• Ujjwal Kumar Chowdhury, Srikant Sharma, Lakshmi Kumari Sankhyan, Niwin George, Sukhjeet Singh, Suruchi Hasija, Niraj Nirmal Pandey, and Mani Kalaivani.
• Cardiothoracic Sciences Centre, 28730All India Institute of Medical Sciences, New Delhi, India.
• World J Pediatr Congenit Heart Surg. 2020 Nov 1; 11 (6): 733-741.

BackgroundRepair of superior sinus venosus defect with high partially anomalous pulmonary venous connection (PAPVC) using an intracardiac baffle may be complicated by systemic or pulmonary venous pathway obstruction and sinus nodal dysfunction (SND). Our surgical strategy for repair of all types of superior sinus venosus defect has evolved chiefly to avoid the abovementioned complications and preserving the growth potential of the superior cavoatrial junction.MethodsBetween 2007 and 2019, fifty consecutive patients aged 2 to 60 (mean, 17.6±16.7) years underwent repair of superior sinus venosus defect using the double-barrel technique as described. The anomalous pulmonary veins drained into the superior cavoatrial junction in 17 patients and more than 2 cm above the cavoatrial junction in 33 patients.ResultsThere were no early or late deaths and no reoperations. At a mean follow-up of 103.9 (±50.2) months, all survived the operation, and actuarial freedom from SND was 97.9% (±standard error, 0.02%; 95% CI: 0.86-0.99). No patient had systemic or pulmonary venous pathway obstruction. A permanent pacemaker was required in one (2%) patient for sick sinus syndrome.ConclusionsThe double-barrel method is an expedient, safe, and effective technique in superior sinus venosus defect. It provides dual drainage of superior vena cava preserving the superior cavoatrial junction without causing systemic or pulmonary venous pathway obstruction and can be utilized in all cases including those with high PAPVC. Preservation of the cavoatrial junction and use of autogenous atrial tissue for systemic venous pathway avoids SND and preserves growth potential.

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