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.     

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.     

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.     

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.     

Full‐blown cardiac manifestations in ankylosing spondylitis

Ankylosing spondylitis often involves a heart, such as aortic or mitral regurgitation, conduction disorder, or cardiomyopathy. We present a 34‐year‐old male patient with ankylosing spondylitis who has severe aortic regurgitation, mild mitral stenosis, and a conduction disturbance of the left bundle branch block, identified using multimodal images.     

Two-dimensional echocardiographic detection of preclinical aortic root abnormalities in rheumatoid variant diseases

Two-dimensional echocardiographic findings of subaortic fibrous ridging, aortic leaflet thickening, and aortic root dilatation and thickening are described in a group of 36 patients with rheumatoid variant diseases. The group consisted of 25 patients with ankylosing spondylitis, nine patients with Reiter's syndrome, and two patients with inflammatory bowel disease and spondylitis. No patient had clinical or laboratory evidence of aortic regurgitation or heart block. Subaortic fibrous ridging or marked leaflet thickening was noted in 11 of 36 patients; In contrast, no such changes were found In an age-matched control group of 29 men. The subgroup of patients with subaortic fibrous ridging or leaflet thickening (11 patients) had significantly longer disease duration (28.1 versus 17.7 years) and higher Incidence of aortic root echo-density (82 versus 36 percent) than the remaining patients. It is concluded that a significant portion of patients with ankylosing spondylitis or Reiter's syndrome have echocardiographic evidence of aortic root Involvement prior to the clinical onset of aortic regurgitation.     

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.     

HLA-B27: an important genetic risk factor for lone aortic regurgitation and severe conduction system abnormalities

PURPOSE: HLA-B27, an immunogenetic marker that is present in 8 percent of the white population around the world, has been found to be an important risk factor for the development of a group of rheumatic disorders, the seronegative spondyloarthropathies. Our objective was to assess the possible role of HLA-B27 and the associated inflammatory disease process in the development of lone aortic regurgitation. PATIENTS AND METHODS: A group of 91 patients with lone aortic regurgitation were studied by HLA typing and clinical and roentgenologic examination. RESULTS: The HLA-B27-associated inflammatory disease process was found to be the probable underlying cause in 15 to 20 percent of patients with lone aortic regurgitation of different degrees of severity. Furthermore, HLA-B27 was found in 88 percent of the male patients with the combination of aortic regurgitation and severe conduction system abnormalities. CONCLUSION: We suggest that this cardiac syndrome should be regarded as an HLA-B27-associated syndrome, sometimes part of ankylosing spondylitis or Reiter's disease, but just as often presenting without obvious rheumatic disease. The marker is thus an important and widely distributed risk factor not only for the development of rheumatic disease but also for acquired aortic regurgitation and sever conduction system abnormalities.     

Clinical, haemodynamic, and coronary angiographic correlates of angina pectoris in patients with severe aortic valve disease.

Of 88 consecutive patients aged 20 to 77 years with severe symptomatic aortic valve disease requiring surgery, 51 patients had angina pectoris; of these 51, 41 had predominant aortic stenosis and 10 had severe aortic regurgitation. All patients with angina pectoris underwent coronary angiography; significant coronary arterial disease was encounted in 24 per cent of those with aortic stenosis and 20 per cent of those with aortic regurgitation. By contrast, of 37 patients without angina pectoris 19 underwent coronary arteriography; none showed significant coronary artery disease (P smaller than 0.05). Among patients with angina pectoris, 17 per cent of those with aortic stenosis experienced prolonged, rest or nocturnal pain, compared to 70 per cent of those with aortic regurgitation (P smaller than 0.005). At the time of onset of angina pectoris, there were features of heart failure in 34 per cent of those with aortic stenosis, and in 90 per cent of those with aortic regurgitation (P smaller than 0.005). Nitroglycerin promptly relieved angina pectoris in 56 percent of patients with aortic stenosis and in 50 per cent of those with aortic regurgitation (P smaller than 0.05). Neither the pattern of angina pectoris nor the response to nitroglycerin was dependent upon the coexistence of significant coronary artery disease. In patients with aortic stenosis, there was not significant difference between those with angina pectoris, and those without angina with regard to left ventricular end-diastolic volume, end-diastolic pressure, ejection fraction, peak systolic pressure, wall thickness, cardiac index, or the product of these factors. In patients with aortic regurgitation, cardiac index was significantly lower (P smaller than 0.05), left ventricular end-diastolic volume tended to be larger, and ejection fraction tended to be lower in patients with angina pectoris as opposed to those without angina pectoris.     

