Coronary artery anomalies are relatively common . It can be either in it’s origin, course , or termination etc.
There are two major sub groups.
- Anomalies associated with other congenital heart diseases (Both cyanotic and acyanotic)
- 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
1 http://asianannals.ctsnetjournals.org
2 http://ats.ctsnetjournals.org
Dr.S.Venkatesan MD 