Pediatr Cardiol 12:44-45, 1991

Pediatric Cardiology 9 Springer-Verlag New York Inc. 1991

A b e r r a n t L e f t P u l m o n a r y A r t e r y with T r a c h e a l S t e n o s i s w i t h o u t V a s c u l a r Sling Felipe Moreno, Luis Garcia-Guereta, Fernando Benito, Carlos Gamallo, F. Campo, and F. Herranz Department of Paediatric Cardiology, La Paz Children's Hospital, Madrid, Spain

SUMMARY: We report a case of abnormal origin of the left pulmonary artery from the right pulmonary artery, associated with tracheal stenosis but with no vascular sling. This is the first such case, to our knowledge, without vascular sling. KEY WORDS: Aberrant left pulmonary a r t e r y - Tracheal stenosis

Tracheal stenosis has frequently been reported in association with the so-called vascular sling [1]. Moreover, in all published cases of vascular sling, the left pulmonary artery (LPA) arose from the right pulmonary artery (RPA) [ 1-12]. The first case to our knowledge of the abnormal origin of the LPA from the RPA, associated with tracheal stenosis without vascular sling, is reported.

Case Report A 2-month-old boy was referred to the Department of Pediatric Cardiology due to respiratory distress since birth. On physical examination, he was found to have mild cyanosis, intercostal retraction and wheezing, liver enlargement, a grade II/VI systolic murmur, and a loud second sound. The electrocardiogram (ECG) showed right ventricular hypertrophy. A chest x-ray film revealed cardiac enlargement and air trapping in the right lower lobe. The esophagogram showed a posterior indentation in its upper third, but failed to reveal any anterior indentation. At bronchoscopy a circular and rigid stenosis was found which made it impossible to pass a 3-ram bronchoscope beyond the lower end of the trachea. Cardiac catheterization revealed a left-to-right shunt through an atrial septal defect, persistent ductus arteriosus, and pulmonary hypertension. Pulmonary artery angiography showed a hypoplastic LPA arising from the RPA. Stop-flow aortography revealed an anomalous origin of the right subclavian artery and an artery arising from the lower thoracic aorta that divided into two branches suppling the lower lobes of both lungs (Fig. 1). The patient died 15 days later in respiratory failure. Au: topsy confirmed the cardiac anomalies. A hypoplastic LPA arose from the RPA 1 cm distal to the ductus and coursed anterior to

Address of[print requests to: Dr. Felipe Moreno, Hospital lnfantil La Paz, Paseo de la Castellana, 261. 28046 Madrid, Spain.

the trachea (Fig. 2) to enter the left lung. A bilobar right lung, unilobar left lung, pulmonary sequestrations at the base of both lungs, and complete cartilaginous rings at the lower end of the trachea were also disclosed.

Discussion In all reported cases of LPA arising from the RPA, the LPA encircles the trachea [1-12]. Therefore, anomalous or aberrant LPA and vascular sling have been used as synonyms. In a pulmonary artery sling, the posterior course of the LPA usually produces tracheobronchial compression and respiratory distress, but frequently the respiratory symptoms are caused by intrinsic stenosis of the trachea, mainly due to complete cartilaginous rings [I, 5, 6, 9, 11]. Cardiac defects are also common [1, 4, 5, 9, ! 1]. In our case, besides the cardiac defects, there were anomalous origins of the LPA and tracheal stenosis, as in a sling, but the LPA did not encircle the trachea. This suggests that both anomalies--the aberrant LPA and tracheal stenosis--have a common or related embryopathogenic mechanism, independent of the course of the LPA. Sade et al. [9] suggested that, when the ventral pulmonary bud of the left sixth arch fails to develop, connection of the left postbranchial plexus can be established by either of two routes across the midline: caudal to the lung bud, resulting in a posterior course of the anomalous vessel, as in a sling; or cephalic to the lung bud, resulting in an anterior course, as in the case reported here. Angiographic diagnosis of pulmonary artery sling has been based on proving the abnormal origin

Moreno et al.: Aberrant Left Pulmonary Artery

45

Fig. 1. Sitting-up pulmonary angiogram shows the origin of the left pulmonary artery from the right pulmonary artery and its anterior course. Hyperinflation of the right lower lobe can also be seen. PA, pulmonary artery trunk. Fig. 2. Anterior view of the specimen with the heart displaced to the left. The left pulmonary artery arises from the right pulmonary artery and passes to the left lung in front of the trachea and left broncbus. LPA, left pulmonary artery; LA, left atrium; RPA, right pulmonary artery; T, trachea.

of the LPA from the RPA, but it is also necessary, as our case illustrates, to demonstrate its course, by means of a sitting-up projection [3], or noninvasively by axial computed tomographic imaging [10] or two-dimensional echocardiography [12]. The clinical course and o u t c o m e of a vascular sling mainly depends on the severity o f associated tracheobronchial anomalies [5, 6, 8, 9]. In the reported asymptomatic patients [2, 3, 7, 10], tracheobronchial anomalies were absent, which has led some authors to think that vascular surgery would not be indicated [3, 7]. On the contrary, when severe tracheobronchial lesions are present, tracheobronchial surgery is essential [6]. References 1. Contro S, Miller RA, White H, Ports WJ (1958) Bronchial obstruction due to pulmonary artery anomalies. 1. Vascular sling. Circulation 17:418-423 2. Dupuis C, Vaksmann G, Pernot C, Gerard R, Martinez J, Van Egmond H (1988) Asymptomatic form of left pulmonary artery sling. Am J Cardio! 61:177-181 3. Gumbiner CH, Mullins CE, McNamara DG (1980) Pulmonary artery sling. Am J Cardiol 45:311-315

4. Jue KL, Raghib G, Amplatz K, Adams P, Edwards J (1965) Anomalous origin of the left pulmonary artery from the right pulmonary artery. A JR 95:598-610 5. Koopot R, Nikaidoh H, ldriss RS (1975) Surgical management of anomalous left pulmonary artery causing tracheobronchial obstruction. J Thorac Cardiovasc Surg 69:239-246 6. Nakayama DK, Harrison MR, Lorimier AA, Brasch RC. Fishman NH (1982) Reconstructive surgery for obstructing lesions of the intrathoracic trachea in infants and small children. J Pediatr Surg 17:854-868 7. Phelan PD, Venables AW (1978) Management of pulmonary artery sling (anomalous left pulmonary artery arising from right pulmonary artery). A conservative approach. Thorax 33:67-72 8. Quero M, Castro MC, Brito JM, Cordovilla G (1973) Arteria pulmonar izquierda aberrante. Cita de un caso y revisi6n de la literatura. An Esp Pediat 6:309-316 9. Sade RM, Rosenthal A, Fellows K, Castafieda AR (1975) Pulmonary artery sling. J Thorac Cardiovasc Surg 69:333346 10. Stone DN, Bein ME, Garris JB (1980) Anomalous left pulmonary artery. Two new adult cases. A JR 135:1259-1263 11. Tesler VF, Balsara RH, Nigidula FN (1974) Aberrant left pulmonary artery (sling): Report of five cases. Chest 66:402407. 12. Yeager SB, Chin AJ, Sanders SP (1986) Two-dimensional echocardiographic diagnosis of pulmonary artery sling in infancy. J Am Coil Cardiol 7:625-629

Aberrant left pulmonary artery with tracheal stenosis without vascular sling.

We report a case of abnormal origin of the left pulmonary artery from the right pulmonary artery, associated with tracheal stenosis but with no vascul...
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