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.     

Ankylosing spondylitis and heart abnormalities: do cardiac conduction disorders, valve regurgitation and diastolic dysfunction occur more often in male patients with diagnosed ankylosing spondylitis for over 15 years than in the normal population?

The objective of this study was to determine the rate of selected cardiac pathologies (conduction disorders, valve regurgitation and diastolic dysfunction) in patients with long-standing ankylosing spondylitis (AS) and compare the results with the prevalence in the normal population. A rheumatologic (structured questionnaire interview) and cardiac evaluation (resting electrocardiography and echocardiography) was performed in 100 male subjects with AS and a disease duration of more than 15 years. The rates for conduction disorders, aortic and mitral valve regurgitation and diastolic dysfunction were compared with the corresponding results in the literature among the normal population. In patients with long-standing AS there was no increased rate for valve regurgitation (mitral and aortic valve) and for arrhythmia. Diastolic dysfunction occurred more often in patients with long-standing AS. However, this might be caused by the presence of other cardiovascular risk factors such as age and hypertension. According to these results, a cardiologic evaluation with echocardiography should not be recommended routinely in patients with long-standing AS. To confirm these results, a large prospective study with patients with long-standing AS and with a matched control group should be performed in the future.     

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.     

Successful repair of a large aortic abscess causing apical displacement of the anterior mitral valve

Anatomic continuity between the anterior mitral leaflet and the aortic root may predispose those patients with aortic root pathology to functional changes of the mitral valve without any involvement of this valve. A 34-year-old man presented with aortic valve endocarditis. Transthoracic echocardiograpy showed severe aortic regurgitation with a large aortic root abscess. The anterior leaflet of the mitral valve was displaced towards the apex of the heart causing moderate mitral regurgitation. The patient underwent aortic valve replacement with reconstruction of the aortic annulus and ventriculoaortic continuity. This procedure alone restored the mitral valve structure and function without any need for intervention on the mitral valve. Aortic abscess is a serious complication of aortic valve endocarditis and may alter the function of other structures of the heart, especially the mitral valve. Restoration of aortic wall integrity and left ventricular – aortic continuity usually restores the mitral valve structure and function if the valve is unaffected by the infection. A decision on the mitral valve should be made following correction of the aortic pathology.     

HLA-B27 positive juvenile arthritis with cardiac involvement preceding sacroiliac joint changes

While cardiovascular disease develops in up to 50% of adult patients with ankylosing spondylitis, it is very uncommon in its juvenile counterpart. Regarding the early stage of the disease, before onset of sacroiliac joint changes, only two cases with aortic incompetence have been published while reports of mitral valve involvement are not available. A 13 year old boy is described with an HLA-B27 positive asymmetric oligoarthritis and enthesitis, without back pain or radiographic evidence of sacroiliitis. Echocardiography showed an echogenic structure measuring 8 × 11 × 20 mm at the fibrous continuity between the aortic and mitral valves extending through a false tendon into an echogenic thickened posterior papillary muscle, causing a grade II aortic and grade I/II mitral regurgitation. Short term corticosteroid and long term non-steroidal anti-inflammatory drug and disease modifying antirheumatic drug treatments efficiently controlled the symptoms. The cardiac findings remained unchanged during a follow up of 20 months. Careful cardiac evaluation appears to be mandatory even in these young patients.


Keywords: juvenile ankylosing spondylitis; HLA-B27; aortic valve insufficiency; mitral valve insufficiency     

