Ann Thorac Surg 2005;80:1917-1918
© 2005 The Society of Thoracic Surgeons
Case report
Anomalous Origin of the Right Pulmonary Artery From the Ascending Aorta With Fibrous Continuity to the Pulmonary Trunk
Martin Kostolny, MD
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,
Ergin Kocyildirim, MD,
Marc R. de Leval, MD,
Robert H. Anderson, MD
Cardiothoracic Unit, Great Ormond Street Hospital for Children NHS Trust, and the Institute of Child Health/University College London, London, United Kingdom
Accepted for publication May 26, 2004.
* Address correspondence to Dr Kostolny, Cardiothoracic Unit, Great Ormond Street Hospital for Children, Great Ormond St, London WC1N 3JH, UK (Email: kostom{at}gosh.nhs.uk).
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Abstract
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A 1-month-old patient was brought to our institution with clinical signs of pulmonary hypertension. Cross-sectional echocardiography suggested a diagnosis of aortopulmonary window. At the time of surgery, we found that the right pulmonary artery was arising anomalously from the left side of the ascending aorta, but was also connected to the pulmonary trunk by a fibrous cord. We reimplanted the right pulmonary artery into the pulmonary trunk, closing the resultant opening in the ascending aorta by direct suture. The postoperative course was uneventful. On follow-up, the patient is asymptomatic without medication.
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Introduction
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Anomalous origin of the right pulmonary artery from the ascending aorta is well described but, as far as we know, there is only one report that describes its origin from the left side of the ascending aorta [1]. We have now encountered a patient in whom the right pulmonary artery, as in that case, took its origin from the left lateral aspect of the ascending aorta. In our case, however, the anomalous artery was additionally connected to the pulmonary trunk by a fibrous cord. We believe this variant is unique. In this report we illustrate our findings and discuss the differential diagnoses.
A 1 month-old female patient was referred to our institution. On examination, she had clinical signs of increased flow of blood to the lungs. The electrocardiogram was suggestive of mild right ventricular hypertrophy. The initial echocardiogram performed at the referring hospital had revealed a structurally normal heart, but the systolic pulmonary arterial pressure had been estimated at 60 mm Hg in the absence of any obstruction in the right ventricular outflow tract. Because of the clinical condition, combined with the echocardiographic features, the initial diagnosis had been of primary pulmonary hypertension.
When examined in our institution, the chest roentgenogram showed moderate cardiac enlargement with increased pulmonary vasculature; and our echocardiogram showed normal intracardiac anatomy, systemic pulmonary arterial pressures, moderate dilation of both ventricles, slight turbulence in the right pulmonary artery, and diastolic retrograde flow in the descending aorta and in the aortic arch, with no signs of coarctation or patency of the arterial duct.
Because of the severe pulmonary hypertension, the patient was commenced on increasing doses of Sildenafil (Pfizer, UK), as much as 1 mg per kilogram given five times daily, and the echocardiogram was reviewed. It was then suggested that the retrograde diastolic flow through the descending aorta and the aortic arch could be due to an aortopulmonary window. A further echocardiogram (Fig 1) was then interpreted as showing a communication between the ascending aorta and the right pulmonary artery, measuring 5 to 6 mm in diameter, and permitting a continuous shunt with a maximum velocity of 2 m per second. In view of these echocardiographic findings, it was decided to proceed with surgical repair. Sildenafil therapy was maintained until the operation.
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Fig 1. The preoperative echocardiogram in apical four-chamber view shows a communication between the right pulmonary artery and the ascending aorta (bracket).
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The operation was performed through a midline sternotomy, using standard aortic and bicaval cannulation. Both right and left pulmonary arteries were snared. The arterial duct was found to be patent, and was ligated. The aorta was clamped, and cold blood cardioplegia was given in antegrade fashion into the ascending aorta. It was then seen that the proximal part of the right pulmonary artery, which arose anomalously from the left side of the ascending aorta, was also connected by a fibrous cord to the pulmonary trunk (Fig 2). The cord was divided, and the adventitia on the pulmonary trunk was reinforced with suture. The anomalous pulmonary artery was disconnected from the ascending aorta, and the resultant opening in the ascending aorta was closed primarily in two layers with continuous 7-0 Prolene (Ethicon, Somerville, NJ) sutures. An opening was created in the pulmonary trunk, to which the right pulmonary artery was reimplanted with continuous 7-0 Prolene (Fig 3).
