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ARCAPA – A rare coronary artery anomaly

Posted Feb 17 2010 9:05am

Coronary artery anomalies are relatively common . It can be either in it’s origin, course ,  or termination etc.

There are two major sub groups.

  1. Anomalies associated with other congenital heart diseases (Both cyanotic and acyanotic)
  2. Isolated coronary artery anomalies .

The second category  which we encounter in cath labs frequently  does not have major implications . RCA and LCA arising  away from it’s respective sinuses ,Separate origin for LCX, or conus, RCA from left sinus or a high take off of RCA are the common anomalies.

While  coronary anomalies are commonly associated  in complex congenital heart disease (TOF, DORV, TGV, etc )

Isolated  complex anomalies of coronary arteries are extremely  rare

This happens , when one coronary artery arises from pulmonary artery instead of aorta and   it becomes a fascinating disease !

The ALCAPAs  and ARCAPAs

When the LCA originates from PA it becomes a  rare cause  of  left to right shunt .it is referred to anomalous origin of LCA from PA (ALCAPA) .

The ALCAPA is many times common than the “ARCAPA”

We report a case of ARCAPA (Anomalous orgin of RCA from PA )

The unique features of ARCAPA  could be

  • Isolated ARCAPA is very rare.
  • Only a handful of  patients reported in literature
  • These children present with more of right heart failure as RV function is compromised .
  • A continuous murmur in 2nd LSCS without cyanosis gives a clue
  • Angina is rare unlike ALCAPA
  • Mitral regurgitation is uncommon as LV function is relatively intact.
  • The q waves in V5 V6 we see in ALCAPA is conspicuous by it’s absence
  • ARCAPA is often ssociated with bicuspid aortivc valve, VSD etc
  • Left to right shunting can be significant .
  • 64 slice MDCT is a great investigation in this entity
  • Surgical ostial transfer is preferred so as to restore twin coronary circulation

Image and video of the ARCAPA will be uploaded shortly

Reference

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