Surgical Approach to Anomalous Left Coronary Artery From the Pulmonary Artery 

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

Overview

Background

Anomalous left coronary artery from the pulmonary artery (ALCAPA), or Bland-White-Garland syndrome,[1]  is a rare but very significant lesion that requires prompt recognition and diagnosis. In most patients with this condition, the origin of the left main coronary artery abnormally originates from the posterior or leftward sinus of the pulmonary artery. Less commonly, the anomalous coronary artery arises from the right pulmonary artery. The branching of the anomalous left coronary artery is typically normal, with normal left anterior descending and circumflex coronary arteries. The origin of the right coronary artery is also normal; however, this vessel is usually enlarged and tortuous. With early diagnosis, the prognosis of ALCAPA is excellent after surgical repair.

ALCAPA affects 1 in 300,000 live US births, representing approximately 0.24-0.46% of congenital heart defects.[2, 3]  No data are available to suggest variance in the frequency of this condition in different countries or between social, economic, or ethnic groups.

 

History of the Procedure

Early surgical intervention for repair of an anomalous left coronary artery from the pulmonary artery (ALCAPA) were palliative, including, but not limited to, the following procedures[3] :

  • 1953: An aortopulmonary anastomosis to increase oxygen saturation in the main pulmonary artery (Potts); a left carotid artery–to–anomalous left coronary artery procedure (Mustard) [4]
  • 1959: Simple ligation of the proximal origin of the anomalous left coronary artery (Sabiston et al) [5]
  • 1966: Saphenous vein grafting from the aorta to the anomalous left coronary artery (Cooley et al) [6]
  • 1968: A left subclavian artery–to–anomalous left coronary artery repair (Meyer et al) [7]

An internal mammary artery–to–ALCAPA procedure has also been performed.[8] Pulmonary artery banding has been attempted to increase perfusion pressure in the anomalous left coronary artery from the pulmonary artery.[9] In addition, procedures to increase collateral circulation, such as poudrage and de-epicardialization, have been tried.[10]

Each of the above procedures has fallen out of favor. At present, establishing a system with today coronary arteries is the goal in definitive surgical repair.

Direct anastomosis of the ALCAPA directly to the aorta has been the procedure of choice since the 1970s.[3] The experience gained in coronary artery transfer during the arterial switch operation has facilitated techniques for coronary transfer to repair the ALCAPA. In most patients, the anomalous left coronary artery is situated in a position that allows for direct transfer of the anomalous coronary artery. When direct transfer of the coronary artery is not feasible, it may be appropriate to create an intrapulmonary aortocoronary tunnel (Takeuchi procedure/repair).[11]

Some patients with ALCAPA and severe cardiac dysfunction may need to undergo cardiac transplantation.

Pathophysiology

The pathophysiology of anomalous left coronary artery from the pulmonary artery (ALCAPA) varies and depends on the patient's age, the pulmonary vascular resistance/pressure, the presence of collateral vessels between the right and left coronary artery systems, and the degree of myocardial ischemia. Four physiologic stages have been described, as outlined below.

Stage 1

In the fetal and early neonatal period, pulmonary vascular resistance is high and pulmonary artery pressure is equal to the aorta pressure. Saturation and perfusion in the  ALCAPA are adequate, and no obvious myocardial ischemia or impairment of left ventricular function is observed.

Stage 2

During the first days to weeks of neonatal life, pulmonary vascular resistance normally decreases. The drop in pressure is inadequate to provide prograde flow into the  ALCAPA.

Flow to the left coronary system is provided by collateral flow from the right coronary artery system. At this time, flow in the  ALCAPA is retrograde.

Collateral flow from the right coronary artery system meets the high resistance of the left ventricular myocardial bed, and preferential flow occurs into the low-resistance pulmonary artery. This leads to the development left ventricular myocardial ischemia. At this time, infants may present with clinical signs of myocardial ischemia.

With the retrograde flow of fully saturated blood into the pulmonary artery, a small left-to-right shunt may be present, detected on cardiac catheterization by a "step up" in oxygen saturation in the pulmonary artery. Usually, the shunt is minimal, and the ratio of pulmonary blood flow (Qp) to systemic blood flow (Qs) ranges from 1 to 1.5.

