eMedicine Specialties > Pediatrics: Cardiac Disease and Critical Care Medicine > Cardiology

Anomalous Left Coronary Artery From the Pulmonary Artery

Mary C Mancini, MD, PhD, Professor, Department of Surgery, Louisiana State University Health Sciences Center
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

Updated: Nov 7, 2008

Introduction

Background

Anomalous origin of the left coronary artery arising from the pulmonary artery (ALCAPA) is a rare but serious congenital anomaly.

ALCAPA was first described in 1866. The first clinical description in conjunction with autopsy findings was described by Bland and colleagues in 1933, so the anomaly is also called Bland-White-Garland syndrome.¹ By 1962, Fontana and Edwards reported a series of 58 postmortem specimens that demonstrated that most patients had died at a young age.²

Presently, the prognosis for patients with ALCAPA is dramatically improved as a result of both early diagnosis using echocardiography with color flow mapping and improvements in surgical techniques, including myocardial preservation.

The ALCAPA anomaly may result from (1) abnormal septation of the conotruncus into the aorta and pulmonary artery, or from (2) persistence of the pulmonary buds together with involution of the aortic buds that eventually form the coronary arteries.

ALCAPA is usually an isolated cardiac anomaly but, in rare incidences, has been described with patent ductus arteriosus, ventricular septal defect, tetralogy of Fallot, and coarctation of the aorta. Extremely rare variations of anomalous origin of the coronary arteries from the main pulmonary artery include the following:

• The left anterior descending or circumflex branches
• The right coronary, often discovered as an incidental finding on autopsy
• Both the right and left coronary arteries, a circumstance not compatible with survival

Pathophysiology

ALCAPA does not present prenatally because of the favorable fetal physiology that includes (1) equivalent pressures in the main pulmonary artery and aorta secondary to a nonrestrictive patent ductus arteriosus, and (2) relatively equivalent oxygen concentrations due to parallel circulations.

This results in normal myocardial perfusion and, therefore, no stimulus for collateral vessel formation between the right and left coronary artery systems is present. Shortly after birth, as the circulation becomes one in series, pulmonary artery pressure and resistance decrease, as does oxygen content of pulmonary blood flow. This results in the left ventricular myocardium being perfused by relatively desaturated blood under low pressure, leading to myocardial ischemia.

Initially, myocardial ischemia is transient, occurring during periods of increased myocardial demands, such as when the infant is feeding and crying. Further increases in myocardial oxygen consumption lead to infarction of the anterolateral left ventricular free wall. This often causes mitral valve papillary muscle dysfunction and variable degrees of mitral insufficiency.

Collateral circulation between the right and left coronary systems ensues. Left coronary artery flow reverses and enters the pulmonic trunk due to the low pulmonary vascular resistance (coronary steal phenomena). As a result, left ventricular myocardium remains underperfused. Consequently, the combination of left ventricular dysfunction and significant mitral valve insufficiency leads to congestive heart failure (CHF) symptoms (eg, tachypnea, poor feeding, irritability, diaphoresis) in the young infant. Inadequate myocardial perfusion likely causes significant chest pain and these symptoms of myocardial ischemia may be misinterpreted as routine infantile colic.

Frequency

United States

ALCAPA is a rare, congenital cardiac anomaly accounting for approximately 0.25-0.5% of all congenital heart disease. The incidence of ALCAPA does not vary geographically. ALCAPA is not considered an inheritable congenital cardiac defect. No risk factors for the occurrence of ALCAPA in any individual family are known, and ALCAPA is not associated with any syndromes or noncardiac conditions.

Mortality/Morbidity

Left untreated, the mortality rate in the first year of life is 90% secondary to myocardial ischemia or infarction and mitral valve insufficiency leading to CHF. Sudden death may occur because of inadequate collateral circulation between the left and right coronary artery systems.

Race

No predilection is known.

Sex

Occurrence is generally similar between males and females and is not considered an inheritable congenital cardiac defect.

Age

Approximately 85% of patients present with clinical symptoms of CHF within the first 1-2 months of life. In unusual cases, the clinical presentation with symptoms of myocardial ischemia may be delayed into early childhood. Rarely, a patient may stabilize following infarction and present with mitral valve regurgitation later in childhood or even adulthood.

