Eur J Cardiothorac Surg 2005;28:900-902
© 2005 Elsevier Science NL
Coexistence of left pulmonary artery sling and aortopulmonary window complicated with difficult airway—a rare congenital cardiopulmonary defect
Huai-Min Chen
a
,
Hsin-I Tseng
b
,
Jiann-Wei Huang
a
,
Zen-Kong Dai
b
,
*
a Division of Cardiovascular Surgery, Kaohsiung Medical University Hospital, Taiwan, ROC
b Department of Pediatrics, Kaohsiung Medical University Hospital, Taiwan, ROC
Received 27 June 2005;
received in revised form 13 August 2005;
accepted 23 August 2005.
* Corresponding author. Present address: Department of Pediatrics, Kaohsiung Municipal Hsiao-Kang Hospital, 482 Shan-Ming Rd, Hsiao-Kang Dist. 812, Kaohsiung, Taiwan, ROC. Tel.: +886 7 803 6783x3730; fax: +886 7 806 6418. (Email: zenkong{at}kmu.edu.tw).
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Abstract
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Both the pulmonary artery sling and the aortopulmonary window are unusual and serious anomalies. In patients with sling the airway might be compromised by associated cartilaginous o-rings in variant segments. In aortopulmonary window the clinical presentations are similar to the large patent ductus arteriosus or ventricular septal defect, but the surgical procedure is quite difficult and different. We operated an infant with a rarely seen cardiac defect, the coexistence of left pulmonary artery sling and Type 3 aortopulmonary window, when he was 35 days old. Postoperative repeated bronchospasm caused prolonged ventilation and hospitalization. Patient was extubated 28 days later and discharged at the age of 80 days without any events. Besides, the reconstructed three-dimensional images of ultrafast computed tomography offered us an interesting view other than surgical findings. A case of two such rare lesions coexisting was not to be found in the literature review, so we report this case because of its rarity and clinical interest.
Key Words: Left pulmonary artery sling • Aortopulmonary window • Bronchospasm • Tracheomalacia • Vascular airway compression
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1. Introduction
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Both the left pulmonary artery (LPA) sling and the aortopulmonary (AP) window are rare anomalies. Affected infants generally present with respiratory distress during the first weeks of life [1]. We had an infant with the diagnosis of LPA sling with tracheal stenosis, AP window, patent ductus arteriosus (PDA), atrial septal defect (ASD), and tracheomalacia. We did not find any case of these two rare lesions coexisting in one patient in the literature, so we described the management and experience of this very rare congenital abnormality.
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2. Patient history and management
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A baby with a gestational age of 39 weeks was born under normal spontaneous delivery and was 2900 g at birth. He suffered from progressive respiratory distress and cyanosis. He was transferred to our hospital at the age of 30 days. Echocardiography revealed anomalous origin of left pulmonary artery from the right one, AP window, PDA, and ASD. Serial examinations including flexible bronchoscopy, barium esophagogram, and chest computed tomography (CT) were performed and all confirmed these diagnoses. CT images showed that the left pulmonary artery originated from the right one with compression over right carina (Fig. 1A) and absence of aortopulmonary septum (Fig. 1B). Flexible bronchoscopy revealed external compression with pulsation near right carina, poor development of tracheal cartilages, and tracheomalacia.
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Fig. 1. (A) The left pulmonary artery is originated from right pulmonary artery and it passes between trachea and esophagus. The diameter of left pulmonary artery is smaller than that of the right one (RPA/LPA: right/left pulmonary artery). (B) Complete absence of aortopulmonary septum is seen between aorta and pulmonary trunk. The arrow indicates the site of AP window (Ao: aorta; PT: pulmonary trunk; RVOT: right ventricular outflow tract).
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3. Surgical result
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The patient underwent the operation at the age of 35 days. A smaller LPA originating from the right one, total defect of aortopulmonary septum (Type 3 AP window) and progressive narrowing of the trachea without specific stricture except the area of the LPA sling were found (Fig. 2A). The LPA was divided and relocated to the anterior of trachea. The aortopulmonary window was incised and each great artery was reconstructed with separate autologus pericardial patch. The LPA was reimplanted into the pulmonary artery patch. ASD was repaired by direct closure. The airway was left untreated because no segmental stricture was found. The patient was weaned from cardiopulmonary bypass without difficulty.
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Fig. 2. (A) Surgical view from the frontal side. The left pulmonary artery originates from the right one and passes leftward behind the trachea. There was no specific stricture of trachea except the region of LPA sling (LAA: left atrium appendage; MPA: main pulmonary artery; TA: trachea; Ao: aorta). (B) Three-dimensional image of isolated airway system: a segment of trachea stenosis in length about 2.2 cm, and a more constrictive segment in length about 8 mm on the region of LPA sling are seen. The dimension of the rulers on the bottom and left is 5 mm in each space.
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4. Results
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Repeated bronchospasm occurred postoperatively when we tried to wean him off the ventilator, and acute elevation of carbon dioxide up to 117 mmHg was noted. Medications were used to decrease the bronchospasm but achieved little success. The value of PaCO2 remained high, so we used manual bagging of the endotracheal tube frequently and continued the sedative to decrease the level of CO2 and the airway spasm. The condition improved. Nine days after the operation, respiratory distress and bronchospasm recurred, and blood gas revealed PaCO2 of up to 170 mmHg. The condition deteriorated and it failed to respond to previous management. In order to understand what happened of the airway, bronchoscopy was performed, and a crest above the carina with a tiny orifice was found. In spite of aggressive management, the respiratory distress worsened. The pressure of airway dropped suddenly, and the respiratory distress improved after we changed the endotracheal tube. Follow-up bronchoscopy revealed no crest. We gradually tapered the sedative, and extubation was performed 28 days after operation and required no sedative. The patient was discharged at the age of 80 days when he did not require additional oxygen. The follow-up echocardiography revealed good pulmonary artery flow.
