DESCRIPTION

• A sinus of Valsalva aneurysm (SVA) is a rare congenital or acquired dilatation of the coronary sinus that, when ruptured, creates a fistula between the aorta and a cardiac chamber, typically the RV. Rupture to extracardiac sites (eg, pericardial space) has been described.
• Congenital aneurysms more common than acquired aneurysms
• Right coronary sinus most commonly affected (65–85%); noncoronary sinus 20–30% of cases
• Left coronary sinus, <5% of cases

EPIDEMIOLOGY

• Prevalence in general population 0.09%
• Incidence in patients with congenital heart disease requiring surgery 0.1–3.5%
• Males > Females (3:1)
• Typically recognized at age 20–40 yr in the setting of acute rupture
• Increased incidence in Asian populations

RISK FACTORS

• Congenital heart disease
• Male gender
• Eastern/Asian populations
• Hereditary connective tissue disorders (eg, Marfan syndrome, Ehlers-Danlos syndrome)

PATHOPHYSIOLOGY

• Most unruptured aneurysms pose no hemodynamic perturbation and are asymptomatic.
• Rate of aneurysm expansion is not defined, highly variable.
• Progressive dilatation of aneurysm may lead to compression of a coronary artery and myocardial ischemia. Arrhythmias, including atrial fibrillation, ventricular tachycardia, and heart block (from extension into interventricular septum) have been described.
• Sudden rupture creates an aortocardiac fistula, leading to acute volume overload and heart failure.
• Infective endocarditis may involve the aneurysm edges or cardiac structures downstream of the fistula (following rupture).

ETIOLOGY

• Congenital: Deficient muscular and/or elastic components in aortic media result in lack of continuity between aortic media and annulus fibrosus of aortic valve:
  • May be associated with: Common supracristal ventricular septal defect (with right sinus aneurysm), atrial septal defect, bicuspid aortic valve, and aortic coarctation
  • Connective tissue diseases (eg, Marfan syndrome and Ehlers-Danlos syndrome)
• Acquired: Degeneration of elastic connective tissue in setting of infection (eg, syphilis, bacterial endocarditis, tuberculosis), inflammatory aortopathy (eg, rheumatoid heart disease), atherosclerosis, or trauma.

COMMONLY ASSOCIATED CONDITIONS

• Associated conditions present in 50–70% of cases
• Congenital heart disease:
  • Ventricular septal defect
  • Atrial septal defect
  • Coarctation of aorta
  • Aortic stenosis
  • Tetralogy of Fallot
• Connective tissue disease:
  • Marfan syndrome, Ehlers-Danlos syndrome
• Acquired diseases:
  • Aortic valve regurgitation
  • Atherosclerosis
  • Endocarditis
  • Rheumatic heart disease
  • Syphilitic aortitis

Outline

• Description
• Epidemiology
• Risk Factors
• Pathophysiology
• Etiology
• Commonly Associated Conditions

History

• Unruptured aneurysms typically asymptomatic:
  • Rarely, anginal symptoms, palpitations, fatigue, and dyspnea on exertion (due to compression or extension to nearby structures eg, coronary arteries)
• With rupture, abrupt onset of chest pain. Acute heart failure due to volume overload (eg, dyspnea, orthopnea, edema, and fatigue)

Physical Exam

• A continuous murmur may be appreciated with large unruptured aneurysm.
• Signs of a fistula (eg, bounding pulses; increased pulse pressure; continuous murmur accentuated in diastole heard best along lower left sternal border; parasternal thrill)
• Depending on downstream cardiac chamber involved, variable signs of acute and/or chronic heart failure

DIAGNOSTIC TESTS & INTERPRETATION

EKG:

• Normal (~20%)
• Sinus tachycardia with rupture
• Conduction disturbances (including bundle branch blocks)
• Ventricular hypertrophy (left and/or right)

Lab

Blood cultures if endocarditis suspected

Imaging

• CXR:
  • Cardiomegaly
  • Pulmonary edema with acute rupture
• Transthoracic echo: Initial imaging modality of choice:
  • 2D echo demonstrates aneurysm in multiple views.
  • Doppler demonstrates flow between aortic sinus and chamber into which fistula empties (eg, RV or atrium).
  • Contrast echo may demonstrate fistula.
• Transesophageal echo:
  • Enhances visualization of fistula
  • Aids in identification of associated cardiac anomalies and planning surgical approach
• Contrast enhanced helical or ultrafast CT:
  • Visualizes aneurysm
• MRI:
  • Visualizes aneurysm, regional anatomy, and associated cardiac anomalies
• Cardiac catheterization:
  • Aortography may demonstrate aneurysm or fistula.
  • Sampling of oxygen saturations reveals step up at level of fistula communication.
• Classification:
  • Type A (ruptured)
  • Type B-I (unruptured and asymptomatic)
  • Type B-II (unruptured and symptomatic)
  • Type C (extracardiac)

