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PubMed This is a summary of 14 peer-reviewed journal articles Updated
Otolaryngology

The Heart-Airway Connection: Understanding Associated Anomalies

At a Glance

Congenital tracheal stenosis (CTS) frequently occurs alongside a blood vessel defect called a pulmonary artery sling, known together as the Ring-Sling Complex. Doctors typically treat this using a synchronous repair, fixing both the misplaced vessel and the narrow windpipe in a single surgery.

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When a child is diagnosed with Congenital Tracheal Stenosis (CTS), it is very common for doctors to also want to check their heart and blood vessels. This is because the windpipe and the heart develop at exactly the same time in the womb, often influenced by the same genetic “blueprint” [1][2].

When these two systems both have anomalies, it is frequently referred to as the Ring-Sling Complex [3].

Understanding the “Ring-Sling Complex”

The name “Ring-Sling” comes from the two specific problems happening at once:

  • The Ring: The “O-shaped” complete tracheal rings that make the windpipe narrow and rigid [3][1].
  • The Sling: A condition called Pulmonary Artery Sling (PAS) [3].

In a typical body, the left pulmonary artery (which carries blood to the lungs) goes straight to the left lung. In a “sling,” this artery takes a detour. It starts from the right side, then loops around the back of the windpipe to get to the left lung [4][1]. This “sling” of a blood vessel physically squeezes the already-narrow windpipe, making it even harder for the child to breathe [5].

Why Your Child Needs a Heart Doctor

Because the blood vessels and the windpipe are literally wrapped around each other, an airway specialist (Otolaryngologist or ENT) and a heart specialist (Cardiothoracic Surgeon) must work as a team [6].

If your child has both a narrow trachea and a pulmonary artery sling, the medical team will usually recommend a synchronous repair [7][8]. This means that in one single operation:

  1. The heart surgeon moves the “slinging” artery to its correct position, taking the pressure off the windpipe [9].
  2. The airway surgeon performs a slide tracheoplasty to widen the trachea [7].

Performing these repairs at the same time is considered safe and effective, and it prevents the need for multiple major surgeries later [8][10].

Looking Ahead

While having both a heart and airway anomaly makes the condition more complex, modern surgical techniques have made these “one-stage” repairs highly successful [7][11].

Because the nerves that control the vocal cords run very close to both the heart and the windpipe, your team will carefully monitor your child’s voice and swallow function after surgery [12]. Children with the “Ring-Sling Complex” may stay in the intensive care unit (ICU) a bit longer to ensure both systems are healing well, but the goal remains the same: a stable, wide airway and a healthy heart for a lifetime of activity [13][14].

Next page: Seeing the Airway: How CTS is Diagnosed and Mapped

Common questions in this guide

What is the Ring-Sling Complex in children?
The Ring-Sling Complex occurs when a child has narrow, O-shaped tracheal rings (the ring) along with a pulmonary artery sling (the sling). This means an abnormally placed blood vessel wraps around and squeezes the already narrow windpipe.
What is a synchronous repair for CTS and heart defects?
A synchronous repair is a single operation where an airway specialist and a heart surgeon work together. They reposition the misplaced blood vessel and widen the windpipe during the exact same surgery, avoiding the need for multiple major procedures.
Why do doctors check my child's heart if they have a narrow windpipe?
The windpipe and heart develop at the same time in the womb, often guided by the same genetic blueprint. It is highly common for a child with congenital tracheal stenosis to also have blood vessel anomalies, such as a pulmonary artery sling.
Will my child's voice be affected by the Ring-Sling Complex surgery?
The nerves that control the vocal cords run very close to both the heart and the windpipe. Because of this close proximity, the surgical team will carefully monitor your child's voice and swallowing function after the synchronous repair is complete.

Questions to Ask Your Doctor

Curated prompts to bring to your next appointment.

  1. 1.Does my child have a 'pulmonary artery sling' or another type of vascular ring?
  2. 2.Will the airway and heart specialists be performing a 'synchronous' or 'one-stage' repair together?
  3. 3.How does the presence of a heart defect change the surgical plan for the 'O-shaped' rings in the trachea?
  4. 4.What specific imaging (like a CT or heart-specific MRI) has been done to see how the blood vessels are pressing on the windpipe?

Questions For You

Tap a prompt to share your answer — we'll use it plus this page's context to start a tailored conversation.

References

References (14)
  1. 1

    Clinical and structural aspects of tracheal stenosis and a novel embryological hypothesis of left pulmonary artery sling.

