Absent right superior vena cava with left superior vena cava draining into unroofed coronary sinus with common atrium in a child with an atrioventricular septal defect

The association of absent right superior vena cava and persistent left superior vena cava draining into unroofed coronary sinus with common atrium and the atrioventricular septal defect is an extremely rare form of the congenital cardiac disorder with only one case reported so far, hence, can be missed preoperatively if not carefully looked for. Failure to detect absent right superior vena cava beforehand may otherwise pose difficulties in carrying out invasive surgical or medical interventions.     

Persistent left superior vena cava draining to left atrium with normal coronary sinus

In embryology, a persistent connection of the left superior vena cava to the left atrium rarely coexists with a coronary sinus. We herein report an unusual case of persistent left superior vena cava terminating in a left atrium with normal coronary sinus, which was revealed at the time of permanent pacemaker implantation after a second operation for recurrent left atrioventricular valve regurgitation. Because this anomaly had gone undiagnosed at the first operation, we were unable to diagnose it prior to the second operation, because the preoperative coronary angiogram clearly demonstrated a coronary sinus that was not dilated. We would have repaired the anomaly using a patch or other procedure had it been diagnosed before the second operation in order to prevent cyanosis or brain complications secondary to right-to-left shunting. One should always be on guard for this rare condition.     

Persistent left superior vena cava draining to left atrium with normal coronary sinus

In embryology, a persistent connection of the left superior vena cava to the left atrium rarely coexists with a coronary sinus. We herein report an unusual case of persistent left superior vena cava terminating in a left atrium with normal coronary sinus, which was revealed at the time of permanent pacemaker implantation after a second operation for recurrent left atrioventricular valve regurgitation. Because this anomaly had gone undiagnosed at the first operation, we were unable to diagnose it prior to the second operation, because the preoperative coronary angiogram clearly demonstrated a coronary sinus that was not dilated. We would have repaired the anomaly using a patch or other procedure had it been diagnosed before the second operation in order to prevent cyanosis or brain complications secondary to right-to-left shunting. One should always be on guard for this rare condition.     

Atretic right superior vena cava with left superior vena cava draining directly into left atrium with absent coronary sinus in an adult with partial atrioventricular canal defect and complete heart block

The association of atretic right superior vena cava with persistent left superior vena cava draining directly into left atrium with absent coronary sinus in atrioventricular canal defect is virtually unknown in adults with no case reported so far. Though atretic right superior vena cava with persistent left superior vena cava is an extremely rare venous anomaly seen in congenital heart disease, it has important clinical implications in cardiac surgery and interventional cardiology. Atrial arrhythmias and right bundle branch block are common with advancing age in partial atrioventricualr canal defect but complete heart block has scarcely been reported in the medical literature.     

19例左上腔静脉引流入左心房的诊治

目的 总结左上腔静脉引流入左心房的诊治体会.方法 1998年2月至2012年1月收治19例先天性心脏病合并左上腔静脉引流入左心房患儿,男10例,女9例,年龄0.5～12.0岁;体重6～28 kg.均经心脏彩色多普勒、左上肢声学造影及心导管心室造影、CT等检查明确诊断.采用直接结扎或切断缝合、自体心包左心房内隧道、房间隔重建、双向Glenn手术治疗.结果 全组病例无死亡.术后恢复过程平稳.术后随访,最长者已11年,情况良好.结论 左上腔静脉引流入左心房术前确诊较难,加深对其特性的认识可以提高术前检出率,手术治疗效果良好.     

Repair of sinus venosus defect in a patient with untreated vein of Galen aneurysmal malformation

The vein of Galen aneurysmal malformation (VGAM) is a rare cerebral arteriovenous shunt, which may be associated with a congenital cardiac defect. Embolisation of the VGAM may be undertaken in the neonatal period if necessary, but is safer in infancy. Recent advances in neuroradiology have changed the prognosis for this group with many patients achieving survival with normal development. This case report describes a patient with a sinus venosus defect (SVD) and a VGAM and considers both the optimal timing of treatment of the two malformations and the conduct of anaesthesia for open repair of the SVD in the presence of an untreated VGAM.     

Surgical repair of common atrium associated with persistent left superior vena cava draining into the left atrium

A case of 54-year-old female of common atrium associated with persistent left superior vena cava (PLSVC) draining into the left atrium and absence of the coronary sinus was experienced. The corrective surgery was done by using a horseshoe-shaped autologous pericardial patch to make a new atrial septum to allow the PLSVC draining into the right atrium. Since the common atrium is frequently associated with maldrainage of the PLSVC, the surgical treatment should be determined according to existence of the PLSVC and its opening site.     