Follow-up & special considerations

• For unruptured aneurysm discovered incidentally (eg, during imaging for other indication), compression of nearby structures (eg, RV outflow tract, coronary arteries) should be assessed by imaging (eg, cardiac catheterization/angiography) or a functional study (ie, stress test).
• Serial imaging (every 6 mo)

DIFFERENTIAL DIAGNOSIS

Rupture can present as abrupt onset of dyspnea, chest pain, weakness, and fatigue. Other cardiovascular disorders can present similarly:

• CHF
• Aortic regurgitation
• Aortic dissection
• Coronary-venous fistula

Outline

• History
• Physical Exam
• Diagnostic Tests & Interpretation
  • Lab
  • Imaging
• Differential Diagnosis

First Line

• Unruptured aneurysm:
  • -Blockers may have beneficial effects by reducing dp/dt, slowing progression of aneurysm expansion and reducing risk of rupture. An antiarrhythmic effect may also be derived:
    • Contraindications: Allergy, acute decompensated heart failure, hypotension, significant bradycardia or high-degree AV block, acute asthma, or COPD exacerbation.
    • Precautions: Avoid abrupt withdrawal, caution in reactive airway disease.
    • Interactions: Caution when combining with other AV nodal-blocking agents (eg, diltiazem) or centrally acting 2 agonists.
  • ACE inhibitors or ARBs may reduce aneurysm expansion.
    • Contraindications: Allergy, progressive renal failure, renal artery stenosis, hypotension
    • Precautions: ACE inhibitors may cause cough; both ACE inhibitors and ARBs may cause or aggravate renal failure, increase serum potassium levels.
    • Interactions: Concomitant use with diuretics may lead to hypotension and renal failure.
• Acute ruptured aneurysms:
  • Sodium nitroprusside: Indicated for rapid afterload reduction in critically ill patients presenting in acute heart failure
    • Contraindications: Allergy, hypotension, HTN in setting of aortic coarctation
    • Precautions: Continuous BP monitoring required (arterial line) to avoid precipitous drops in BP that may lead to end organ ischemia (eg, stroke), cyanide toxicity (venous blood pH and oxygen saturation can be monitored to screen for toxicity), renal failure predisposes to thiocyanate toxicity. Use lowest possible dose for shortest duration possible.
• ACE inhibitors or ARBs should be considered for patients sufficiently stable to tolerate oral medications with evidence of CHF.
• Antibiotics for endocarditis, if present

Second Line

• Nicardipine (IV formulation as an alternative to nitroprusside)
• Nitrates plus hydralazine (IV or PO)
• Loop diuretics for pulmonary congestion (eg, furosemide)
• Inotropic support with milrinone or dobutamine
• Verapamil or diltiazem (alternative to -blockers in unruptured aneurysms)
• Aspirin may be considered for prevention of stroke in unruptured aneurysm
• Treatment of coexistent arrhythmias

Outline

• First Line
• Second Line

ADDITIONAL TREATMENT

General Measures

• Acute rupture is emergency requiring admission to ICU. Diagnostic procedures are indicated to define exact location of aneurysm, as well as associated conditions. Immediate surgical consultation:
  • Adequate oxygenation (intubation and mechanical ventilation as needed)
  • Afterload reduction with medical therapies
  • Diuresis if pulmonary congestion present
  • Inotropic support may be required if cardiogenic shock is present.
  • Antibiotics for endocarditis, if present
• For unruptured aneurysm, avoidance of strenuous physical activity. Medical therapy, described earlier, may reduce rate of aneurysm expansion and risk of acute rupture.

SURGERY

• Cardiac surgery for ruptured aneurysm
• Operative techniques include simple plication, patch repair, aortic root replacement and aortic valve replacement, and reimplantation of coronary arteries.
• Rarely, percutaneous closure devices have been successful if surgery not an option.

IN-PATIENT CONSIDERATIONS

Patient with acute ruptured sinus of Valsalva aneurysm:

• Mechanical ventilator support may be required
• IV access
• Admission to the intensive care unit
• Analgesia (morphine or other opioids)
• Consider insertion of Foley catheter
• Strict bed rest
• NPO while diagnostic workup performed and evaluation by surgeons completed
• Chronic sinus of Valsalva aneurysm does not require hospitalization.

