Ann Thorac Surg 2000;69:1259-1261
© 2000 The Society of Thoracic Surgeons
CASE REPORTS
Anomalous pulmonary artery from the aorta via a patent ductus arteriosus: repair in a premature infant
Khaled J. Salaymeh, MDa,
Thomas R. Kimball, MDa,
Peter B. Manning, MDb
a Division of Cardiology, Children’s Hospital Medical Center, Cincinnati, Ohio, USA
b Division of Cardiothoracic Surgery, Children’s Hospital Medical Center, Cincinnati, Ohio, USA
Address reprint requests to Dr Salaymeh, Division of Cardiology, Children’s Hospital Medical Center, 3333 Burnet Ave, Cincinnati, OH 45229-3039
e-mail: salaymeh{at}fuse.net
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Abstract
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A successful repair of anomalous left pulmonary artery from the ascending aorta via a left ductus arteriosus in a 1 kg baby is reported. Repair was performed at an early age to avoid pulmonary hypertension and left pulmonary artery occlusion. Utilizing the right ductus to perfuse the right lung, surgery was performed without cardiopulmonary bypass.
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Introduction
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The left pulmonary artery (LPA) arising from the aorta is a rare anomaly, occasionally being associated with tetralogy of Fallot. As an isolated lesion, anomalous LPA from the aortic arch is extremely uncommon and was first described in 1941 [1]. Weintraub and colleagues [2] reported the first successful surgical repair in a 7-year-old child. We report a unique case in which diagnosis and successful repair were performed in a 1,010 g 10-day-old infant.
C.D. was born at 28 weeks gestational age by cesarean section due to late decelerations with a birth weight of 1,055 g. He developed respiratory distress syndrome and was subsequently intubated and ventilated. Physical examination demonstrated vital signs of heart rate = 176, respiratory rate = 66, blood pressure = 94/58, and oxygen saturations = 85% to 90% on room air. Cardiovascular examination showed slightly increased precordial activity, normal S1 and S2, and a grade 2/6 systolic ejection murmur at the left mid and upper sternal border. An electrocardiogram demonstrated sinus rhythm and right atrial enlargement. A chest roentgenogram demonstrated mild to moderate cardiomegaly and a symmetric increase in pulmonary vascular markings.
An echocardiogram was obtained at 5 days of life because of suspicion of a patent ductus arteriosus. This demonstrated situs solitus vicera and atria, D-looped ventricular topology, normal intracardiac anatomy, normally related great vessels, and a right-sided aortic arch. There was an isolated LPA arising from a left-sided ductus proximal to the origin of the left brachiocephalic artery (Fig 1). The right pulmonary artery (RPA) originated normally from the main pulmonary artery. In addition, there was a right-sided patent ductus arteriosus (PDA) between the transverse aortic arch and the RPA.
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Fig 1. (A) Subcostal coronal echocardiographic view demonstrating the left pulmonary artery (LPA) arising from the transverse aorta (AO) via the left patent ductus arteriosus (LPDA). (B) Suprasternal short axis view with color Doppler demonstrating the LPDA and the descending aorta (DAo). (LV = left ventricle; RA = right atrium; RPA = right pulmonary artery; RV = right ventricle.)
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At 10 days old, the patient developed signs of excessive pulmonary blood flow. A repeat echocardiogram demonstrated narrowing of the left-sided ductus arteriosus, whereas the right-sided ductus remained widely patent.
Due to concerns that left ductus arteriousus closure would adversely affect LPA development, prostaglandin E1 (PGE) was begun and surgery was scheduled. During the surgical procedure, the anatomy was confirmed (Fig 2A). PGE infusion allowed intraoperative occlusion of the main pulmonary artery without cardiopulmonary bypass during LPA reimplantation, with pulmonary blood flow to the right lung supplied by the right ductus arteriosus. Ductal tissue of slightly more than 2 mm was excised from the LPA. The LPA was then directly reimplanted to the main pulmonary artery with a running 8-0 Prolene (Ethicon, Somerville, NJ) suture (Fig 2B). The patient was maintained on minimal oxygen and hypoventilated in an effort to keep pCO2 between 45 and 48 mm Hg. PGE was discontinued in the operating room after ligating the right ductus arteriosus.
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Fig 2. (A) Intraoperative view demonstrating the left pulmonary artery (LPA) arising from the distal ascending aorta (AAo). (B) Intraoperative view demonstrating the implantation of the LPA into the main pulmonary artery (MPA).
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After surgery, the patient remained intubated for 1 week. A radionuclide pulmonary perfusion scan performed 2 weeks after surgery demonstrated right lung blood flow = 67% and left lung blood flow = 33%. The discrepancy was expected due to the marked size difference noted between the RPA and LPA. A follow-up echocardiogram performed 3 months postoperatively demonstrated a slightly small LPA and a normal right ventricular pressure. At 10 months postoperatively the patient had normal findings upon cardiac examination and electrocardiography.
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Comment
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In a review of the literature [1–7], there are only seven reported cases of isolated LPA arising from a right-sided aortic arch with normal intracardiac structures. In our report the diagnosis was made by echocardiogram in a 1,010 g infant. The most useful views were the suprasternal long and short axis and the subxiphoid sagittal and coronal views. Particularly helpful were the suprasternal sweeps anteriorly and posteriorly in which the branching pattern of the normal and aberrant (left ductus arteriosus to LPA and right ductus arteriosus to RPA) vessels could be delineated clearly. Color Doppler echocardiography was especially useful in tracking the distal course of the anomalous vessels. The superior-inferior orientation of the left ductus arteriosus and its course may be misinterpreted initially as a left descending aorta which when placed in the perspective of a right aortic arch may lead to a false diagnosis of double aortic arch. Careful delineation of the course of the left ductus arteriosus must be performed.
It is evident from review of previously reported cases [1–7] and our case that early repair is the key in treating this anomaly because of the risk of early pulmonary obstructive disease. Further, closure of the left ductus would eventually sacrifice the LPA. Our patient developed increased pulmonary blood flow at 10 days of life, and by 14 days had evidence of left ductus stenosis and LPA hypoplasia. The unique feature in our case was the patient size (1,010 g), and the successful repair without bypass, using the right ductus to perfuse the RPA intraoperatively. It is recommended that patients with this anomaly be repaired as early as possible to avoid pulmonary hypertension. In addition, LPA occlusion can be avoided by intervening as soon as possible.
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References
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Thomas H.W. Cardiovascular anomalies, congenital cardiac malformations. J Tech Methods 1960;58:21-29.
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Weintraub R.A., Fabian C.E., Adams D.F. Ectopic origin of one pulmonary artery from the ascending aorta. Radiology 1966;86:666-676.[Medline]
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Mittal P.K., Agarwal S.K., Ghosh P.K. Isolated anomalous origin of left pulmonary artery from ascending aorta in an adult. J Thorac Cardiovasc Surg 1993;106:1220-1223.[Medline]
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Accepted for publication August 25, 1999.
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Abstract
Introduction
