Aortitis in ankylosing spondylitis: Early detection of aortic root abnormalities with two dimensional echocardiography

Mild aortic root dilatation, cusp thickening and subvalvular fibrous ridges have been reported as characteristic in patients with ankylosing spondylitis and aortic regurgitation. Thirty-five patients with ankylosing Spondylitis (10 also had Reiter's syndrome) without clinically apparent cardiac involvement were studied using phased array two dimensional and sector-directed M mode echocardiography to determine the prevalence of aortic abnormalities. Aortic root dimensions were measured at the aortic anulus, at the tip of the cusps and 0.5 to 1.5 cm above the cusps. The two dimensional echocardiographic study was also analyzed for qualitative abnormalities. The dimensions were compared with those in 20 normal men and among patient subgroups separated according to age, duration and severity of ankylosing spondylitis and presence of qualitative abnormalities. With one exception, no abnormally increased aortic dimensions suggestive of aortic dilatation were found in any group. However, two patients had aortic dimensions greater than 4.2 cm at the valve (normal 4.0 cm or less). Also, six patients had discrete areas of increased bright echoes below the left or noncoronary cusps suggestive of a subaortic “bump” and two of the six patients had increased aortic cusp echoes suggestive of thickening or fibrosis, or both. These changes tended to occur more commonly in older patients and those with more severe disease. It is concluded that aortic root changes suggestive of inflammation or fibrosis, or both, occur in asymptomatic patients with ankylosing spondylitis and are detectable on two dimensional echocardiography. Dilatation usually does not occur without aortic regurgitation.     

Spondyloarthropathy-associated aortitis and massive thickening of the aortic-mitral curtain: diagnosis by echocardiography

Cardiac involvement in reactive arthritis is well-recognized, and usually results in aortic regurgitation, proximal aortitis, and conduction system abnormalities. Aortitis is usually recognized in conjunction with aortic regurgitation, but can be diagnosed in isolation as aortic root thickening and subaortic fibrous ridging. We report a case of spondyloarthropathy-associated aortitis diagnosed by transesophageal echocardiography. The case illustrates the aortic root pathology and highlights the unique morphologic echocardiographic feature of this condition, prominent thickening of the aortic-mitral curtain.     

Combined mitral and aortic regurgitation in ankylosing spondylitis: Angiographic and anatomic features

Clinical and cardiac morphologic features are described in a man with combined aortic and mitral regurgitation associated with ankylosing spondylitis. Although aortic regurgitation is a recognized accompaniment of ankylosing spondylitis, the occurrence of hemodynamically-significant mitral regurgitation in this arthritic condition has not been documented previously. Histologic study disclosed changes in the anterior mitral leaflet identical to those observed in the wall of the aorta and base of the aortic valve cusps in patients with ankylosing spondylitis. Thus, ankylosing spondylitis may be associated with characteristic lesions in anterior mitral leaflet in addition to those in the ascending aorta and aortic valve. The subaortic bump at the base of the anterior mitral leaflet, the most characteristic cardiovascular lesion of ankylosing spondylitis, may be visualized during life by left ventricular angiography, and its identification allows proper etiologic diagnosis of the valvular regurgitation.     

Aortic root disease and valve disease associated with ankylosing spondylitis

Objectives. This study sought to determine the prevalence, characteristics, relation to clinical features and evolution of aortic root disease and valve disease associated with ankylosing spondylitis (AKS).Background. Aortic root disease and valve disease are common in patients with AKS, but their clinical and prognostic implications have not been well defined.Methods. Forty-four outpatients with AKS and 30 age- and gender-matched healthy volunteers underwent initial transesophageal echocardiography and rheumatologic evaluations. Twenty-five patients underwent clinical and echocardiographic follow-up 39 ± 10 months later.Results. Aortic root disease and valve disease were common in patients (82%) as compared with controls (27%; p < 0.001). Aortic root thickening, increased stiffness and dilatation were seen in 61%, 61% and 25% of patients, respectively. Valve thickening (41% for the aortic and 34% for the mitral valve) manifested predominantly (74%) as nodularities of the aortic cusps and basal thickening of the anterior mitral leaflet, forming the characteristic subaortic bump. Valve regurgitation was seen in almost half of patients, and 40% had moderate lesions. Except for the duration of AKS, aortic root disease and valve disease were unrelated to the activity, severity or therapy of AKS. During follow-up of 25 patients, in up to 24% new aortic root or valve abnormalities developed, in 12% existing valve regurgitation worsened significantly and in 20% abnormalities resolved. Twenty percent of patients developed heart failure, underwent valve replacement, had a stroke or died, as compared with 3% of control subjects.Conclusions. Aortic root disease and valve disease are common in patients with AKS, are unrelated to clinical features of AKS, can resolve or progress over time and are associated with clinically important cardiovascular morbidity.     

