Case Report

Oblique Coronary Artery Prolongation Approach in Anomalous Left Coronary Artery From the Pulmonary Artery in a Low-Birth-Weight Neonate

World Journal for Pediatric and Congenital Heart Surgery 2015, Vol. 6(2) 328-331 ª The Author(s) 2014 Reprints and permission: sagepub.com/journalsPermissions.nav DOI: 10.1177/2150135114563768 pch.sagepub.com

Joseph J. Franco, DO1, Cesar Igor Mesia, MD2, Ricardo O. Escarcega, MD1, Randy Stevens, MD2, and Achintya Moulick, MD2

Abstract A 19-day-old newborn girl weighing 1.9-kg was born with anomalous origin of the left coronary artery (LCA) from the pulmonary artery (PA). Because of the small anatomic structures and long distance between the anomalous origin of the LCA high within the left posterior sinus of the main PA, a simple transfer of the anomalous LCA to the aorta was deemed impossible. A coronary button was created with an oblique flap of tissue cut from the posterior pulmonary arterial wall. An oblique flap was created from the anterior aortic wall. The two flaps were used to create a tubular prolongation that connected the LCA to the aorta. This created a tension-free anastomosis with potential for growth. The aorta and PA were then patched with pericardium. A left coronary angiogram three years after surgery demonstrated a patent coronary tube. Keywords ALCAPA, coronary anomaly, Takeuchi, oblique aortic flap Submitted April 24, 2014; Accepted October 22, 2014.

Introduction Anomalous origin of the left coronary artery (LCA) arising from the pulmonary artery (ALCAPA) is a rare congenital defect occurring in 1 of the 300,000 live births. The clinical spectrum, originally described in 1933 by Bland et al,1 may include profoundly compromised myocardial perfusion. Mortality in the first year of life approaches 90% in the absence of surgical treatment. A number of successful surgical approaches have been described to treat this condition.2 We present a case of a 19-day-old low-birth-weight (LBW) newborn girl with ALCAPA who underwent a successful reparative operation, despite the challenge related to her small size.

were seen by two-dimensional echocardiography but the coronary flow direction could not be ascertained by color Doppler. A left heart catheterization and aortic root angiogram were performed to define the coronary artery anatomy, which had not been clearly delineated by transthoracic echocardiogram. It demonstrated a patent right coronary artery (RCA) and no evidence of a LCA. A right heart catheterization with angiogram of the main pulmonary artery (PA) showed that the LCA arose from the posterior leftward aspect of the main PA (Figure 1). Our diagnostic approach in young infants with left ventricular dilation and dysfunction is to perform a cardiac catheterization to confirm or exclude the diagnosis of ALCAPA. We follow this approach because the pulmonary vascular resistance in young infants is still high and this may cause echocardiographic

Case Report A 19-day-old 1.9-kg newborn girl was admitted to the neonatal intensive care unit for management of LBW and a left polycystic kidney. She developed progressive respiratory failure. A chest X-ray demonstrated cardiomegaly and increased pulmonary vascular markings. Her echocardiogram revealed a dilated left ventricle with an ejection fraction of less than 10% and severe mitral regurgitation. Two coronary arteries

1 Section of Cardiology, Department of Medicine, Temple University Hospital, Philadelphia, PA, USA 2 Heart Center, St Christopher’s Hospital for Children, Philadelphia, PA, USA

Corresponding Author: Cesar Igor Mesia, Heart Center, St Christopher’s Hospital for Children, 3601 ‘‘A’’ Street, Philadelphia, PA 19134, USA. Email: [email protected]

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Abbreviations and Acronyms ALCAPA LBW LCA PA RCA

anomalous origin of the left coronary artery (LCA) arising from the pulmonary artery low birth weight left coronary artery pulmonary artery right coronary artery

Figure 1. Pulmonary angiogram showing the anomalous left coronary artery from the pulmonary artery (PA).

