14-years old asymptomatic boy with holosystolic murmur heard over the lower left sternal border. Zoomed apical 5 chamber view revealed a perimembranous ventricular septal defect (VSD) which was closed by an aneurysmal leaflet of the tricuspid valve (TV) (Figure A) .Color Doppler across the defect showed left to right shunt (Figure B) Short axis view at the level of the aortic valve showed that VSD was extending from 10 to 11 o’clock . (Figure C). Injection of bubbles showed negative contrast inside the right ventricle indicating incomplete defect closure. (Figure D) 3D Transesophageal Echocardiography (TOE) with zoomed mode showed the entry point from the left ventricular side and exit point from the right ventricular side.(Figure E) En-face viewing of the TV from the right ventricular perspective showed an aneurysm-like pouch formed by the anterior leaflet (Figure F) The left atrial and ventricular size was normal and the shunt ratio was 1.3 so he was treated conservatively. Ventricular septal defect is the most common congenital anomaly.The incidence of spontaneous closure is still undetermined .Different mechanisms for anatomical closure have been proposed such as the adherence of the septal leaflet and/or the anterior leaflet of the tricuspid valve to the margins of the defect forming an aneurysm-like pouch that maybe mistaken for an aneurysm of the membranous septum. Echocardiography is the gold standard for evaluation of the site , size and type of VSD and its relation to the tricuspid valve. Recently, transcatheter closure is feasible and efficacious in properly selected patients.
57-years old man presented with exertional dyspnea. An early systolic murmur was heard over the aortic areas 2D and 3D Echocardiography revealed unicuspid , unicommissural aortic valve (UAV) with a characteristic “teardrop” lateral orifice (Figure A) and moderate valve stenosis (3D planimetered aortic valve area (AVA) is 1.1cm2) (Figure B) Continuous wave Doppler across aortic valve (AV) showed high peak and mean systolic gradients of 85 and 60mmHg respectively.(Figure C). 2D /3D Transesophageal Echocardiography (TOE) revealed a subaortic ridge attached to the posterior annulus (Arrow) (Figure D) Further En-face viewing of the aortic valve from the left ventricular outflow tract (LVOT) perspective showed a shelf-like ridge extending from the commissure to the cusp (Arrow) (Figure E) Zoomed mode of the aortic- LVOT junction confirmed the presence of the subaortic ridge seen attached to the posterior aortic annulus near the commissural opening (Figure F) The patient was referred for surgical consultation .. Unicupid aortic valve (UAV) is a rare congenital anomaly that has.2 subtypes ; unicomissural and acommissural subtypes. Both can present with variable degrees of the aortic stenosis (AS) and/or aortic valve regurgitation (AR).UAV has more early, accelerated and severe valvular degeneration in addition to smaller orifice in comparison with bicuspid and tricuspid aortic valve. Echocardiography is the gold standard for diagnosis and evaluation of the AV morphology and function and the associated disorders such as ventricular septal defect , aortopathy and subaortic obstruction.. Surgical aortic valve replacement (AVR) and repair of the associated anomalies are the most common treatment modality .