• Users Online: 348
  • Home
  • Print this page
  • Email this page
Home About us Editorial board Ahead of print Current issue Search Archives Submit article Instructions Subscribe Contacts Login 

 Table of Contents  
Year : 2022  |  Volume : 8  |  Issue : 1  |  Page : 59-61

Anomalous left coronary artery from the right pulmonary artery with an intramural course

1 Sri Padmavathi Children Heart Centre, Tirupati, Andhra Pradesh, India
2 Department of Cardiothoracic and Vascular Surgery, SMS Medical College and Attached Hospitals, Jaipur, Rajasthan, India
3 Consultant Congenital and Paediatric Cardiac Surgeon, Bhanubhai and Madhuben Patel Cardiac Centre, Bhaikaka University, Karamsad, Anand, Gujrat, India
4 Department of Paediatric Cardiology, Children's Brain and Heart Institute, Andhra Hospitals, Vijayawada, Andhra Pradesh, India

Date of Submission28-Feb-2022
Date of Decision01-Apr-2022
Date of Acceptance06-Apr-2022
Date of Web Publication26-Apr-2022

Correspondence Address:
Dhruva Sharma
Department of Cardiothoracic and Vascular Surgery, SMS Medical College and Attached Hospitals, J L N Marg, Jaipur - 302 001 Rajasthan
Login to access the Email id

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jpcs.jpcs_12_22

Rights and Permissions

Anomalous left coronary artery from the right pulmonary artery (RPA) is a rare congenital coronary anomaly and is one of the surgically treatable causes of ventricular dysfunction in infants. The left coronary artery when it arises from the RPA or near its base tends to follow the intramural course. Careful echocardiographic evaluation of the course of the coronary artery is necessary under sedation to avoid missing this anomaly. Unroofing of this coronary artery and closing of the pulmonary artery origin are recommended for treatment. We report a case where the intramural course was retrocommissural and unroofing would have resulted in aortic incompetence. We describe how a 90° rotation is possible by augmenting the coronary button with an anterior pericardial hood. The reconstruction of the RPA should be done with adequate mobilization and redundancy to prevent compression and bowstringing of the reimplanted coronary artery.

Keywords: Anomalous left coronary artery from the right pulmonary artery, intramural coronary artery, retrocommissural course, severe left ventricular dysfunction in infants

How to cite this article:
Subramaniam KG, Sharma D, Bhende VV, Kudumula V, Reddy SN. Anomalous left coronary artery from the right pulmonary artery with an intramural course. J Pract Cardiovasc Sci 2022;8:59-61

How to cite this URL:
Subramaniam KG, Sharma D, Bhende VV, Kudumula V, Reddy SN. Anomalous left coronary artery from the right pulmonary artery with an intramural course. J Pract Cardiovasc Sci [serial online] 2022 [cited 2023 Mar 30];8:59-61. Available from: https://www.j-pcs.org/text.asp?2022/8/1/59/344125

  Introduction Top

The anomalous origin of the left coronary artery from the base of the right pulmonary artery (RPA) is a known anatomical variant that tends to follow an intramural course. The close association of the course of the artery to the aortic wall may create diagnostic confusion about the origin. We report a 3-month-old child with severe left ventricular (LV) dysfunction where the diagnosis was missed initially. The described surgical approach for this condition consists of laying open the intramural course on the side of the aorta and re-endothelialization and closure of the coronary ostia. This technique was not possible in our case due to the close relationship of the commissural pillars of the very hypoplastic left coronary sinus to the intramural course. Laying open the intramural course could have resulted in commissural injury and aortic regurgitation. We describe a technique where 90° rotation of the coronary button was carried out using a portion of the pulmonary wall posteriorly and a patch of autologous pericardium anteriorly to prevent kinking during rotation of the coronary button. The reconstruction of the pulmonary artery also needs attention to prevent compression of the intramural course. This can be done by good mobilization of the pulmonary arteries, using a generous patch not only posteriorly but also anteriorly, or shifting the anastomosis of the proximal pulmonary artery to the left. This would prevent bowstringing of the transferred coronary artery by the right side of the reconstructed pulmonary artery.

