Surgical Approach to Anomalous Left Coronary Artery From the Pulmonary Artery Workup

Updated: May 22, 2017
  • Author: Mary C Mancini, MD, PhD, MMM; Chief Editor: Suvro S Sett, MD, FRCSC, FACS  more...
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Workup

Laboratory Studies

Perform arterial blood gas measurements, including an assessment for acidosis and carbon dioxide retention, in the setting of respiratory distress.

Levels of cardiac enzymes (eg, troponin I, creatine kinase–MB fraction) may be elevated in patients with myocardial ischemia, but these results are not specific for anomalous left coronary artery from the pulmonary artery (ALCAPA).

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Imaging Studies

Chest radiography

Chest radiography in patients with anomalous left coronary artery from the pulmonary artery (ALCAPA) reveals cardiomegaly, left atrial and left ventricular enlargement, and pulmonary venous congestion.

Echocardiography

Currently, most cases of ALCAPA can be diagnosed by echocardiography. In infants presenting with left ventricular dilatation and dysfunction, special attention should be directed to the coronary artery anatomy during echocardiographic evaluation.

Two-dimensional (2D) imaging alone is usually inadequate to thoroughly evaluate for ALCAPA. The anomalous coronary may course very close to the aortic sinus and create the false impression of a normal anatomic origin of the left coronary artery. Usually, 2D imaging identifies an enlarged right coronary artery at its origin and proximal course. Coupled with color-flow Doppler imaging, 2D imaging greatly increases the diagnostic findings of echocardiography.

Color-flow Doppler imaging demonstrates abnormal retrograde flow in the anomalous left coronary artery and into the main pulmonary artery segment. The color flow into the pulmonary artery should not be confused with a shunt from a ductus arteriosus or a coronary-cameral fistula.

Transesophageal echocardiography may be useful in the rare adult patient in whom ALCAPA is suspected, but this examination is usually unnecessary in infants.

Examples of echocardiography findings are shown in the images below.

This is a parasternal long-axis view, two-dimensio This is a parasternal long-axis view, two-dimensional echocardiogram of the pulmonary artery. The anomalous left coronary artery and first-order branches of the anomalous left coronary artery (LCA) are identified.
This is also a parasternal long-axis view, two-dim This is also a parasternal long-axis view, two-dimensional echocardiogram. A very dilated left ventricle (LV) with mitral regurgitation is noted.
This is a parasternal long-axis view, two-dimensio This is a parasternal long-axis view, two-dimensional, color-flow Doppler echocardiogram. Normal flow is noted in the pulmonary artery, but note the abnormal retrograde flow (*) in the anomalous left coronary artery from the pulmonary artery (ALCAPA).
This is a parasternal short-axis view, two-dimensi This is a parasternal short-axis view, two-dimensional, color-flow Doppler echocardiogram. Normal antegrade flow in the proximal right coronary artery (RCA) is observed.
This is a modified parasternal, long-axis echocard This is a modified parasternal, long-axis echocardiogram with color-flow Doppler. Abnormal retrograde flow in the left anterior descending (LAD) coronary artery is seen. LV = left ventricle; RV = right ventricle.
This is an apical four-chamber two-dimensional ech This is an apical four-chamber two-dimensional echocardiogram. Note the very dilated left atrium and left ventricle. LA = left atrium; LV = left ventricle; RA = right atrium; RV = right ventricle.

Ultrasonography

Examples of ultrasonography in anomalous left coronary artery from the pulmonary artery are shown in the images below.

This is an intraoperative transesophageal, transve This is an intraoperative transesophageal, transverse plane, four-chamber view, two-dimensional, color-flow Doppler sonogram. Note the dilated left atrium, dilated left ventricle, and mitral regurgitation. LV = left ventricle; RV = right ventricle.
This is an intraoperative transesophageal, transve This is an intraoperative transesophageal, transverse plane, two-dimensional sonogram showing the main pulmonary artery (MPA) with origin of the anomalous left coronary artery. Note the first-order branching into the left anterior descending (LAD) and circumflex coronary arteries. LMAC = left main coronary artery.
This is an intraoperative transesophageal, transve This is an intraoperative transesophageal, transverse plane, two-dimensional, color-flow Doppler ultrasound image demonstrating the main pulmonary artery (MPA) with origin of the anomalous left coronary artery. Abnormal retrograde flow is noted in the left anterior descending (LAD) coronary artery. LMAC = left main coronary artery.
This is an intraoperative transesophageal, transve This is an intraoperative transesophageal, transverse plane, two-dimensional ultrasound image. It reveals completed repair of the left main coronary artery (LMCA) anastomosed to the aorta. LAD = left anterior descending coronary artery.
This is an intraoperative transesophageal, transve This is an intraoperative transesophageal, transverse plane, two-dimensional, color-flow Doppler ultrasound image. Note the completed repair with normal antegrade flow in the circumflex and left anterior descending (LAD) coronary arteries. LMCA = left main coronary artery.

Computed tomography angiography (CTA)

Coronary CTA can reveal anomalous left coronary artery from the pulmonary artery in adult patients; however, it does not eliminate the need for cardiac catheterization.

Multidector CT (MDCT) angiography appears to be superior to conventional angiography for defining the ostial origin and proximal course of anomalous coronary arteries, demonstrating the association between the abnormal coronary arteries with the aorta and pulmonary artery, as well as visualization of the intrinsic anatomy and termination of these arteries. [13]  

 

 

 

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Electrocardiology

Electrocardiography (ECG) can reveal an infarct pattern, typically in an anteroseptal distribution. Wide and/or deep Q waves are typically present in leads I and aVL. Loss of normal R-wave progression in the precordial leads and T-wave inversion in leads I, aVL, and the left precordial leads may be observed.

Note: The ECG changes noted above are nonspecific for anomalous left coronary artery from the pulmonary artery (ALCAPA) and may be encountered in other forms of cardiomyopathy.

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Diagnostic Procedures

Cardiac catherization and angiography

If the diagnosis is unclear in a patient with suspected anomalous left coronary artery from the pulmonary artery (ALCAPA), cardiac catheterization and angiography may be indicated to definitively evaluate the coronary arteries.

Typically, right ventricular, pulmonary artery and left ventricular end-diastolic, and pulmonary artery wedge pressures are increased. A small shunt (Qp/Qs of approximately 1-1.5) may be present.

Angiography images delineate the ALCAPA. Aortic root, left ventricular, and balloon occlusion angiography of the pulmonary artery can be used to delineate the anatomy in patients with ALCAPA.

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