Interrupted Aortic Arch — #5 Biventricular Repair

Date
November 11, 2025
URL
https://x.com/WithAScalpel/status/1988234747282071708?s=20
Categories
Interrupted Aortic ArchCardiac Surgery

Interrupted Aortic Arch — #5 Biventricular Repair (Arch Repair + VSD Closure vs Yasui)

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Overview

Interrupted aortic arch (IAA) with VSD spans a spectrum from a well-formed left heart to intrinsic hypoplasia of the LVOT/aortic root. The operative goal is to reconstruct an unobstructed systemic outflow and restore a durable, physiologic two-ventricle circulation. When the left heart (MV, LV, LVOT, AoV/ascending aorta) is adequate, arch repair with VSD closure re-establishes native LV-to-aorta continuity. When the LVOT is too small or obstructed to support systemic flow, the Yasui operation (LV–PA baffle + DKS neo-aorta + RV–PA conduit) provides the definitive two-ventricle pathway. Mid-term outcomes after contemporary one-stage repair of arch + VSD are excellent, but the major failure mode remains late LVOT obstruction, motivating careful preoperative selection and intraoperative strategy [1].

Patient selection and decision framework

Key anatomic predictors. Across multiple series, the aortic annulus dimension indexed to body weight is the most practical discriminator for strategy: an annulus (mm) smaller than patient weight (kg) + 1–1.5 mm is associated with a high risk of LVOT reoperation after conventional arch repair with VSD closure, and many such patients benefit from a primary or staged Yasui pathway [2,10]. Additional markers that shift decision-making toward Yasui include small mitral and aortic valves and global arch hypoplasia on preoperative echocardiography [3], as well as type B IAA, bicuspid aortic valve, and aberrant right subclavian artery, all of which correlate with the need for LVOT intervention [4,6].

Algorithm (practical).

  • Adequate left heart and LVOT (favorable annulus threshold, reassuring valve and LV metrics): proceed with arch repair + VSD closure; aim for direct LV–Ao continuity via the native LVOT.
  • Borderline/small LVOT (annulus below weight-based threshold; small AoV/ascending aorta; adverse arch phenotypes): select Yasui as the durable two-ventricle strategy—either primary in the OR or staged after physiologic stabilization.

Operative strategies

A. Arch repair with VSD closure (two-ventricle repair through the native LVOT)

  1. Arch reconstruction. Under antegrade cerebral perfusion and hypothermia, mobilize the descending aorta and complete an extended end-to-side anastomosis to the ascending aorta with liberal patch augmentation to eliminate any isthmic narrowing; ligate/divide the ductus.
  2. VSD closure. Patch close the VSD, deliberately preserving the conduction axis and avoiding subaortic crowding. The LV then ejects across the native LVOT/AoV into the reconstructed arch.
  3. Quality checks. Post-repair echo targets include a laminar LVOT with trivial subaortic turbulence and an arch velocity <2 m/s without diastolic runoff. Contemporary series show low mortality for single-stage arch + VSD repair, with reinterventions driven by recurrent coarctation or late LVOT obstruction [1].

B. Yasui operation (two-ventricle repair bypassing an inadequate LVOT)

  1. LV–PA baffle. Through the VSD, construct a short, non-angulated baffle from the LV to the pulmonary valve/main PA (future neo-aortic valve/outflow).
  2. DKS anastomosis. Perform a generous Damus–Kaye–Stansel to unite the native aorta with the MPA, creating the neo-aorta that receives LV output.
  3. RV–PA conduit. Re-establish pulmonary flow via an RV–PA conduit sized for somatic growth; expect conduit exchange over time.
  4. Technical priorities. Keep the LV–PA tunnel straight and roomy; avoid conduction tissue at the VSD margin; protect the coronaries during DKS orientation; ensure hemiarch/arch work is tension-free.

Perfusion and myocardial protection (both strategies)

  • Cannulation and ACP. Use perfusion strategies that provide continuous cerebral protection and, when needed, intermittent or continuous lower-body perfusion to minimize visceral ischemia during arch work.
  • Ischemia minimization. Limit cross-clamp time, maintain meticulous deairing (especially with intracardiac baffles), and avoid excessive hypertension on wean.