  Case Report Top

A 3-month-old male child weighing 2.7 kg presented with feeding difficulty, poor weight gain, irritability, and vomiting. An echocardiogram done had missed the diagnosis, as the left coronary artery seemed to arise from the aorta and antegrade flow was noticed. The child, however, had severe LV dysfunction (ejection fraction of 20%). An echocardiogram after sedation helped clinch the diagnosis as the retrograde flow could be appreciated which was entering into the base of the RPA [Figure 1a-d]. The papillary muscles were hyperechoic, and there was moderate mitral regurgitation. The child was planned for high-risk coronary reimplantation onto the aorta.
Figure 1: (a) Showing echocardiogram four-chamber view with dilated left atrium and left ventricle with bright papillary muscle due to ischemia (arrow). (b) Showing parasternal short axis view with left coronary artery arising from main pulmonary artery with intramural course (arrow) of aorta (Ao). (c) Showing parasternal short axis view with retrograde flow (red color) in LCA (arrow) and LAD (blue color) into MPA. (d) Showing parasternal short axis view with dilated RCA arising from aorta with forward flow. LA: Left atrium, LV: Left ventricle, LCA: Left coronary artery, MPA: Main pulmonary artery, RCA: Right coronary artery, Ao: Aorta, RA: Right atrium, RV: Right ventricle

Click here to view

Intraoperatively, the tiny leash of collaterals could be appreciated going across the pulmonary artery. Suspecting intramural course, complete dissection between the aorta and the pulmonary artery was not done to avoid injury inadvertent injury to the coronary artery. After mobilization of the RPA post dividing the ligamentum, cardiopulmonary bypass (CPB) was instituted with aorto-bicaval cannulation and the branch PAs were immediately snared to prevent runoff into the pulmonary circulation. Aorta was cross clamped and antegrade plegia was delivered through the aortic root. The pulmonary artery was opened anteriorly to confirm the diagnosis, which showed the opening of the coronary ostia at the base of the right side of the pulmonary artery. The patient was cooled to 26° and gentle probing of the ostia with a 2-mm arteriotomy cannula confirmed the intramural course in the aortic wall. Additional plegia was delivered through the ostia from the pulmonary artery. The aorta was opened longitudinally to identify the site for opening up the intramural course.

We were surprised to find an extremely hypoplastic left coronary sinus with almost fusion of the commissure between the noncoronary sinus, the presumed left coronary, and the right coronary sinus. The intramural course was behind this commissure and laying open its course had a risk of injury to the commissure and possible subsequent aortic regurgitation.

It was decided to reimplant the coronary artery by rotating it to the aorta. The left main ostia were harvested with a generous wall of the pulmonary artery after transection of the pulmonary artery. The longitudinal incision was extended obliquely to reach the area above the commissure. The coronary artery was reimplanted by rotating it by about 90°, the pulmonary artery wall was sutured posteriorly, and a patch of the fixed autologous pericardium was used to patch anteriorly. It was made sure that the intramural course did not kink during this process with the help of a 2-mm olive tip arteriotomy cannula. The aortotomy was closed and plegia was repeated to ensure satisfactory filling of the left main coronary artery.

The pulmonary artery reconstruction began by filling the area created by the defect with autologous glutaraldehyde-fixed pericardium. The reconstruction was done by approximating the left pulmonary artery base to the left lateral aspect of the proximal pulmonary artery and patching the right aspect anteriorly with another pericardial patch. This was done to ensure there was redundancy along the right aspect of the pulmonary artery to prevent compression of the rotated and reimplanted left coronary artery.

The child was gradually weaned off CPB with 0.1 ug/kg/min of adrenaline and 0.5 ug/kg/min of milrinone. The child was extubated on day 5 post surgery to a high flow nasal cannula, which was gradually weaned over the next week, and the child was discharged after 20 days with near-normal ejection fraction and mild mitral regurgitation.

  Discussion Top

The incidence of intramural anomalous left coronary artery from the right pulmonary artery

The incidence of the anomalous coronary artery from the RPA is about 4%–22% in different series.[1],[2] Mishra et al[1] in a single centre experience over a period of 14 years reported 105 cases of anomalous coronary artery. Coronary artery was found to be arising from the right pulmonary artery among 5 cases, 4 of these 5 cases had an anomalous course. Dr Zhang et al[2] in their report of 10 cases of intramural ALCAPA highlighted that when in the presence of a significant extramural course with a short intramural course direct reimplantation is possible. In cases that are arising more distally from RPA, the intramural course could be significant and an unroofing technique was considered the procedure of choice. Barbero-Marcial et al.[3] reported a series of four cases where the origin was from RPA and all those who underwent unroofing procedures did well and one case of reimplantation had sudden cardiac death following a prolonged postoperative recovery.

Our case was unique in that the intramural course was long and just behind two commissures of non and left coronary sinus and left and right coronary sinus. The left coronary sinus was extremely small explaining the retrocommissural course. This made unroofing technically dangerous and the very short length of the artery before it enters the aortic wall made rotation and direct reimplantation also prone to kinking.