Echocardiographic features of mitral regurgitation due to ankylosing spondylitis

A report of echocardiographic features of an extension of "subaortic bump" producing mitral regurgitation in a patient with ankylosing spondylitis and left-sided bivalvular regurgitation is described for the first time. The anatomic and echocardiographic features of "subaortic bump," specifically found in patients with ankylosing spondylitis, have been previously reported. A review of the literature on ankylosing spondylitis and mitral regurgitation is discussed.     

Cardiac involvement in patients with primary antiphospholipid syndrome

To evaluate cardiac involvement in primary antiphospholipid syndrome, two-dimensional and Doppler echocardiographic studies were performed in 34 consecutive patients with this syndrome. All patients had an increased level of serum anticardiolipin antibodies with no evidence of malignancy or systemic lupus erythematosus. The clinical manifestations of primary antiphospholipid syndrome were arterial thrombosis in 14 patients, venous thrombosis in 6 and recurrent fetal loss in 14. Valvular lesions were observed on two-dimensional echocardiography in 11 patients (32%) (9 women and 2 men), aged 24 to 57 years (mean +/- 1 SD 36 +/- 10). Abnormal echocardiographic findings were observed in 9 (64%) of 14 patients with arterial thrombosis versus 1 (17%) of 6 patients with venous thrombosis and 1 (7%) of 14 patients with recurrent fetal loss. The most common echocardiographic abnormality was mitral leaflet thickening, found in five patients; this was associated with mitral regurgitation in three and with combined mild mitral stenosis and regurgitation in one patient. Localized subvalvular mitral thickening was observed in one patient and calcification of the anulus in another. Aortic valve thickening was observed in two patients, one of whom also had a moderate degree of aortic regurgitation. Vegetation-like lesions on the mitral or aortic valve were found in two patients. It is concluded that valvular lesions are commonly found in primary antiphospholipid syndrome, particularly when the syndrome is manifested by peripheral arterial thrombosis. The location and appearance of valvular lesions in this syndrome are heterogeneous. Most patients have no clinically significant valvular disease. Two-dimensional and Doppler echocardiographic studies are often informative in these patients.     

Yersinia-Related Arthritis in the Pacific Northwest

Serologic evidence of Yersinia enterocolitica infection was sought by agglutination testing in serum samples from several populations, including Haida Indians, Red Cross blood donors, and Caucasian patients with rheumatoid arthritis, ankylosing spondylitis, and Reiter's syndrome. No evidence was found to indicate that yersinial infection was etiologically related to Haida spondylitis or Reiter's syndrome. Four of 28 patients with acute arthritis were diagnosed from serologic evidence as having Yersinia-related arthritis.     

Echocardiographic abnormalities in ankylosing spondylitis.

Twenty four patients with ankylosing spondylitis of 10 or more years' duration were assessed for evidence of cardiac disease. Seven patients (29%) had evidence of cardiac disease, including one patient with a pericardial effusion, three with conduction abnormalities, and two with aortic incompetence. Aortic incompetence in one patient was clinically silent and was detected only with Doppler echocardiography. This patient had, in addition, thickening of the posterior aortic wall, an echocardiographic feature not previously described in ankylosing spondylitis. There was no evidence of aortic valve disease in a control group matched for age and sex. Patients with ankylosing spondylitis and cardiac abnormalities were older, had a longer disease duration, and more peripheral joint disease than those without cardiac abnormalities. Doppler echocardiography is a useful technique in the assessment of cardiac disease in ankylosing spondylitis and may detect aortic valve disease at an early preclinical stage.     

Severe Mitral and Aortic Regurgitation in Association with Ankylosing Spondylitis

Aortic disease and aortic valve regurgitation are well documented in association with ankylosing spondylitis, although involvement of the mitral valve occurs more rarely. We report a case of severe mitral and aortic regurgitation in association with ankylosing spondylitis. We then discuss the characteristic cardiac manifestations that may occur in association with ankylosing spondylitis and the associated echocardiographic features.     

