Anomalous Left Coronary Artery From the Pulmonary Artery Clinical Presentation

  • Author: Mary C Mancini, MD, PhD; Chief Editor: Steven R Neish, MD, SM   more...
 
Updated: Dec 1, 2011
 

History

  • Infants with anomalous left coronary artery from the pulmonary artery (ALCAPA) usually do well for a short period then gradually become fussy and irritable. Typically, they may display pallor, irritability, and diaphoresis after feeding, which are often attributed to colic.
  • Signs and symptoms of congestive heart failure (CHF), including tachypnea, tachycardia, diaphoresis, and poor feeding, eventually ensue, leading to poor weight gain. Usually no obvious evidence of a systemic illness is noted.
  • In rare instances, children outgrow these symptoms and gradually become asymptomatic, although periodic dyspnea, angina pectoris, syncope, or sudden death may still occur in adulthood.
Next

Physical

  • If CHF is present, the infant appears distressed and exhibits tachypnea, tachycardia, diaphoresis, and irritability.
  • Auscultation may demonstrate a systolic murmur of mitral valve regurgitation and, possibly, a diastolic rumble of relative mitral stenosis best located at the apical left precordial region.
  • Rarely, a soft continuous murmur may be detected at the upper left sternal border that is reminiscent of a coronary artery fistula or a small patent ductus arteriosus.
  • The left ventricular precordial impulse may appear prominent and displaced both inferiorly and laterally.
  • The second heart sound may seem narrowly split with increased intensity of the pulmonic component, if left ventricular failure causes pulmonary artery hypertension secondary to elevated left atrial pressure.
  • In cases of severe CHF, hepatic enlargement may be observed, and the peripheral pulses may be diminished in intensity secondary to low cardiac output.
Previous
Next

Causes

  • Inheritance is not a factor. For example, if 2 family members are affected, the fact that they are within the same family did not have a role in their development of the condition.
  • In utero exposure to teratogens, chromosomal abnormalities, or other risk factors are unrelated to ALCAPA.
  • An isolated congenital cardiac defect, including patent ductus arteriosus, ventricular septal defect, tetralogy of Fallot, or coarctation of the aorta, rarely may be associated with ALCAPA. No specific association with any noncardiac anomalies is noted.[4]
Previous
Proceed to Differential Diagnoses
 
 
Contributor Information and Disclosures
Author

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

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

Disclosure: Nothing to disclose.

Coauthor(s)

Howard S Weber, MD, FAAP, FACC, FSCAI  Professor, Assistant Chief, Section of Pediatric Cardiology, Penn State University School of Medicine; Director, Pediatric Catheterization Laboratory, Milton S Hershey Medical Center

Howard S Weber, MD, FAAP, FACC, FSCAI is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, and Society for Cardiac Angiography and Interventions

Disclosure: Nothing to disclose.

Specialty Editor Board

Paul M Seib, MD  Associate Professor of Pediatrics, University of Arkansas for Medical Sciences; Medical Director, Cardiac Catheterization Laboratory, Co-Medical Director, Cardiovascular Intensive Care Unit, Arkansas Children's Hospital

Paul M Seib, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, American Heart Association, Arkansas Medical Society, International Society for Heart and Lung Transplantation, and Society for Cardiac Angiography and Interventions

Disclosure: Nothing to disclose.

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.

Alvin J Chin, MD  Professor of Pediatrics, University of Pennsylvania School of Medicine; Attending Physician, Cardiology Division, Children's Hospital of Philadelphia

Alvin J Chin, MD, is a member of the following medical societies: American Association for the Advancement of Science, American Heart Association, and Society for Developmental Biology

Disclosure: Nothing to disclose.

Gilbert Z Herzberg, MD  Assistant Professor, Department of Pediatrics, Section of Pediatric Cardiology, New York Medical College; Consulting Staff, Department of Pediatrics, Sound Shore Medical Center

Gilbert Z Herzberg, MD is a member of the following medical societies: American Academy of Pediatrics

Disclosure: Nothing to disclose.

Chief Editor

Steven R Neish, MD, SM  Director of Pediatric Cardiology Fellowship Program, Associate Professor, Department of Pediatrics, Baylor College of Medicine

Steven R Neish, MD, SM is a member of the following medical societies: American Academy of Pediatrics, American College of Cardiology, and American Heart Association

Disclosure: Nothing to disclose.

