Total Anomalous Pulmonary Venous Return (TAPVR): Surgical Considerations

In TAPVR, none of the pulmonary veins (PVs) connect to the left atrium (LA); all pulmonary venous return reaches the LA only through an atrial communication. Surgical repair must establish a non-obstructive pathway from all PVs to the LA, close the interatrial communication appropriately, and eliminate obstructed venous segments. The presence and severity of pulmonary venous obstruction (PVO) is the dominant driver of urgency and early risk [1, 2].

Classification and urgency

Classify TAPVR by the vertical vein (VV) pathway—supracardiac, cardiac (to coronary sinus/right atrium), infracardiac, or mixed. Across population-based and single-center cohorts, supracardiac is the most frequent, followed by infracardiac, cardiac, then mixed variants [1, 2]. Any obstruction along the common pulmonary venous channel (CPV), VV, or the systemic-venous entry elevates pulmonary venous/capillary pressures and mandates urgent—often neonatal—repair. In unobstructed forms, early elective repair is favored to limit RV volume overload and evolving pulmonary vascular changes [2, 3].

Preoperative assessment: four anatomic domains

1. Pulmonary veins (PVs). Confirm the presence and course of all four PVs and scrutinize for intrinsic hypoplasia, ostial/segmental stenosis, or long-segment narrowing. Intrinsic PV disease is the strongest predictor of postoperative PVS and should lower the threshold for a sutureless/pericardial marsupialization strategy [4, 5].
2. Common pulmonary venous channel (CPV). Define whether PVs coalesce into a CPV, its caliber/length, and its relationship to the LA. A long or narrow CPV raises shear and restenosis risk; plan the landing site (posterior LA dome/roof) for a short, straight anastomosis [4, 7].
3. Vertical vein (VV). Map the CPV–VV junction and the VV course (supracardiac/cardiac/infracardiac/mixed). Typical pinch points include bronchial/PA crossings (supracardiac) and the diaphragmatic hiatus/hepatic venous bed (infracardiac). Preoperative VV obstruction correlates with worse hypoxemia and perioperative risk and must be definitively relieved by the repair [2].
4. Heart chambers & atrial communication. Assess LA/LV size and compliance. Chronic unloading can yield small or stiff LV, where immediate full preload may be poorly tolerated—consider temporary VV patency (“pop-off”) and/or ASD fenestration. Size and restrictiveness of PFO/ASD influence pre-op oxygenation and post-op LA filling strategy [3, 5].

Operative strategy

Core steps (conventional repair).

1. Unobstructed LA anastomosis. Create a wide, tension-free anastomosis between the CPV (or unroofed coronary sinus in cardiac TAPVR) and the LA. Favor a posterior, non-angulated, straight-line route to minimize turbulence and restenosis [4, 7].
2. Vertical vein (VV) management.
• In unobstructed TAPVR with adequate LA/LV compliance, ligate the VV to prevent residual L→R shunt.
• With preoperative obstruction, labile pulmonary hypertension, or small/noncompliant LA/LV, leave the VV partially patent and close later after hemodynamic stabilization [2, 5].
3. Atrial septum. Complete closure is standard; use a small fenestration selectively when LA pressure is labile or LV compliance is questionable to avoid post-op pulmonary edema/low output [3, 5].

Sutureless (pericardial marsupialization) repair. For diffuse intrinsic PV hypoplasia, a narrow/long CPV, or recurrent PVS, open the posterior LA and marsupialize the LA edges to the pericardium around the confluence, leaving PV/CPV tissue untouched. This reduces circumferential PV suturing, traction, and scar, improving freedom from restenosis compared with conventional resection/re-anastomosis in high-risk anatomy [4, 6].

Technical pearls

• Keep the LA–CPV anastomosis short, wide, straight; mobilize both channels adequately to avoid tension/kinks.
• Ensure meticulous hemostasis and deairing in the deep posterior field.
• In cardiac TAPVR, completely unroof the coronary sinus and reclose the atrial septum without narrowing the CS–LA communication.
• Before separation from bypass, confirm by TEE: laminar PV inflow to the LA, no gradients/kinks, acceptable LA pressure, and balanced RV/LV function [3–5].

Postoperative management and surveillance

• Prevent pulmonary hypertensive crises (oxygenation/ventilation, deep sedation, inhaled NO as needed).
• Trend LA pressure, systemic saturation, and Dopplers for early obstruction (rising PV/CPV velocities, edema, RV dysfunction).
• Pulmonary venous stenosis (PVS) is the major late hazard—often declares within the first 6–12 months and carries a marked survival penalty; early detection and reintervention (balloon, stent, or reoperation—often sutureless) are crucial [4–6].
• Expect RV reverse remodeling after unobstructed repair; LV filling may be transiently abnormal early post-op [3, 5].

Summary

Durable TAPVR repair hinges on complete anatomic mapping, a generous, non-angulated LA connection, judicious VV management, and tailored atrial septal closure. Sutureless techniques expand options for small or diseased PVs. Because pre-op PVO and intrinsic PV disease strongly predict post-op PVS and survival, vigilant PH management and lifelong imaging surveillance are essential [1–6].

References

[1] Seale AN, Uemura H, Webber SA, Partridge J, Roughton M, Ho SY, McCarthy KP, Jones S, Shaughnessy L, Sunnegårdh J, Hanseus K, Berggren H, Johansson S, Rigby ML, Keeton BR, Daubeney PEF. Total anomalous pulmonary venous connection: morphology and outcome from an international population-based study. Circulation. 2010;122(25):2718–2726.

[2] Wang JK, Wu MH, Lin MT, Chiu IS, Chen WJ, Lue HC. Clinical spectrum of total anomalous pulmonary venous connection: 15 years’ experience in one medical center. J Chin Med Assoc. 2004;67(7):331–335.

[3] Frommelt PC, Sheridan DC, Deatsman S, Yan K, Simpson P, Frommelt MA, Litwin SB, Tweddell JS. Unobstructive total anomalous pulmonary venous return: impact of early elective repair on the need for prolonged mechanical ventilatory support. Pediatr Cardiol. 2010;31(8):1191–1197.

[4] White BR, Ho DY, Faerber JA, Katcoff H, Glatz AC, Mascio CE, Stephens P Jr, Cohen MS. Repair of total anomalous pulmonary venous connection: risk factors for postoperative obstruction. Ann Thorac Surg. 2019;108(1):122–129.

[5] Seale AN, Uemura H, Webber SA, Partridge J, Roughton M, Ho SY, McCarthy KP, Jones S, Shaughnessy L, Sunnegårdh J, Hanseus K, Berggren H, Johansson S, Rigby ML, Keeton BR, Daubeney PEF. Outcome of postoperative pulmonary venous obstruction after repair of total anomalous pulmonary venous connection. J Thorac Cardiovasc Surg. 2013;145(5):1255–1262.

[6] Harada T, Nakano T, Oda S, Kado H. Surgical results of total anomalous pulmonary venous connection in 256 patients. Interact Cardiovasc Thorac Surg. 2019;28(3):421–426.