Close
New

Medscape is available in 5 Language Editions – Choose your Edition here.

 

Surgical Approach to Anomalous Left Coronary Artery From the Pulmonary Artery Treatment & Management

  • Author: Mary C Mancini, MD, PhD, MMM; Chief Editor: Jonah Odim, MD, PhD, MBA  more...
 
Updated: Jan 30, 2015
 

Medical Therapy

Medical therapy should be used only to stabilize the patient for surgery. Intubation and mechanical ventilation often are needed in infants who present with shock and cardiac failure. This allows for adequate sedation and analgesia. The goal of analgesia and sedation are to minimize oxygen demands of the failing myocardium. Oxygen therapy is used to treat or prevent hypoxia.

Inotropic support is often necessary. Agents such as dobutamine or milrinone are beneficial to augment cardiac function; however, milrinone should be used cautiously because it may lower afterload/blood pressure to a degree that may impair coronary perfusion.

Diuretics (eg, furosemide) are useful to decrease pulmonary venous congestion. Transfusion of packed red blood cells may be useful to increase the oxygen-carrying capacity in patients who have severe anemia.

Next

Surgical Therapy

Direct transfer of the left coronary artery

Temporary cardiopulmonary bypass and cold blood cardioplegia are used. The pulmonary artery is transected, and the anomalous coronary artery is removed as a button of tissue around the ostium of the anomalous coronary artery. This technique is similar to the technique used in the arterial switch operation. The proximal coronary is mobilized, and the button is turned posteriorly for direct anastomosis into the aortic root. A slightly smaller button of aortic root is removed, and the coronary button is transposed and sewn into place on the aortic root. The pulmonary artery is then repaired with autologous pericardium.

Takeuchi repair

This technique is rarely needed today because most surgeons perform direct transfer of the anomalous left coronary artery from the pulmonary artery (ALCAPA) even when the anomalous vessel is transferred over some distance. In the Takeuchi repair, an aortopulmonary window is created. The pulmonary artery is opened, creating an anterior transverse flap of native pulmonary artery tissue, which creates a baffle to carry the aortic oxygenated blood to the anomalous coronary artery. The pulmonary artery is then repaired with autologous pericardium. Complications of the Takeuchi repair include obstruction of the baffle created between the anomalous coronary artery and supravalvar pulmonary stenosis.

Bypass grafting

The proximal anomalous coronary can be ligated, and bypass grafting may be used to reestablish coronary perfusion. In the past, carotid artery, subclavian artery, and saphenous vein grafts were used. Currently, internal mammary grafting or saphenous venous grafting can be used when direct transfer or the Takeuchi repair is not feasible.

Variations of direct transfer of the anomalous left coronary artery from the pulmonary artery

Several reports have documented variations of the direct transfer of the anomalous left coronary artery from the pulmonary artery, as follows:

  • The transected main pulmonary artery is used as a conduit tube in a variation of coronary angioplasty. A conduit tube of native pulmonary artery is anastomosed side to side to the aorta.
  • Enlarged autogenous aortic and pulmonary arterial flaps are used to create an extended left main stem coronary artery during anastomosis of the anomalous left coronary artery from the pulmonary artery to the aorta.
  • Elongated flaps of the aorta and pulmonary artery are sewn side to side to create a tunnel from the anomalous left coronary artery from the pulmonary artery to the aorta.

The advantage is that none of these techniques use prosthetic material to repair the anomalous left coronary artery from the pulmonary artery.

Previous
Next

Preoperative Details

See Medical Therapy.

Previous
Next

Intraoperative Details

Upon initial exposure, the dilated dysfunctional left ventricle may be susceptible to fibrillation during manipulation of the heart.

During cardioplegia, both the ascending aorta and the main pulmonary artery are cannulated and cross-clamped. This provides antegrade cardioplegia in the right coronary artery and the anomalous left coronary artery. If cardioplegia is instilled in the ascending aorta only, runoff and steal of cardioplegia into the main pulmonary artery via the anomalous left coronary artery may occur. With the advent of the technique of retrograde cardioplegia, cannulation of the pulmonary artery may be eliminated in some cases.

When choosing the incision site on the aorta for the aortocoronary anastomosis, transverse aortotomy is used to visualize the aortic sinus. This insures optimal location and placement of the coronary button for the aortocoronary anastomosis.

Some centers advocate performing a mitral annuloplasty to treat severe mitral regurgitation. This technique remains controversial because the mitral regurgitation is usually caused by annular dilation or papillary muscle dysfunction, both of which may improve after revascularization of the left ventricular myocardium and improvement of left ventricular function.

