PAPVR with Sinus Venosus ASD — Double-Patch Technique

Partial anomalous pulmonary venous return (PAPVR) of the right upper pulmonary vein (RUPV) to the SVC/right atrium (RA) with a superior sinus venosus ASD produces a left-to-right shunt of oxygenated blood into the right heart, driving RA/RV volume overload, RV dilatation, and functional TR. Repair must reroute anomalous PV flow to the LA and close the sinus venosus ASD, while preserving SVC patency and protecting the sinus node (SN). Comparative series underscore a trade-off among techniques: the double-patch strategy tends to reduce venous pathway narrowing relative to single-patch repairs, but may increase sinus node dysfunction (SND); Warden repairs generally minimize SN injury at the expense of a caval anastomosis that can rarely narrow over time [1, 2, 3, 4].

Operative Strategy (Double-Patch/Two-Patch)

Exposure and Cannulation

• Bicaval cannulation with high SVC control.
• Lateral RA atriotomy extended anteriorly onto the SVC, deliberately anterior to the SN and its arterial supply to mitigate rhythm injury [2].

Step 1 — Define the Defects

• Identify the RUPV orifice at the SVC/RA junction and delineate the high, posterior sinus venosus ASD; use stay sutures for orientation.

Step 2 — First Patch (Intracardiac Baffle to LA)

• Sew a wide, gently curved baffle (autologous pericardium or synthetic) from the SVC/RA junction across the sinus venosus ASD to the LA septal rim, ensuring:
• Non-angulated geometry to avoid PV turbulence/gradients,
• Bites away from the SN region near the SVC–RA junction,
• No encroachment on SVC lumen.

These maneuvers address failure modes observed with narrower baffles (PV gradients, SVC crowding) and align with data showing lower venous gradients when the PV tract is generously routed [1].

Step 3 — Second Patch (Cavo-atrial Augmentation)

• Augment the SVC–RA pathway with a second patch to restore a wide, laminar inflow channel and further protect the SN by keeping the reconstruction anterior and capacious [1, 2].

What the double-patch accomplishes. (i) Dependable rerouting of anomalous PV flow into the LA and (ii) re-expansion of the SVC–RA pathway, reducing the two main failure modes of single-patch repairs—pulmonary venous obstruction and SVC narrowing [1].

Intraoperative Pitfalls & Avoidance

• Pulmonary venous obstruction

Use a large, redundant baffle with smooth curves; test with saline/TEE for unobstructed laminar LA inflow [1].

• SVC stenosis

Always augment the cavo-atrial junction with the second patch; avoid tight/posterior suture lines that narrow the pathway [1].

• Sinus node dysfunction

Keep all incisions and bites anterior; avoid the terminal groove and SN artery territory where rhythm changes were most associated in clinical series [2, 3].

• Residual shunt

Inspect the baffle–septal interface circumferentially; test under filling and close any fenestrations.

Postoperative Management & Surveillance

• Rhythm monitoring. Junctional rhythms may appear early; protect and surveil the SN given higher SND risk historically reported after two-patch repairs [2, 3].
• Echocardiography at discharge and follow-up:
• Baffle patency (no PV gradient; laminar LA inflow),
• SVC pathway (Doppler gradient, venous congestion),
• RV size/TR—expected regression as volume load abates.
• Cross-sectional imaging (CT/MR) when Doppler is equivocal or symptoms suggest SVC pathway narrowing (facial edema, headaches, exercise intolerance).

Alternatives & Variations

• Warden procedure. For high SVC entry of anomalous PVs, division of the SVC with distal SVC–RA appendage anastomosis plus intracardiac baffling avoids SN injury near the cavo-atrial junction and has excellent early/late survival; late SVC obstruction is uncommon and often managed percutaneously [4]. Pediatric series report low SVC obstruction (~2.4%) with very low SND [5].
• Modified double-patch with right atrial appendage. In contemporary series, a right-atrial-appendage–assisted augmentation achieved no SVC or PV stenosis and only transient SND (13.6%), with recovery on follow-up—supporting geometry-first, SN-sparing reconstruction [6].
• Single-patch techniques. Feasible in selected anatomies, but higher risk of SVC narrowing when the SVC–RA junction is already tight; double-patch augmentation is preferred when that junction is at risk [1, 2].

Expected Outcomes (What to Tell the Team)

• Shunt elimination with restored LA drainage of RUPV,
• Progressive RV reverse remodeling and improvement in functional TR,
• Low reintervention for PV/SVC obstruction when baffle geometry is generous and cavo-atrial augmentation is routine [1, 4, 5],
• Rhythm outcomes depend on technique and incision planning: two-patch repairs reduce venous gradients but have shown higher SND than single-patch/Warden in several series; programs increasingly favor single-patch or Warden when anatomy allows to maximize normal sinus rhythm [2, 3, 4].

Practical Selection Heuristics

• Favor double-patch when: the SVC–RA junction is narrow, multiple RUPV orifices require a wide intracardiac baffle, or a single-patch would compromise SVC caliber [1].
• Favor Warden when: anomalous PVs enter high in the SVC or when SN protection is paramount; anticipate excellent survival and rare, manageable SVC issues [4, 5].
• Incision planning matters regardless of technique—anterior SVC/RA incisions and SN-sparing bites reduce rhythm complications [2, 3].

Summary

The double-patch repair for PAPVR with superior sinus venosus ASD is a durable, anatomy-respecting strategy: a first patch to route all anomalous PV flow to the LA and a second patch to reconstruct a generous SVC–RA channel. Evidence shows it minimizes venous gradients compared with single-patch but carries a higher SND signal than single-patch/Warden in some cohorts. When entry is high, a Warden offers SN protection with low, often correctable SVC narrowing. Tailor the choice to venous geometry and SN risk, and execute with generous patching and anterior incisions to optimize patency, rhythm, and RV remodeling [1–6].

References

[1] Iyer AP, Somanrema K, Pathak S, Manjunath PY, Pradhan S, Krishnan S. Comparative study of single- and double-patch techniques for sinus venosus atrial septal defect with partial anomalous pulmonary venous connection. J Thorac Cardiovasc Surg. 2007;133(3):656-659.

[2] Stewart RD, Bailliard F, Kelle AM, Backer CL, Young L, Mavroudis C. Evolving surgical strategy for sinus venosus atrial septal defect: effect on sinus node function and late venous obstruction. Ann Thorac Surg. 2007;84(5):1651-1655.

[3] Stephens EH, Mongé MC, Eltayeb O, Patel A, Webster G, Cornicelli MD, et al. Evolution and current results of a unified strategy for sinus venosus surgery. Ann Thorac Surg. 2021;111(3):980-986.

[4] Griffeth EM, Dearani JA, Mathew J, Graham GC, Connolly HM, King KS, Schaff HV, Stephens EH. Early and late outcomes of the Warden and modified Warden procedure. Ann Thorac Surg. 2022;114(5):1723-1729.

[5] Yong MS, Griffiths S, Robertson T, Brink J, d’Udekem Y, Brizard C, Konstantinov IE. Outcomes of the Warden procedure for partial anomalous pulmonary venous drainage in children. Interact Cardiovasc Thorac Surg. 2018;27(3):422-426.

[6] Elzein C, Pawale A, Garcia CS, Takahashi M, Nayfeh T, Geirsson A, et al. Repair of superior sinus venosus atrial septal defect using a modified two-patch technique. Ann Thorac Surg. 2020;109(2):583-587.