Related Experiment Video
Updated: Jan 29, 2026
Shunt Surgery, Right Heart Catheterization, and Vascular Morphometry in a Rat Model for Flow-induced Pulmonary Arterial Hypertension
Published on: February 11, 2017
Long-term outcomes of surgery for pulmonary artery sling in children
Matthew S Yong1,2,3, Michael Z L Zhu1, Douglas Bell4
1Department of Cardiac Surgery, Royal Children's Hospital, Melbourne, Australia.
Insights
Pulmonary artery sling repair outcomes show survival depends on concurrent tracheal surgery. Long-term survival is good, but respiratory monitoring is essential for patients with PA sling and tracheal repair.
Area of Science:
- Cardiovascular Surgery
- Pediatric Surgery
- Thoracic Surgery
Background:
- Pulmonary artery (PA) sling is a rare congenital vascular anomaly.
- It is frequently associated with congenital tracheal stenosis, complicating surgical repair.
- Long-term outcomes of PA sling repair, especially when combined with tracheal surgery, require further elucidation.
Purpose of the Study:
- To describe the long-term outcomes of surgical repair for pulmonary artery sling.
- To evaluate the impact of concomitant tracheal surgery on mortality and survival.
- To assess the functional status and respiratory outcomes in survivors.
Main Methods:
- A retrospective study of 33 patients who underwent PA sling repair between 1984 and 2018 at two institutions.
- Analysis of operative mortality, long-term survival, functional class, and respiratory function (spirometry).
- Comparison of outcomes between patients who required PA sling repair alone versus those needing combined PA sling and tracheal repair.
Main Results:
- Operative mortality decreased over time, with higher rates in patients requiring tracheal surgery (slide tracheoplasty used in 52%).
- 15-year survival was 100% for PA sling repair alone versus 76% for combined PA sling and tracheal repair (P=0.08).
- Survivors had excellent functional status (NYHA class I/II), but spirometry revealed obstructive lung defects, necessitating long-term follow-up.
Conclusions:
- The need for concurrent tracheal surgery significantly impacts early mortality in PA sling repair.
- While late survival is generally excellent, long-term respiratory sequelae are common.
- Ongoing respiratory assessment and follow-up are crucial for patients who have undergone PA sling and tracheal repair.
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.
Related Concept Videos
01:28Treatment for Pulmonary Arterial Hypertension: Phosphodiesterase Inhibitors
Among the PDE5 inhibitors, sildenafil (Revatio) stands out as a competitive and selective inhibitor. It operates by elevating cellular levels of cGMP and augmenting signaling through the cGMP-PKG pathway, promoting vasodilation. Upon oral...
01:18Treatment for Pulmonary Arterial Hypertension: Endothelin Receptor Antagonists
ETs are synthesized through a complex sequence of enzymatic steps, primarily involving an enzyme referred to as endothelin-converting enzyme...
01:23Treatment for Pulmonary Arterial Hypertension: Prostacyclin Receptor Agonists
These agonists bind to the IPR receptor situated on the plasma membrane of the pulmonary artery smooth muscle cells. This binding triggers a cascade of reactions known as the GS-AC-cAMP-PKA pathway. This pathway results in the relaxation of smooth muscle...
02:24Predicting Reaction Outcomes
01:16Treatment for Pulmonary Arterial Hypertension: Oxygen Therapy for Respiratory Failure
Oxygen therapy is vital in increasing and maintaining blood oxygen levels in PAH patients. As a result, it aids in reducing fatigue,...
01:13Outcomes of Glycolysis
Cellular respiration can occur aerobically (with oxygen) or anaerobically (without oxygen). In the presence of oxygen, cellular respiration starts with glycolysis and continues with pyruvate...