Ventricular Septal Defect (VSD) (see Ventricular Septal Defect, [[Ventricular Septal Defect]]): most commonly associated cardiac defect
Normal Anatomy of the Sinuses of Valsalva
Anatomy: 3 distinct outpouchings of the aortic wall located above, each associated with an aortic valve cusp
Physiology: first recognized by Leonardo da Vinci in the 15th century, the sinuses of Valsalva separate the aortic wall from the edges of the aortic valve leaflets during systole
Allows the aortic valve leaflets to close during diastole without the interference of surface tension
Allows aortic valve leaflets to open during systole by creating a low-pressure system via the Venturi effect
Physiology
Sinus of Valsalva Aneurysm Formation
General Mechanism of Formation: defect in aortic media, resulting in separation of the aortic media from the aortic annulus fibrosus
Congenital: most common type
Represents 0.1%-3.5% of all congenital heart defects
Likely due to muscular or elastic tissue deficiencies in the aortic wall behind the sinus of Valsalva
Acquired: due to conditions that weaken the aortic wall
Doppler Flow: continuous high-velocity unidrectional flow through fistula
Note: turbulent jet from the rupture may mask the jet from a concomitant VSD, if present (in contrast to sinus of Valsalva aneurysmal rupture, VSD usually produces a high-velocity systolic flow + low-velocity diastolic flow)
Transesophageal Echocardiogram (TEE) (see Echocardiogram)
Usually Diagnostic
“Windsock” Appearance
Doppler Flow: continuous high-velocity unidrectional flow through fistula
Note: turbulent jet from the rupture may mask the jet from a concomitant VSD, if present (in contrast to sinus of Valsalva aneurysmal rupture, VSD usually produces a high-velocity systolic flow + low-velocity diastolic flow)
Physiology: due to rupture into RV just below the tricuspid valve
Treatment
Surgical Patch Closure
Patch technique is probably the most efficacious with lower rate of fistula recurrence than primary suture closure technique
Low-Risk: operative mortality is 1.9%-11.8%
Surgical closure generally has a good long-term prognosis
Early surgical intervention is probably indicated in cases with rupture
Operative Complications
Intraoperative CHF or Cardiogenic Shock
Intraoperative Complete Heart Block
Intraoperative Tamponade
Post-Op CHF or Cardiogenic Shock
Post-Op Multi-Organ Failure
Post-Op Sepsis
Post-Op Septic Peritonitis
Recurrent, Refractory Ventricular Arrhthymias
Toxic Epidermonecrosis with DIC
Non-Invasive Clamshell Device Closure
Alternative to surgical closure
References
Ruptured aneurysms of the sinus of Valsalva. Ann Thorac Surg 1986; 42:81-5
Sinus of Valsalva aneurysms. Clin Cardiol 1990; 13:831-6.
The Four Seasons of Ruptured Sinus of Valsalva Aneurysms: Case Presentations and Review. The Heart Surgery Forum #2004-11287 (6), 2004 [Epub November 2004]
A ten-year review of ruptured sinus of Valsalva: clinico-pathological and echo-doppler features. Singapore Med J 2001; 42(10): 473-476