Intraoperative pitfalls and avoidance

  • Residual LVOT/baffle stenosis (Yasui). Oversize the baffle and avoid kinks at the VSD edge.
  • Coronary compromise (DKS). Plan the hood and orientation to prevent torsion/compression.
  • Conduction injury (VSD work). Respect the septal leaflet hinge and posteroinferior rim.
  • Arch mismatch/tension. Favor liberal augmentation to lower recoarctation risk.

Early postoperative priorities

  • Afterload and perfusion. Support systemic pressure without excessive hypertension across fresh arch and DKS suture lines.
  • PVR control. Optimize ventilation and acid–base to keep PVR low—particularly important with an RV–PA conduit.
  • Completion echo before ICU transfer. Confirm unobstructed systemic pathway (native LVOT or LV–PA baffle/DKS), competent valves, low arch gradient, free RV–PA conduit flow, and good biventricular function.

Long-term surveillance and expected reinterventions

  • LVOT/neo-aortic pathway. After conventional arch + VSD repair, late LVOT obstruction remains the main reason for reintervention; risk is highest when the preoperative aortic annulus and aortic root are small [2,5,11]. In a recent multi-institutional cohort, 5-year freedom from LVOT reoperation was ~77% after either isolated IAA repair or Yasui, and 100% after Ross–Konno; conal resection at the primary repair had the lowest freedom from LVOT reoperation (~47%) [6].
  • RV–PA conduit (Yasui). Reoperation is expected for conduit replacement; single-center experience reports reoperation in roughly 40–45% by mid-term follow-up, most commonly conduit exchange, with excellent preservation of ventricular function [7,8].
  • Valvular status. Track native AoV competence (arch + VSD) and neo-aortic valve function (Yasui).
  • Rhythm and ventricular performance. Screen for conduction disturbances related to septal patching and follow biventricular function longitudinally.
  • Global outcomes. When anatomy is amenable, Yasui provides a robust two-ventricle solution and compares favorably with single-ventricle pathways in mid-term survival; contemporaneous single-institution data showed lower operative mortality and superior early/mid-term survival for Yasui versus Norwood cohorts [9].

References

[1] 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. PubMed

[2] Hirata Y, Quaegebeur JM, Mosca RS, Takayama H, Chen JM. 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(2):588-592. PubMed

[3] Abarbanell G, Border WL, Schlosser B, Morrow G, Tretter JT, Tweddell JS, et al. Preoperative echocardiographic measures in interrupted aortic arch: Which ones best predict surgical approach and outcome? Congenit Heart Dis. 2018;13(3):409-417. PubMed

[4] Alsoufi B, Manlhiot C, Ehrlich A, McCrindle BW, Jaeggi E, Honjo O, et al. Predictors of late left ventricular outflow tract obstruction after interrupted aortic arch repair. J Thorac Cardiovasc Surg. 2016;151(4):1070-1079.e1.

[5] Szaflik K, Goreczny S, Ostrowska K, Kaźmierczak P, Moll M, Moll JA. Predictors of Left Ventricular Outflow Tract Obstruction After Conventional Repair for Patients with Interrupted Aortic Arch or Coarctation of the Aorta, Combined with Ventricular Septal Defect: A Single-Center Experience. Pediatr Cardiol. 2022;43(3):607-618. PubMed

[6] Luo S, Schoof PH, Hickey E, Alsoufi B. The Fate of the Left Ventricular Outflow Tract Following Interrupted Aortic Arch Repair. World J Pediatr Congenit Heart Surg. 2024;15(5):641-651. PubMed+1

[7] Kanter KR, Kirshbom PM, Kogon BE. Biventricular repair with the Yasui operation (Norwood/Rastelli) for systemic outflow tract obstruction with two adequate ventricles. Ann Thorac Surg. 2012;93(6):1999-2005. PubMed

[8] Nakano T, Kado H, Tatewaki H, Hinokiyama K, Machida D, Ebuoka N, Yasui H. 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. PubMed

[9] Carrillo SA, Mainwaring RD, Schaffer JM, Hanley FL, Reddy VM. Contemporaneous comparison of the Yasui and Norwood procedures at a single institution. J Thorac Cardiovasc Surg. 2015;149(2):508-513. PubMed

[10] Riggs KW, He X, Forbess JM, et al. How Small Is Too Small? Decision-Making and Outcomes in Neonates with Interrupted Aortic Arch and a Small Left Ventricular Outflow Tract. World J Pediatr Congenit Heart Surg. 2019;10(3):371-381. PubMed