PMC8168023_01
Transthoracic echocardiogram demonstrating right sinus of Valsalva (SoV) defect in parasternal long-axis view (Panels A and B). Subaortic area of calcification noted in the interventricular septum (asterisk, Panel B). Short-axis view demonstrates windsock deformity representing ruptured right SoV into the right ventricular outflow tract (Panel C). Color flow Doppler demonstrates left-to-right flow (Panel D). Color M-Mode through the ruptured SoV aneurysm demonstrates systolic and diastolic flow (Panel E).
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In parasternal long-axis views, a dilated right coronary SoV was noted with evidence of systolic and diastolic left to right flow by color Doppler imaging (Figure 1A, B, and D). Additionally, a calcified area was noted in the supracristal portion of the interventricular septum immediately inferior to the aortic valve and RVOT (Figure 1B).
Doppler echocardiography demonstrates flow across ruptured right sinus of Valsalva in systole and diastole (A). Flow Doppler in the descending thoracic aorta shows diastolic flow reversal (B).
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Doppler echocardiography showed peak systolic and diastolic velocities of 4.2 m/s and 3.6 m/s, respectively, across the defect (Figure 2A). Diastolic flow reversal in the descending thoracic aorta was noted (Figure 2B).
Multi-planar reconstruction of cardiac computed tomography angiography. Ruptured right sinus of Valsalva aneurysm into the right ventricular outflow tract in sagittal projection (arrow, Panel A). Membranous interventricular septal calcification (asterisk, Panel A). Quadricuspid pulmonary valve depicted in axial view (Panel B).
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CCTA confirmed normal coronary arteries, tricuspid aortic valve, and ruptured right SoV aneurysm (with windsock deformity also noted on TTE) into the RVOT just below a quadricuspid pulmonic valve (Figure 3).
Right and left heart catheterization simultaneous hemodynamic tracings demonstrate equalization of end-diastolic left and right ventricular pressures (A) and reduced diastolic gradient between the aorta and the pulmonary artery (B).
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Right heart catheterization revealed a mean pulmonary artery pressure of 24 mm Hg with equalization of left ventricular and pulmonary end-diastolic pressures, as well as an aortic to pulmonic diastolic gradient of 25 mm Hg (Figure 4).
Intraoperative transesophageal echocardiogram re-demonstrates the ruptured right sinus of Valsalva defect into the right ventricular outflow tract in 60  (A) and 120  (B). Color Doppler echocardiography demonstrates flow across the defect in 120  (C).
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Additionally, the previous finding of calcifications in the subaortic interventricular septum was noted without any evidence of color flow across the septum (Figure 5).
Intraoperative findings demonstrating ruptured sinus of Valsalva defect (A) and underlying supracristal ventricular septal defect (B).
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Intraoperative findings demonstrated severe and bulky exophytic calcifications of right aortic annulus at the right cusp hinge and at the membranous septum just below the right coronary cusp associated with a VSD (Figure 6A and B).