Intraoperative transesophageal echocardiography may be used to help identify and document abnormal flow in the anomalous left coronary artery from the pulmonary artery and normal flow in the repaired/transposed coronary artery. Transesophageal echocardiography is also useful for postoperative monitoring of ventricular function and mitral valve regurgitation.

Intraoperative images are shown below.

(1) Cardioplegia catheter in ascending aorta. (2) (1) Cardioplegia catheter in ascending aorta. (2) Cross-clamp on ascending aorta. (3) Cross-clamp on main pulmonary artery. (4) Arterial bypass cannula in the main pulmonary artery. (5) Cardioplegia catheter in the main pulmonary artery. (6) Dilated conal branch of the right coronary artery. (7) Venous bypass cannula in the right atrial appendage. (8) Left heart vent.
(1) Transverse anterior incision in the main pulmo (1) Transverse anterior incision in the main pulmonary artery trunk. (2) Probe is in the orifice of the anomalous left coronary artery.
(1) Divided distal main pulmonary artery. (2) Left (1) Divided distal main pulmonary artery. (2) Left coronary artery button. (3) Divided proximal main pulmonary artery.
(1) Left coronary artery button. (2) Divided proxi (1) Left coronary artery button. (2) Divided proximal main pulmonary artery. (3) Bypass sucker in transverse aortotomy (to visualize the aortic sinuses). (4) Incision in aortic sinus for site of aortocoronary anastomosis.
(1) Completing the anastomosis of the left coronar (1) Completing the anastomosis of the left coronary artery to the aortic sinus. (2) Divided proximal main pulmonary artery.
(1) Completed anastomosis of the left coronary art (1) Completed anastomosis of the left coronary artery to the aortic sinus. (2) Divided proximal main pulmonary artery. (3) Ascending aorta, transverse aortotomy.
(1) Suture closure of the aortotomy. (1) Suture closure of the aortotomy.
(1) Distal divided main pulmonary artery. (2) Begi (1) Distal divided main pulmonary artery. (2) Beginning re-anastomosis (posterior wall) of the main pulmonary artery. (3) Proximal main pulmonary artery.
(1) Completed repair of the main pulmonary artery (1) Completed repair of the main pulmonary artery re-anastomosis.
Previous
Next

Postoperative Details

Standard postoperative care is performed in the cardiac or pediatric intensive care unit. Blood products may be needed to control or decrease postoperative bleeding. Mechanical ventilation and inotropic support are typically required in the initial postoperative period. Afterload reduction therapy (eg, nitroprusside) is often used to control postoperative hypertension. Milrinone and epinephrine are used liberally in the immediate postoperative period. In patients in whom separation from cardiopulmonary bypass is difficult, further support with extracorporal membrane oxygenation (ECMO) may be needed. Intra-aortic balloon pump therapy may be used in older children and adults. Serial echocardiography is used to assess for improvement in the left ventricular function and mitral regurgitation.

A retrospective study by Weigand et al indicated that immediate postoperative morbidity in the repair of anomalous left coronary artery from the pulmonary artery is associated with patient size, the left ventricular end diastolic dimension (LVEDD), and the preoperative left ventricular shortening fraction. The investigators, who conducted the study on 44 patients, also found that the LVEDD Z-score independently predicted the length of time needed for left ventricular function to normalize and that in infants, this score independently predicted how long the patient would require postoperative intravenous inotropic support.[6]

Previous
Next

Follow-up

Standard follow-up care is required after surgical repair of anomalous left coronary artery from the pulmonary artery. Local care of the sternotomy incision is advised, and infants and small children should not be lifted by their arms for 6-8 weeks. Outpatient therapy with diuretics (eg, furosemide) and/or afterload reduction (eg, captopril, enalapril) is often used.

Long-term follow-up care includes the use of electrocardiography and echocardiography. In older children and adults, exercise stress testing,[7] including stress echocardiography and nuclear medicine perfusion scans, are useful to assess the patient's functional capacity postoperatively.

Previous
Next

Complications

Surgical complications include bleeding, infection, cardiac arrest/failure, stroke, and the need for further surgery. Most congenital heart surgery programs quote surgical mortality rates at less than 5-10%.