Echographic early systolic partial closure (notching) of the aortic valve in congestive cardiomyopathy

We investigated the prevalence and significance of aortic valve early systolic partial closure (notching) in congestive cardiomyopathy by reviewing clinical and M-mode echocardiographic findings in 33 patients. We also compared their echocardiographic aortic root and valve findings to those in 17 aortic regurgitation patients and 24 normal subjects. Thirteen cardiomyopathy patients (39%) exhibited aortic valve partial closure--similar to the prevalence in the aortic regurgitation (41%) and normal (33%) groups. However, patients with dilated cardiomyopathy and aortic valve notching exhibited a higher mean percentage of partial closure (18% +/- 10) than those with notching in either the aortic regurgitation (8% +/- 9) or normal (5% +/- 2) group. There was no significant difference in age, body surface area, left ventricular dimension, systolic function, or presence of mitral regurgitation between cardiomyopathy patients with and without aortic valve notching, but the former had slightly greater aortic root dimensions and maximal aortic leaflet separation. Although the reason for this difference is unknown, a wider aortic root may result in low-pressure areas bordering the aortic flow stream during early systole, which may favor partial aortic valve closure.     

Postoperative follow-up of fibromuscular subaortic stenosis

Objectives. This study attempted to determine whether early subaortic resection at lower levels of obstruction reduces the rate of recurrence of subaortic stenosis or reduces secondary damage to the aortic valve, or both.Background. Fibromuscular subaortic stenosis is a progressive condition, and at present it is unclear whether early operation reduces the recurrence rate along with decreasing the incidence of aortic insufficiency.Methods. Thirty-seven patients with fibromuscular subaortic stenosis and no other significant cardiac abnormality who under-went open subaortic resection were evaluated. The preoperative, early and late postoperative catheterization or echocardiographic findings as well as the operative reports were reviewed. The median age at operation was 6.4 years (range 1.1 to 17.3). The entire group has been followed up postoperatively for a median of 5.2 years (range 1.1 to 11). Mean systolic gradients across the left ventricular outflow tract were used for the purpose of this study.Results. There was a significant correlation between the preoperative mean systolic gradient and the incidence of preoperative aortic regurgitation and late postoperative aortic valve thickening as well as the incidence and degree of late postoperative aortic regurgitation. Late postoperative gradient and degree of aortic regurgitation correlated significantly with the follow-up interval. Aortic regurgitation was progressive in some patients despite subaortic resection. A preoperative mean gradient >30 mm Hg provided a reasonable cutoff for the likelihood postoperatively of needing a reoperation, having a postoperative shelf, a thickened aortic valve, moderate aortic regurgitation or a gradient of > 10 mm Hg.Conclusions. Our results suggest that although early subaortic resection may not reduce the rate of recurrence of fixed subaortic stenosis, it is likely to reduce acquired damage to the aortic valve.     

Ankylosing spondylitis in association with mitral stenosis,mitral regurgitation,and aortic regurgitation: a case report and review of the literature

We report on a patient with ankylosing spondylitis in association with mitral stenosis, mitral regurgitation, and aortic regurgitation. Despite extensive search of literature, we could not find association of mitral stenosis with ankylosing spondylitis. This report is the first to describe this association. Our findings are based on clinical and echocardiographic findings.     

Lone aortic regurgitation, sacroiliitis, and HLA B27. Case history and frequency of association.

An account of aortic regurgitation complicating ankylosing spondylitis is given. Twenty patients with lone aortic regurgitation and without overt spondylitis were examined clinically and radiologically and tissue typed. No evidence of sacroiliitis could be found in any patient. HLA B27 was absent from this group, and no significant disturbance in antigen frequency was noted.     

Lone aortic regurgitation, sacroiliitis, and HLA B27. Case history and frequency of association.

An account of aortic regurgitation complicating ankylosing spondylitis is given. Twenty patients with lone aortic regurgitation and without overt spondylitis were examined clinically and radiologically and tissue typed. No evidence of sacroiliitis could be found in any patient. HLA B27 was absent from this group, and no significant disturbance in antigen frequency was noted.     

