A weakness of the aortic wall that leads to focal bulging, aneurysm formation or windsock deformity.

• Male:Female 4:1
• Western population 0.14 – 0.23%
• Asian population 0.46 – 3.5%

• Congenital – Majority of cases and usually affects single sinus only.
• Acquired – Less common. Acquired defects occur secondary to conditions which affect the aortic wall leading to degeneration of the elastic and connective tissue elements. This often results in diffuse dilatation of all 3 sinuses which contrasts to the single isolated sinus lesion seen among congenital patients. Associated conditions include:
  • Syphilis
  • Trauma
  • Endocarditis
  • Atherosclerosis
  • Marfans (diffuse dilatation)
  • Uncommon – Ehlers Danlos (other connective tissue disorders), Takayasu arteritis (other vasculitides), post AV replacement

• Congenital SOVA results from a developmental defect of the bulbar cordis causing a discontinuity and lack of elastin between the media of the aortic sinus and hinge line of the aortic annulus.
• Acquired SOVA occurs due to aortic wall degeneration of elastin and connective tissue elements.
• In both conditions, prolonged exposure to high aortic pressures leads to aneurysm formation.

• Right sinus 65 – 85% (fig. 1a and 1b)
• Noncoronary sinus 10 – 30% (fig. 2a, 2b, 2c)
• Left sinus < 5%

• VSD (30-60%) - supracristal (majority) or perimembranous
• Bicuspid AV (15-20%)
• Aortic insufficiency (40-50%) from cusp prolapse (usually RCC with VSD)
• Other - Pulmonary stenosis (usually subvalvular), coarctation of the aorta, PDA, ASD

Asian series demonstrate a higher incidence of right SOVA which more commonly rupture into the RV, higher incidence of VSD and lower incidence of bicuspid AV compared to Western populations.

• Unruptured - Largely asymptomatic. Occasionally present with arrhythmias (CHB, AF, VT) from compression of atrioventricular node (AVN), IVS and bundle of His or myocardial ischemia from obstruction of coronary blood flow (CBF).
• Ruptured - There are 3 patient groups defined based on size of ruptured orifice, rapidity of rupture and which chamber the rupture has occurred into
  • 35% acute symptoms (dyspnea, chest pain)
  • 45% gradual dyspnea
  • 20% asymptomatic and diagnosed with a new murmur

• Rupture (fig. 3a, 3b, 3c)
• Endocarditis
• Unruptured large or complex SOVA – Controversial. Generally, repair recommended if:
  • Complications of VSD, AI, RVOT obstruction, MR, arrhythmias and infection
  • Large SOVA which have high potential for late complications of myocardial ischemia and rupture
• Unruptured, small and asymptomatic SOVA – No clear guidelines but serial echo evaluation advisable

• For congenital SOVA, windsock excision, closure of SOVA defect, and if needed VSD patch repair, closure of fistula and aortic valve repair.
• Aortic valve replacement maybe required in patients with calcified bicuspid AV or moderate-severe AI causing cusp retraction, thickening and calcification.
• Root replacement or Ross procedure maybe used if severe local septic etiology.

• Rupture into RV > RA > LV > IVS/PA leads to formation intracardiac fistulae or tamponade. Congenital SOVA most commonly rupture between 15 – 30 years either spontaneously or secondary to strenuous physical activity, trauma or endocarditis.
• Myocardial ischemia from decreasing CBF or coronary thrombosis
• Endocarditis
• Sudden death from arrhythmia or left main coronary artery compression

10 year survival post-surgical repair 94%

• Fig. 1a Colour compare midesophageal AV LAX view depicting rupture of a right Sinus of Valsalva aneurysm into the right ventricle with an associated subaortic ventricular septal defect.
• Fig. 1b Colour compare midesophageal AV SAX view of a rightSinus of Valsalva aneurysm showing flow into the right ventricle.
• Fig. 2a Colour compare 2D midesophageal AV SAX depicting rupture of non-coronary Sinus of Valsalva aneurysm into the right atrium with unidirectional turbulent flow.
• Fig. 2b 3D full volume of the AV SAX displaying a non-coronary Sinus of Valsalva aneurysm. Drop out at tip of windsock is an echo feature consistent with rupture.
• Fig. 2c 3D colour Doppler demonstrating a ruptured non-coronary SOVA windsock to right atrial fistula with unidirectional turbulent flow.
• Fig. 3a Non-Coronary sinus – Right atrial fistula pulled inside-out into the aorta.
• Fig. 3b Ruptured non-coronary Sinus of Valsalva aneurysm. Arrows depict direction of blood flow through the defect between the aorta and right atrium.
• Fig. 3c Excision of fistula and aneurysm with pericardial patching of the non-coronary sinus. Non-Coronary Cusp (black arrow). Non-Coronary Sinus bovine pericardial patch (white arrow).
• Fig. 4 Colour compare 2D midesophageal 5Ch demonstrating the midesophageal five chamber view of a non-coronary SOVA.
• Fig. 5a 2D midesophageal AV SAX demonstrating a windsock deformity of non-coronary SOVA.
• Fig. 5b Full volume 3D demonstrating a windsock deformity of non-coronary SOVA.
• Fig. 6a 2D midesophageal AV SAX demonstrating a ruptured left Sinus of Valsalva aneurysm with surrounding thrombus.
• Fig. 6b 2D colour midesophageal AV SAX demonstrating a ruptured left Sinus of Valsalva aneurysm with surrounding thrombus.
• Fig. 7a 2D midesophageal 4Ch demonstrating a moderately dilated right ventricle.
• Fig. 7b 3D midesophageal 4Ch demonstrating a moderately dilated right ventricle.

• ME AV SAX (fig. 1b, 2a, 2b)
• ME AV LAX (fig. 1a)
• ME 5 Ch (fig. 4)
• ME RV in/outflow
• Deep TG LAX

• Sinus dilatation
• Single (congenital) or mutiple (acquired) sinus involvement
• SOVA location + measure size
• Identify cardiac chamber which SOVA expands into
• Identify presence of windsock deformity of the SOVA which expands during systole (fig. 5a, 5b)
• Echo dropout at tip of the windsock may indicate rupture⁷(fig. 2b)
• Sinus thrombus (fig. 6a, 6b)
• RV/LV volume overload /dilatation (fig. 7a, 7b)
• RV/LV systolic function

• Presence of ruptured versus unruptured SOVA
• Flow is typically turbulent or laminar in thrombosed and low flow state
• Identify location of rupture and fistula formation
• Determine shunt direction

• Determine peak/mean pressure gradients across intracardiac fistulae
• Determine the timing and duration of fistula flow with reference to the ECG
• Aortic-intracardiac fistula demonstrates continuous systolic + diastolic high velocity unidirectional flow
• VSD demonstrates high velocity systolic + low velocity diastolic flow
• If both conditions are present, continuous high velocity aortic-intracardiac fistula flow may mask the VSD Doppler spectrum⁷

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• ASD
• PDA
• Coarctation of the aorta
• Gerbode effect (shunt LV to RA)