Clinical

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.

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.

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.³

Differential Diagnoses

Cardiomyopathy, Dilated
Coronary Artery Fistula
Mitral Valve Insufficiency
Myocarditis, Viral

Other Problems to Be Considered

Congenital mitral valve disorder
Idiopathic dilated cardiomyopathy

Workup

Laboratory Studies

• Cardiac isoenzymes
  • Laboratory blood tests are not definitive in the diagnosis of anomalous left coronary artery arising from the pulmonary artery (ALCAPA).
  • Although elevation of creatine kinase (CK), MB, or troponin occurs following infarction of cardiac muscle, these tests should not be used for diagnostic purposes.

Imaging Studies

• Chest radiography: This usually demonstrates cardiomegaly, with or without pulmonary venous congestion, although this is not diagnostic for ALCAPA.
• Two-dimensional echocardiography with Doppler color flow mapping
  • This test often is diagnostic and, in some situations, replaces the need for cardiac catheterization and angiography.
  • Echo without Doppler may identify abnormal origin of the left coronary artery from the main pulmonary artery (Media file 3). In unusual circumstances, the anomalous coronary may arise from a branch pulmonary artery, making echocardiographic diagnosis difficult, even with Doppler.
• The use of color flow velocity mapping can be diagnostic, demonstrating retrograde flow from the anomalous left coronary into the pulmonary trunk. The retrograde flow into the pulmonary trunk is typically directed in an unusual orientation within the main pulmonary artery (Media file 4), distinguishing it from the diagnosis of a patent ductus arteriosus.
• Doppler mapping of an abnormal color flow jet will usually identify abnormal retrograde flow within the main pulmonary artery in both late systole and diastole (Media file 5). The mapping image partially depends on pulmonary artery pressure.
• The presence of retrograde flow is dependent on the development of collaterals between the left and right coronary artery systems. If collateralization has not occurred, as may be the case with a very early age presentation, this finding may be absent.
• Abnormal dilation of the proximal right coronary artery, when present, reflects development of extensive collateralization between the right and left coronary artery systems in those patients who present later in infancy or in childhood.
• An additional finding, which is not sensitive but highly specific, is abnormal "brightness" (echogenicity) of left ventricular papillary muscles and sharply delimited sectors of the left ventricular endocardial surface.
• Variable degrees of mitral valve regurgitation, left ventricular dysfunction, and wall motion abnormalities may be identified.⁴

Other Tests

• Twelve-lead electrocardiography
  • Typically, an anterolateral infarct pattern with abnormal deep (>3 mm) and wide (>30 msec) q waves is observed in leads I, aVL, V₅, and V₆, absent q waves in leads II, III, and aVF, and poor R wave progression across the precordial leads, with sudden shift to qR. Electrocardiography (ECG) detects abnormalities of repolarization in the form of ST-segment depression or inversion, both inferior and lateral (Media file 1). The QRS axis is typically normal, although, in some cases, a left superior axis is seen.
  • Following successful surgical revascularization, the ECG may revert to normal findings with the disappearance of the pathologic q waves and ST-T wave changes (Media file 2).

Procedures

• Cardiac catheterization and angiography
  • Angiographic evaluation of the coronary artery system should be performed despite a negative echocardiogram if either the clinical history or ECG is strongly suggestive.
  • Hemodynamic measurements are usually consistent with low cardiac output and elevated left atrial pressures secondary to reduced left ventricular compliance or significant mitral valve insufficiency.
  • Oximetry may show a small left-to-right shunt into the pulmonary arteries.
  • Aortography or selective right coronary arteriography usually demonstrates an enlarged right coronary artery system with collateralization to the left coronary artery and eventual reflux of contrast into the pulmonary arterial system (Media file 6).
  • If collateralization has not occurred, identification of the anomalous left coronary artery may not be evident by aortography or selective right coronary arteriography.
• Stop flow angiography
  • With a large bolus of contrast under high pressure, an alternative approach is to perform a balloon occlusion angiogram within the distal main pulmonary artery, which retrogradely should fill the anomalous left coronary artery (Media file 7).
  • Though rare, false-negative results with this technique may be caused by incomplete occlusion of the main pulmonary artery or by balloon malposition. A balloon positioned in the proximal main pulmonary artery may occlude the orifice of the anomalous left coronary. Alternatively, if the anomalous left coronary artery arises from the left pulmonary artery, positioning the balloon in the distal main pulmonary artery may prevent contrast from entering the coronary artery.⁵