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5. Discussion
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Before operation, whether to correct the problem of tracheobronchial tree was an issue of concern. Because no specific stricture was found on bronchoscopy, we decided to correct the sling and relieve the external compression only. The definitive treatment of LPA sling is LPA translocation/reimplantation, relief of vascular airway compression, and reconstruction of tracheal stenosis [2]. A number of techniques to repair tracheal stenosis have been utilized, but the optimal technique remains controversial. The primary concerns include the growth potential of reconstructed trachea, the incidence of granulation formation, the fate of cartilaginous or pericardium surface, and the long-term functional outcome [3]. Because mortality and morbidity are so high in patients of less than 6 months of age with LPA sling and tracheal stenosis after tracheal reconstruction [3,4], we decided not to perform the tracheoplasty for his tracheal narrowing and performed the decompression only.
Rutter et al. [5] reported the non-operative management of complete tracheal rings in infants in 2004 and concluded that not all patients with complete tracheal rings required tracheoplasty and that some have satisfactory airway growth and do not require airway reconstruction. For tracheomalacia, tracheopexy or stent insertion may decrease the severity [6–8], but the ensuing complications remain a challenge. For the tracheal stenosis, if there is presence of another stenosis other than of the carinal region, the combination of tracheal, and LPA reconstructions is recommended [9].
After operation, the tracheobronchial tree was not found to be extremely narrow on the flexible bronchioscopy, but he was frequently attacked by bronchial spasm. We tried to reconstruct the three-dimensional images of preoperative CT of the tracheobronchial tree. The images presented a segmental stricture of about 2.2 cm above carina and a more constrictive segment of about 8 mm on the region of LPA sling (Fig. 2B); it presented more precise and detailed information than two-dimensional pictures. If we had been able to review the detailed anatomy before operation, we might have chosen a different surgical plan. The traditionally reconstructed three-dimensional images from ultrafast CT are full of the surrounding tissue, which limits their practical use. If the isolated system, especially the vascular and airway system can be reconstructed into three-dimensional images, the images can render more precise and detailed surgical information, so the preoperative CT scan should be performed.
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6. Conclusion
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To our knowledge, a case such as ours has never been reported in the literature. The infant was successfully treated with pulmonary artery reimplantation without impairment of pulmonary flow, external decompression of the airway, and reconstruction of two great vessels. He was also successfully managed with long-term sedation to overcome the problem of the airway spasm and the potential for pulmonary hypertension crisis. Despite prolonged hospitalization, the patient was ultimately discharged without sequelae. Questions remain about the potential for airway growth and possible life-threatening airway spasm, and it needs long-term follow-up to obtain the answer. Besides, for patients with vascular airway compression, the ultrafast CT with three-dimensional reconstruction of the vascular or airway system is very useful for the diagnosis and preoperative evaluation of the condition.
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References
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- Dihleman C, Mantel K, Vogl T, Nitolai T. Pulmonary sling: morphological findings, pre- and postoperative course. Eur J Pediatr 1995;154:2-14.[CrossRef][Medline]
- Chen HM, Chin IS, Chen SJ, Wu SJ, Chen CH. Concomitant management of airway and its adjacent vascular pathology in addition to repair of congenital cardiac defects. Int J Cardiol 2000;13:181-189.
- Fiore AC, Brown JW, Weber TR, Turrentine MW. Surgical treatment of pulmonary artery sling and tracheal stenosis. Ann Thorac Surg 2005;79:38-46.[Abstract/Free Full Text]
- Woods RK, Sharp RJ, Holcomb JW, Snyer CL, Lofland GK. Vascular anomalies and tracheoesphageal compression: a single institution's 25-year experience. Ann Thorac Surg 2001;72:434-439.[Abstract/Free Full Text]
- Rutter M, Willging P, Cotton RT. Nonoperative management of complete tracheal rings. Arch Otolaryngol Head Neck Surg 2004;130:450-452.[Abstract/Free Full Text]
- Jacobs JP, Quintessenza J, Botero L, Van Gelder HM, Giroud J, Elliott M. The role of airway stents in the management of paediatric tracheal, carinal, and bronchial disease. Eur J Cardiothorac Surg 2000;18:505-512.[Abstract/Free Full Text]
- Furman R, Backer CL, Dumham ME, Donaldson J, Mavroudis C, Helinger LD. The use of balloon-expandable metallic stents in the treatment of paediatric tracheomalacia and bronchomalacia. Arch Otolaryngol Head Neck Surg 1999;125:203-207.[Abstract/Free Full Text]
- Conti VR, Lobe TE. Vascular sling with tracheomalacia: surgery management. Ann Thorac Surg 1989;47:310-311.[Abstract]
- Jonas RA, DiNildo J, Laussen PC. Vascular rings, slings, and tracheal anomalies. In: Koster J, Burrows S, editors. Comprehensive surgical management of congenital heart disease. London: Arnold; 2004. pp. 497-509.
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Abstract
1. Introduction
