Anomalous Coronary Artery

Anomalous Coronary Artery: Excerpt from The 5-Minute Pediatric Consult

Geoffrey L. Bird, MD

Anomalous Coronary Artery - BASICS

Anomalous Coronary Artery - description

In this disorder, most commonly the left (rarely the right) coronary artery arises from the pulmonary artery rather than the aorta.

Anomalous Coronary Artery - epidemiology

• Incidence: Rare anomaly
• Prevalence:
  • The majority of patients with this anomaly present in infancy, at around the age of 2 months.
  • Of note, the literature includes many single case reports of newly diagnosed patients presenting as old as during the 4th to 7th decade of life.

Anomalous Coronary Artery - pathophysiology

• Collateral flow runoff tends to “steal” blood from the myocardial blood vessels into the pulmonary artery, resulting in myocardial ischemia.
• The diastolic BP in the pulmonary artery is typically much lower than the main driving force for myocardial perfusion in patients with normal anatomy, namely, diastolic aortic pressure.
• The fact that the left ventricle may be perfused with desaturated blood plays a less important role than the overall perfusion-related imbalance between myocardial oxygen demand and supply.

Anomalous Coronary Artery - etiology

• Abnormal septation of the conotruncus into aorta and pulmonary artery
• Persistence of the pulmonary buds and involution of the aortic buds that will eventually form the coronary arteries
• As-yet-unspecified genetic predisposition

Anomalous Coronary Artery - DIAGNOSIS

Anomalous Coronary Artery - signs & symptoms

Anomalous Coronary Artery - history

• Paroxysms of poor feeding, pallor, and sweating
• Irritability (crying), especially after meals
• May have intractable CHF
• Sometimes asymptomatic
• Occasionally may be symptomatic in infancy and then gradually improve (with adequate coronary collateralization)
• Older children and adults may have dyspnea, syncope, or angina pectoris on effort
• Sudden death

Anomalous Coronary Artery - physical exam

• Signs of CHF (e.g., cachexia, tachycardia, tachypnea, lethargy, diaphoresis)
• Loud P2 component of S2
• Gallop rhythm
• Murmur: Mitral incompetence, or a continuous murmur reminiscent of a coronary arteriovenous fistula
• Diagnosis should be entertained in any infant presenting with cardiomegaly or perplexing cardiorespiratory symptoms.

Anomalous Coronary Artery - tests

Anomalous Coronary Artery - imaging

• Chest radiograph study: Cardiomegaly, pulmonary edema
• Nuclear imaging: Thallium myocardial perfusion imaging shows reduced uptake in ischemic regions.
• Electrocardiography: Anterolateral infarct pattern in an infant (Q in I, aVL, V4-V6), abnormal R wave progression in precordial leads
• Echocardiogram: Attachment of coronary artery to pulmonary artery by 2-dimensional imaging. Doppler interrogation shows flow passing from coronary artery to great artery rather than vice versa.
  • Large right coronary artery
  • Functional impairment, wall-motion abnormalities, and dilation of the left ventricle
  • Echogenic papillary muscles
  • Mitral regurgitation

Anomalous Coronary Artery - diag proced-surgery

• Cardiac catheterization: Angiographic and hemodynamic parameters may correlate with degree of cardiovascular dysfunction.
  • Low cardiac output
  • High filling pressures
  • Pulmonary hypertension
• Aortic root angiography shows passage of contrast medium from normally connected right coronary artery to the left coronary arterial system to the pulmonary artery.
• Pulmonary artery angiogram shows reflux of contrast medium into the left coronary artery and/or a “negative wash-in” of unopacified blood flowing from left coronary to pulmonary artery.
• Coronary CT angiography and cardiac MRI: Newer modalities that have not surpassed sensitivity of current gold standard of angiography

Anomalous Coronary Artery - differencial diagnosis

• Cardiomyopathy
• Mitral valve incompetence
• Left ventricular failure from other causes
• Colic
• Bronchiolitis

Anomalous Coronary Artery - TREATMENT

Anomalous Coronary Artery - initial stabilization

Attention to basic life support measures (airway, breathing, circulation) and prompt referral to a pediatric cardiac center. An excess of procedures, interventions, and manipulation are poorly tolerated by this group of patients. Even with the full support of a tertiary center’s experienced team, these measures are fraught with peril.