Treatment

Medical Care

• Initial management of anomalous left coronary artery from the pulmonary artery (ALCAPA) is both supportive and temporary. Treatment of congestive heart failure (CHF) includes carefully using diuretics, afterload reduction medications, and inotropic drugs.
• Although systemic oxygen transport may be reduced in the presence of low systemic blood flow, using 100% oxygen may be deleterious. Oxygen may further reduce pulmonary vascular resistance and magnify coronary steal from the right coronary artery into the pulmonary arteries.
• A similar phenomenon occurs with aggressive afterload reduction, during which right coronary artery perfusion may be reduced, leading to decreased left coronary blood flow.
• Inotropic support, on the other hand, may significantly increase myocardial oxygen consumption, which, in the presence of reduced myocardial blood flow, may result in worsening ischemia.
• Increasing reports of catheter intervention for this lesion are emerging. The results in these instances remain conflicting. Surgical intervention remains the procedure of choice.

Surgical Care

• Spontaneous resolution of CHF symptoms is rare. Surgical revascularization of the left coronary artery system is usually necessary.
• Once the patient is stabilized, perform surgical revascularization to create a 2 coronary artery system. Over the years, the following techniques have been advocated:
  • Ligation of the left coronary artery at its origin from the main pulmonary artery is an original technique, performed without the use of cardiopulmonary bypass. The long-term results were not optimal since myocardial perfusion remained solely dependent on extensive collateralization from the right coronary artery, and the patient remained at risk for ischemic episodes and sudden death.
  • Current surgical procedures are directed at establishing revascularization by creating a 2 coronary artery system via either (1) a left subclavian artery-coronary artery anastomosis, (2) a saphenous vein bypass graft, (3) Takeuchi procedure (creation of an aortopulmonary window and an intrapulmonary tunnel extending from the anomalous ostium to the window), or (4) direct reimplantation. By establishing a patent 2 coronary artery system, most patients experience normalization of left ventricular systolic function, thereby improving long-term survival.
  • The need for simultaneous mitral valve reconstruction, in the presence of severe insufficiency, is controversial because spontaneous improvement of mitral valve function often occurs following surgical revascularization.
  • Once revascularization to a 2 coronary artery system is accomplished, most patients demonstrate improved left ventricular systolic function, decreased mitral valve insufficiency, and resolution of CHF symptoms. In many cases, the classic infarct pattern on electrocardiography eventually disappears following normalization of left coronary blood flow (see Media file 2). Occasionally, persistent refractory mitral regurgitation will necessitate delayed mitral valve repair or replacement.
• Postoperative care, precautions, and complications
  • Diuretics, and afterload reduction may be necessary until there is significant improvement in left ventricular systolic and diastolic function with resolution of mitral valve insufficiency. These medications improve cardiac output and eliminate the preoperative symptoms of congestive heart failure.
  • Although unusual, there remains a risk of cardiac dysrhythmia secondary to preoperative myocardial ischemia or infarction. Monitor continuously in the immediate postoperative period.^3,6,7,8

Consultations

Pediatric cardiologist; Pediatric cardiothoracic surgeon

Diet

No specific postoperative restrictions are usually necessary.

Activity

• Restrictions are directly related to the severity of left ventricular dysfunction and postoperative mitral valve insufficiency.
• No specific recommendations are necessary since the majority of patients are infants. For patients who are able to participate in exercise or competitive sports or those with residual postoperative hemodynamic problems, consider recommending avoidance of significant isometric activities.

