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CASE REPORT
Year : 2023 | Volume
: 22
| Issue : 4 | Page : 554-556
Rare association of aneurysm of anterior mitral leaflet with bicuspid aortic valve
Vivek Mohanty, Shubham Kumar Sharma, Surender Deora
Department of Cardiology, All India Institute of Medical Science, Jodhpur, Rajasthan, India
Correspondence Address:
Vivek Mohanty
Cath Lab, 6th Floor IPD Block, All India Institute of Medical Science, Jodhpur - 342 005, Rajasthan
India
Source of Support: None, Conflict of Interest: None
CheckDOI: 10.4103/aam.aam_142_22
Abstract
Mitral valve aneurysm is defined as outpouching of the mitral valve which expands in systole and collapse in diastole. It was first reported in 1729 by Morand. Most of the cases have been described in the African population. It is rare in the Indian population. Its chief presentations are congestive heart failure, palpitations, arrhythmia, or in rare cases an embolic phenomenon. It can be diagnosed either by echocardiography or cardiac imaging (magnetic resonance imaging/computed tomography). It can either occur as an isolated defect or with other associated valvular abnormalities. Here, we present a case of a 40-year-old male who was referred for the evaluation of palpitation who on the investigation was found to have a bicuspid aortic valve with severe Aortic Regurgitation (AR) along with aneurysm in the anterior mitral leaflet causing severe mitral regurgitation. This case highlights the importance of keeping a submitral aneurysm as a differential diagnosis for mitral regurgitation and searching for other associated valvular defects which may affect the management and treatment outcomes.
Abstract in French sRésumé
L'anévrisme de la valve mitrale est défini comme un gonflement de la valve mitrale qui se dilate en systole et s'effondre en diastole. Il a été signalé pour la première fois en. 1729 par Morand. La plupart des cas ont été décrits dans la population africaine. Il est rare dans la population indienne. Ses principales présentations sont insuffisance cardiaque congestive, palpitations, arythmie, ou dans de rares cas un phénomène embolique. Elle peut être diagnostiquée soit par échocardiographie, soit imagerie cardiaque (imagerie par résonance magnétique/tomodensitométrie). Il peut se produire soit comme un défaut isolé, soit avec d'autres défauts valvulaires associés. anomalies. Nous présentons ici le cas d'un homme de 40 ans qui a été référé pour l'évaluation de palpitations et qui, à l'examen, a été s'est avéré avoir une valve aortique bicuspide avec une insuffisance aortique sévère ainsi qu'un anévrisme dans le feuillet mitral antérieur provoquant une insuffisance mitrale sévère. Ce cas met en évidence l'importance de garder un anévrysme subsectoriel comme diagnostic différentiel d'une insuffisance mitrale et de rechercher d'autres défauts valvulaires associés pouvant affecter la prise en charge et les résultats du traitement.
Mots-clés: Insuffisance aortique, valve aortique bicuspide, insuffisance mitrale, anévrisme submitral
Keywords: Aortic regurgitation, bicuspid aortic valve, mitral regurgitation, submitral aneurysm
Introduction 
Submitral aneurysm is a rare valvular anomaly. Most of the cases worldwide have been reported in Africa.[1] Very few cases have been reported in the Indian subcontinent.[2] The exact mechanism for the development of these aneurysms is not known. Proposed etiologies include congenital weakness of the fibrous annulus of the valve which leads to outpouching of the left ventricular wall.[3],[4] Mitral valve aneurysm has also been reported following aortic valve endocarditis.[5] Most of these cases involve the posterior mitral valve and anterior mitral subvalvular aneurysms are very rare.[6]
Case Report A 40-year-old male presented with chief complaints of palpitations, nocturnal angina, and intermittent dyspnea for the past 3 years. There was a past history of multiple episodes of febrile illness for which the patient had prolonged hospitalizations. On examination, blood pressure was 104/50 mm Hg in the right upper limb in the supine position and the pulse rate was 86/min. Signs of severe aortic regurgitation in the form of pulsus bisferiens, water hammer pulse, Landolfi sign, Quincke sign, Traube pistol shot over the femoral artery, and Duroziez's sign were present. The apex was in the sixth left intercostal space 2 cm outside the mid-clavicular line and hyperkinetic in character. On auscultation, S1-soft, S2-wide variable split with loud P2, and LVS3 were present. A grade 4/6 pansystolic murmur radiating toward the axilla was heard. There was also decrescendo diastolic murmur in the neoaortic area. Electrocardiogram showed normal sinus rhythm with evidence of left ventricle pressure overload in form of left ventricle hypertrophy with left ventricle strain pattern. Chest X-ray revealed cardiomegaly without evidence of pulmonary venous congestion. Transthoracic echocardiography showed submitral aneurysm in the anterior mitral leaflet [Figure 1]. Color Doppler showed severe mitral regurgitation with an eccentric jet toward left atrium [Figure 2]. Furthermore, there was the presence of bicuspid aortic valve with the fusion between the left and right coronary cusp (Type 1 bicuspid aortic valve) with severe aortic regurgitation with an eccentric jet [Figure 3]a and [Figure 3]b. Furthermore, there was the presence of false tendon in the left ventricle [Figure 4]. The patient underwent cardiac computed tomography which confirmed the presence of submitral aneurysm [Figure 5].
Figure 1: Transthoracic echocardiography parasternal long-axis view showing submitral aneurysm
Figure 2: Color Doppler showed severe MR with an eccentric jet. MR: Magnetic resonance
Figure 3: (a) Color Doppler showed severe AR with an eccentric jet. (b) Transthoracic echocardiography parasternal short-axis view showing fusion between the left and right coronary cusp (Type 1 bicuspid aortic valve)
Figure 4: Transthoracic echocardiography apical 4 chamber view showing false tendon in the left ventricle
Figure 5: Cardiac CT coronal view showing submitral aneurysm. CT: Computed tomography Discussions Submitral Aneursym (SMA) is a rare cardiac anomaly mostly seen in the black population. The presence of bicuspid aortic valves in this patient makes it an extremely rare association. The most common presentation is mitral regurgitation, but it can rarely present as life-threatening complications such as ventricular tachycardia due to compression of the left main coronary artery.[2] The presence of severe aortic regurgitation with concomitant severe mitral regurgitation can lead to further increase in left ventricular end-diastolic pressure (LVEDP) and such patients may develop acute heart failure. The presence of bicuspid aortic valve makes the patient more vulnerable to subacute bacterial endocarditis than the general population.[7] Infection may spread along the aortic mitral continuity and may predispose to the development of submitral aneurysm. Although the most common etiology is congenital, infective endocarditis has been documented as a cause of submitral aneurysm.[8] There has been no study on the association between the subaortic mitral aneurysm with bicuspid aortic valve although case reports have been reported in the literature before.[9] Patient of bicuspid aortic valve have higher association with ascending aorta aneurysm compared to general population. It could be possible certain genetic factors play a role in this and these patients can have aneurysm at other sites including submitral aneurysms. Color Doppler echocardiography is the most valuable tool to diagnose SMAs in such clinical settings. The presence of severe aortic regurgitation with concomitant severe mitral regurgitation can lead to further increased LVEDP and such patients may develop acute heart failure. In the Indian subcontinent, the most common cause of mitral regurgitation is rheumatic, so SMA, although uncommon, should always be considered an etiology of mitral regurgitation, especially in young patients. The definitive diagnosis is made by echocardiography and definitive treatment is surgical.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
References 
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