Sinus of Valsalva Aneurysm

Epidemiology

History

  • 1835: sinus of Valsalva aneurysm was first found on autopsy
  • 1956: first successful surgical repair of ruptured sinus of Valsalva aneurysm
  • 1974: first reported case of Echo-diagnosed ruptured sinus of Valsalva aneurysm

Geography

  • There is a 5-fold higher incidence of ruptured sinus of Valsalva aneurysms in Asian countries than in Western countries

Age

  • Age of rupture is generally younger in Asian countries than in Western countries
  • Age of rupture is distributed across age groups in Western coutries

Sex

  • There is a 4:1 male:female ratio (regardless of ethnicity)

Clinical Detection

  • Most sinus of Valsalva aneurysms are clinically undetected until rupturing

Associated Cardiovascular Lesions

  • General Comments: Eastern case series report associated VSD and AI, while Western case series report a wide variety of cardiac defects
  • Aortic Insufficiency (see Aortic Insufficiency, [[Aortic Insufficiency]]): commonly associated cardiac defect
  • Aortic Coarctation (see Aortic Coarctation, [[Aortic Coarctation]])
  • Aortic Stenosis (AS) (see Aortic Stenosis, [[Aortic Stenosis]])
  • Atrial Septal Defect (see Atrial Septal Defect, [[Atrial Septal Defect]])
  • Bicuspid Aortic Valve (see Bicuspid Aortic Valve, [[Bicuspid Aortic Valve]])
  • Left Superior Vena Cava Septal Defect
  • Left Ventricular Outflow Tract Obstruction
  • Mitral Regurgitation (MR) (see Mitral Regurgitation, [[Mitral Regurgitation]])
  • Patent Ductus Arteriosus (PDA) (see Patent Ductus Arteriosus, [[Patent Ductus Arteriosus]])
  • Patent Foramen Ovale (PFO) (see Patent Foramen Ovale, [[Patent Foramen Ovale]])
  • Pulmonic Stenosis (see Pulmonic Stenosis, [[Pulmonic Stenosis]])
  • Tetralogy of Fallot (see Tetralogy of Fallot, [[Tetralogy of Fallot]])
  • 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
    • Aortic Atherosclerosis
    • Behcet’s Disease (see Behcet’s Disease, [[Behcets Disease]])
    • Blunt/Penetrating Trauma
    • Endocarditis (see Endocarditis, [[Endocarditis]])
    • Marfan Syndrome (see Marfan Syndrome, [[Marfan Syndrome]])
    • Senile Aortic Dilation
    • Syphilis (see Syphilis, [[Syphilis]])
    • Tuberculosis (see Tuberculosis, [[Tuberculosis]])
  • Due to Cystic Medial Necrosis
    • Mucoid Degeneration and Fragementation within Aortic Wall Media

Sinus of Valsalva Aneurysm Rupture

  • Aneurysm of Either the Right or Non-Coronary Sinuses of Valsalva
    • Rupture into the Right Ventricle (RV): most common location -> creates an aortocardiac fistula with L->R shunt
    • Rupture into the Right Atrium (RA): second most common location -> creates an aortocardiac fistula with L->R shunt
    • Rupture into the Pericardial Space: rare location -> tamponade
  • Aneurysm of the Left Sinus of Valsalva
    • Rupture into the Left Ventricle (LV): less common location -> creates an aortocardiac fistula with L->L shunt

Diagnosis

Echocardiogram (see Echocardiogram)

  • May Be 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)

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)

Cardiac Catheterization with Coronary Angiogram (see Cardiac Catheterization)

  • Diagnostic

Cardiac MRI (see Cardiac MRI)