A Common Color Flow Doppler Finding in the Mitral Regurgitation of Acute Rheumatic Fever

Between October 1986 and May 1991, 19 patients with acute rheumatic fever who were hospitalized in the Department of Pediatrics at Soroka Medical Center underwent echocardiographic color flow Doppler examination. Five patients had an echocardiographic picture of mitral valve prolapse, with or without leaflet thickening, and one had a flail anterior leaflet. Six had mitral valve thickening without prolapse, and seven patients had an echocardiographically normal appearing mitral valve. All 19 patients had a posterolaterally directed jet of mitral regurgitation demonstrated on color flow Doppler. All patients had echocardiographically normal aortic valves. However, mild aortic regurgitation was noted in eight of them. The posterolateral jet of mitral insufficiency in acute rheumatic fever may be the most common echo-Doppler finding in this condition. (ECHOCARDIOGRAPHY, Volume 8, November 1991)
Summary and Conclusions     

IgA nephropathy associated with seronegative spondylarthropathies

Three patients with a seronegative spondylarthropathy were found to have IgA nephropathy. Two patients had ankylosing spondylitis (one with psoriasis), and one had incomplete Reiter's syndrome. All three had a focal proliferative glomerulonephritis with IgA-dominant mesangial immune deposits. One patient had a leukocytoclastic vasculitis. This association of IgA nephropathy with seronegative spondylarthropathies raises the possibility of a common or related pathogenesis. There is evidence to suggest that both diseases are mediated by genetically controlled immune responses to mucosal contact with etiologic antigens.     

Echocardiographic manifestations of discrete subaortic stenosis

The echocardiographic manifestations of discrete membranous subaortic stenosis are described in three cases. The diagnosis was confirmed by cardiac catheterization in all and at operation in two. The characteristic finding in these patients was abnormal movement of the aortic valve leaflets. The leaflets opened rapidly with the onset of systole, then exhibited abrupt premature closure shortly after ventricular ejection. The valve remained partially closed throughout the remainder of systole. This premature leaflet closure Is believed to be caused by obstruction to aortic valve flow produced by the band-like fibrous subaortic tissue. The valve leaflets also exhibited a gross fluttering motion possibly caused by a jet stream effect of the turbulent blood hitting the leaflets. In the two patients treated surgically, postoperative echograms continued to show premature leaflet closure but the degree of closure was decreased. The echocardlogram of one patient had the characteristic systolic anterior bulge of the mitral valve observed in muscular subaortic stenosis or idiopathic hypertrophic subaortic stenosis. Our data suggest that the following echocardiographic findings are suggestive if not specific for discrete subaortic stenosis: aortic valve closure early in systole, persistent valve closure throughout the remainder of systole, coarse fluttering of the leaflets, and absence of asymmetric septal hypertrophy.     

风湿性主动脉瓣病变行主动脉瓣成形术34例临床分析

目的 总结34例风湿性主动脉瓣病变行主动脉瓣成形手术的经验.方法 2005年6月至2010年9月,共34例风湿性主动脉瓣患者行主动脉瓣成形术,其中男性20例,女性14例,年龄22～70（43.1±14.8）岁.成形方法：主动脉瓣交界切开6例,交界切开并纤维增厚结节切除6例,单纯纤维增厚结节切除2例,瓣膜心包延伸术2例,瓣叶小体修复术4例,瓣叶交界折叠6例,瓣叶交界折叠并夹闭环缩术6例,瓣膜楔形切除术2例.结果 无死亡病例,并发症发生率5.9%,其中低心排1例、急性肾功能不全1例.26例无反流,8例轻度反流.结论 风湿性主动脉瓣病变掌握好成形的指征,选择恰当的成形方式,可取得良好的治疗效果.     

A search for viruses and mycoplasmas in connective tissue diseases

Taking advantage of the distinctive buoyant densities of viral and mycoplasmal nucleic acids, methods were developed for detecting a noncytolytic virus or mycoplasma in synovial cell cultures. No evidence of viral or mycoplasmal infection was found in the study of 20 cell strains derived from 13 patients with rheumatoid arthritis, 3 patients with noninflammatory joint disease, and one patient each with Reiter's syndrome, Behcet's syndrome, vasculitis with hepatitis 6 antigenemia, and ankylosing spondylitis with peripheral arthritis. Further, neither the treatment of synovial-VERO cocultured cells with 5-iodo-2'-deoxyuridine in dimethyl sulfoxide nor radiolabeling at the time of explanation permitted the detection of a virus or mycoplasma.