References

  1. Bland EF. Congenital anomalies of the coronary arteries: report of an unusual case associated with cardiac hypertrophy. 1933;8:787-801.

  2. Fontana RS, Edwards JE. Congenital Cardiac Disease: a Review of 357 Case Studies Pathologically. WB Saunders; 1962:291.

  3. Su LS, Burkhart HM, O'Leary PW, Dearani JA. Mitral valve arcade with concomitant anomalous left coronary artery from the pulmonary artery. Ann Thorac Surg. Dec 2011;92(6):e121-3. [Medline].

  4. Arciniegas E, Farooki ZQ, Hakimi M, Green EW. Management of anomalous left coronary artery from the pulmonary artery. Circulation. Aug 1980;62(2 Pt 2):I180-9. [Medline].

  5. Secinaro A, Ntsinjana H, Tann O, Schuler PK, Muthurangu V, Hughes M, et al. Cardiovascular magnetic resonance findings in repaired anomalous left coronary artery to pulmonary artery connection (ALCAPA). J Cardiovasc Magn Reson. May 16 2011;13:27. [Medline]. [Full Text].

  6. Pisacane C, Pinto SC, De Gregorio P, et al. "Steal" collaterals: an echocardiographic diagnostic marker for anomalous origin of the left main coronary artery from the pulmonary artery in the adult. J Am Soc Echocardiogr. Jan 2006;19(1):107.e3-107.e6. [Medline].

  7. Juan CC, Hwang B, Lee PC, Meng CC. Diagnostic application of multidetector-row computed tomographic coronary angiography to assess coronary abnormalities in pediatric patients: comparison with invasive coronary angiography. Pediatr Neonatol. Aug 2011;52(4):208-13. [Medline].

  8. Piechaud JF, Shalaby L, Kachaner J, et al. Pulmonary artery "stop-flow" angiography to visualize the anomalous origin of the left coronary artery from the pulmonary artery in infants. Pediatr Cardiol. 1987;8(1):11-5. [Medline].

  9. Erdinc M, Hosgor K, Karahan O. Repair of anomalous origin of the left coronary artery arising from right pulmonary artery with rolled-conduit-extended reimplantation in an adult. J Card Surg. Nov 2011;26(6):604-7. [Medline].

  10. McNamara DG. Treatment of anomalous origin of left coronary artery arising from the pulmonary artery. 1973;1:497-499.

  11. 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. Jul 1968;56(1):104-7. [Medline].

  12. Takeuchi S, Imamura H, Katsumoto K, et al. New surgical method for repair of anomalous left coronary artery from pulmonary artery. J Thorac Cardiovasc Surg. Jul 1979;78(1):7-11. [Medline].

  13. Canale LS, Monteiro AJ, Rangel I, et al. Surgical treatment of anomalous coronary artery arising from the pulmonary artery. Interact Cardiovasc Thorac Surg. Oct 8 2008;[Medline].

  14. Champsaur G, Bozio A, Joffre B, et al. [Anomalous origin of the left coronary artery from the pulmonary artery. Treatment by left subclavian-left main coronary artery anastomosis]. Nouv Presse Med. Apr 5 1980;9(16):1167-9. [Medline].

  15. Chhatriwalla AK, Younoszai A, Latson L, Jaber WA. An 8-month-old girl with an anomalous left coronary artery from the pulmonary artery complicated by myocardial ischemia after surgical reimplantation. J Nucl Cardiol. May-Jun 2006;13(3):432-6. [Medline].

  16. el-Said GM, Ruzyllo W, Williams RL, et al. Early and late result of saphenous vein graft for anomalous origin of left coronary artery from pulmonary artery. Circulation. Jul 1973;48(1 Suppl):III2-6. [Medline].

  17. George JM, Knowlan DM. Anomalous origin of the left coronary artery from the pulmonary artery in anadult. N Engl J Med. Nov 12 1959;261:993-8. [Medline].

  18. Heifetz SA, Robinowitz M, Mueller KH, Virmani R. Total anomalous origin of the coronary arteries from the pulmonary artery. Pediatr Cardiol. 1986;7(1):11-8. [Medline].

