PAPVR with Superior Sinus Venosus ASD — The Warden Procedure

In the common variant of PAPVR, the right upper pulmonary vein (RUPV) drains to the high SVC and coexists with a superior sinus venosus ASD (SV-ASD). In this geometry, intracardiac baffling alone risks narrowing the SVC. The Warden procedure—division of the SVC above the PV entry, intracardiac baffling of RUPV flow to the left atrium (LA), and reimplantation of the distal SVC to the right-atrial appendage (RAA)—was designed to preserve SVC caliber and protect the sinus node (SN) by avoiding suture lines across the cavo-atrial junction [1]. Contemporary series reinforce its role when the anomalous PV enters ≥10 mm above the cavo-atrial junction and highlight low rates of durable rhythm problems and manageable late SVC issues [2].

Preoperative Assessment

Define (i) the vertical height of RUPV entry into the SVC, (ii) SV-ASD size/location, (iii) RAA size and mobility (for a tension-free cavo-atrial reconstruction), and (iv) any additional anomalous veins. Transthoracic/TEE echocardiography establishes RV volume load and delineates inflow/outflow relationships; intraoperative TEE is invaluable for confirming SVC level, baffle plan, and detecting anastomotic narrowing in real time [3].

Operative Steps (Warden)

1. Confirm lesions and plan levels. After bicaval cannulation, confirm a superior SV-ASD and high RUPV entry into the upper RA/SVC.
2. Divide the SVC at/just above the RUPV level to free a tension-free distal SVC limb.
3. Baffle PV flow to the LA. Close the proximal SVC stump and patch the SV-ASD to route the RUPV stream across the defect into the LA; keep the baffle wide, smooth, and gently curved to avoid pulmonary venous (PV) gradients.
4. Cavo-atrial reconstruction. Open the RAA, remove trabeculations, and anastomose the distal SVC to the RAA with the line kept anterior to the SN region.
5. Anterior augmentation. Patch-augment the anterior aspect of the SVC–RAA anastomosis to maintain a nonrestrictive channel, then close the atriotomy and de-air carefully.

Rationale. By moving the caval anastomosis away from the terminal groove/SA nodal artery and eliminating intracaval baffle crowding, Warden reduces two principal failure modes of intracardiac single-/double-patch repairs—SVC stenosis and SN injury [1, 2, 4].

Technical Nuances and Pitfalls

• Sinus node protection. Keep atrial/SVC incisions and bites anterior to the SN and its artery; avoid deep posterior sutures at the cavo-atrial junction [4].
• Pulmonary venous patency. Use a generous, non-angulated baffle (thin pericardium or PTFE) and confirm laminar RUPV-to-LA flow by TEE and saline testing [3].
• SVC reach/tension. Mobilize distal SVC cranially and RAA caudally; if reach is limited, consider an interposition graft or extended anterior augmentation. Small SVC size (<10 mm) predicts higher risk of SVC obstruction and may favor augmentation or grafting [5].
• Hemostasis and residual shunt. Inspect the baffle–septal interface and posterior SVC–RAA line (common occult bleeders); pressure-test and close any residual interatrial communications.

Early Postoperative Management

• Rhythm surveillance. Junctional rhythms are common early; persistent SN dysfunction is infrequent with anterior incisions and tends to improve by discharge or follow-up [2].
• SVC pathway monitoring. Watch for upper-body venous congestion; Doppler should demonstrate low-gradient, laminar SVC–RAA flow.
• PV pathway. Ensure low Doppler velocities across the baffle; escalate to CT/MR for equivocal Doppler or suggestive symptoms.

Outcomes and Comparison to Alternatives

• Children. Dedicated pediatric series demonstrate excellent early and late outcomes with very low SVC obstruction and no operative mortality, supporting Warden when PV entry is high [6].
• Mixed/Adult populations. Large single-center experiences show no early mortality, low persistent SND, and rare late SVC obstruction that is typically amenable to stenting, with >90% survival at 5–10 years and modest reintervention rates [2].
• Technique comparisons. When anatomy allows a pure intracardiac repair, both double-patch and Warden achieve excellent anatomic results; however, Warden is consistently associated with less early sinus node dysfunction than double-patch, with similar mid-term rhythm status thereafter [7].
• Risk modifiers. Younger age at operation independently associates with higher reintervention risk after Warden, underscoring the importance of growth-friendly geometry and generous augmentation in small patients [8].

Choose Warden for high SVC entry of the RUPV (≈ ≥10 mm above the cavo-atrial junction) or when SVC caliber would be compromised by an intracardiac baffle—prioritizing SN-sparing anterior incisions, non-tortuous PV baffling, and liberal anterior augmentation to maintain durable patency and sinus rhythm [1, 2, 4–7].

References

[1] Warden HE, Gustafson RA, Tarnay TJ, Neal WA. An alternative method for repair of partial anomalous pulmonary venous connection to the superior vena cava. Ann Thorac Surg. 1984;38(6):601–605.

[2] 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.

[3] Aggarwal N, Gadhinglajkar S, Sreedhar R, Dharan BS, Chigurupati K, Babu S. Warden repair for superior sinus venosus atrial septal defect and anomalous pulmonary venous drainage in children: Anesthesia and transesophageal echocardiography perspectives. Ann Card Anaesth. 2016;19(2):293–299.

[4] Okonta KE, Agarwal V. Does Warden’s procedure reduce sinus node dysfunction after surgery for partial anomalous pulmonary venous connection? Interact Cardiovasc Thorac Surg. 2012;14(6):839–842.

[5] Lin H, Yan J, Wang Q, Gao Y, Fang J, Li Y. Outcomes of the Warden procedure for partial anomalous pulmonary venous drainage. Pediatr Cardiol. 2020;41(1):134–140.

[6] 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.

[7] Zubritskiy A, Naberukhin Y, Arkhipov A, Karaskov A, Mascetta G, Murzi B, Salciccia A, Farneti P, Troise G, Glauber M, et al. Outcomes of double-patch and Warden techniques in patients with supracardiac partial anomalous pulmonary venous connection. Heart Lung Circ. 2020;29(2):156–161.

[8] Binsalamah ZM, Ibarra C, Edmunds EE, Rios R, Nathan M, Baird CW, Emani SM, Del Nido PJ, McElhinney DB. Younger age at operation is associated with reinterventions after the Warden procedure. Ann Thorac Surg. 2021;111(6):2059–2065.