Abstract
A 27-year-old man had an incidental finding of sisto-diastolic murmur during a screening medical evaluation. Transthoracic and subsequent transesophageal echocardiography revealed a saccular enlargement of non-coronary (NC) sinus of Valsalva. The aneurysm extended into the right atrium (RA) and presented a large wall discontinuity, with continuous sisto-diastolic flow from the aorta to the right atrium. Computed tomography confirmed the findings and excluded other associated abnormalities. The patient underwent urgent surgical intervention. Intraoperatively, findings reported a floppy and cribrose saccular aneurysm sprouting from the NC sinus inside the RA. The correction turned out to be very challenging due to the close connection between the aneurysmal wall and the tricuspid valve (TV) and aortic valve (AV) annuli. The aortic NC sinus was excised and replaced with a Dacron patch. Damaging of the TV septal leaflet required repair through a consolidating suture involving the septal leaflet of TV and the corresponding annular insertion. AV replacement was also needed due to damage of the AV NC cusp. No complications occurred during the postoperative stay. Histopathology revealed severe atrophy of muscular and elastic fibres of the aortic wall, elastic fiber fragmentation, replacement fibrosis and extensive deposition of mucopolysaccharides.
Sinus of Valsalva aneurysm (SoVA) is a rare condition characterized by an enlargement of the aortic root between the aortic valve and the sinotubular junction. SoVA can be either congenital, as a consequence of weakness of the elastic lamina, or acquired, due to infective, degenerative, or traumatic conditions. A prevalence of 0.09% was described in autopsy series and males are more frequently affected. Acute rupture of SoVA requires emergent surgery because of acute life-threatening haemodynamic instability. Congenital and chronic ruptured SoVA could be asymptomatic. Nevertheless, such incidental finding requires urgent surgical correction due to the possibility of unexpected further rupture, generating massive left-right shunt. The close relationship with nearby anatomical structures is a major issue which should be taken into account. In this perspective multimodality imaging is of paramount importance, allowing for a fine surgical planning and avoidance of complications.
Imaging, surgical, and histological features of ruptured non-coronary sinus of Valsalva aneurysm. (A, B) Two-dimensional and color-Doppler transesophageal echocardiography, showing the aneurysmal enlargement of non-coronary sinus of Valsalva, and pathological color-Doppler signal between the aorta and the right atrium. (C, D) Computed tomography multiplanar reconstruction and 3D volume rendering representation of the described aneurysm with contrast medium leakage. (E) Surgical specimen of the excised aneurysm. (F) Histological sample of the aortic wall (Azan–Mallory trichrome stain), showing severe atrophy of muscular and elastic fibers and replacement fibrosis. AO, aorta; LV, left ventricle; SoVA, sinus of Valsalva aneurysm; RA, right atrium; RV, right ventricle; TV, tricuspid valve.
