Matthew S Yong1,2,3, Michael Z L Zhu1, Douglas Bell4, Nelson Alphonso4, Johann Brink1,2, Yves d'Udekem1,2,3, Igor E Konstantinov1,2,3,5. 1. Department of Cardiac Surgery, Royal Children's Hospital, Melbourne, Australia. 2. Department of Paediatrics, University of Melbourne, Melbourne, Australia. 3. Murdoch Children's Research Institute, Melbourne, Australia. 4. Department of Cardiac Surgery, Queensland Children's Hospital, Brisbane, Australia. 5. Melbourne Children's Centre for Cardiovascular Genomics and Regenerative Medicine, Melbourne, Australia.
Abstract
OBJECTIVES: Pulmonary artery (PA) sling is a rare vascular anomaly often associated with congenital tracheal stenosis. We describe the long-term outcomes with repair of this condition. METHODS: A retrospective study was conducted at 2 institutions. From 1984 to 2018, 33 patients with PA sling underwent repair. RESULTS: The median age at the time of surgery was 5.9 months (quartile 1-3: 2.5-12 months). Concomitant tracheal surgery was required in 21 patients (64%) where slide tracheoplasty was used in 11 patients (52%). There were no early deaths in patients who did not require tracheal surgery (n = 12). Operative mortality was 22% (2 of 9 patients) between 1984 and 1993, 11% (1 of 9 patients) between 1994 and 2003 and 6.7% (1 of 15 patients) between 2004 and 2018. The 15-year probability of survival for patients who had PA sling repair alone was 100%, and for patients who required PA sling and tracheal repair was 76 ± 10% (95% confidence interval 51-89%) (P = 0.08). The mean follow-up for survivors was 14 ± 9.8 years (3 months-33 years). All survivors were in the New York Heart Association functional class I/II at the last follow-up. Spirometry performed at a median age of 10.4 years after PA sling and tracheal surgery demonstrated obstructive lung defects with median forced expiratory volume in 1 s of 1.0 l (48% predicted), forced vital capacity of 1.5 l (74% predicted) and forced expiratory volume in 1 s/forced vital capacity of 0.69 (78% predicted). CONCLUSIONS: Early mortality after PA sling repair is determined by the need for tracheal surgery. Though late survival was excellent, and the majority of survivors remained asymptomatic, long-term respiratory assessment and follow-up is warranted for these patients.
OBJECTIVES: Pulmonary artery (PA) sling is a rare vascular anomaly often associated with congenital tracheal stenosis. We describe the long-term outcomes with repair of this condition. METHODS: A retrospective study was conducted at 2 institutions. From 1984 to 2018, 33 patients with PA sling underwent repair. RESULTS: The median age at the time of surgery was 5.9 months (quartile 1-3: 2.5-12 months). Concomitant tracheal surgery was required in 21 patients (64%) where slide tracheoplasty was used in 11 patients (52%). There were no early deaths in patients who did not require tracheal surgery (n = 12). Operative mortality was 22% (2 of 9 patients) between 1984 and 1993, 11% (1 of 9 patients) between 1994 and 2003 and 6.7% (1 of 15 patients) between 2004 and 2018. The 15-year probability of survival for patients who had PA sling repair alone was 100%, and for patients who required PA sling and tracheal repair was 76 ± 10% (95% confidence interval 51-89%) (P = 0.08). The mean follow-up for survivors was 14 ± 9.8 years (3 months-33 years). All survivors were in the New York Heart Association functional class I/II at the last follow-up. Spirometry performed at a median age of 10.4 years after PA sling and tracheal surgery demonstrated obstructive lung defects with median forced expiratory volume in 1 s of 1.0 l (48% predicted), forced vital capacity of 1.5 l (74% predicted) and forced expiratory volume in 1 s/forced vital capacity of 0.69 (78% predicted). CONCLUSIONS: Early mortality after PA sling repair is determined by the need for tracheal surgery. Though late survival was excellent, and the majority of survivors remained asymptomatic, long-term respiratory assessment and follow-up is warranted for these patients.