van Son J A, Black M D, Haas G S, Falk V, Hambsch J, Onnasch J F, Mohr F W
Department of Cardiac Surgery, Herzzentrum, University of Leipzig, Germany.
Thorac Cardiovasc Surg. 1998 Dec;46(6):371-4. doi: 10.1055/s-2007-1010257.
Complex unroofed coronary sinus with a persistent left superior vena cava has as its commonest major associated intracardiac anomaly a partial or complete atrioventricular canal defect. In this clinical setting, biventricular repair with construction of a complex intra-atrial baffle from the pulmonary veins to the mitral valve has a reported mortality rate of as high as 50%. Looking for an improvement, we have carried out an extracardiac repair of the anomalous systemic venous component with atrial septation. In 2 infants (aged 7 and 12 weeks) with unroofed coronary sinus, bilateral superior venae cavae, right isomerism, and complete atrioventricular canal, in addition to patch closure of the ventricular component of the atrioventricular septal defect, a baffle was constructed between the pulmonary veins and the mitral valve. In four subsequent infants (aged 7,10,16, and 20 weeks) with unroofed coronary sinus, bilateral superior venae cavae, complete atrioventricular canal, right isomerism (n = 2), and mild infundibular stenosis (n = 1), repair consisted of end-to-side anastomosis of the left superior vena cava to the right superior vena cava and complete repair of the atrioventricular canal and associated conditions. There was no mortality. The early postoperative course in the two patients with intra-atrial baffle was characterized by increased left-atrial pressure (18 and 20 mm Hg), with varying degrees of pulmonary venous congestion, supraventricular tachycardias, and systemic hypotension. The pulmonary venous congestion increased, so that one patient was successfully converted 10 weeks postoperatively to an extracardiac repair with septation of the atria and the other will probably follow. In the 4 patients with a primary extracardiac repair, the hemodynamic result was excellent, with a median left-atrial pressure of 11 mm Hg on the first postoperative day. At a median follow-up of 12 months, all 5 patients with an extracardiac repair are clinically well with widely patent anastomoses between the left and right superior venae cavae. The extracardiac repair technique for complex unroofed coronary sinus, as opposed to the intra-atrial baffle repair, avoids creation of a small and low-compliance left-atrial compartment with the potential for development of pulmonary venous congestion.
合并永存左上腔静脉的复杂无顶冠状静脉窦最常见的主要相关心内异常是部分或完全性房室通道缺损。在这种临床情况下，采用从肺静脉到二尖瓣构建复杂心房内挡板的双心室修复术，据报道死亡率高达50%。为寻求改善，我们采用了对异常体静脉成分进行心外修复并同时进行房间隔造口术。对于2例患有无顶冠状静脉窦、双侧上腔静脉、右心异构和完全性房室通道的婴儿（年龄分别为7周和12周），除了用补片闭合房室间隔缺损的心室部分外，还在肺静脉和二尖瓣之间构建了一个挡板。在随后的4例患有无顶冠状静脉窦、双侧上腔静脉、完全性房室通道、右心异构（2例）和轻度漏斗部狭窄（1例）的婴儿（年龄分别为7周、10周、16周和20周）中，修复包括左上腔静脉与右上腔静脉的端侧吻合以及房室通道和相关病症的完全修复。无一例死亡。采用心房内挡板修复的2例患者术后早期病程表现为左心房压力升高（分别为18和20 mmHg），伴有不同程度的肺静脉淤血、室上性心动过速和全身性低血压。肺静脉淤血加重，因此1例患者在术后10周成功转换为采用心房分隔的心外修复，另1例可能也会如此。在4例最初采用心外修复的患者中，血流动力学结果极佳，术后第一天左心房压力中位数为11 mmHg。在中位随访12个月时，所有5例行心外修复的患者临床情况良好，左右上腔静脉之间的吻合口广泛通畅。与心房内挡板修复不同，复杂无顶冠状静脉窦的心外修复技术避免了形成一个小且顺应性低的左心房腔，从而有可能避免肺静脉淤血的发生。
