Asian Cardiovasc Thorac Ann 2005;13:77-78
© 2005 Asia Publishing EXchange Ltd
Pulmonary Artery Sling Associated with Tetralogy of Fallot
Yuko Takeda, MD,
Toshihide Asou, MD,
Dicky Fakhri, MD1,
Anna U Rahayoe, MD1,
Hirokuni Yoshimura, MD,
Jusuf Rachmat, MD1
Department of Thoracic and Cardiovascular Surgery, Kitasato University, Kanagawa, Japan
1 Harapan Kita National Cardiac Center, Jakarta, Indonesia
For reprint information contact: Yuko Takeda, MD Tel: 81 45 711 2351 Fax: 81 45 742 7821 Email: surgeon{at}pf7.so-net.ne.jp, Department of Cardiovascular Surgery, Kanagawa Children’s Medical Center, 2-138-4 Mutsukawa, Minami-ku, Yokohama, Kanagawa 232-8555, Japan.
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ABSTRACT
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We describe a rare case of pulmonary artery sling occurring simultaneously with tetralogy of Fallot. This report describes the successful concomitant repair of both intracardiac anomalies.
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INTRODUCTION
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Pulmonary artery sling is a rare congenital condition in which an anomalous left pulmonary artery arises from the right pulmonary artery and courses behind the bifurcation of the trachea reaching the hilum of the left lung.1,2 Thus, it forms a sling around the trachea and may compromise the bronchial airway by compressing the trachea. It presents solitarily or in association with other cardiac anomalies.3 We describe the management and outcome of a case of pulmonary artery sling associated with tetralogy of Fallot.
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CASE REPORT
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A 2-and-a-half-year-old boy was referred to Harapan Kita National Cardiac Center, Jakarta, Indonesia, for total correction of tetralogy of Fallot. Body weight was 10.5 kg. The patient presented with cyanosis and squatting. A diagnosis of tetralogy of Fallot was made by echocardiography and cardiac catheterization. Although he had no symptoms of airway obstruction, pulmonary artery sling was suspected on pulmonary angiogram which showed the anomalous origin of the left pulmonary artery arising from the right pulmonary artery coursing to the left lung hilum (Figure 1
). The pressure of the main pulmonary artery was 27/11(16) mm Hg. The left pulmonary artery was 5.6 mm in diameter and the right pulmonary artery 6.5 mm. The systemic arterial oxygen saturation was 92% and the hemoglobin level was 17.6 g·dL–1.
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Figure 1. Right ventriculography showed that the left pulmonary artery arose from the right pulmonary artery and coursed behind the trachea and to the left lung hilum. The left pulmonary artery was 5.6 mm in diameter and the right pulmonary artery was 6.5 mm.
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At surgery, a median sternotomy was performed. The right pulmonary artery was dissected. The left pulmonary artery arose from the right pulmonary artery behind the ascending aorta and coursed between the trachea and the esophagus and reached the left lung hilum. The left pulmonary artery was dissected and further mobilized free from the trachea during cardiopulmonary bypass. When crystalloid cardioplegic arrest was achieved, the muscle was resected from the right ventricular outflow tract and a perimembranous ventricular septal defect was closed using a trans-atrial and trans-pulmonary approach.4 Commissurotomy was performed on the trileaflet pulmonary valve. After unclamping the aorta, the left pulmonary artery was transected and the stump of the right pulmonary artery was transversely closed with a continuous 6–0 monofilament suture. The left pulmonary artery was anastomosed end-to-side to the left lateral aspect of the main pulmonary artery in front of the trachea so that direct anastomosis was achieved posteriorly and an autologous non-transannular pericardial patch was used anteriorly. Aortic cross-clamp time was 43 minutes and bypass time 125 minutes.
The patient was weaned from bypass without use of inotropes and extubated 6 hours after surgery in the intensive care unit without any signs of airway obstruction. Postoperative echocardiogram showed the widely opened right ventricular outflow tract and bifurcation of the pulmonary artery. No significant pulmonary valve regurgitation was noted (Figure 2). He was discharged from hospital on the sixth postoperative day following an uneventful postoperative period.
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Figure 2. Subcostal coronal view of postoperative echocardiogram showed that both the right ventricular outflow tract and the bifurcation of the pulmonary artery were widely opened.
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DISCUSSION
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Pulmonary artery sling may be concomitantly discovered with associated cardiovascular anomalies as in our case.2,3 According to the literature, in 30% of cases the pulmonary artery sling is associated with major cardiovascular anomalies such as ventricular septal defect, atrial septal defect and patent ductus arteriosus. Tetralogy of Fallot is an extremely rare cardiovascular anomaly associated with pulmonary artery sling.3,5 Murdison and colleague5 reviewed a case of tetralogy of Fallot with severe pulmonary valvar stenosis and pulmonary artery sling discovered at postmortem examination. Among 130 cases reviewed by Gikonyo and colleagues,3 three cases demonstrated tetralogy of Fallot and pulmonary artery sling. In all of them, however, the diagnosis was made at necropsy. To our knowledge, this is the first case of pulmonary artery sling successfully repaired concomitantly with tetralogy of Fallot. In pulmonary artery sling, the left pulmonary artery arises anomalously from the right pulmonary artery and courses between the trachea and the esophagus reaching the left lung hilum and forming a sling around the trachea. Thus, respiratory symptoms such as wheezing and stridor may develop due to tracheobronchial compression by the left pulmonary artery. According to Gikonyo’s review of the literature, however, fourteen (12%) of the 130 patients surveyed were asymptomatic.3 Although our patient did not present with any respiratory symptoms, cyanosis due to tetralogy of Fallot was noted. A diagnosis of pulmonary artery sling was incidentally made by preoperative angiogram during the evaluation of heart disease. While the mechanism of the absence of cardiorespiratory symptoms is not clearly known in the present case, the relatively small pulmonary artery due to tetralogy of Fallot may have prevented the trachea from being compressed by the left pulmonary artery. This case illustrates the unusual presentation of tetralogy of Fallot in association with pulmonary artery sling and the successful repair of both cardiac anomalies.
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REFERENCES
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- Potts WJ, Holinger PH, Rosenblum AH, Anomalous left pulmonary artery causing obstruction to right main bronchus: report of a case. JAMA
1954;155:1409–1.[Abstract/Free Full Text]
- Woods RK, Sharp RJ, Holcomb III GW, Snyder CL, Lofland GK, Ashcraft KW, et al. Vascular anomalies and tracheoesophageal compression: a single institution’s 25-year experience. Ann Thorac Surg
2001;72:434–9.[Abstract/Free Full Text]
- Gikonyo BM, Jue KL, Edwards JE. Pulmonary vascular sling: Report of seven cases and review of the literature. Pediatric Cardiology
1989;10:81–9.[Medline]
- Asou T, Rachmat J. Slicing technique of the RV outflow tract in transatrial-transpulmonary repair for tetralogy of Fallot. J Cardiovasc Surg
2001;42:639–42.[Medline]
- Murdison KA, Weinberg PM. Tetralogy of Fallot with severe pulmonary valvar stenosis and pulmonary vascular sling (Anomalous origin of the left pulmonary artery from the right pulmonary artery). Pediatric Cardiology
1991;12:189–91.[Medline]
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ABSTRACT
INTRODUCTION