    Fukushima N, Shimojima N, Ishitate M, et al.

    Pediatric pulmonology 2020; (55(3)):747-753 doi:10.1002/ppul.24661.

    PMID: 31975532
  2. 2

    TBX5 pathogenic variant in a patient with congenital heart defect and tracheal stenosis.

    Yamoto K, Kato F, Yamoto M, et al.

    Congenital anomalies 2024; (64(1)):23-27 doi:10.1111/cga.12548.

    PMID: 38062907
  3. 3

    A rare case of 'ring-sling complex' in a symptomatic adult patient: A left pulmonary artery sling with complete tracheal rings.

    Lee M, Baranowski R, Oo A, Waller D

    JRSM open 2023; (14(11)):20542704231205388 doi:10.1177/20542704231205388.

    PMID: 38314403
  4. 4

    A left pulmonary artery sling with left bronchiectasis in an adult patient: A case report and review of literature.

    Lv L, Cheng X, Yu X, et al.

    Respirology case reports 2023; (11(1)):e01072 doi:10.1002/rcr2.1072.

    PMID: 36523544
  5. 5

    Image characteristics and main types of abnormal branching of fetal pulmonary artery in prenatal echocardiography - a retrospective study.

    Yan H, Zhang C, He L

    Ginekologia polska 2024; doi:10.5603/gpl.96884.

    PMID: 38334346
  6. 6

    Retrospective analysis of factors contributing to poor prognosis among 271 pediatric patients with tracheal stenosis treated with slide tracheoplasty.

    Chen Y, Wen W, Du X, et al.

    The Journal of thoracic and cardiovascular surgery 2025; (170(1)):36-45.e3 doi:10.1016/j.jtcvs.2024.11.017.

    PMID: 39581311
  7. 7

    Tracheoplasty should be proactively considered in the surgical strategy for treating the ring-sling complex.

    Du XW, Wang PH, Wang H, et al.

    The Journal of thoracic and cardiovascular surgery 2025; (169(2)):375-384.e4 doi:10.1016/j.jtcvs.2024.08.011.

    PMID: 39159883
  8. 8

    Treatment of long-segment congenital tracheal stenosis with congenital cardiovascular defects in infancy.

    Dai L, Zhao L, Shen L, et al.

    International journal of pediatric otorhinolaryngology 2023; (172()):111691 doi:10.1016/j.ijporl.2023.111691.

    PMID: 37536159
  9. 9

    Relationship between a Tracheal and Left Pulmonary Artery Stenosis Index and the Prognosis of Pulmonary Artery Sling with Tracheal Stenosis.

    Shen X, Tan W, Jia B, Ye M

    Pediatric cardiology 2021; (42(7)):1585-1593 doi:10.1007/s00246-021-02643-9.

    PMID: 34046721
  10. 10

    Surgical management of congenital tracheal stenosis associated with complex cardiovascular anomalies.

    Morita K, Hatakeyama T

    Pediatric surgery international 2022; (38(12)):1903-1908 doi:10.1007/s00383-022-05206-y.

    PMID: 36083305
  11. 11

    [Surgical treatment strategy of pulmonary artery sling: a series of 110 cases].

    Xu HH, Yang YL, Bi SM, et al.

    Zhonghua wai ke za zhi [Chinese journal of surgery] 2023; (61(11)):994-1000 doi:10.3760/cma.j.cn112139-20221214-00530.

    PMID: 37767666
  12. 12

    Incidence and risk factor of vocal cord paralysis following slide tracheoplasty for congenital tracheal stenosis: a retrospective observational study.

    Kaneko N, Hasegawa T

    Cardiology in the young 2022; (32(4)):579-583 doi:10.1017/S1047951121002663.

    PMID: 34247683
  13. 13

    Closed-PICU perioperative management of congenital tracheal stenosis.

    Aoki K, Kurosawa H, Seino Y, et al.

    Pediatrics international : official journal of the Japan Pediatric Society 2022; (64(1)):e15085 doi:10.1111/ped.15085.

    PMID: 34865290
  14. 14

    Congenital tracheobronchial stenosis.

    Hewitt RJ, Butler CR, Maughan EF, Elliott MJ

    Seminars in pediatric surgery 2016; (25(3)):144-9.

    PMID: 27301600

This page provides educational information about Congenital Tracheal Stenosis and associated heart conditions. Always consult your child's pediatric cardiothoracic and airway specialists for personalized medical advice.

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