Cardiovascular involvement in Crohn’s disease in the absence of ankylosing spondylitis

We describe a patient who had aortic regurgitation associated with Crohn’s disease in the absence of ankylosing spondylitis. Aortitis and aortic insufficiency are fairly uncommon in Crohn’s disease. The patient required aortic valve replacement because of severely uncoated cusps secondary to inflammation of the aortic wall and aortic valve. There was a saccular formation just above the right non-coronary commissure. This sac was closed with a pericardial patch. Pledgeted sutures were used for implantation of the prosthetic valve to avoid periprosthetic leakage. The right coronary ostium had narrowed due to aortic wall thickening. A right internal thoracic artery to right coronary artery bypass was done since there was no necessity for proximal anastomosis.     

Comparison of echocardiography and angiography in determining the cause of severe aortic regurgitation.

To assess the accuracy of echocardiography in determining the cause of aortic regurgitation M mode and cross sectional echocardiography were compared with angiography in 43 patients with predominant aortic regurgitation. Each patient had all three investigations performed during the same admission to hospital. In each instance, the cause of aortic regurgitation was confirmed at surgery or necropsy. Seventeen patients had rheumatic aortic valve disease, 13 bacterial endocarditis with a perforated or partially destroyed cusp, five a bicuspid aortic valve (four with a history of endocarditis), and eight aortic regurgitation secondary to aortic root dilatation or aneurysm. Overall sensitivity of echocardiography and aortography was 84% in determining the cause of aortic regurgitation. Thus, rheumatic valve disease and endocarditis appear to be the most common causes of severe aortic regurgitation in this hospital based population. Furthermore, echocardiography is a sensitive non-invasive technique for determining the cause of aortic regurgitation and allows differentiation of valvular from root causes of aortic regurgitation.     

Pathology of aortic valve stenosis and pure aortic regurgitation a clinical morphologic assessment—part I

This two-part article examines the histologic and morphologic basis for stenotic and purely regurgitant aortic valves. Part I discusses stenotic aortic valves and Part II will discuss causes of purely regurgitant aortic valves. In over 95% of stenotic aortic valves, the etiology is one of three types: congenital (primarily bicuspid), degenerative, or rheumatic. Other rare causes of stenotic aortic valves include active infective endocarditis, homozygous type II hyperlipoproteinemia, and systemic lupus erythematosis. The causes of pure aortic regurgitation are multiple but can be separated into diseases affecting the valve (normal aorta) (infective endocarditis, congenital bicuspid, rheumatic, floppy), diseases affecting the walls of aorta (normal valve) (syphilis, Marfan's, dissection), disease affecting both aorta and valve (abnormal aorta, abnormal valve) (ankylosing spondylitis), and diseases affecting neither aorta nor valve (normal aorta, normal valve) (ventricular septal detect, systemic hypertension). Diseases affecting the aortic valve alone are the most common subgroup of conditions producing pure aortic valve regurgitation.     

Assessment of pulsed Doppler echocardiography in detection and quantification of aortic and mitral regurgitation.

Pulsed Doppler echocardiography was employed to detect disturbed or turbulent flow diagnostic of aortic or mitral regurgitation. Sensitivity, specificity, diagnostic accuracy, and predictive value were assessed by the independent interpretation and comparison of aortic root angiograms (91 patients) and left ventriculograms (94 patients) to the time interval histogram display of the pulsed Doppler. Sensitivity of Doppler in detecting mitral regurgitation was 94 per cent, with specificity 89 per cent, predictive value 81 per cent, and diagnostic accuracy 90 per cent (32 patients with, 62 without regurgitation). In aortic regurgitation, sensitivity was also 94 per cent, specificity 82 per cent, predictive value 94 per cent, and the diagnostic accuracy was 91 per cent (69 patients with, 22 without aortic regurgitation). Additionally, no Doppler evidence of mitral or aortic regurgitation was present in 20 normal subjects. The aetiology of left-sided valvular regurgitation varied widely, with prosthetic valvular insufficiency being the cause of mitral and aortic regurgitation in seven and 10 patients, respectively. Sixteen of 17 (94%) paraprosthetic leaks were correctly identified by pulsed Doppler. In patients with aortic regurgitation the flow-velocity curve recorded in the ascending aorta frequently showed a negative (or reversed) diastolic component, the magnitude of which (expressed as percentage negative area) correlated significantly with angiographic severity of regurgitation. Thus, pulsed Doppler echocardiography is a highly accurate and objective non-invasive technique for detecting mitral and aortic regurgitation. In aortic regurgitation, estimation of severity is possible from inspection of the Doppler ascending aortic flow velocity curve.     