Single (left) superior vena cava draining into the left atrium. Surgical repair

Anomalous drainage of a single left superior vena cava into the left atrium is rare, and correction presents a technical problem. This report describes a patient with absent right superior vena cava, a persistent large left superior vena cava draining to the left atrium, and an intact interatrial septum.Surgical correction was accomplished by removing the entire septum and placing a cloth baffle to cover the orifices of the pulmonary veins and mitral valve, thus partitioning pulmonary venous blood into the left ventricle. This technique of septal excision and replacement is applicable to other complicated anomalies involving the systemic and pulmonary venous channels and their relationship to the two atria.The surgical management of related anomalies of the superior vena cava is also discussed.     

Atrial septal defect with drainage of the inferior vena cava into the left atrium

Atrial septal defect (ASD) with drainage of the inferior vena cava (IVC) into the left atrium (LA) is a rare congenital anomaly. Few cases have been reported in the literature. We present a 17-year-old female with an ASD and an anomalous drainage of the IVC into the LA leading to cyanosis since early childhood. Diagnosis was documented by computed tomography (CT) angiography and confirmed intra-operatively. The patient underwent successful surgical correction with an uneventful postoperative course.     

Light-guided surgery to repair coronary sinus orifice atresia with left superior vena cava

An 8-month-old male with coronary sinus orifice atresia, left superior vena cava, and single ventricle underwent light-guided coronary sinus unroofing concomitant with bidirectional cavopulmonary anastomosis to circumvent coronary sinus hypertension. During surgery, a 2.25 Fr angioscopic catheter was inserted into the coronary sinus via the left superior vena cava. The coronary sinus, lit by the illumination obtained from the catheter, was readily located from the left atrial interior, and unroofed. Light-guided coronary sinus unroofing is an easy, safe, and quick technique for the creation of unobstructed coronary sinus drainage in patients with coronary sinus orifice atresia and left superior vena cava.     

冠状窦顶重建治疗无顶综合征合并心内膜垫缺损和永存左上腔静脉

目的 总结冠状静脉窦顶重建在无顶冠状静脉窦综合征合并心内膜垫缺损和永存左上腔静脉的外科治疗经验.方法 2000年1月至2008年1月共753例心内膜垫缺损病人行畸形矫治手术,15例(2%)同时合并永存左上腔静脉和无顶冠状静脉窦综合征,包括部分型心内膜垫缺损12例,完全型心内膜垫缺损3例.均在行心内膜垫缺损矫治术同期重建冠状静脉窦顶,1例直接结扎左上腔静脉,14例将左上腔静脉隔入右房,其中10例用自体心包补片,4例通过折叠左房后壁完成冠状静脉窦顶的重建,最后在重建的冠状静脉窦开口的左侧缝合修补房间隔缺损.结果 术后早期1例死于严重肺部感染.术后随访3个月～6年,无远期死亡病例.14例术后均未见腔静脉、肺静脉回流梗阻和心房水平残余分流.1例行左房后壁折叠分隔左上腔静脉者术后早期超声心动图提示左上腔静脉入左房顶处血流速度偏快.结论 合并心内膜垫缺损和永存左上腔静脉的无顶冠状静脉窦综合征外科治疗有难度,冠状窦顶重建技术能够取得良好的治疗效果.     

Right superior vena cava draining in the left atrium: anatomical, embryological, and surgical considerations

A newborn, with SDS (S = situs viscero-atrialis solitus, D = D-loop of the ventricles, S = solitus, normally related great arteries) anomalous drainage of the right superior vena cava in the left atrium, intact atrial septum, and anomalous drainage of the right superior pulmonary veins in the right superior vena cava, underwent surgical repair at our institution. This rare cyanotic, congenital, cardiac malformation is herein described with particular regard to its anatomical, embryological, and surgical implications.     

The triad of persistent left superior vena cava connected to the coronary sinus, right superior vena cava draining into the left atrium, and atrial septal defect: report of a successful operation for a rare anomaly

The triad of right superior vena cava connecting to the left atrium, persistent left superior vena cava draining into the right atrium (coronary sinus), and atrial septal defect is a rare malformation. Recently, we successfully corrected this anomaly in a 47-year-old man.