Stage 3

Rarely, a large collateral circulation may be located between the right and left coronary systems, which may provide adequate myocardial perfusion, allowing infants to have little or no clinical difficulties. These extensive collateral vessels can provide enough coronary flow to allow patients to live to adulthood.

Stage 4

In the final stage, collateral flow is inadequate, retrograde flow into the pulmonary artery persists, and myocardial steal continues. At this stage, adults may present with signs of myocardial ischemia.

Myocardial ischemia

Myocardial ischemia occurs in an anterolateral distribution, resulting in global left ventricular dilatation and dysfunction. Mitral valve regurgitation is common secondary to papillary muscle infarction, mitral annular dilation, or both. Left atrial dilation and pulmonary venous congestion ensue, adding congestive symptoms to those of angina pectoris.

Etiology

The coronary arterial circulation is established by 45 days' gestation in the fetus. Anomalous left coronary artery from the pulmonary artery (ALCAPA) is caused either by abnormal division of the conotruncus or by abnormal involution of endothelial buds that are present on all six sinuses of Valsalva of the great vessels. Usually, all but two of the endothelial buds involute, leaving two buds in the aortic sinuses to eventually become the coronary arteries. With ALCAPA, an endothelial bud sometimes persists on a pulmonary sinus and attaches to the developing left main coronary artery. The left coronary artery can also connect to other locations in the pulmonary artery and has even been reported to connect to one of the branch pulmonary arteries.

Although ALCAPA typically occurs as an isolated defect, it has been associated with congenital defects, including ventricular septal defect, patent ductus arteriosus, and coarctation of the aorta.[3] One case report documented ALCAPA in a patient with hypoplastic left heart syndrome.[12]

Presentation

Historically, the defect of anomalous left coronary artery from the pulmonary artery (ALCAPA) was termed Bland-Garland-White syndrome. In 1933, Bland et al first eloquently described the clinical presenting signs in infants with ALCAPA.[1] The following description is of a 10-week-old infant:

…while nursing from the bottle, the onset of an unusual group of symptoms occurred, which consisted of paroxysmal attacks of acute discomfort precipitated by the exertion of nursing. The infant appeared at first to be in obvious distress, as indicated by short expiratory grunts, followed immediately by marked pallor and cold sweat with a general appearance of severe shock. Occasionally, with unusually severe attacks, there appeared to be a transient loss of consciousness…[1]

Symptoms

Infants present with respiratory distress, feeding intolerance, or failure to thrive. In the rare case involving an older child or adult, the patient may have exertional chest pain, dyspnea, or syncope. Unfortunately, sudden death occurs in some patients following exertion.

Signs

Upon physical examination, infants have an enlarged heart and displaced apical impulse. A gallop rhythm or the holosystolic murmur of mitral regurgitation may be present. Signs of congestive heart failure may be apparent.

The clinical signs of ALCAPA are nonspecific. Myocarditis and cardiomyopathy are other considerations in infants presenting with left ventricular dilation and heart failure. Careful evaluation for the presence of ALCAPA is necessary in any infant presenting with left ventricular dilatation and heart failure.

 

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).

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]  

 

 

 

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.

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.

 

Treatment

Medical Therapy

Medical therapy should be used only to stabilize the patient for surgery. Intubation and mechanical ventilation often are needed in infants who present with shock and cardiac failure. This allows for adequate sedation and analgesia. The goal of analgesia and sedation are to minimize oxygen demands of the failing myocardium. Oxygen therapy is used to treat or prevent hypoxia.

Pharmacotherapy

Inotropic support is often necessary in patients with anomalous left coronary artery connected to the pulmonary artery (ALCAPA). Inotropic agents such as dobutamine or milrinone help to augment cardiac function. Use caution with administering milrinone, because it may lower afterload/blood pressure to such a degree that coronary perfusion may be impaired.