The natural history of aortic regurgitation. A clinical and hemodynamic study

A combined prospective and retrospective analysis of clinical and hemodynamic data in 150 patients with aortic regurgitation was undertaken to review the natural history of this lesion. One hundred twenty-six patients were classified as having chronic aortic regurgitation and 24 as having acute aortic regurgitation. Serial hemodynamic studies were performed one to eight years apart in 24 patients with chronic aortic regurgitation.Clinical symptoms included dyspnea and fatigability as a consequence of reduced cardiac reserve, and chest pain and palpitations, symptoms not directly related to deteriorating cardiac function. As a rule, patients in younger age groups were free of symptoms; disability usually appeared in the fourth and fifth decades of life. Disability was poorly related to the degree of aortic regurgitation and extent of ventricular hypertrophy or cardiac enlargement. Hemodynamic abnormalities almost always preceded the development of clinical disability, but normal or near normal performance was usual in younger asymptomatic subjects despite severe degrees of aortic regurgitation and pronounced left ventricular hypertrophy.In patients with acute aortic regurgitation, findings ranged from near normal to severe cardiac decompensation requiring emergency surgical treatment.In our study (1) the protracted clinical course of chronic aortic regurgitation is confirmed: the asymptomatic state is present for decades in patients with severe aortic regurgitation even though serious hemodynamic deterioration can be documented; (2) the late appearance of clinical disability at a stage when irreversible myocardial damage may be present imposes, at present, an insoluble therapeutic dilemma with regard to the timing of surgical treatment; (3) except for the most severe, intolerable acute aortic regurgitation, there is considerable similarity between chronic and acute forms of aortic regurgitation; (4) ischemic cardiac pain is rare in aortic regurgitation, and syncope does not occur as part of this disease.     

Aortic root dilatation as a cause of isolated, severe aortic regurgitation. Prevalence, clinical and echocardiographic patterns, and relation to left ventricular hypertrophy and function

To evaluate the relation of aortic root dilatation to aortic regurgitation, we examined clinical, echocardiographic, and radionuclide cineangiographic findings in 102 patients with severe aortic regurgitation. Aortic root dilatation was the only apparent cause in 31 patients (30%), exceeding in prevalence any valvular cause, and was independently associated only with older age (p less than 0.001). Echocardiography showed dilatation to be either localized to the sinuses of Valsalva or to be generalized. At initial evaluation, patients with generalized dilatation had severer abnormalities of left ventricular size and function than those with localized or no dilatation. Aortic valves were subsequently replaced in more patients with generalized than localized dilatation during 28 +/- 17 month follow-up (9 of 15 patients compared with 2 of 15, p less than 0.03). Thus, idiopathic aortic root dilatation is the commonest definable cause of severe aortic regurgitation; aortic root dilatation is associated independently with age but not blood pressure; and generalized aortic root dilatation is associated with marked ventricular dilatation, hypertrophy, and dysfunction.     

The preoperative and operative assessment of aortic regurgitation. Cineaortography vs. electromagnetic flowmeter

Supravalvular Cineaortography, the most commonly employed procedure for the assessment of aortic regurgitation, is a semiquantitative technique. The surgeon's estimation of aortic regurgitation present at surgery may differ appreciably from the cineaortographic prediction. To evaluate the aortographic technique, a sine wave electromagnetic flowmeter was applied to the ascending aorta of 25 patients who underwent thoracotomy after Cineaortography had demonstrated aortic regurgitation graded 1+ to 4+ by conventional grading criteria. The percent of retrograde to forward aortic flow and the mean volume regurgitation (ml/diastole) was compared with the cineaortographic grade. In 5 patients with a cineaortographic grade of 1+, aortic retrograde flow ranged from 5 to 17 percent of forward flow and mean volume regurgitation from 3 to 13 ml/ diastole. Seven patients with a cineaortographic grade of 2+ had retrograde flow ranging from 8 to 75 percent with volume regurgitation ranging from 6 to 60 ml/diastole. In 12 patients with 3+ regurgitation, the retrograde flow varied from 17 to 86 percent of forward flow with a volume regurgitant range of 6 to 121 ml. The single patient with grade 4+ aortic regurgitation had a retrograde flow of 49 percent and a mean volume regurgitation of 31 ml.     