Previous
Next

Outcome and Prognosis

Even in patients with severe left ventricular dilation, global left ventricular dysfunction, and mitral regurgitation, outcome and prognosis is frequently excellent after surgical reimplantation of anomalous left coronary artery from the pulmonary artery (ALCAPA).[8] Prompt diagnosis, medical stabilization, a coordinated team approach in the operating room and postoperative intensive care unit can facilitate excellent outcomes for this relatively rare congenital defect.

Previous
Next

Future and Controversies

Direct transfer of the anomalous left coronary artery from the pulmonary artery (ALCAPA) is the surgical procedure of choice. As specialists at most congenital heart surgery centers have gained more experience with coronary artery transfer with the arterial switch operation, surgical repair of anomalous left coronary artery from the pulmonary artery has benefited from refinement of these surgical techniques. With appropriate diagnosis, presurgical stabilization, and team-oriented postoperative care, patients with anomalous left coronary artery from the pulmonary artery are expected to have an excellent outcome. Further refinement of long-term follow-up care with specialized stress and functional testing (eg, nuclear medicine perfusion, stress echocardiography) is anticipated.

Previous
 
Contributor Information and Disclosures
Author

Mary C Mancini, MD, PhD, MMM Professor and Chief of Cardiothoracic Surgery, Department of Surgery, Louisiana State University School of Medicine in Shreveport

Mary C Mancini, MD, PhD, MMM is a member of the following medical societies: American Association for Thoracic Surgery, American College of Surgeons, American Surgical Association, Society of Thoracic Surgeons, Phi Beta Kappa

Disclosure: Nothing to disclose.

Coauthor(s)

Christopher Mart, MD Associate Professor, Pediatric Echocardiography, Department of Pediatrics, Division of Pediatric Cardiology, University of Utah, Primary Children's Medical Center

Christopher Mart, MD is a member of the following medical societies: American College of Cardiology, American Society of Echocardiography, Society of Pediatric Echocardiography

Disclosure: Nothing to disclose.

John Myers, MD Director, Pediatric and Congenital Cardiovascular Surgery, Departments of Surgery and Pediatrics, Professor, Penn State Children's Hospital, Milton S Hershey Medical Center

John Myers, MD is a member of the following medical societies: American Association for Thoracic Surgery, American College of Cardiology, American College of Surgeons, American Heart Association, American Medical Association, Congenital Heart Surgeons Society, Pennsylvania Medical Society, Society of Thoracic Surgeons

Disclosure: Nothing to disclose.

Specialty Editor Board

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Mary C Mancini, MD, PhD, MMM Professor and Chief of Cardiothoracic Surgery, Department of Surgery, Louisiana State University School of Medicine in Shreveport

Mary C Mancini, MD, PhD, MMM is a member of the following medical societies: American Association for Thoracic Surgery, American College of Surgeons, American Surgical Association, Society of Thoracic Surgeons, Phi Beta Kappa

Disclosure: Nothing to disclose.

Chief Editor

Jonah Odim, MD, PhD, MBA Section Chief of Clinical Transplantation, Transplantation Branch, Division of Allergy, Immunology, and Transplantation, National Institute of Allergy and Infectious Diseases (NIAID), National Institutes of Health (NIH)

Jonah Odim, MD, PhD, MBA is a member of the following medical societies: American College of Cardiology, American College of Chest Physicians, American Association for Physician Leadership, American College of Surgeons, American Heart Association, American Society for Artificial Internal Organs, American Society of Transplant Surgeons, Association for Academic Surgery, Association for Surgical Education, International Society for Heart and Lung Transplantation, National Medical Association, New York Academy of Sciences, Royal College of Physicians and Surgeons of Canada, Society of Critical Care Medicine, Society of Thoracic Surgeons, Canadian Cardiovascular Society

Disclosure: Nothing to disclose.

Additional Contributors

Daniel S Schwartz, MD, FACS Medical Director of Thoracic Oncology, St Catherine of Siena Medical Center, Catholic Health Services

Daniel S Schwartz, MD, FACS is a member of the following medical societies: Society of Thoracic Surgeons, Western Thoracic Surgical Association, American College of Chest Physicians, American College of Surgeons

Disclosure: Nothing to disclose.

References
  1. Mustard WT. Anomalies of the coronary arteries. Pediatric Surgery. Chicago, IL: Mosby-Year Book; 1953. Vol 1: 433-40.

  2. Sabiston DC, Neill CA, Taussig HB. The direction of blood flow in anomalous left coronary artery arising from the pulmonary artery. Circulation. 1960. 22:591.

