Surgical Approach to Anomalous Left Coronary Artery From the Pulmonary Artery

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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.

 

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] :

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.

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.

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.

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.

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.

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 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.

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]

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]

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.

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.

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Mary C Mancini, MD, PhD, MMM Surgeon-in-Chief and Director of Cardiothoracic Surgery, Christus Highland

Mary C Mancini, MD, PhD, MMM is a member of the following medical societies: American Association for Thoracic Surgery, American College of Surgeons, American Surgical Association, Phi Beta Kappa, Society of Thoracic Surgeons

Disclosure: Nothing to disclose.

Christopher Mart, MD Associate Professor, Pediatric Echocardiography, Department of Pediatrics, Division of Pediatric Cardiology, University of Utah, Primary Children’s Medical Center

Christopher Mart, MD is a member of the following medical societies: American College of Cardiology, American Society of Echocardiography, Society of Pediatric Echocardiography

Disclosure: Nothing to disclose.

John Myers, MD Director, Pediatric and Congenital Cardiovascular Surgery, Departments of Surgery and Pediatrics, Professor, Penn State Children’s Hospital, Milton S Hershey Medical Center

John Myers, MD is a member of the following medical societies: American Association for Thoracic Surgery, American College of Cardiology, American College of Surgeons, American Heart Association, American Medical Association, Congenital Heart Surgeons Society, Pennsylvania Medical Society, Society of Thoracic Surgeons

Disclosure: Nothing to disclose.

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Mary C Mancini, MD, PhD, MMM Surgeon-in-Chief and Director of Cardiothoracic Surgery, Christus Highland

Mary C Mancini, MD, PhD, MMM is a member of the following medical societies: American Association for Thoracic Surgery, American College of Surgeons, American Surgical Association, Phi Beta Kappa, Society of Thoracic Surgeons

Disclosure: Nothing to disclose.

Suvro S Sett, MD, FRCSC, FACS Professor of Surgery, Dalhousie University Faculty of Medicine; Head, Division of Pediatric Cardiac Surgery, Izaak Walton Killam Hospital for Children, Canada

Suvro S Sett, MD, FRCSC, FACS is a member of the following medical societies: American College of Surgeons, Congenital Heart Surgeons Society, Western Thoracic Surgical Association

Disclosure: Nothing to disclose.

Daniel S Schwartz, MD, MBA, FACS Medical Director of Thoracic Oncology, St Catherine of Siena Medical Center, Catholic Health Services

Daniel S Schwartz, MD, MBA, FACS is a member of the following medical societies: American College of Chest Physicians, American College of Surgeons, Society of Thoracic Surgeons, Western Thoracic Surgical Association

Disclosure: Nothing to disclose.

Surgical Approach to Anomalous Left Coronary Artery From the Pulmonary Artery

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