Cardiothoracic Sciences Centre, 28730All India Institute of Medical Sciences, New Delhi, India.
World J Pediatr Congenit Heart Surg. 2020 Nov;11(6):733-741. doi: 10.1177/2150135120938352.
Repair 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.
Between 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.
There 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.
The 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.
使用心内隔瓣修复伴有高位部分性肺静脉异常连接（PAPVC）的上腔静脉窦缺损可能会导致体循环或肺静脉通路阻塞和窦房结功能障碍（SND）。我们修复所有类型上腔静脉窦缺损的手术策略主要是为了避免上述并发症，并保留上腔房交接处的生长潜力。
在 2007 年至 2019 年期间，50 例年龄在 2 至 60 岁（平均 17.6±16.7 岁）的患者接受了双筒技术修复上腔静脉窦缺损。异常肺静脉在 17 例患者中引流至上腔房交接处，在 33 例患者中引流至上腔房交接上方 2 厘米以上。
无早期或晚期死亡，无再次手术。平均随访 103.9（±50.2）个月后，所有患者均存活，SND 无事件生存率为 97.9%（±标准误差，0.02%；95%CI：0.86-0.99）。无患者发生体循环或肺静脉通路阻塞。1 例（2%）患者因病态窦房结综合征需要永久性起搏器。
双筒法是一种方便、安全、有效的上腔静脉窦缺损修复方法。它提供了上腔静脉的双重引流，保留了上腔房交接处，不会导致体循环或肺静脉通路阻塞，可用于包括高位 PAPVC 在内的所有病例。保留腔房交接处并用自体心房组织重建体循环静脉通路可避免 SND 并保留生长潜力。