Diagnostic consideration

Surgical considerations

Unroofing of the intramural portion has been suggested as a simple procedure once the diagnosis is made. Suspecting an intramural course preoperatively, we hoped to unroof the left coronary artery gaining access through a longitudinal incision on the aorta as previously described.[3] However, in our case, the relationship of the intramural portion to the commissure made the unroofing procedure impossible with the danger of aortic regurgitation. Hence, once we decided to reimplant, the two main considerations were as follows:

  1. The coronary should not kink during the process as the intramural portion acts like a fixed portion and rotating the coronary ostia by more than 110°–120° can result in kinking. An easily understandable analogy would be using the palm of the left hand as the coronary ostia and the forearm as the intramural portion which is fixed, the amount of rotation of the palm gives us the rotational freedom we can get while reimplanting the coronary artery
  2. The coronary artery should not be compressed by the reconstructed pulmonary artery. A distended reconstructed pulmonary artery can compress the coronary, especially if the coronary is implanted anteriorly on the aorta, to avoid this, the coronary should be implanted as posteriorly as possible. Although not highlighted, the recommendation of LeCompte maneuver[4] and the Florida patch[5] closure of the pulmonary artery defect are measured to avoid this complication.

This can be avoided by:

  1. Mobilizing the RPA as extensively as possible
  2. Making sure that there is redundancy on the right lateral aspect of the reconstructed pulmonary artery – this can be achieved by placing an anterior patch in addition to the posterior patch so that it does not cause “bowstringing” of the reimplanted coronary artery by the RPA
  3. Displacing the anastomosis to the left side by cutting into the left pulmonary artery and closing the right aspect of the distal pulmonary artery.

  Conclusion Top

Any anomalous left coronary artery arising close to the right aspect of the pulmonary artery should be suspected to have an intramural course. Dissection between the aorta and the pulmonary artery should be avoided or needs to be done extremely cautiously to prevent inadvertent injury to the intramural course.

There are a few case reports about intramural coronary artery when the left coronary artery is arising from the base of the RPA; this is the first case that highlights the presence of severely hypoplastic left coronary sinus with a retrocommissural course of the coronary artery which prevented the unroofing manoeuver. We highlight how a kink and compression-free transfer could be accomplished using autologous pericardium to reduce the amount of rotation needed. The reconstruction of the pulmonary artery requires as much attention as the coronary transfer to ensure a successful outcome.

Animal and human rights statement

All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki Declaration and its later amendments or comparable ethical standards.

Informed consent

Informed consent was obtained from the participant included in the study.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient's guardian has given consent for images and other clinical information to be reported in the journal. The patient's guardian understands that name and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.

Ethics clearance

Institutional Ethical Committee (IEC) Clearance was not required for this case report.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

  References Top

Mishra A. Surgical management of anomalous origin of coronary artery from pulmonary artery. Indian J Thorac Cardiovasc Surg 2021;37 Suppl 1:131-43.  Back to cited text no. 1
Zhang H, Cheng P, Jin G, Han D, Luo Y, Li J. Surgical strategies for anomalous origin of the left coronary artery from the right pulmonary artery with an intramural aortic course: A report of 10 cases. J Thorac Cardiovasc Surg 2017;153:648-53.  Back to cited text no. 2
Barbero-Marcial M, Tanamati C, Atik E, Ebaid M, Jatene A. Anomalous origin of the left coronary artery from the pulmonary artery with intramural aortic route: Diagnosis and surgical treatment. J Thorac Cardiovasc Surg 1999;117:823-5.  Back to cited text no. 3
Malankar DP, Chauhan S, Das S, Bisoi AK. How to do it: Repair of anomalous left coronary artery from pulmonary artery (ALCAPA) with coronary button transfer and Lecompte maneuver. J Card Surg 2010;25:225-7.  Back to cited text no. 4
Otaki Y, Ungerleider R. Half pantaloon (Florida – Shaped) patch for repair of anomalous left coronary artery from the pulmonary artery. CTSNet 2016. Available from: https://www.ctsnet.org/article/half-pantaloon-florida-shaped-patch-repair-anomalous-left -coronary-artery-pulmonary-artery. [Last accessed on 2022 Feb 11].  Back to cited text no. 5


  [Figure 1]


Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
Access Statistics
Email Alert *
Add to My List *
* Registration required (free)

  In this article
  Case Report
   Article Figures

 Article Access Statistics
    PDF Downloaded66    
    Comments [Add]    

Recommend this journal