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Airway compression management in late-presenting absent pulmonary valve syndrome

  • Anastasia Martinez-Esteve Melnikova (a1), Tornike Sologashvili (a2), Maurice Beghetti (a1), Cécile Tissot (a1), Afksendiyos Kalangos (a2), Regula Corbelli (a3), Yacine Aggoun (a1), Dominique Didier (a4) and Patrick O. Myers (a2)...


Introduction: Patients with absent pulmonary valve syndrome often present early with airway compression from aneurysmal pulmonary arteries. This study reviews our experience in managing absent pulmonary valve syndrome in later presenting children, and techniques used for managing airway compression. Methods: This study is a retrospective chart review of all patients who underwent repair of absent pulmonary valve syndrome from 2000 to 2012 at our institution. The primary endpoints were post-operative bronchoscopic and clinical evidence of persistent airway compression and need for reinterventions on the pulmonary arteries. Results: A total of 19 patients were included during the study period. The mean age at repair was 4.1±3.0 years (range 10 months–11 years). In all, seven patients had pre-operative bronchoscopic evidence of airway compression, which was managed by pulmonary artery reduction plasty in four patients and Lecompte manoeuvre in three patients. There were no peri-operative deaths. In patients with pulmonary artery plasty, two had no post-operative airway compression, one patient had improved compression, and one patient had unchanged compression. In patients managed with a Lecompte manoeuvre, two patients had no or trivial airway compression and one had improved compression. There were six late reinterventions or reoperations on the pulmonary arteries – two out of four in the pulmonary artery plasty group and one out of three in the Lecompte group. Conclusions: Most late-presenting patients with absent pulmonary valve syndrome do not have airway compression. Either pulmonary artery reduction plasty or the Lecompte manoeuvre can relieve proximal airway compression, without a significantly different risk of pulmonary artery reintervention between techniques.


Corresponding author

Correspondence to: Dr P. O. Myers, MD, Division of Cardiovascular Surgery, Faculty of Medicine, Geneva University Hospitals & School of Medicine, 4 rue Gabrielle-Perret-Gentil 1211 Geneva 14, Switzerland. Tel: +41 (22) 372-7638; Fax: +41 (22) 372-7634; E-mail:


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Presented at the AEPC 47th annual meeting, 22–25 May 2013, London, United Kingdom.



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1. Snir, E, de Leval, MR, Elliott, MJ, Stark, J. Current surgical technique to repair Fallot’s tetralogy with absent pulmonary valve syndrome. Ann Thorac Surg 1991; 51: 979982.
2. Brown, JW, Ruzmetov, M, Vijay, P, Rodefeld, MD, Turrentine, MW. Surgical treatment of absent pulmonary valve syndrome associated with bronchial obstruction. Ann Thorac Surg 2006; 82: 22212226.
3. Hraska, V. Absent pulmonary valve repair. Op Techn Thorac Cardiovasc Surg 2007; 12: 3646.
4. Chen, JM, Glickstein, JS, Margossian, R, et al. Superior outcomes for repair in infants and neonates with tetralogy of Fallot with absent pulmonary valve syndrome. J Thorac Cardiovasc Surg 2006; 132: 10991104.
5. Conte, S, Serraf, A, Godart, F, et al. Technique to repair tetralogy of Fallot with absent pulmonary valve. Ann Thorac Surg 1997; 63: 14891491.
6. Hraska, V, Kantorova, A, Kunovsky, P, Haviar, D. Intermediate results with correction of tetralogy of Fallot with absent pulmonary valve using a new approach. Eur J Cardiothorac Surg 2002; 21: 711714; discussion 714–715.
7. Nolke, L, Azakie, A, Anagnostopoulos, PV, Alphonso, N, Karl, TR. The Lecompte maneuver for relief of airway compression in absent pulmonary valve syndrome. Ann Thorac Surg 2006; 81: 18021807.
8. Tissot, C, Aggoun, Y, Beghetti, M, et al. The Lecompte maneuver as an alternative to reduction pulmonary arterioplasty for relief of airway compression in absent pulmonary valve syndrome. Ann Thorac Surg 2007; 83: 727; author reply 727–728.
9. Myers, PO, Kalangos, A, Didier, D, Tissot, C. Tetralogy of Fallot with absent pulmonary valve, supracardiac total anomalous pulmonary venous connection, and infradiaphragmatic systemic to pulmonary collateral artery. Eur Heart J 2010; 31: 3005.
10. Chevers, N. Recherches sur les maladies de l'artère pulmonaire. Arch Gen Med 1847; 15: 488508.
11. Volpe, P, Paladini, D, Marasini, M, et al. Characteristics, associations and outcome of absent pulmonary valve syndrome in the fetus. Ultrasound Obstet Gynecol 2004; 24: 623628.
12. Kirshbom, PM, Kogon, BE. Tetralogy of Fallot with absent pulmonary valve syndrome. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu 2004; 7: 6571.



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