findings that may be equivocal or incomplete, which could lead to unnecessary delay in surgical therapy in a patient with potential ongoing myocardial damage. She was referred for urgent surgical correction with preparations for postoperative extracorporeal membrane oxygenation if needed. It was felt that the mitral valve should only be addressed if the degree of mitral regurgitation did not improve after establishment of normal LCA perfusion. The chest was opened through midline sternotomy. The patient was cooled to 18 C for optimal myocardial protection, given the severe ventricular dysfunction. The aorta was cross clamped, and a single dose of cardioplegia was introduced antegrade through the aortic root. The branch pulmonary arteries were snared. The pulmonary trunk was transected, and the left coronary ostium was noted high within the left posterior sinus of the main PA. It was felt that direct transfer and primary implantation of the LCA to the aorta was not possible, given the significant distance between the anomalous LCA and the aorta and extensive tissue edema. It was also felt that a tunnel repair in the

style of a Takeuchi operation could create significant obstruction within the small main PA. A coronary button was created with an oblique flap of tissue that was cut out of the posterior wall of the PA. Similarly, a generous oblique flap was created from the aortic root to the distal ascending aorta. The two flaps were constructed into a tube that connected the LCA to the aorta. This created a tension-free anastomosis of the coronary artery to the aorta utilizing only native tissue, with potential for growth (Figure 2). The aorta was then patched with glutaraldehydetreated autologous pericardium. The posterior wall of the PA was also patched with glutaraldehyde-treated pericardium, and the two ends of the pulmonary trunk were reconnected using 6-0 Prolene. We prefer to use glutaraldehyde-treated pericardium as we believe it may decrease potential for aneurysmal formation. Upon completion of the anastomosis, the cross-clamp was removed and the heart spontaneously converted to sinus rhythm after a brief period of ventricular fibrillation. The patient was gradually separated from bypass and was transferred to the cardiac care unit. The patient required delayed sternal closure secondary to significant swelling of the heart. Left ventricular function returned to normal within one week of surgery, and there was mild mitral valve regurgitation within two weeks of surgery. The patient was discharged home from the hospital six weeks after surgery. Discharge was delayed due to medical problems mainly related to slow feeding progress and left multicystic kidney. The patient has maintained normal left ventricular and mitral valve function as seen on serial echocardiograms. The patient underwent a cardiac catheterization and balloon dilation of the main PA at 29 months of age. The left main coronary tube was widely patent (Figure 3). The main PA was moderately narrowed at the site of the previously placed patch, with a peak systolic gradient of 40 mm Hg. The patient responded well to balloon dilation, with a trivial residual peak systolic gradient of 10 to 15 mm Hg at her last follow-up at 44 months of age.

Discussion The ALCAPA was first described in 1866. However, the first clinical description in conjunction with autopsy findings was described by Bland et al in 1933.1 The majority of cases present early in infancy, although very rare cases may present in adulthood. At birth, the infant is typically asymptomatic. At times of stress, particularly with feeding, the child may develop myocardial ischemia in the territory of the LCA.3 This may promote the development of collateral vessels between the RCA and LCA and thus between the aorta and PA. Prolonged ischemia may lead to myocardial hibernation and stunning or to myocardial infarction, which may involve the papillary muscles, resulting in mitral valve regurgitation and congestive cardiac failure.4 Prompt surgical treatment aimed at restoring a two coronary artery system has become the treatment of choice, as our collective understanding of the disease process has evolved. Mitral regurgitation is a common finding in patients with ALCAPA caused by ischemic papillary muscles and left

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World Journal for Pediatric and Congenital Heart Surgery 6(2)

AO

PA

ALCAPA AO AO

RA

OBLIQUE INCISION OBLIQUE INCISION

A RA RA ALCAPA

AO

B

INCISION FURTHER POSTERIORLY

C

PA

AO AO OBLIQUE INCISION PERICARDIAL PATCH

RA

CORONARY BUTTON ROTATED

RA

CORONARY BUTTON ROTATED

E

D Figure 2. Artist’s rendering of operative technique.

ventricular dilation. We do not routinely plan to repair the mitral valve, in view of the fact that improved ventricular function following surgical repair of the coronary anomaly is often accompanied by regression of the mitral valve regurgitation. In one of the earliest reports of surgical therapy, Potts and colleagues created an aortopulmonary anastomosis. The resulting left-to-right shunt had the effect of increasing pulmonary blood flow and increasing the oxygen saturation of blood perfusing the LCA.5 Since that time, a variety of techniques have been developed to treat this anomaly, including carotid or subclavian artery bypass grafting, saphenous vein grafts, internal mammary artery grafting, or isolated ligation of the LCA at its anomalous origin from the PA, a procedure which relegates the entirety of myocardial perfusion to the RCA and its collaterals. In the era of cardiopulmonary bypass, the most popular operation for anatomical correction of ALCAPA is reimplantation of the anomalous LCA by transferring a button of PA tissue to the

