insertion. Aortic regurgitation is usually associated with other arterial localisations of the disease, but may also be found alone when the disease seems limited to the ascending aorta. Coronary artery disease, especially ostial, is observed in 30% of patients with this type of aortic regurgitation. Coronary angiography is therefore mandatory before surgery. The arterial involvement may be investigated initially and followed-up by 2 non-invasive investigations = digitalised intravenous angiography (DIVA) and M mode and 2D echocardiography. The two investigations give comparable results with regards to the study of the aorta, but the branches of the aorta and pulmonary arteries can only be investigated by DIVA.(ABSTRACT TRUNCATED AT 250 WORDS)

Unlabelled: The long term outcome of patients undergoing aortic valve replacement (AVR) for chronic aortic regurgitation (AR) is mainly determined by the reversibility or permanence of left ventricular dysfunction. We analysed the echocardiogram of 49 patients before and after surgery to identify the patients whose left ventricular dysfunction regressed completely after AVR. The patients were divided into 2 groups according to the results of the last postoperative echocardiogram: Group I: 25 patients whose left ventricular dimensions and wall motion reverted to normal; Group II: 24 patients with dilated and/or hypokinetic left ventricles. The two groups of patients were comparable for sex (Group I: 19 men, 6 women; Group II: 20 men, 4 women), age (Group I: 50,8 years, Group II: 53,9 years) and length of postoperative follow-up (Group I: 32 months, Group II: 34 months). The following parameters were measured and compared: diastolic and systolic left ventricular dimensions, myocardial mass and ventricular wall motion., Results: Patients in Group I had less left ventricular dilatation than those in Group II (+35% vs +60%, p less than 0,001) and left ventricular contraction was better (FE: 62% vs 45%, p less than 0,001; %FS: 35% vs 23%, p less than 0,001). This study establishes that patients with chronic AR and % delta Dd less than 60%, an EF greater than 50% or %FS greater than 25%, have about a 90% probability of normalisation of LV function after AVR. If one of the indices exceeds these threshold values, the probability of permanent LV dilatation and/or hypokinesia after AVR is also about 90%.(ABSTRACT TRUNCATED AT 250 WORDS)