Echocardiographic versus Histologic Findings in Marfan Syndrome

This retrospective study attempted to establish the prevalence of multiple-valve involvement in Marfan syndrome and to compare echocardiographic with histopathologic findings in Marfan patients undergoing valvular or aortic surgery.We reviewed echocardiograms of 73 Marfan patients who underwent cardiovascular surgery from January 2004 through October 2009. Tissue histology was available for comparison in 29 patients.Among the 73 patients, 66 underwent aortic valve replacement or the Bentall procedure. Histologic findings were available in 29 patients, all of whom had myxomatous degeneration. Of 63 patients with moderate or severe aortic regurgitation as determined by echocardiography, 4 had thickened aortic valves. The echocardiographic findings in 18 patients with mitral involvement included mitral prolapse in 15. Of 11 patients with moderate or severe mitral regurgitation as determined by echocardiography, 4 underwent mitral valve repair and 7 mitral valve replacement. Histologic findings among mitral valve replacement patients showed thickened valve tissue and myxomatous degeneration. Tricuspid involvement was seen echocardiographically in 8 patients, all of whom had tricuspid prolapse. Two patients had severe tricuspid regurgitation, and both underwent repair. Both mitral and tricuspid involvement were seen echocardiographically in 7 patients.Among the 73 patients undergoing cardiac surgery for Marfan syndrome, 66 had moderate or severe aortic regurgitation, although their valves manifested few histologic changes. Eighteen patients had mitral involvement (moderate or severe mitral regurgitation, prolapse, or both), and 8 had tricuspid involvement. Mitral valves were most frequently found to have histologic changes, but the tricuspid valve was invariably involved.     

Images in cardiovascular medicine: prosthetic aortic valve and conduit dehiscence with large periconduit cavity, ascending aortic aneurysm and severe mitral regurgitation

Prosthetic aortic valve and conduit dehiscence with periconduitcavity and ascending aortic aneurysm is an uncommon complicationof aortic root surgery. It is usually recognizable at echocardiographydue to an abnormal position of the prosthetic valve and conduitin relation to the native aortic annulus in conjunction withan abnormal echolucent periconduit space that fills with colorflow. Mitral regurgitation is an unusual complication of thiscondition. We present a patient with severe mitral regurgitation secondaryto prosthetic aortic valve and conduit dehiscence with a largepericonduit cavity and aneurysm of the intervalvular fibrosa.The mechanism of mitral regurgitation is secondary to functionalinvolvement of the anterior mitral valve leaflet and intervalvularfibrosa with anterior mitral leaflet restriction in conjunctionwith mild left ventricular remodeling. Significant mitral regurgitationpersisted post resection of the periconduit cavity and aorticvalve replacement, requiring mitral valve replacement. This case study reports a new mechanism of mitral regurgitationin the setting of prosthetic aortic valve and conduit dehiscence.     

Parachute mitral valve with severe mitral regurgitation in an adult patient

Parachute mitral valve (PMV) is rarely seen in the adult population, in isolation or in association with other congenital heart defects, since most patients may have had milder lesions previously that were asymptomatic early in life, or were not detected due to lack of a comprehensive examination. We report a case of an 18-year-old woman with a history of exertional dyspnea, atypical chest pain, and cough for about 1 year. The echocardiographic examination identified a PMV with severe mitral regurgitation associated with bicuspid aortic valve and coarctation of the aorta. Most patients present with mitral stenosis of varying degree of severity, and rarely present with severe mitral regurgitation as seen in our patient.     

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.     

False Diagnosis of Aortic Stenosis Due to Doppler Recording of Mitral Regurgitation from the Suprasternal Notch

The suprasternal notch provides an important window for estimating aortic valve gradient with a nonimaging continuous-wave transducer. Since the Doppler beam is highly angulated with respect to mitral flow, it is generally assumed that mitral regurgitation cannot simulate significant aortic stenosis in this window. We report on an 88-year-old patient with clinically suspected aortic stenosis and heavy valve calcification, in whom suprasternal notch Doppler with a nonimaging probe showed a high-velocity flow that appeared to confirm the diagnosis of aortic stenosis. Cardiac catheterization demonstrated moderate mitral regurgitation due to flail leaflet and no aortic gradient. Awareness of the potential to image mitral regurgitation from the suprasternal notch is stressed to avoid misdiagnosis. (ECHOCARDIOGRAPHY, Volume 8, January 1991)     

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.     

Sensitivity and specificity of pulsed Doppler echocardiography in detection of aortic and mitral regurgitation

In order to assess the value of pulsed Doppler echocardiographyin detection of valvular regurgitation, 63 patients were evaluatedfor aortic and/or mitral regurgitation using pulsed Dopplerechocardiography and selective cineangiography. The Dopplerstudy was considered as positive when a turbulent flow was detectedbelow the aortic valve for aortic insufficiency and behind themitral valve for mitral insufficiency on a graphic display (timeinterval histogram) when technically adequate andor on an audiosignal.These results were compared with standard angiographic evaluationof the regurgitation: pulsed Doppler echocardiography had 94%sensitivity and the specificity rate was very high (87.5%) evenfor mild regurgitation. Thus, Doppler technique is highly specificand sensitive in detection of aortic and mitral regurgitationwhen both audiosignal and time interval histogram are simultaneouslyperformed.     