Outline

• Additional Treatment
  • General Measures
• Surgery
• In-Patient Considerations

FOLLOW-UP RECOMMENDATIONS

• For unruptured aneurysm without symptoms, clinical follow-up every 6 mo with imaging
• Guidelines for elective surgical intervention have not been defined. Surgical repair should be considered when rapid expansion of aneurysm is documented or when compression of nearby structures is associated with symptoms or hemodynamic consequences.

DIET

Regular, unless on a special diet or NPO due to another disorder. Sodium restriction is advised with CHF.

PATIENT EDUCATION

• Bed rest for ruptured aneurysm
• Mild to moderate aerobic activity (~6–8 METS) as tolerated for unruptured aneurysm. Strenuous physical activity and exercise should be avoided.
• Following aneurysm repair, activity as tolerated.

PROGNOSIS

• Ruptured sinus of Valsalva aneurysm carries a grave prognosis unless surgically repaired.
• Repaired aneurysms carry an 85%±7.4% long-term survival rate.
• Prognosis poorer in patients with Marfan syndrome or in individuals requiring concomitant aortic valve replacement due to aortic valve insufficiency.

COMPLICATIONS

• Acute rupture
• Pulmonary edema with respiratory failure
• Circulatory collapse with shock
• Sudden cardiac death due to: Tamponade, coronary compression/ischemia, arrhythmias
• Endocarditis
• Aortic valve regurgitation

Outline

• Follow-Up Recommendations
• Diet
• Patient Education
• Prognosis
• Complications

CODES

ICD9

747.29 Other congenital anomalies of aorta

SNOMED

54160000 congenital aneurysm of sinus of Valsalva (disorder)

CLINICAL PEARLS

• Sinus of Valsalva aneurysm is a rare congenital or acquired dilatation of coronary sinus that, when ruptured, creates a fistula between the aorta, a cardiac chamber, and/or extracardiac space or structure.
• Sudden rupture usually occurs between 20 and 40 yr. Most patients complain of chest pain and have symptoms and signs of acute volume overload and heart failure. Diagnosis usually made with transthoracic echo. Other imaging modalities, including transesophageal echo, CT, or MRI may be useful in defining anatomy and planning surgical intervention.
• Sudden rupture of sinus of Valsalva aneurysm is medical emergency requiring admission to ICU and immediate surgical consultation.
• Progressive dilatation of aneurysm may lead to compression of nearby structures, including coronary arteries.
• Clinical follow-up with imaging every 6 mo is recommended for asymptomatic patients.

Outline

• Codes
  • ICD9
  • SNOMED
• Clinical Pearls

ADDITIONAL READING

• Ahimastos AA, Aggarwal A, D’Orsa KM, et al. Effect of perindopril on large artery stiffness and aortic root diameter in patients with Marfan syndrome: Arandomized controlled trial. JAMA. 2007;3298(13):1539–1547.
• Cheitlin MD, Alpert JS, Armstrong WF, et al. ACC/AHA guidelines for the clinical application of echocardiography. Circulation. 1997;95:1686–1744.
• Feigenbaum H. Diseases of the aorta. In: Feigenbaum H, ed. Echocardiography, 6th ed. Philadelphia: Williams & Wilkins, 2005:679–83.
• Feldman DM and Roman MJ. Aneurysms of The Sinuses of Valsalva. Cardiology. 2006;106(2):73–81.
• Shores J, Berger KR, Murphy EA, et al. Progression of aortic dilatation and the benefit of long-term beta-adrenergic blockade in Marfan's syndrome. N Engl J Med. 1994;330(19):1335–1341.
• Takach TJ, Reul GJ, Duncan JM, et al. Sinus of Valsalva aneurysm or fistula: Management and outcome. Ann Thorac Surg. 1999;68:1573–1577.
• Vural KM, Sener E, Tasdemir O, et al. Approach to sinus of Valsalva aneurysms: A review of 53 cases. Eur J Cardiothorac Surg. 2001;20:71–76.
• Webb GD, Smallhorn JF, Therrien J, et al. Congenital heart disease. In: Braaunwald E, Libby P, Bonow R, Mann D, Zipes D, ed. Heart disease: A textbook of cardiovascular medicine, 8th ed. Philadelphia: Elsevier Saunders, 2008:1609.

Gad A. Silberman

Nanette K. Wenger