Anomalous coronary arteries

Background

• Coronary arteries arise from the truncus arteriosus as endothelial buds, during this process abnormal involution, bud positioning, or septation of the truncus arteriosus may lead to abnormal origin of the coronary arteries
• Symptoms occur due to the path that these arteries take to supply the heart, some coursing between the aorta and pulmonary arteries which results in compression, or taking abrupt turns causing acute angles that are predisposed to obstruction
• Incidence is 0.64% of births

Types

• Left circumflex from right sinus of valsalva
• Single coronary artery from the left sinus of valsalva
• Both coronary arteries from the right sinus of valsalva
• LAD from the right sinus of valsalva

Clinical Features

• Anginal chest pain
• Syncope
• Sudden death
  • In one study accounted for 13% of cases of sudden death in competitive athletes

Differential Diagnosis

Syncope (peds)

• Seizure
• Breath-holding spell
• Hyperventilation syndrome
• Pregnancy (especially ectopic)
• Hypoglycemia
• Cardiac disease
  • Tet spell
  • Hypertrophic cardiomyopathy
  • Brugada Syndrome
  • Dysrhythmias
  • Long QT syndrome
  • Anomalous coronary arteries
• Toxicologic exposure (stimulants or depressant)
  • CO poisoning
  • Ethanol toxicity
  • Drugs of abuse

Chest pain (peds)

• Idiopathic (most common)
• Precordial catch syndrome
  • Sudden, intense pain with deep inspiration, self-resolving
  • Usually located to one finger point and the intercostal space (often at apex of heart)
• Musculoskeletal
  • Costochondritis, trauma
• Pulmonary
  • Pneumothorax
  • Pneumonia (peds)
  • Pulmonary embolism
  • Aortic dissection
    • Uncommon but consider with history of connective tissue disease
• GI
  • Esophagitis
  • Esophageal spasm
  • Gastritis
• Psychosomatic
  • Panic attack
  • Anxiety
• Cardiac (1%)
  • Left-sided obstructive lesions
  • Dysrhythmias
  • HOCM
  • Pericarditis
  • Prinzmetal's angina (vasospasm)
  • MI
    • Rare, even post-Kawasaki
    • Consider coronary artery dissection
  • Mitral valve prolapse
  • Anomalous coronary arteries]

Evaluation

• EKG
• CXR
• Troponin
• Echocardiography
• Noninvasive coronary magnetic resonance angiography (CMRA)
  • The best option if available
• Coronary angiography

Management

• Definitive treatment is surgery

Disposition

• Cardiology consult