  3. Cooley DA, Hallman GL, Bloodwell RD. Definitive surgical treatment of anomalous origin of left coronary artery from pulmonary artery: indications and results. J Thorac Cardiovasc Surg. 1966 Dec. 52(6):798-808. [Medline].

  4. Meyer BW, Stefanik G, Stiles QR, et al. A method of definitive surgical treatment of anomalous origin of left coronary artery. A case report. J Thorac Cardiovasc Surg. 1968 Jul. 56(1):104-7. [Medline].

  5. Bland ER, White PD, Garland J. Congenital anomalies of the coronary arteries: report of an unusual case associated with cardiac hypertrophy. Am Heart J. 1933. 8:787.

  6. Weigand J, Marshall CD, Bacha EA, et al. Repair of Anomalous Left Coronary Artery From the Pulmonary Artery in the Modern Era: Preoperative Predictors of Immediate Postoperative Outcomes and Long Term Cardiac Follow-up. Pediatr Cardiol. 2014 Oct 10. [Medline].

  7. [Guideline] Paridon SM, Alpert BS, Boas SR, et al. Clinical stress testing in the pediatric age group: a statement from the American Heart Association Council on Cardiovascular Disease in the Young, Committee on Atherosclerosis, Hypertension, and Obesity in Youth. Circulation. 2006 Apr 18. 113(15):1905-20. [Medline].

  8. Ojala T, Salminen J, Happonen JM, et al. Excellent functional result in children after correction of anomalous origin of left coronary artery from the pulmonary artery--a population-based complete follow-up study. Interact Cardiovasc Thorac Surg. 2010 Jan. 10(1):70-5. [Medline].

  9. Backer CL, Stout MJ, Zales VR, et al. Anomalous origin of the left coronary artery. A twenty-year review of surgical management. J Thorac Cardiovasc Surg. 1992 Jun. 103(6):1049-57; discussion 1057-8. [Medline].

  10. Conte G, Pellegrini A. On the development of the coronary arteries in human embryos, stages 14- 19. Anat Embryol (Berl). 1984. 169(2):209-18. [Medline].

  11. Cowles RA, Berdon WE. Bland-White-Garland syndrome of anomalous left coronary artery arising from the pulmonary artery (ALCAPA): a historical review. Pediatr Radiol. 2007 Jul 3. [Medline].

  12. Edwards JE. The direction of blood flow in coronary arteries arising from the pulmonary trunk. Circulation. 1964. 29:163-6.

  13. Grace RR, Angelini P, Cooley DA. Aortic implantation of anomalous left coronary artery arising from pulmonary artery. Am J Cardiol. 1977 Apr. 39(4):609-13. [Medline].

  14. Houston AB, Pollock JC, Doig WB, et al. Anomalous origin of the left coronary artery from the pulmonary trunk: elucidation with colour Doppler flow mapping. Br Heart J. 1990 Jan. 63(1):50-4. [Medline].

  15. Katsumata T, Westaby S. Anomalous left coronary artery from the pulmonary artery: a simple method for aortic implantation with autogenous arterial tissue. Ann Thorac Surg. 1999 Sep. 68(3):1090-1. [Medline].

  16. Keith JD. The anomalous origin of the left coronary artery from the pulmonary artery. Br Heart J. 1959. 21:149-61.

  17. Kittle CF, Diehl AM, Heilbrunn A. Anomalous left coronary ariseing from the pulmonary artery: report of a case and surgical consideration. J Pediatr. 1955. 47:198.

  18. Lilje C, Le TP, Ntalakoura K, Weil J, Lacour-Gayet F. Noninvasive Follow-up of Complications After the Takeuchi Operation. J Am Soc Echocardiogr. 2007 Jul 11. [Medline].

  19. Lumia D, Carrafiello G, Lagana D, et al. MDCT coronary angiography for diagnosis of anomalous origin right coronary artery: a case report. Emerg Radiol. 2007 Sep. 14(4):237-40. [Medline].

  20. Murthy KS, Krishnanaik S, Mohanty SR, et al. A new repair for anomalous left coronary artery. Ann Thorac Surg. 2001 Apr. 71(4):1384-6. [Medline].

  21. Nisim AA, Spivak J, Margulies DR, et al. Anomalous origin of the left main coronary artery: a rare cause of respiratory distress in an adult trauma patient with a lower extremity injury. J Trauma. 2007 Jun. 62(6):1504-6. [Medline].