Anomalous Coronary Artery - general measures

The 1st priority is to safely institute supportive care measures while expeditiously planning for surgical intervention. Medical therapy alone has a very limited role in the current era.

Anomalous Coronary Artery - surgery

• Direct reimplantation of the left coronary artery into the aorta using a button of pulmonary arterial tissue and/or an extension-tube graft of anterior and posterior pulmonary arterial wall tissue sewn into a narrow cylinder to avoid tension, distortion, and stenosis
• Creation of an aortopulmonary window and tunnel that directs blood from aorta to the left coronary ostium (Takeuchi procedure)
• Ligation of the origin of the left coronary artery (to prevent flow runoff into the pulmonary artery or “steal”) is less frequently used, even in very ill infants.
• Ligation of origin of left coronary artery and reconstitution of flow with saphenous or internal mammary graft is less frequently used in the current era.

Anomalous Coronary Artery - FOLLOW UP

Anomalous Coronary Artery - prognosis

• Untreated, 65–85% of those who present in infancy will die before the age of 1 year, usually after 2 months of age (when pulmonary vascular resistance falls).
• Few of those who present early improve spontaneously.
• Late results after surgery are excellent in many centers. Hospital mortality in larger selected series of these frequently moribund patients is ≤5%, with very little subsequent attrition.
• Mitral regurgitation usually improves after surgery establishes a patent dual-coronary system, but this may take 6–12 months to be fully realized. Follow-up evaluation is warranted, as mitral regurgitation may progress despite surgery, and valve repair may be required later.

Anomalous Coronary Artery - bibliography

Allen HD, Gutgesell HP, Clark EB, et al., eds. Moss and Adams’ Heart Disease in Infants, Children and Adolescents Including the Fetus and Young Adult. 6th ed. Baltimore: Williams & Wilkins; 2000:679–682.
1. Azakie A, Russell JL, McCrindle BW, et al. Anatomic repair of anomalous left coronary artery from the pulmonary artery by aortic reimplantation: Early survival, patterns of ventricular recovery and late outcome. Ann Thorac Surg. 2003;75(5):1535–1541.
2. Dodge-Khatami A, Mavroudis C, Backer CL. Anomalous origin of the left coronary artery from the pulmonary artery: Collective review of surgical therapy. Ann Thorac Surg. 2002;74(3):946–955.
Keane JF, Lock JE, Fyler DC, eds. Nadas’ Pediatric Cardiology. Philadelphia: WB Saunders; 2006.
3. Lange R, Vogt M, Horer J, et al. Long-term results of repair of anomalous origin of the left coronary artery from the pulmonary artery. Ann Thorac Surg. 2007;83(4):1463–1471.
4. Michielon G, Di Carlo D, Brancaccio G, et al. Anomalous coronary artery origin from the pulmonary artery: Correlation between surgical timing and left ventricular function recovery. Ann Thorac Surg. 2003;76(2):581–588, discussion 588.
5. Pelliccia A. Congenital coronary artery anomalies in young patients: New perspectives for timely identification. J Am Coll Cardiol. 2001;37(2):598–600.

Anomalous Coronary Artery - CODES

Anomalous Coronary Artery - icd9

746.85 Coronary artery anomaly

Anomalous Coronary Artery - FAQ

• Q: How do you differentiate crying from colic?
• A: This is not easy, but clinical assessment should manifest the signs of CHF, shock, and low cardiac output, which are decidedly atypical for the usual patient with colic. If the patient is still feeding, the crying in patients with this lesion classically occurs after meals, when blood is shunted to the liver and intestines. This is not a highly sensitive finding, and concern should lead to further objective evaluation.
• Q: How does one proceed with the evaluation?
• A: In addition to history and physical examination findings, results of chest radiography, an ECG, and echocardiography may show the typical features discussed above.

Book Source Details

• Book Title: The 5-Minute Pediatric Consult
• Author(s): M. William Schwartz MD; et al.
• Year of Publication: 2008
• Copyright Details: The 5-Minute Pediatric Consult, Copyright © 2008 Lippincott Williams & Wilkins.

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Copyright notice for book excerpts: Copyright © 2008 Lippincott Williams & Wilkins. All rights reserved.

More About This Book: Title: The 5-Minute Pediatric Consult Authors: M. William Schwartz MD; et al. Publisher: Lippincott Williams & Wilkins Copyright: 2008 ISBN: 0-7817-7577-9

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