Diuretics (eg, furosemide) are useful to decrease pulmonary venous congestion. Drugs that reduce the workload of the heart include beta-blockers, which aid in neurohormonal modification, left ventricular ejection fraction (LVEF) improvement, arrhythmia prevention, and ventricular rate control, and angiotensin-converting enzyme (ACE) inhibitors, which are also useful for neurohormonal modification, vasodilatation, and LVEF improvement;

Transfusion of packed red blood cells may be useful to increase the oxygen-carrying capacity in patients who have severe anemia.

Indications and Contraindications for Surgical Repair

Indications

Demonstration of the lesion and diagnosis of anomalous left coronary artery from the pulmonary artery (ALCAPA) are an indication for surgical intervention. Prompt preparations should be made for surgical repair. Medical therapy provides a bridge to surgery and should be used to optimize the hemodynamics in the patient during the preoperative period.

Contraindications

Very few contraindications for surgical repair of ALCAPA have been identified. Even in patients with severe disease and poor left ventricular function, revascularization after repair of ALCAPA usually results in improved left ventricular function.

Contraindications for surgical repair include multisystemic end-organ failure and a poor prognosis for survival with or without surgical intervention for the ALCAPA.

Surgical Therapy

Direct transfer of the left coronary artery

Temporary cardiopulmonary bypass and cold blood cardioplegia are used for direct transfer of the left coronary artery. The pulmonary artery is transected, and the anomalous coronary artery is removed as a button of tissue around the ostium of the anomalous coronary artery. This technique is similar to the one used in the arterial switch operation. The proximal coronary is mobilized, and the button is turned posteriorly for direct anastomosis into the aortic root. A slightly smaller button of aortic root is removed, and the coronary button is transposed and sewn into place on the aortic root. The pulmonary artery is then repaired with autologous pericardium.

A retrospective review of long-term data (1980-2012; mean follow-up of 8.16 ± 6.7 years) of 30 patients with ALCAPA who underwent surgical repair with coronary transfer (n = 19), Takeuchi repair (n = 9), or closure (n = 2), demonstrated good survival rates (no deaths) and long-term ventricular function for both coronary transfer and Takeuchi repair.[14]  The 10-year rate of freedom from reintervention was 94.1% and 71.1%, respectively, for coronary transfer and Takeuchi repair. However, at 8-year follow-up, infants who underwent Takeuchi repair were significantly more likely to have at least moderate pulmonary regurgitation compared to those who underwent coronary transfer (79.9% vs 0%, P <0.001).

Takeuchi procedure/repair

Currently, the Takeuchi technique is rarely needed because most surgeons perform direct transfer of the anomalous left coronary artery from the pulmonary artery (ALCAPA), even when the anomalous vessel is transferred over some distance. In the Takeuchi procedure/repair, an aortopulmonary window is constructed. The pulmonary artery is opened, creating an anterior transverse flap of native pulmonary artery tissue, which forms a baffle (conduit) to carry the aortic oxygenated blood to the anomalous coronary artery. The pulmonary artery is then repaired with autologous pericardium.

Complications of the Takeuchi procedure/repair include obstruction of the baffle created between the anomalous coronary artery and supravalvar pulmonary stenosis.

Bypass grafting

The proximal anomalous coronary can be ligated, and bypass grafting may be used to reestablish coronary perfusion. In the past, carotid artery, subclavian artery, and saphenous vein grafts were used. Currently, internal mammary grafting or saphenous venous grafting can be used when direct transfer or the Takeuchi procedure/repair is not feasible.

Variations of direct transfer of the ALCAPA

Several reports have documented variations of the direct transfer of the ALCAPA, including the following:

  • The transected main pulmonary artery is used as a conduit tube in a variation of coronary angioplasty. A conduit tube of native pulmonary artery is anastomosed side-to-side to the aorta.

  • Enlarged autogenous aortic and pulmonary arterial flaps are used to create an extended left main stem coronary artery during anastomosis of the ALCAPA to the aorta.

  • Elongated flaps of the aorta and pulmonary artery are sewn side-to-side to create a tunnel from the ALCAPA to the aorta.