  • Diagnostic, But Not Frequently Used

Clinical Presentations

Upruptured Sinus of Valsalva Aneurysm

  • Aneurysmal Burrowing Into Intraventricular Septum
  • Aortic Insufficiency (AI) (see Aortic Insufficiency, [[Aortic Insufficiency]])
    • Physiology: due to compression and distortion of structures around aortic valve
  • Asymptomatic: 20% of sinus of Valsalva aneurysms are unruptured and found incidentally on surgery or autopsy
  • Atrioventricular Heart Blocks
  • Cardiomegaly
    • Diagnosis
      • CXR/Chest CT: enlargement of left heart border
  • Coronary Artery Ostial Thrombotic Obstruction (see Coronary Artery Disease, [[Coronary Artery Disease]])
    • Clinical: acute MI
  • Sudden Cardiac Death/Syncope (see Sudden Cardiac Death, [[Sudden Cardiac Death]] and Syncope, [[Syncope]]): may occur
    • Physiology: due to aneurysmal enlargement with LV or RV outflow tract obstruction, etc
  • Tricuspid Regurgitation (see Tricuspid Regurgitation, [[Tricuspid Regurgitation]])
    • Physiology: due to aneurysm extending into RV just below the tricuspid valve

Ruptured Sinus of Valsalva Aneurysm

  • General Features
    • Onset of Symptoms: symptom onset is gradual in >50% of cases (likely due to gradual enlargement of a small hole)
    • Severity of Symptoms: variable (based on co-existence of VSD or AI)
    • Palpable Thrill: along the right or left lower parasternal border
    • Bounding Pulses
  • Aortic Insufficiency (AI) (see Aortic Insufficiency, [[Aortic Insufficiency]])
    • Physiology: due to compression and distortion of structures around aortic valve
  • Arrhythmias
  • Congestive Heart Failure/Cardiogenic Shock (see Congestive Heart Failure, [[Congestive Heart Failure]] and Cardiogenic Shock, [[Cardiogenic Shock]])
    • Aneurysmal Rupture Into RV with L->R shunt
      • Diagnosis
        • Echo/TEE: usually diagnostic
        • Tricupid Regurgitation: may occur when rupture occurs into RV just below the tricuspid valve
        • Swan: RA -> RV step-up in SaO2
      • Clinical
        • CHF Symptoms: dyspnea, paroxysmal nocturnal dyspnea, fatigue, peripheral edema
    • Aneurysmal Rupture Into RA with L->R shunt
      • Diagnosis
        • Echo/TEE: usually diagnostic
        • Swan: venous -> RA step-up in SaO2
      • Clinical
        • CHF Symptoms: dyspnea, paroxysmal nocturnal dyspnea, fatigue, peripheral edema
    • Aneurysmal Rupture Into LV with L->L shunt
      • Diagnosis
        • Echo/TEE: usually diagnostic
      • Clinical
        • CHF Symptoms: dyspnea, paroxysmal nocturnal dyspnea, fatigue, peripheral edema
  • Coronary Artery Ostial Thrombotic Obstruction (see Coronary Artery Disease, [[Coronary Artery Disease]])
    • Clinical: acute MI
  • Endocarditis (see Endocarditis, [[Endocarditis]])
  • Hypertension (see Hypertension, [[Hypertension]])
  • Isolated Heart Murmur (see Heart Murmurs, [[Heart Murmurs]])
    • Harsh Systolic Murmur (see Heart Murmurs, [[Heart Murmurs]]): at left sternal border
    • Loud, Continuous “Machine-Type” Murmur (Accentuated in Diastole): present in 40% of patients
  • Palpitations (see Palpitations, [[Palpitations]])
  • Sudden Cardiac Death/Syncope (see Sudden Cardiac Death, [[Sudden Cardiac Death]] and Syncope, [[Syncope]]): may occur
    • Physiology: due to aneurysmal enlargement with ventricular outflow tract obstruction, etc
  • Tamponade (see Tamponade, [[Tamponade]])
    • Physiology: due to aneurysmal rupture into pericardial space
  • Tricuspid Regurgitation (see Tricuspid Regurgitation, [[Tricuspid Regurgitation]])
    • 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