  19. Hershey J, Isada L, Fenster MS. Emergent primary PCI of anomalous LAD. J Invasive Cardiol. May 2006;18(5):E152-3. [Medline].

  20. Johnsrude CL, Perry JC, Cecchin F, et al. Differentiating anomalous left main coronary artery originating from the pulmonary artery in infants from myocarditis and dilated cardiomyopathy by electrocardiogram. Am J Cardiol. Jan 1 1995;75(1):71-4. [Medline].

  21. Menahem S, Venables AW. Anomalous left coronary artery from the pulmonary artery: a 15 year sample. Br Heart J. Oct 1987;58(4):378-84. [Medline].

  22. Mesurolle B, Qanadli SD, Mignon F, Lacombe P. Anomalous origin of the left coronary artery arising from the pulmonary trunk. AJR Am J Roentgenol. Apr 2006;186(4):1202; author reply 1202. [Medline].

  23. Murala JS, Cooper S, Duffy B, et al. Anomalous left coronary artery arising from the left pulmonary artery, aortic coarctation, and a large ventricular septal defect. J Thorac Cardiovasc Surg. Apr 2006;131(4):911-2. [Medline].

  24. Murala JS, Sankar MN, Agarwal R, et al. Anomalous origin of left coronary artery from pulmonary artery in adults. Asian Cardiovasc Thorac Ann. Feb 2006;14(1):38-42. [Medline]. [Full Text].

  25. Murala JS, Sankar MN, Agarwal R, et al. Anomalous origin of left coronary artery from pulmonary artery in adults. Asian Cardiovasc Thorac Ann. Feb 2006;14(1):38-42. [Medline].

  26. Neufeld HN, Schneeweiss A. Coronary Artery Disease in Infants and Children. Philadelphia, PA: Lea and Febiger; 1983:1-30.

  27. Schreiber C, Lange R. Creation of a dual-coronary system for anomalous origin of the left coronary artery from the pulmonary artery utilizing the trapdoor flap technique. Eur J Cardiothorac Surg. May 2003;23(5):851-2. [Medline].

 
Previous
Next
 
Preoperative electrocardiogram in a 2-month-old infant with anomalous origin of the left coronary artery from the pulmonary artery demonstrating pathologic Q waves in leads I and aVL and diffuse ST-T wave changes consistent with an anterolateral infarction.
Electrocardiogram in 2-month-old infant with anomalous origin of the left coronary artery from the pulmonary artery 17 months following successful surgical revascularization, demonstrating complete resolution of the anterolateral infarction pattern and ST-T wave changes.
Two-dimensional echocardiographic image (parasternal short axis view) in a patient with anomalous origin of the left coronary artery arising from the pulmonary artery (ALCAPA). The left coronary artery (white arrow) appears to course towards the main pulmonary artery (MPA) just above the pulmonary valve and not to the aortic root (Ao). RV = Right ventricle.
Two-dimensional echocardiographic image with color flow mapping (parasternal short axis view) in the same patient with anomalous origin of the left coronary artery arising from the pulmonary artery (ALCAPA). The addition of color flow mapping to the 2-dimensional image demonstrates abnormal flow reversal within the left coronary artery (white arrows) towards the main pulmonary artery (MPA) just above the pulmonary valve. RV = Right ventricle. Ao = Aortic root.
Doppler interrogation of the abnormal color flow jet is depicted, demonstrating abnormal flow within the main pulmonary artery towards the transducer in diastole, which represents runoff from the anomalous left coronary artery (large white arrowhead). Small white arrow: Normal antegrade main pulmonary artery flow in systole. MPA = Main pulmonary artery.
Aortogram in a patient with suspected anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA). Frontal (left panel) and lateral (right panel) images demonstrating an enlarged right coronary artery (small white arrow), which fills a small left coronary system (solid arrow head) via collaterals with eventual faint opacification of the main pulmonary artery (not demonstrated in this frame).
Main pulmonary artery angiogram demonstrating the technique of stop flow angiography. There is retrograde opacification of the entire left coronary artery system, which originates from the distal main pulmonary artery (MPA), including the anterior descending (solid white arrowhead) and circumflex (small white arrow) branches. Left panel: Frontal image. Right panel: Lateral image.
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2012 by WebMD LLC.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.