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Paper text:
pseudoaneurysm of the mitral - aortic fibrosa ( pmaf ) or intervalvular fibrosa is an uncommon condition , consisting of a pulsatile cavity in the mitral - aortic junction communicating with the left ventricular outflow tract ( lvot ) .
when intervalvular fibrosa pseudoaneurysm communicates to both the left ventricle ( lv ) and left atrium ( la ) , eccentric jet flow from the anterior to posterior mitral annulus may occur during systole and mimic mitral regurgitation due to perforation in the anterior leaflet .
a 24-year - old male was referred to our emergency room with a history of acute dyspnea of 2 days duration associated with orthopnea . at admission , he had nyha class iv dyspnea .
he had no history of prolonged fever , cough , or chest pain . on physical examination ,
examination of the lungs and heart revealed extensive crackles , grade 4/6 pan systolic murmur at the apex and gallops .
no skin lesions were noted and the extremities did not show any peripheral edema . there was no clinical evidence of acute rheumatic activity or infective endocarditis .
the chest x - ray showed mild cardiomegaly with a cardiothoracic ratio of 0.55 with evidence of pulmonary edema .
the erythrocyte sedimentation rate ( esr ) was 35 mm at 1 h. the c - reactive protein ( crp ) level was 2.2 mg / dl and antistreptolysin o ( aslo ) titer was normal .
two - dimensional transthoracic echocardiogram ( tte ) demonstrated a false aneurysm - like structure in the maif at the lvot [ figure 1 , video 1 ] .
it also demonstrated the neck of pseudoaneurysm communicating with the lvot and also a defect in the aneurysmal wall communicating with the la .
color doppler examination showed a communication between the echo - free space and lvot and a turbulent flow through the defect in the aneurysmal wall into the la [ figure 2 , video 2 ] suggestive of supra - annular mitral regurgitation .
the mitral , aortic valve , and tricuspid valves were normal and there were no vegetations over mitral or aortic valves and no annular abscess .
the diagnosis of a pseudoaneurysm of the maif was confirmed using transesophageal echocardiography ( tee ) .
it demonstrated a large pseudoaneurysm of maif measuring 3.0  4.2 cm communicating with the lvot through a narrow neck [ figure 3 , video 3 ] and also rupture in its wall [ figure 4 , video 4 ] resulting in communication between the lvot and la causing a large shunt .
transthoracic echocardiography in a parasternal long - axis view showing an aneurismal sac posterior to aorta ( arrow ) transthoracic echocardiography in an apical four - chamber view showing an aneurismal sac in la with rupture ( arrow ) into la with color doppler showing a turbulent jet resulting in supra annular mr and valvular mr transesophageal echocardiography in a four - chamber view showing aneurismal sac with neck ( arrow ) communicating with the lvot transesophageal echocardiography in four chamber view showing aneurismal sac in maif with rupture ( arrow ) into la with color doppler showing a turbulent jet resulting in supra - annular mr
maif is the junction between the left half of the non - coronary cusp and the adjacent third of the left coronary cusp of the aortic valve and the anterior mitral leaflet .
pseudoaneurysm of the maf is a rare but potentially life - threatening complication of aortic valve endocarditis , aortic valve surgery , or chest trauma .
aortic valve endocarditis is usually the main reason that predisposes the maif to perforate and form a pseudoaneurysm .
both direct extension of the infection from the aortic wall and the aortic jet striking the subaortic structures and anterior mitral leaflet may damage and infect the maif .
the relatively avascular tissue of this region contributes to the extension of infection and subsequent distortion .
rupture of an maif pseudoaneurysm may give rise either to a pericardial tamponade , an eccentric jet of mitral regurgitation , or a direct communication between the lvot and the la .
in some instances , the pseudoaneurysm remains intact and progressive enlargement may compress the coronary arteries causing symptomatic coronary obstruction.[68 ] these false aneurysms are prone to rupture , embolize , or even cause further destruction of the aortic or mitral valve apparatus . when the pseudoaneurysm communicates with the ascending aorta or lv or la ; it may present as heart failure with a clinical picture similar to that of aortic or mitral regurgitation , respectively .
karalis and colleagues described 24 ( 44% ) complications involving 55 consecutive cases of aortic endocarditis , including 8 abscesses and aneurysms in interfibrosa , 7 interfibrosa perforations into the adjacent la , and 9 anterior mitral aneurysms and perforations .
however , in the present case , there was no evidence of the above - mentioned risk factors .
tee is known to be superior to tte in the visualization of valvular vegetations , abscesses , and other complications in patients with infective endocarditis .
tte may show the aneurysm located behind the aortic root by the occurrence of systolic expansion and diastolic collapse seen by echocardiography .
this should be differentiated from a typical aortic ring abscess which does not show this phenomenon .
both tte and tee can detect a pseudoaneurysm of the maif with sensitivity rates of 43% and 90% , respectively .
3d echocardiography may provide excellent three - dimensional anatomical information and also help in guiding the appropriate surgical therapy .
the recommended treatment for pmaf is surgery even in asymptomatic patients in order to prevent the development of complications .
resection and repair of the defect can be done with or without aortic valve replacement or homograft replacement of the aortic root .
surgery for this condition is technically complex since patients often have a background of prior surgery ; hence the associated morbidity and mortality are not negligible
. if the anatomy of the maif aneurysm is suitable even percutaneous device closure of the aneurysm can be an option .
to the best of our knowledge , it is rare for maif aneurysm to develop in the absence of aortic valve disease , infective endocarditis , prosthetic aortic valve , or trauma .
maif aneurysm can rupture into la and can result in large lv to left atrial shunt .
both tte and tee are quite sensitive in identifying the relationship as well as complication resulting from rupture into surrounding structures , and also help in guiding the appropriate surgical therapy .
aneurysm of the mitral - aortic intervalvular fibrosa ( maif ) is an exceptionally rare but a potentially catastrophic complication , commonly following aortic valve endocarditis . 
 we present a 24-year - old male presenting with acute onset dyspnea secondary to maif aneurysm rupturing into a left atrium causing large shunt which was diagnosed on echocardiography . 
 the maif aneurysm in the absence of infective endocarditis rupturing into left atrium is extremely rare .