Medication

Medications used at the time of presentation in patients with anomalous left coronary artery from the pulmonary artery (ALCAPA), including the judicious use of diuretics, focus on afterload reduction and inotropic support for the treatment of congestive heart failure (CHF) symptoms. Except for diuretics, medications may have immediate deleterious effects that could lead to worsening myocardial ischemia, further reductions in cardiac output, and the potential for ventricular arrhythmias. Following surgical revascularization, these same medications may be used more aggressively for the continued treatment of CHF, left ventricular dysfunction, and mitral valve insufficiency.

Diuretics

These agents promote excretion of water and electrolytes by the kidneys. They are used to treat heart failure or hepatic, renal, or pulmonary disease when sodium and water retention has resulted in edema or ascites. These medications ease the work of breathing by decreasing the degree of pulmonary venous congestion (pulmonary edema) secondary to mitral valve insufficiency or elevated left atrial pressures resulting from diminished left ventricular compliance. Diuretics also may decrease systemic venous congestion (preload reduction) if right heart failure also has occurred.

Furosemide (Lasix)

Increases excretion of water by interfering with chloride-binding cotransport system, which, in turn, inhibits sodium and chloride reabsorption in ascending loop of Henle and distal renal tubule. Diuretic effect occurs within 10-20 min following an IV dose and peaks 1-1.5 hours later.

Dosing

Adult

1-2 mg/kg/dose PO/IV; may be repeated q6-8h prn

Pediatric

Administer as in adults

Interactions

Metformin decreases concentrations; interferes with hypoglycemic effect of antidiabetic agents and antagonizes muscle-relaxing effect of tubocurarine; auditory toxicity appears to be increased with coadministration of aminoglycosides; hearing loss of varying degrees may occur; anticoagulant activity of concurrent warfarin may be enhanced; increased plasma lithium levels and toxicity are possible when taken concurrently

Contraindications

Documented hypersensitivity; hepatic coma; anuria; severe electrolyte depletion

Precautions

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

Avoid use in presence of significant hypokalemia, which increases risk of cardiac arrhythmias

Afterload-reducing agents

These agents improve preoperative or postoperative cardiac output by reducing systemic vascular resistance and increasing systemic blood flow resulting from myocardial dysfunction and/or significant mitral valve insufficiency. Nitrates are peripheral and coronary vasodilators used in the management of angina pectoris, heart failure, and myocardial infarction. ACE inhibitors are beneficial in all stages of chronic heart failure. Pharmacologic effects result in a decrease in systemic vascular resistance, reducing blood pressure, preload, and afterload. Dyspnea and exercise tolerance are improved.

Nitroprusside (Nitropress)

Vasodilator of choice for severe, low-output, left-sided heart failure, providing that the arterial pressure is reasonably maintained. Rapidly acts and has a balanced effect, dilating both arterioles and veins. Because of an increase in stroke volume, considerable hemodynamic improvement without much hypotension may occur. In general, some decrease in blood pressure occurs, which may limit therapeutic effect. No PO equivalent is available.

Dosing

Adult

0.3-0.5 mcg/kg/min IV initially; titrate to effect in increments of 0.5 mcg/kg/min; average dose is 1-6 mcg/kg/min
Infusion rates >10 mcg/kg/min may lead to cyanide toxicity

Pediatric

Administer as in adults

Interactions

Synergistic with other systemic vasodilators

Contraindications

Documented hypersensitivity; subaortic stenosis; idiopathic hypertrophic and atrial fibrillation or flutter

Precautions

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

Caution in increased intracranial pressure, hepatic failure, severe renal impairment, and hypothyroidism; in renal or hepatic insufficiency, levels may increase and can cause cyanide toxicity; sodium nitroprusside has the ability to lower blood pressure and, thus, should be used only in those patients with mean arterial pressures >70 mm Hg

Captopril (Capoten)

Angiotensin converting enzyme (ACE) inhibitors have a major role as a peripheral vasodilator in hypertension and CHF. They act on angiotensin-renin-aldosterone system by inhibition of ACE. Prevents conversion of angiotensin I to angiotensin II, a potent vasoconstrictor, resulting in increased levels of plasma renin and a reduction in aldosterone secretion. This is most beneficial when CHF is accompanied by high plasma renin activity that leads to increased sympathetic activity, aldosterone release, and peripheral vasoconstriction. Use of ACE inhibitors usually is reserved for long-term postoperative management, at which point, the severity of myocardial dysfunction and mitral valve insufficiency has improved significantly to allow the use of PO medications.