aorta.2 However, a number of situations exist where modification on this method may be necessary, including unfavorable coronary anatomy or inadequate length of the native artery. Takeuchi and colleagues described the creation of a coronary tunnel within the body of the PA, made from a baffle of PA wall. The PA is then reconstructed with a patch of pericardium or homograft.6 Although not without complications (supravalvular pulmonary stenosis, baffle leak, or aortic insufficiency), the Takeuchi procedure remains a viable surgical option for patients with anatomy that is considered less than optimal for coronary transfer and reimplantation.7 A number of other surgical techniques have evolved out of a need for creating a tension-free anastomosis. The use of a flap of PA wall with a corresponding flap from the aorta to lengthen the implanted coronary artery was first described in 1992.8 Another iteration involves the use of a noncircumferential segment of the PA to lengthen the coronary artery. This creates a wide tunnel of

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Franco et al

331 it prevents tension on the anastomosis.7,9 Importantly, the coronary tube is made of native tissue with potential for growth.9 Using a tube constructed from PA and ascending aortic tissue, the patient was provided with a durable two coronary system. The variable nature of ALCAPA requires surgical ingenuity in an experienced center. This iteration of surgical repair of the disease may offer an alternative option in select cases. Declaration of Conflicting Interests The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Funding The author(s) received no financial support for the research, authorship, and/or publication of this article.

References

Figure 3. Follow-up left coronary angiogram demonstrating a patent left coronary tube from the aorta.

autologous tissue, allowing the coronary artery to reach the aorta. Reviews of this type of procedure suggest it is a durable repair with a low risk of vessel closure or mortality.8 The distant takeoff of the anomalous LCA and the small PA in this LBW patient precluded the performance of a standard reconstructive operation. Therefore, she required a modified surgical approach. It seemed intuitive to try to create a tube made up of native tissue as the proximal LCA, similar to a technique described by Murthy et al in 20019 but in an oblique fashion. The technique employed by Murthy on a 6-kg baby utilized a horizontal incision and used a flap of aorta as a posterior wall of the neoleft main coronary artery. In contrast, by using a pericardial patch on the aorta and rotating the oblique flap of tissue, we were able to extend the primary anastomosis rather than creating a neotube in our patient. The use of an oblique incision approach helped us compensate for the long distance between the anomalous coronary artery and the aorta. Our novel modification has two advantages. Like other approaches that involve coronary elongation,

1. Bland EF, White PD, Garland J. Congenital anomalies of the coronary arteries: report of an unusual case associated with cardiac hypertrophy. Am Heart J. 1933;8: 787-801. 2. Dodge-Khatami A, Mavroudis C, Backer CL. Anomalous origin of the left coronary artery from the pulmonary artery: collective review of surgical therapy. Ann Thorac Surg. 2002;74(3): 946-955. 3. Keith JD. The anomalous origin of the left coronary artery from the pulmonary artery. Br Heart J. 1959;21(2): 149-161. 4. Brotherton H, Philip RK. Anomalous left coronary artery from pulmonary artery (ALCAPA) in infants: a 5-year review in a defined birth cohort. Eur J Pediatr. 2008;167(1): 43-46. 5. Kittle CF, Diehl Am, Heilbrunn A. Anomalous left coronary artery arising from the pulmonary artery. J Pediatr. 1955;46: 196-203. 6. Takeuchi S, Imamura H, Katsumoto K, et al. New surgical method for repair of anomalous left coronary artery from pulmonary artery. J Thorac Cardiovasc Surg. 1979;78(1): 7-11. 7. Isomatsu Y, Imai Y, Shin’oka T, Aoki M, Iwata Y. Surgical intervention for anomalous origin of the left coronary artery from the pulmonary artery: the Tokyo experience. J Thorac Cardiovasc Surg. 2001;121(4): 792-797. 8. Alsoufi B, Sallehuddin A, Bulbul Z, et al. Surgical strategy to establish a dual-coronary system for the management of anomalous left coronary artery origin from the pulmonary artery. Ann Thorac Surg. 2008;86(1): 170-176. 9. Murthy KS, Krishnanaik S, Mohanty SR, Varghese R, Cherian KM. A new repair of anomalous left coronary artery. Ann Thorac Surg. 2001;71(4): 1384-1386.

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Oblique coronary artery prolongation approach in anomalous left coronary artery from the pulmonary artery in a low-birth-weight neonate.

A 19-day-old newborn girl weighing 1.9-kg was born with anomalous origin of the left coronary artery (LCA) from the pulmonary artery (PA). Because of ...
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