  • In the rare variant of ALCAPA with aortic fusion, unroofing of the intramural portion has been successful. If there is no intramural component, then direct transfer may be required, with care taken to avoid kinking of the artery.[15, 16]

  • The Lecompte maneuver has been used to reduce tension on the anastomosis between the left coronary artery and the aorta, and to prevent compression of the reimplanted left coronary artery by the posterior wall of the pulmonary artery when the left coronary artery arises from the nonfacing sinus.[17, 18]

The advantage of these techniques is that none use prosthetic material to repair the ALCAPA.[17, 18] ​[19]

Intraoperative Details

Upon initial exposure, the dilated dysfunctional left ventricle may be susceptible to fibrillation during manipulation of the heart.

During cardioplegia, both the ascending aorta and the main pulmonary artery are cannulated and cross-clamped. This provides antegrade cardioplegia in the right coronary artery and the anomalous left coronary artery. If cardioplegia is instilled in the ascending aorta only, runoff and steal of cardioplegia into the main pulmonary artery via the anomalous left coronary artery may occur. With the advent of the technique of retrograde cardioplegia, cannulation of the pulmonary artery may be eliminated in some cases.

When choosing the incision site on the aorta for the aortocoronary anastomosis, transverse aortotomy is used to visualize the aortic sinus. This insures optimal location and placement of the coronary button for the aortocoronary anastomosis.

Some centers advocate performing a mitral annuloplasty to treat severe mitral regurgitation. This technique remains controversial because the mitral regurgitation is usually caused by annular dilatation or papillary muscle dysfunction, both of which may improve after revascularization of the left ventricular myocardium and improvement of left ventricular function.

Intraoperative transesophageal echocardiography may be used to help identify and document abnormal flow in the anomalous left coronary artery from the pulmonary artery (ALCAPA) and normal flow in the repaired/transposed coronary artery. Transesophageal echocardiography is also useful for postoperative monitoring of ventricular function and mitral valve regurgitation.

Intraoperative images are shown below.

Intraoperative photograph. (1) A cardioplegia cath Intraoperative photograph. (1) A cardioplegia catheter in the ascending aorta. (2) A cross-clamp on the ascending aorta. (3) A cross-clamp on the main pulmonary artery. (4) An arterial bypass cannula in the main pulmonary artery. (5) A cardioplegia catheter in the main pulmonary artery. (6) The dilated conal branch of the right coronary artery. (7) A venous bypass cannula in the right atrial appendage. (8) A left heart vent.
Intraoperative photograph. (1) A transverse anteri Intraoperative photograph. (1) A transverse anterior incision in the main pulmonary artery trunk. (2) A probe is in the orifice of the anomalous left coronary artery.
Intraoperative photograph. (1) The divided distal Intraoperative photograph. (1) The divided distal main pulmonary artery. (2) The left coronary artery button. (3) The divided proximal main pulmonary artery.
Intraoperative photograph. (1) The left coronary a Intraoperative photograph. (1) The left coronary artery button. (2) The divided proximal main pulmonary artery. (3) A bypass sucker in the transverse aortotomy (to visualize the aortic sinuses). (4) An incision in the aortic sinus for the site of the aortocoronary anastomosis.
Intraoperative photograph. (1) Completing the anas Intraoperative photograph. (1) Completing the anastomosis of the left coronary artery to the aortic sinus. (2) The divided proximal main pulmonary artery.
Intraoperative photograph. (1) The completed anast Intraoperative photograph. (1) The completed anastomosis of the left coronary artery to the aortic sinus. (2) The divided proximal main pulmonary artery. (3) The ascending aorta, transverse aortotomy.
Intraoperative photograph. (1) Suture closure of t Intraoperative photograph. (1) Suture closure of the aortotomy.
Intraoperative photograph. (1) The distal divided Intraoperative photograph. (1) The distal divided main pulmonary artery. (2) Beginning the reanastomosis (posterior wall) of the main pulmonary artery. (3) The proximal main pulmonary artery.
Intraoperative photograph. (1) The completed repai Intraoperative photograph. (1) The completed repair of the main pulmonary artery reanastomosis.

