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
- First Degree Atrioventricular Block (First Degree Heart Block) (see First Degree Atrioventricular Block, [[First Degree Atrioventricular Block]])
- Second Degree Atrioventricular Block-Mobitz Type I (Wenckebach) (see Second Degree Atrioventricular Block-Mobitz Type I, [[Second Degree Atrioventricular Block-Mobitz Type I]])
- Second Degree Atrioventricular Block-Mobitz Type II (see Second Degree Atrioventricular Block-Mobitz Type II, [[Second Degree Atrioventricular Block-Mobitz Type II]])
- Third Degree Atrioventricular Block (Third Degree Heart Block, Complete Heart Block) (see Third Degree Atrioventricular Block, [[Third Degree Atrioventricular Block]])
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
- Atrial Fibrillation (AF) (see Atrial Fibrillation, [[Atrial Fibrillation]])
- Supraventricular Tachycardia (SVT) (see Supraventricular Tachycardia, [[Supraventricular Tachycardia]])
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