congenital mitral valve anomalies are very rare clinical entities involving one or more components of the mitral valve apparatus .
the majority of these abnormalities are causing mitral valve insufficiency and commonly presenting in the early part of life .
common clinical presentations include left ventricular outflow tract obstruction , valvular insufficiency and heart failure .
we report a rare and unique case of congenital mitral valve anomaly associated with anomalous coronary artery in a middle age man who presented with stroke .
a 55-year - old male , smoker and previously healthy , presented to our emergency department for abrupt onset of difficulty reading and right sided weakness , signs and symptoms suggestive of a new onset stroke .
stat computed tomography ( ct ) and computed tomography angiogram ( cta ) of the brain revealed no abnormality .
magnetic resonance imaging of the brain showed acute infarction of the left posterior insular cortex and left parietal subcortical area .
he underwent a routine transthoracic echocardiogram ( tte ) as to rule out any possible cardiac source of embolism .
tte revealed an abnormally thickened anterior mitral leaflet associated with mild eccentric mitral regurgitation ( mr ) .
tee revealed some form of redundant tissue along the anterior mitral leaflet and the base of the anterior leaflet in the left ventricular outflow tract ( lvot ) .
it is thin and supple , the appearance was neither consistent with a fibroelastoma , nor a tumor , an abscess , a true aneurysm of the sinus of valsalva nor perimembranous septum .
the anterior mitral leaflet is itself thickened and prolapses , which might represent myxomatous disease .
a mild eccentric posteriorly directed mr jet was noted , likely originating from the mild prolapse of the anterior leaflet . at the very distal end of the anterior leaflet , there is an echo bright , independently mobile soft tissue entity , 2 - 3 mm in size .
the left circumflex coronary artery ( lcx ) appears to have both an anomalous origin ( very low in the left sinus of valsalva immediately above the annulus , in closer association to the mitral valve than usual ) .
the ostium of the right coronary artery appears larger than the left coronary artery ( fig . 1 , 2 , and 3 , supplementary movie 1 , 2 , and 3 ) .
coronary cta revealed ; anomalous lcx originating from the proximal right coronary artery off the right sinus of valsalva with a retro - aortic benign course .
the mitral valve was described as ; thickened anterior mitral valve leaflet with a band like attachment to the membranous inter - ventricular septum , the portion of the band closer to the anterior leaflet forms a thin curvilinear band ( fig . 4 and 5 ) .
this abnormal appearance could represent a congenital anomaly , myxomatous changes are still probable although may not explain the atypical attachment site .
the patient had no fever , blood cultures were negative and all other laboratory investigations were within normal limits .
the patient remained in hospital for one week and discharged later with a minor neurological deficit . in the absence of obstruction of the lvot , the patient is being followed up without surgical intervention .
most commonly , mitral valve prolapse due to myxomatous degenerative disease and connective tissue diseases , congenital mitral stenosis , accessory valvular tissue , isolated cleft mitral valve , double orifice and ebstein - like malformation were also reported .
supra - mitral ring is one of the components of shone 's complex . in addition
, there are also anomalies of the tensor apparatus i.e. , archade or hammock valve , straddling mitral valve with abnormal attachment of mitral valve chordae to both ventricles and parachute mitral valve characterized by unifocal attachment to a single or fused papillary muscle.1,2,3 ) blood cyst of the sub - valvular apparatus of the mitral valve are commonly reported as postmortem findings in infants and they regress spontaneously by age of 6 months , few cases in adults resulting in embolisation and valvular dysfunction have been reported.4 ) accessory mitral valve tissue is a rare congenital cardiac anomaly , commonly associated with other congenital heart diseases , such as ventricular septal defects and transposition of the great arteries .
prifti et al.5 ) have classified accessory mitral valve tissue as type i - fixed type ( a : nodular , b : membranous ) , type ii - mobile type ( a : pedunculated , b : leaflet - like ) .
type iib is divided into 1 ) rudimentary chordae and 2 ) developed chordae . in this case
, the appearance might fit into type iib - mobile type ( leaflet like ) .
however , they further described attachment of the mitral accessory tissue into six different locations in the left ventricle through the accessory tissue 's chordae . in our case
, the accessory tissue was attached to the interventricular septum directly , not through a chordae , confirmed by echocardiogram and ct scan . in the majority of cases of accessory mitral valve tissue
, presentations were left ventricular outflow tract obstruction , significant valvular insufficiency , palpitation and heart failure .
the lvot obstruction is the result of systolic ballooning into the lvot producing a mass effect and the continuing turbulence producing this effect , results in a fibrous tissue reaction with permanent deposits of fibrous tissue and progressive lvot obstruction.6,7,8 ) in this case , there was no lvot obstruction and the presentation was stroke .
however , infective endocarditis was excluded in this case on the basis of negative blood culture and absence of other criteria suggesting endocarditis .
we reviewed all echocardiographic features of reported mitral valve anomalies , and none have shown similar appearance to the anomalous mitral valve presented in this case .
proximal course of coronary arteries.9 ) the transesophageal echocardiogram revealed an anomalous course of the lcx , later confirmed by cta .
pathological diagnosis of this uncommon mitral valve anomaly is unavailable , which might be considered as a limitation .
however , given the clinical presentation of stroke , there is an ongoing debate , as to consider this anomaly being a definite cardiac source of embolism .
in addition , there was no lvot obstruction and the final decision was to treat the patient conservatively with regular follow - up , which was also the patient 's preference .
this case highlights an under - recognized anomaly of the mitral valve associated with anomalous coronary arteries .
we advise regular clinical and echocardiographic evaluation in this situation to identify any progression . in future
, such anomaly might be considered as potential source of embolism and surgical removal might be considered as a reasonable indication .
 a 55-year - old male presented with stroke . 
 transesophageal echocardiogram and cardiac computed tomography revealed an unrecognized congenital malformation of the anterior mitral leaflet associated with anomalous left coronary circumflex artery , arising from the right coronary artery , diagnosed first by echocardiogram . 
 this case represents a unique unforeseen mitral valve anomaly that might be considered as potential cardiac source of embolism . 
 this finding broadens the spectrum of known mitral valve anomalies . 
Introduction
Case
Discussion
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