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Fig 2. Operative view of the right pulmonary artery arising from the left side of the ascending aorta. Note the fibrous string connecting to the pulmonary trunk (arrow).
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The patient had an uncomplicated postoperative recovery, and was discharged home on the fifth postoperative day. The echocardiogram before discharge showed a good repair, with laminar flow across both pulmonary arteries, and regurgitant jet across the tricuspid valve with low velocity. At follow-up 5 months after the surgical repair, the heart sounds were normal and no murmurs were heard. There was no hepatomegaly. Cross-sectional echocardiography demonstrated good function of the heart, with only mild right ventricular hypertrophy. The flow was laminar in both pulmonary arteries, with no tricuspid regurgitation. The oval foramen was minimally patent, permitting some flow from left-to-right. The electrocardiogram showed normal sinus rhythm, with no obvious right ventricular hypertrophy.
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Comment
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It is now well established that anomalous origin of the right pulmonary artery from the ascending aorta can now be established using echocardiography alone. Angiography is usually reserved for older patients to assess pulmonary vascular changes [1, 2], since it is also well established that the malformation is associated with pulmonary hypertension not only in the lung perfused by the anomalous artery, but also in the lung fed by the pulmonary artery conveying blood from the right ventricle. Early surgical correction, therefore, is mandatory to prevent permanent hypertensive changes in the pulmonary vasculature. Direct reimplantation is the surgical technique of choice, albeit that this could be difficult or impossible if the anomalous origin of the pulmonary artery is distant from the pulmonary trunk. Numerous techniques using autologous tissue or interposition grafts have been reported to address this problem [3, 4].
We believe our case is unique in that the anomalous right pulmonary artery arises from the left side of the ascending aorta, and there also is a fibrous strand connecting the right pulmonary artery to the pulmonary trunk. We believe that these findings suggest that, early in the embryologic development of this patient, the right pulmonary artery did take its origin from the pulmonary trunk, but was also fed from the aorta through an aortopulmonary window. It is well recognized that overriding of the right pulmonary artery is a frequent accompaniment of aortopulmonary fenestrations [5]. We presume that, in our patient, with ongoing development the right pulmonary artery became walled into the left side of the aorta through the window, but at the same time the segment of artery connecting to the pulmonary trunk became atretic, remaining only as a fibrous cord. It is hardly surprising that, in these circumstances, the arrangement was initially diagnosed echocardiographically as an aortopulmonary fenestration. In summary, therefore, we have described a unique morphologic association, with an excellent result after surgical repair, and proffered an explanation for its morphogenesis.
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Acknowledgments
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Research at the Institute of Child Health and Great Ormond Street Hospital for Children NHS Trust benefits from Research and Development funding received from the NHS Executive.
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References
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- Prifti E, Crucean A, Bonacchi M, et al. Postoperative outcome in patients with anomalous origin of one pulmonary artery branch from the aorta Eur J Cardiothorac Surg 2003;24:21-27.[Abstract/Free Full Text]
- Reyes de la Cruz L, Vizcaino Alarcon A, Arevalo Salas A, Espinosa Islas G, Bolio Cerdan A, Arteaga Martinez M. Echocardiographic diagnosis of anomalous origin of one pulmonary artery from the ascending aorta Arch Cardiol Mex 2003;73:115-123.[Medline]
- Prifti E, Frati G, Crucean A, Vanini V. A modified technique for repair of the anomalous origin of the right pulmonary artery from the ascending aorta Eur J Cardiothorac Surg 2002;22:148-150.[Abstract/Free Full Text]
- Miyazaki K, Murashita T, Kubota T, Matsuzaki K, Miyazaki YJ, Yasuda K. Neonatal repair of anomalous origin of the right pulmonary artery from the ascending aorta. A case report and review in the literature J Cardiovasc Surg (Torino) 2000;41:863-868.[Medline]
- Ho SY, Gerlis LM, Anderson C, Devine WA, Smith A. The morphology of aortopulmonary windows with regard to their classification and morphogenesis Cardiol Young 1994;4:146-155.
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Abstract
Introduction