Exacerbation of mitral regurgitation after tricuspid valve replacement for isolated tricuspid regurgitation

A 74-year-old woman, with a history of aortic valve replacement and open mitral commissurotomy due to rheumatic aortic and mitral stenosis, presented with dyspnea. She developed severe tricuspid regurgitation (TR), requiring tricuspid valve replacement (TVR). Despite an uneventful postoperative course, she was readmitted for dyspnea 2 months later. Trans-thoracic echocardiogram revealed severe mitral regurgitation (MR), despite mild MR at the time of TVR, which has not been previously reported. The main MR mechanism was increased left ventricular preload due to improved TR. Increased diuresis has controlled her congestive heart failure, but her MR remained moderate.     

Ruptured mitral valve aneurysm: A devastating complication of aortic valve endocarditis

Mitral valve aneurysm is a rare clinical entity that is mostly resulted from infective endocarditis, in particular, aortic valve endocarditis. Once mitral valve aneurysm ruptures and severe mitral regurgitation and hemodynamic instability develop, prompt surgery should be considered. Here we report a patient with ruptured mitral valve aneurysm associated with native aortic valve endocarditis that was improved after a successful mitral and aortic valve replacement surgery associated with antibiotic therapy for 6 weeks.     

Aortic valve sclerosis as a marker of coronary artery atherosclerosis; a multicenter study of a large population with a low prevalence of coronary artery disease

There are no studies analyzing the association between aortic valve sclerosis (AVS) and coronary artery disease (CAD) in a large and multicenter patient population with an overall low prevalence of CAD. We hypothesized that AVS could predict the presence and degree of CAD in patients with severe organic mitral regurgitation.     

“Holes-in-one”: A case of concurrent windsock mitral valve,root abscess,and Gerbode defect

Infective endocarditis is a heterogeneous disease with a wide array of pathological lesions. We present a 55-year-old man with severe mitral and aortic regurgitation on transthoracic echocardiography. Transesophageal echocardiogram characterized the mechanisms detecting a windsock mitral valve perforation, aortic root abscess, and Gerbode ventricular septal defect, with the deep transgastric view showing all three pathologies concurrently. The etiologies of mitral valve perforation and Gerbode defects are discussed. Transesophageal echocardiography remains a critical imaging modality to diagnose and evaluate the extent of infective endocarditis with superior sensitivity to transthoracic echocardiography.     

Aorto-mitral inflammation in rheumatological disease: transoesophageal echocardiographic presentation.

Thickening of the aortic root and anterior mitral leaflet has been described in patients with aortic valve insufficiency associated with ankylosing spondylitis. We report identical findings made by transthoracic and transoesophageal echocardiography in three patients with inflammatory, non-infective aorto-mitral pathology as manifestation of various rheumatological disorders. All three patients had significant aortic valve incompetence. All three had thickened walls of the sinuses of Valsalvae and a thickened anterior mitral leaflet, in one case with chordal rupture. These findings were confirmed at open heart surgery, and further characterized by histological examination.     

Mitral prosthetic inflow mimicking severe aortic regurgitation

We describe two patients in whom mitral valve prosthetic inflow mimicked severe aortic regurgitation. Repeat transthoracic echocardiography using multiple transducer angulations and a narrow color Doppler sector angle in one patient and a color Doppler M-Mode study in the second patient proved useful in excluding the presence of severe aortic regurgitation.     

MitraClip used for severe mitral regurgitation after surgery for type A aortic dissection combined with Marfan syndrome

Mitral regurgitation is a rare but catastrophic condition in patients after surgery for type A aortic dissection. The second thoracotomy to complete the mitral valve operation could be fatal. Here, we report a case of severe mitral regurgitation treated with MitraClip in a 53-year-old woman after surgery for type A aortic dissection combined with Marfan syndrome. She was discharged uneventfully, and a significant reduction of regurgitation of mitral valve and tricuspid valve was observed at the 6-month follow-up. MitraClip could be an alternative device for such high-risk patients.