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← Section IV · Congenital Heart Disease
IV.G

Coronary Arteries Anomalies

7 cards

Notes

ALCAPA (anomalous LCA from PA)

  • LCA arises from the main pulmonary artery instead of the left sinus of Valsalva.
  • Infantile presentation: heart failure and ischemia as PVR falls after birth (coronary flow steals into low-resistance PA).
  • Adult presentation (rare survivors): dilated coronary collaterals from RCA; retrograde flow in LAD/LCx; ischemic mitral regurgitation from papillary dysfunction.
  • Echo clue: LCA absent from left sinus; dilated RCA and retrograde flow visible on color Doppler.
  • Treatment: reimplantation of LCA into aortic sinus.

Anomalous origin of a coronary artery from the opposite sinus

  • Anomalous LCA from right sinus - highest SCD risk when the LCA takes an interarterial course (between aorta and PA).
  • Anomalous RCA from left sinus - similar risk if interarterial course.
  • Mechanism: slit-like ostium, acute takeoff angle, and possible aortic compression during exercise.
  • SCD risk highest in young athletes.
  • CT coronary angiography or cardiac MRI to define anatomy.

Coronary AV fistula

  • Direct communication between a coronary artery and a cardiac chamber, coronary sinus, or pulmonary artery.
  • Most drain into the right heart (chamber or CS).
  • May cause a continuous murmur, high-output failure, or steal.
  • Fistulae into the coronary sinus: rare but can cause an "isolated coronary sinus IE" (associated with prosthetic devices, tunneled HD catheters).

Myocardial bridging

  • Segment of coronary artery (usually LAD) tunnels through myocardium rather than lying on the epicardium.
  • Systolic compression of the artery; usually asymptomatic but can cause angina in severe cases.

Coronary arteries in specific CHDs

  • d-TGA post-arterial switch - reimplanted coronaries at risk of ostial stenosis.
  • TOF - anomalous LAD from RCA occurs in ~5 %; important pre-op because of RVOT surgical incision.
  • Bicuspid AV - no specific coronary anomaly, but coronary ostia may be malpositioned.

Cards

  • basicIV.G-001
    What does 'ALCAPA' stand for and where does the anomalous artery originate?
    → Anomalous Left Coronary Artery from the Pulmonary Artery. The LCA originates from the main PA instead of the left sinus of Valsalva.
  • basicIV.G-002
    Typical infantile presentation of ALCAPA?
    → Heart failure and myocardial ischemia (cardiomyopathy) as pulmonary vascular resistance falls after birth. Coronary flow reverses into the low-resistance pulmonary circulation (coronary steal).
  • basicIV.G-003
    How does ALCAPA present in adult survivors?
    → Dilated collateral RCA supplying the anomalous LCA territory, with retrograde flow in the LAD and LCx. Chronic ischemia produces ischemic mitral regurgitation from papillary muscle dysfunction and LV dysfunction.
  • basicIV.G-004
    Which anomalous coronary origin carries the highest SCD risk in young athletes?
    → Anomalous coronary artery (LCA or RCA) arising from the OPPOSITE sinus of Valsalva with an INTERARTERIAL course between the aorta and the pulmonary artery. Slit-like ostium, acute angle, and aortic compression during exercise cause ischemia.
  • basicIV.G-005
    What is myocardial bridging?
    → A segment of a coronary artery (most commonly LAD) tunnels through myocardium rather than remaining on the epicardial surface. Systolic compression of the artery is seen; usually asymptomatic but can produce angina in severe cases.
  • basicIV.G-006
    Which anomalous coronary origin should be sought pre-op in tetralogy of Fallot?
    → Anomalous LAD arising from the RCA (or right sinus) crossing the RVOT. Present in ~5% of TOF; important because the RVOT surgical incision could sever the vessel.
  • basicIV.G-007
    After arterial switch operation for d-TGA, which coronary complication should you monitor for on follow-up?
    → Coronary ostial stenosis at the site of reimplantation, which may cause ischemia or SCD. Also branch PA stenosis and neo-aortic root dilation.