Postoperative Details

Standard postoperative care is performed in the cardiac or pediatric intensive care unit (ICU). Blood products may be needed to control or decrease postoperative bleeding. Mechanical ventilation and inotropic support are typically required in the initial postoperative period. Afterload reduction therapy (eg, nitroprusside) is often used to control postoperative hypertension. Milrinone and epinephrine are used liberally in the immediate postoperative period. In patients in whom separation from cardiopulmonary bypass is difficult, further support with extracorporal membrane oxygenation (ECMO) may be needed. Intra-aortic balloon pump therapy may be used in older children and adults. Serial echocardiography is used to assess for improvement in the left ventricular function and mitral regurgitation.

A retrospective study by Weigand et al indicated that immediate postoperative morbidity in the repair of anomalous left coronary artery from the pulmonary artery (ALCAPA) is associated with patient size, the left ventricular end diastolic dimension (LVEDD), and the preoperative left ventricular shortening fraction. The investigators, who conducted the study on 44 patients, also found that the LVEDD Z-score independently predicted the length of time needed for left ventricular function to normalize and that, in infants, this score independently predicted how long the patient would require postoperative intravenous inotropic support.[20]

Follow-up

Standard follow-up care is required after surgical repair of anomalous left coronary artery from the pulmonary artery (ALCAPA). Local care of the sternotomy incision is advised, and infants and small children should not be lifted by their arms for 6-8 weeks. Outpatient therapy with diuretics (eg, furosemide) and/or afterload reduction (eg, captopril, enalapril) is often used.

Long-term follow-up care includes the use of electrocardiography and echocardiography. In older children and adults, exercise stress testing,[21] including stress echocardiography and nuclear medicine perfusion scans, are useful to assess the patient's functional capacity postoperatively.

Outcome and Prognosis

Even in patients with severe left ventricular dilatation, global left ventricular dysfunction, and mitral regurgitation, the outcome and prognosis is frequently excellent after surgical reimplantation of anomalous left coronary artery from the pulmonary artery (ALCAPA).[22] Prompt diagnosis, medical stabilization, and a coordinated team approach in the operating room and postoperative intensive care unit can facilitate excellent outcomes for this relatively rare congenital defect.

A retrospective study (1994-2015) of 96 Chinese patients with ALCAPA who underwent surgical repair and had a mean follow-up of 10.45 ± 8.96 years found early and late improvement of left ventricular function in most patients.[23] Three patients underwent ligation, six patients underwent ligation and coronary artery bypass grafting, 14 underwent transpulmonary baffling, and 73 patients underwent direct implantation of the anomalous coronary artery into the aorta. Death occurred in three patients (1 early, 2 late).[23]

A retrospective study of data from 33 Japanese infant and older patients who underwent surgical repair for ALCAPA after 1980 and had a median follow-up of 16 years demonstrated overall good postoperative clinical outcomes, but myocardial damage persisted over the long term.[24]  The following were among the study's findings[24] :

  • Postoperative left ventricular ejection fraction improved in infants younger than age 1 year but remain unchanged in those older than 1 year.
  • Postoperative left ventricular asynergy occurred in more than one third (37%) of patients older than 1 year but not in any of the infants younger than 1 year.
  • Both infants and older patients showed postoperative improvement in the severity of mitral regurgitation, but the difference was not significant between the two groups.
  • Both groups had postoperative perfusion defects.
  • Older patients (age >1 year) were more likely to have postoperative cardiovascular events (eg, cardiac death, arrhythmias)

Complications

Surgical complications include bleeding, infection, cardiac arrest/failure, stroke, and the need for further surgery. Most congenital heart surgery programs quote surgical mortality rates at less than 5-10%.

Future and Controversies

Direct transfer of the anomalous left coronary artery from the pulmonary artery (ALCAPA) is the surgical procedure of choice. As specialists at most congenital heart surgery centers have gained more experience with coronary artery transfer with the arterial switch operation, surgical repair of ALCAPA has benefited from refinement of these surgical techniques. With appropriate diagnosis, presurgical stabilization, and team-oriented postoperative care, patients with ALCAPA are expected to have an excellent outcome. Further refinement of long-term follow-up care with specialized stress and functional testing (eg, nuclear medicine perfusion, stress echocardiography, assessment of myocardial strain) is anticipated.[25]