Dosing

Adult

6.25-12.5 mg PO tid; not to exceed 150 mg tid

Pediatric

0.5-2 mg/kg/d PO divided tid
Test dose to evaluate the patient's blood pressure response may be necessary before institution of the normal daily dose

Interactions

NSAIDs may reduce hypotensive effects; ACE inhibitors may increase digoxin, lithium, and allopurinol levels; rifampin decreases levels; probenecid may increase levels; the hypotensive effects of ACE inhibitors may be enhanced when given concurrently with diuretics

Contraindications

Documented hypersensitivity; renal impairment; bilateral renal artery stenosis

Precautions

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

Caution in renal impairment, valvular stenosis, or severe CHF; first-dose hypotension may occur, especially in patients with high renin states

Phosphodiesterase Enzyme Inhibitor

Milrinone

Bi-pyridine positive inotrope and vasodilator with little chronotropic activity. Different in mode of action from both digitalis glycosides and catecholamines. Selectively inhibits phosphodiesterase type III (PDE III) in cardiac and smooth vascular muscle, resulting in reduced afterload, reduced preload, and increased inotropy.

Dosing

Adult

50 mcg/kg IV loading dose over 10 min followed by continuous infusion at 0.375-0.75 mcg/kg/min

Pediatric

Administer as in adults; although used as DOC in many pediatric intensive care units, safety and efficacy not well established. Typical dosing is 0.25-0.50 mcg/kg/min. There is some evidence, in adults, that doses about 0.4 mcg/kg/min increase myocardial oxygen supply-demand mismatch, which could be deleterious in ALCAPA. The use of a loading dose at initiation of therapy is controversial.

Interactions

Incompatible with furosemide when administered within same IV (forms precipitates)

Contraindications

Documented hypersensitivity to milrinone, any component, or inamrinone

Precautions

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

Monitor fluids, electrolyte changes and renal function during therapy (decrease dose with insufficient renal function); excessive diuresis may increase potassium loss and predispose digitalized patients to arrhythmias; important to correct hypokalemia with potassium supplementation prior to treatment; patients showing excessive decreases in blood pressure should have infusion rates slowed or stopped; previous vigorous diuretic therapy has caused significant decreases in cardiac filling pressure, cautiously administer milrinone and monitor blood pressure, heart rate, and clinical symptomatology

Follow-up

Further Inpatient Care

• The severity of symptoms in patients with anomalous left coronary artery from the pulmonary artery (ALCAPA) at presentation determines whether the patient is admitted to an intensive care unit (ICU) for aggressive medical management of congestive heart failure (CHF) before surgical revascularization.
• Initial postoperative management occurs in a pediatric ICU until the patient is extubated and no longer requires intravenous inotropic support or antiarrhythmics.
• Following surgical revascularization, postoperative care includes the use of inotropes, diuretics, and afterload reduction medication to improve cardiac output and to eliminate the preoperative symptoms of CHF.
• Monitor continuously during the immediate postoperative period because there is a risk, although unusual, of cardiac dysrhythmia secondary to preoperative myocardial ischemia or infarction.

Further Outpatient Care

• The clinical status of the patient, in relation to residual CHF symptoms, determines the frequency of postoperative outpatient follow-up visits.
• Most patients do not require frequent cardiac evaluation following surgical revascularization once ventricular function and mitral valve insufficiency is dramatically improved.

Inpatient & Outpatient Medications

• The short-term use of oral digoxin, diuretics, and ACE inhibitors is common following surgical revascularization.