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.     

Aortic regurgitation shortens Doppler pressure half-time in mitral stenosis: clinical evidence, in vitro simulation and theoretic analysis

Mitral valve areas determined by Doppler pressure half-time were compared with areas obtained by planimetry in two groups of patients with mitral stenosis: 24 patients without aortic regurgitation and 32 patients with more than grade 1 aortic regurgitation. The severity of aortic regurgitation was assessed by color flow mapping; 17 patients had grade 2, 10 had grade 3 and 5 had grade 4 aortic regurgitation. Regression equations for pressure half-time area versus planimetry mitral valve area were calculated separately for the aortic regurgitation (r = 0.88) and the nonaortic regurgitation group (r = 0.86); analysis of covariance revealed a significant (p less than 0.001) difference between the two groups leading to overestimation of planimetry area by the pressure half-time method in the aortic regurgitation group. The mitral valve areas in the group without regurgitation were best calculated with the expression 239/T1/2 (r = 0.77) as compared with a best fit of 195/T1/2 (r = 0.85) for the aortic regurgitation group. To elucidate the mechanisms affecting pressure half-time in aortic regurgitation, an in vitro model of mitral inflow in the presence of varying regurgitant volumes and different ventricular chamber compliances was used. Aortic regurgitation shortened directly measured pressure half-time proportional to the regurgitant fraction but an increase in left ventricular compliance could offset this effect. Finally, in a mathematic model of mitral inflow the competing effects of aortic regurgitation and chamber compliance could be confirmed. In conclusion, aortic regurgitation results clinically in a significant net shortening of pressure half-time leading to mitral valve area overestimation. However, the effect is moderate and individually unpredictable because of changes in chamber compliance.     

Semiquantitation of aortic regurgitation by different Doppler echocardiographic techniques and comparison with ultrafast computed tomography.

Fourteen patients with chronic aortic regurgitation were studied by several two-dimensional and Doppler echocardiographic methods to determine the severity of aortic regurgitation. Semiquantitation of aortic regurgitation was performed by various color-flow imaging measurements, diastolic half-time of the continuous-wave regurgitation jet, and pulsed-wave velocity curve in the descending aorta. These measurements were compared with regurgitant volume and fraction by ultrafast computed tomography. All Doppler methods demonstrated a significant correlation for severity of aortic regurgitation with regurgitant fraction by ultrafast computed tomographic scanning, but scatter was present with each method. The methods with the closest correlation were at the lowest level of obtainable results. In clinical practice, all Doppler methods must be used to determine the severity of aortic regurgitation.     

Aortic valve prolapse with aortic regurgitation assessed by Doppler color-flow echocardiography.

The incidence of and the Doppler color-flow echocardiographic characteristics of aortic valve prolapse with nonrheumatic aortic regurgitation were examined. Aortic valve prolapse was observed in 21 of 243 patients (15 men and 6 women) with aortic regurgitation as detected by Doppler color-flow echocardiography (rheumatic, 112; nonrheumatic, 131) in 1247 consecutive patients. Patients with aortic valve prolapse included three patients with essential hypertension and one with annuloaortic ectasia. The remaining 17 patients (7% of those with aortic regurgitation) had no other associated cardiovascular disease (idiopathic aortic valve prolapse). Prolapse of the mitral or the tricuspid valve or both was associated with aortic valve prolapse in seven patients. Aortic regurgitation jet was markedly deviated from the axis of left ventricular outflow tract toward the anterior mitral leaflet or the interventricular septum in 17 of 21 (81%) patients with aortic valve prolapse, whereas 28 of 110 (25%) patients with nonrheumatic aortic regurgitation without prolapse and 17 of 112 (15%) patients with rheumatic aortic regurgitation without prolapse showed the deviation of regurgitant jet (p < 0.001). In conclusion, idiopathic aortic valve prolapse is one of the significant causes of aortic regurgitation, and a marked deviation of regurgitant jet is a characteristic Doppler color-flow echocardiographic finding of aortic regurgitation that results from aortic valve prolapse.