Approach Considerations
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 short-term use of inotropes (eg, oral digoxin), diuretics, and afterload reduction medication (eg, angiotensin converting enzyme [ACE] inhibitors) 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.
Medical Care
Initial management of anomalous left coronary artery from the pulmonary artery (ALCAPA) is both supportive and temporary. Treatment of congestive heart failure 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, however, 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.
Consultations
Consult with a pediatric cardiologist and a pediatric cardiothoracic surgeon.
Diet and activity
No specific postoperative dietary restrictions are usually necessary.
Activity restrictions are directly related to the severity of left ventricular dysfunction and postoperative mitral valve insufficiency. No specific activity recommendations are necessary because 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.
Surgical Care
Spontaneous resolution of congestive heart failure (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 two–coronary artery system. Over the years, various techniques for this 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. However, this surgical option is no longer recommended: 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 two–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. [17] By establishing a patent two–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 two–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 the image below). Occasionally, persistent refractory mitral regurgitation will necessitate delayed mitral valve repair or replacement.
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.
A study of 23 infants indicated that in patients with anomalous left coronary artery from the pulmonary artery (ALCAPA), aortic reimplantation of the anomalous coronary artery is an effective means of improving myocardial function but is a less effective tool for treating severe mitral valve regurgitation. In 16 infants, the anomalous artery was directly implanted into the ascending aorta, while the seven remaining patients underwent repair with a trapdoor flap or tubular extension technique. The investigators evaluated left ventricular function and degree of mitral valve regurgitation over a 10-year follow-up period. Four of the patients died early in the postoperative period (within 12 days after surgery), but improvement in myocardial function was seen in all of the remaining patients. However, of five infants diagnosed preoperatively with severe mitral valve regurgitation, only one demonstrated improvement in this condition; two patients required mitral valve replacement. [18]
In another study, a comparison of coronary transfer and Takeuchi repair (intrapulmonary tunnel) revealed equal improvement of left ventricular function and resolution of mitral valve regurgitation in both groups. [19] However, patients undergoing the Takeuchi technique developed significant pulmonary regurgitation, whereas those that had coronary transfer did not develop this complication. [19]
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. [4, 20, 21, 22]
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. However, follow-up reevaluation, although infrequent should be performed.
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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.
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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.
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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.
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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.
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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.
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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).
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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.
