ARCAPA – A rare coronary artery anomaly

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