  22. Sarris GE, Drummond-Webb JJ, Ebeid MR, et al. Anomalous origin of left coronary from right pulmonary artery in hypoplastic left heart syndrome. Ann Thorac Surg. 1997 Sep. 64(3):836-8. [Medline].

  23. Sercelik A, Mavi A, Karben Z, Batyraliev T, Gumusburun E. Anomalous left anterior descending coronary artery originating from the right coronary artery: a case report and review of the current literature. Minerva Cardioangiol. 2007 Jun. 55(3):428-9. [Medline].

  24. Tashiro T, Todo K, Haruta Y, et al. Anomalous origin of the left coronary artery from the pulmonary artery. New operative technique. J Thorac Cardiovasc Surg. 1993 Oct. 106(4):718-22. [Medline].

 
Previous
Next
 
A 3-month-old child presenting with anomalous left coronary artery from the pulmonary artery (ALCAPA). Note the large prominent Q waves in leads I, aVL, and V4-V6.
Parasternal long-axis 2-dimensional echocardiogram view of the pulmonary artery. The anomalous left coronary artery and first order branches of the anomalous left coronary artery (LCA) are identified.
Parasternal long-axis 2-dimensional echocardiogram. Very dilated left ventricle with mitral regurgitation.
Parasternal long-axis 2-dimensional, color-flow Doppler echocardiogram. Normal flow in the pulmonary artery. Abnormal retrograde flow (*) in the anomalous left coronary artery from the pulmonary artery (ALCAPA).
Parasternal short-axis 2-dimensional, color-flow Doppler echocardiogram. Normal antegrade flow in the proximal right coronary artery.
Modified parasternal long-axis echocardiogram with color-flow Doppler. Abnormal retrograde flow in the left anterior descending (LAD) coronary artery.
Apical 4-chamber 2-dimensional echocardiogram. Note the very dilated left atrium and left ventricle.
Intraoperative transesophageal, transverse plane, 4-chamber view, 2-dimensional, color-flow Doppler ultrasound image. Note the dilated left atrium, dilated left ventricle, and mitral regurgitation. LV=left ventricle; RV=right ventricle.
Intraoperative transesophageal, transverse plane, 2-dimensional ultrasound image. Main pulmonary artery with origin of the anomalous left coronary artery. Note the first-order branching into the left anterior descending and circumflex coronary arteries.
Intraoperative transesophageal, transverse plane, 2-dimensional, color-flow Doppler ultrasound image. Main pulmonary artery with origin of the anomalous left coronary artery. Abnormal retrograde flow is noted in the left anterior descending (LAD) coronary artery.
Intraoperative transesophageal, transverse plane, 2-dimensional ultrasound image. Completed repair of the left main coronary artery (LMCA) anastomosed to the aorta. LAD=left anterior descending coronary artery.
Intraoperative transesophageal, transverse plane, 2-dimensional, color-flow Doppler ultrasound image. Completed repair with normal antegrade flow in the circumflex and left anterior descending (LAD) coronary arteries. LMCA=left main coronary artery.
(1) Cardioplegia catheter in ascending aorta. (2) Cross-clamp on ascending aorta. (3) Cross-clamp on main pulmonary artery. (4) Arterial bypass cannula in the main pulmonary artery. (5) Cardioplegia catheter in the main pulmonary artery. (6) Dilated conal branch of the right coronary artery. (7) Venous bypass cannula in the right atrial appendage. (8) Left heart vent.
(1) Transverse anterior incision in the main pulmonary artery trunk. (2) Probe is in the orifice of the anomalous left coronary artery.
(1) Divided distal main pulmonary artery. (2) Left coronary artery button. (3) Divided proximal main pulmonary artery.
(1) Left coronary artery button. (2) Divided proximal main pulmonary artery. (3) Bypass sucker in transverse aortotomy (to visualize the aortic sinuses). (4) Incision in aortic sinus for site of aortocoronary anastomosis.
(1) Completing the anastomosis of the left coronary artery to the aortic sinus. (2) Divided proximal main pulmonary artery.
(1) Completed anastomosis of the left coronary artery to the aortic sinus. (2) Divided proximal main pulmonary artery. (3) Ascending aorta, transverse aortotomy.
(1) Suture closure of the aortotomy.
(1) Distal divided main pulmonary artery. (2) Beginning re-anastomosis (posterior wall) of the main pulmonary artery. (3) Proximal main pulmonary artery.
(1) Completed repair of the main pulmonary artery re-anastomosis.
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2016 by WebMD LLC. This website also contains material copyrighted by 3rd parties.