Interrupted Aortic Arch — #3 One-Stage Repair

Date
November 9, 2025
URL
https://x.com/WithAScalpel/status/1987504375229481353?s=20
Categories
Interrupted Aortic ArchCardiac Surgery

Interrupted Aortic Arch — #3 One-Stage Repair

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Clinical context

Neonates with interrupted aortic arch (IAA) commonly present with a large VSD and a spectrum of left-sided hypoplasia. Contemporary series report >90% neonatal survival with either a conventional two-ventricle repair or a Yasui pathway when appropriately selected, with most late reinterventions related to the RV–PA conduit rather than recurrent LVOT problems [1]. PubMed

The operative objective in a one-stage strategy is to (i) secure durable systemic outflow, (ii) reconstruct the arch, and (iii) favor a biventricular circulation when the left heart can sustain it; otherwise, to bypass a prohibitive LVOT with a Yasui repair.

Perfusion and cannulation strategy

  • Dual arterial perfusion. Upper-body cannulation (innominate/axillary) enables antegrade cerebral perfusion (ACP) during arch work; a second arterial cannula to the descending aorta (direct or femoral) protects the viscera and lower body [1,2]. annalsthoracicsurgery.org+1
  • Temperature/brain protection. Moderate–deep hypothermia with ACP during circulatory arrest is standard; lower-body perfusion may be continuous or intermittent based on exposure and team preference [2]. PMC
  • Venous drainage. Bicaval cannulation is typical when a VSD patch or intracardiac baffle is planned.

Pathway A — Arch repair with VSD closure

Indications. Choose when LV, LVOT, and aortic valve dimensions and function are adequate to support systemic output [1]. PubMed

Key steps.

  1. Arch reconstruction. Resect ductal tissue; perform end-to-end/extended end-to-side repair; augment with pericardium or homograft for a generous arch without a juxtaductal shelf.
  2. VSD closure. Close the malalignment/perimembranous VSD to route LV output through the native LVOT; protect the conduction axis along the posteroinferior rim.
  3. Assessment. Confirm unobstructed arch/LVOT and aortic valve competence by pressure measurements and echo.

Pitfalls and pearls.

  • Failure to recognize a long subaortic tunnel or small annulus risks late LVOT obstruction and reoperation [3,4]. PubMed+1
  • Ensure smooth, generous arch geometry; residual juxtaductal narrowing predisposes to recoarctation.

Pathway B — Yasui operation (for significant LVOT obstruction)

When to choose. If closing the VSD to the native LVOT would leave critical LVOT obstruction (tiny aortic annulus, long tunnel, dysplastic aortic valve), the Yasui provides a biventricular systemic pathway without neonatal subaortic enlargement.

Concept.

  1. DKS anastomosis (Norwood-type) to create a neo-aorta.
  2. Intraventricular LV-to-PA baffle through the VSD so the LV ejects to the neo-aorta.
  3. RV–PA conduit for pulmonary blood flow.

Evidence and outcomes.

  • The aortic annulus rule helps triage: when annulus diameter is < patient’s weight (kg) + 1.0 mm, LVOT bypass (Yasui/Norwood) is recommended; reoperation for LVOT is common if this threshold is ignored [2]. PubMed
  • Single-center and multicenter reports show excellent early survival and good mid- to long-term results after Yasui, with most late reoperations for RVOT conduit maintenance rather than LVOT/baffle issues [1,5,6]. PubMed+2PubMed+2
  • Primary vs staged Yasui. A recent series found no survival difference between primary and staged strategies but suggested a trend toward longer freedom from RVOT reoperation with a staged approach; overall ~85–90% survival at 10 years was reported [7]. PubMed

Technical notes. Plan baffle geometry to minimize turbulence and avoid LVOT/baffle kinking; size and route the RV–PA conduit to preserve branch PA geometry and future access. Video expositions of the dual-cannulation, single-stage IAA+VSD technique can be useful for team rehearsal and checklisting [8]. PubMed

Practical decision framework

  1. Left-sided adequacy present (LV, AoV, LVOT): perform arch reconstruction + VSD closure (two-ventricle).
  2. LVOT inadequate by metrics (e.g., annulus smaller than weight+1.0–1.5 mm or long tunnel): favor Yasui to bypass the LVOT [2]. PubMed
  3. Borderline anatomy: use institutional algorithms (z-scores, echo/CT, cath data) and team consensus; balance durability of systemic pathway against predictable RV–PA conduit maintenance [1,6]. PubMed+1

Postoperative focus points

  • Perfusion surveillance: right-arm vs lower-extremity pressures, NIRS, lactate, urine output.
  • Pre-discharge echo: rule out arch re-narrowing, LVOT/baffle gradients, conduit dysfunction, and valve regurgitation.
  • Follow-up: anticipate balloon/stent for recoarctation in infancy; staged conduit upsizing in Yasui patients is expected [6,7]. PubMed+1

Bottom line: A one-stage IAA strategy rises and falls with an honest appraisal of the left-sided complex. If the LVOT can carry the load, arch reconstruction with VSD closure restores near-normal physiology. If not, the Yasui safely reroutes systemic flow from the LV via a DKS and intraventricular baffle, accepting conduit maintenance in exchange for durable systemic output.

References

[1] Howell AJ, Argo MB, Barron DJ. Aortic Atresia or Complex Left Outflow Tract Obstruction in the Presence of a Ventricular Septal Defect. World J Pediatr Congenit Heart Surg. 2022;13(5):624-630.

[2] Hirata Y, Jonas RA, del Nido PJ, Turrentine MW, Brown JW, et al. Impact of aortic annular size on rate of reoperation for left ventricular outflow tract obstruction after repair of interrupted aortic arch and ventricular septal defect. Ann Thorac Surg. 2010;90(3):820-827.

[3] Luciani GB, Sfriso M, Faggian G, et al. One-stage repair of interrupted aortic arch, ventricular septal defect, and subaortic stenosis in the neonate. Ann Thorac Surg. 1996;62(1):175-181.

[4] Jonas RA, Quaegebeur JM, Kirklin JW, et al. Outcomes in neonates with interrupted aortic arch and ventricular septal defect: a multi-institutional study. J Thorac Cardiovasc Surg. 1994;107(4):1099-1113.

[5] Nakano T, Kado H, Tatewaki H, et al. The Yasui operation for patients with adequate-sized ventricles and ventricular septal defect associated with obstructions of the aortic arch and left ventricular outflow tract. Eur J Cardiothorac Surg. 2014;45(5):e166-e172.

[6] Ongoing single-institution experience over three decades: The Yasui operation—survival and reintervention profile. JTCVS Open. 2023;14:xxx-xxx. (PubMed PMID: 37808042).

[7] McKiernan MA, et al. Surgical Reinterventions After Primary vs Staged Yasui Procedure. Ann Thorac Surg. 2025;119(4):e??-e??.

[8] Reyes K, et al. Single-stage repair of interrupted aortic arch type B with ventricular septal defect: operative video tutorial. Multimedia Manual of Cardiothoracic Surgery. 2019; 2019:mmz???

[9] Gaynor JW, Wernovsky G, Rychik J, Rome JJ, DeCampli WM, Spray TL. Outcome following single-stage repair of coarctation with ventricular septal defect. Eur J Cardiothorac Surg. 2000;18(1):62-67.