Complications

• Complications are rare. The need for future valve surgery depends on the occurrence of hemodynamic complications (eg, residual mitral valve insufficiency precipitated by permanent damage of the mitral valve architecture) following surgery.
• Late complications related to coronary artery insufficiency are more likely to occur if revascularization was accomplished by any of the following:
  • Surgical ligation
  • Bypass grafts that may become occluded or stenotic
  • Intrapulmonary tunnel technique, which may cause supravalvar pulmonary stenosis or, less commonly, become obstructed at the surgically created aortopulmonary window
• Inadequate growth of the coronary anastomosis is possible, although unlikely, if surgical reimplantation of the left coronary artery was performed. This occurrence is similar to the rare reports of late coronary artery problems following the arterial switch procedure for transposition of the great vessels that also requires direct coronary transfer and reimplantation.

Prognosis

• Early diagnosis using echocardiography with color flow mapping and improvements in surgical techniques (eg, myocardial preservation) dramatically improve prognosis.

Patient Education

• All patients should undergo formal exercise stress testing at an appropriate age as an aid in determining an appropriate exercise program.
• Long-term physical restrictions, including restrictions of participation in competitive sports, are a direct function of whether myocardial ischemia is evident at rest or during exercise.
• No dietary restrictions are necessary following successful surgical revascularization with subsequent clinical improvement.

Miscellaneous

Medicolegal Pitfalls

• The most serious, yet avoidable, medicolegal complication is the mistaking of symptoms in the patient older than several months who presents with anomalous left coronary artery from the pulmonary artery (ALCAPA) as idiopathic dilated cardiomyopathy or end stage myocarditis. The initial presentation may be entirely similar but the treatments are dramatically different. The clinical and initial laboratory findings may be remarkably similar. If there is significant pulmonary artery hypertension, the diagnostic ECG findings may not be apparent.
• A high index of suspicion is necessary to make the diagnosis of ALCAPA, since surgical treatment is extremely successful and results in an excellent long-term prognosis.

Special Concerns

• With successful surgical revascularization and resolution of myocardial dysfunction in female patients, pregnancy and normal vaginal delivery are not contraindicated.
• In patients with persistent myocardial dysfunction, mitral valve insufficiency, or coronary ischemia, the ability to carry a pregnancy to term varies. These patients should be considered high risk and require close observation throughout the pregnancy by both a cardiologist and a perinatologist because the increase in blood volume may be deleterious to the patient's hemodynamic status. A normal vaginal delivery may also be contraindicated in this situation.

Multimedia

Media file 1: 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.

Media file 2: 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.

Media file 3: 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.

Media file 4: 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.

Media file 5: 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.

Media file 6: 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).

Media file 7: 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.

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Keywords

anomalous left coronary artery from the pulmonary artery, ALCAPA, Bland-White-Garland syndrome, abnormal septation of the conotruncus into the aorta and pulmonary artery, persistence of the pulmonary buds together with involution of the aortic buds that form the coronary arteries, cardiac anomaly, myocardial ischemia, infarction of the anterolateral left ventricular free wall, congestive heart failure symptoms, CHF symptoms, heart defect, colic-like symptoms, coliclike symptoms, patent ductus arteriosus, ventricular septal defect, tetralogy of Fallot, coarctation of the aorta

Contributor Information and Disclosures

Author

Mary C Mancini, MD, PhD, Professor, Department of Surgery, Louisiana State University Health Sciences Center
Mary C Mancini, MD, PhD is a member of the following medical societies: American Heart Association, American Medical Association, American Thoracic Society, Association for Academic Surgery, Association for Surgical Education, International College of Surgeons, International Society for Heart and Lung Transplantation, New York Academy of Sciences, Phi Beta Kappa, and Southern Thoracic 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.

Medical Editor

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.

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine
Disclosure: Pfizer Inc Stock Investment from broker recommendation; Avanir Pharma Stock Investment from broker recommendation

Managing Editor

Alvin J Chin, MD, Professor of Pediatrics, Division of Cardiology, The Children's Hospital of Philadelphia, University of Pennsylvania School of Medicine
Alvin J Chin, MD is a member of the following medical societies: American Association for the Advancement of Science and American Heart Association
Disclosure: Nothing to disclose.

CME Editor

Gilbert Herzberg, MD, Assistant Professor, Department of Pediatrics, Section of Pediatric Cardiology, New York Medical College
Gilbert 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.

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