Bertram H, Paul T, Kaulitz R, Luhmer I, Kallfelz H C
Pädiatrische Kardiologie Kinderklinik, Medizinischen Hochschule Hannover.
Z Kardiol. 1996 Dec;85(12):899-905.
A coronary sinus defect results in a communication of variable size between the coronary sinus (CS) and the left atrium (LA). It is in most cases associated with a persistent left superior vena cava (l-SVC) and an atrial septal defect of the coronary sinus type and often part of a more complex cardiac malformation. If the CS to LA fenestration is the single cardiac defect, this anomaly provides the basis of an unusual form of interatrial communication, which may cause diagnostic difficulties. Two patients with this rare anomaly are reported. A 9-year-old boy presented with history of mild cyanosis pronounced after physical activity (SaO2 < 90%) since the age of 3. Comprehensive pulmonary and cardiological diagnostic procedures in the referring hospital yielded normal findings. Finally, a 1-SVC draining into a mildly dilated coronary sinus could be demonstrated echocardiographically. Contrast echocardiography revealed a right-to-left-shunt at atrial level. Diagnosis of a partially unroofed coronary sinus was subsequently confirmed during cardiac catheterization. The second patient was primarily diagnosed echocardiographically to have an atrial septal defect of the primum type (ASD I) in the first year of life. After pulmonary vascular markings on chest-x-ray had increased, she was assigned to our hospital for cardiac catheterization. Slight arterial desaturation (SaO2 88%) was present due to a large l-SVC anomalously draining into the LA, the innominate vein was missing. The roof of the coronary sinus was predominantly absent. Combined with a large atrial septal defect of the coronary sinus type, this resulted in significant left-to-right-shunt and right-to-left-shunt at atrial level due to a pure unroofed coronary sinus.
A coronary sinus defect with partial or complete unroofing of the CS and a persistent l-SVC should be considered in the differential diagnosis of an otherwise unexplained systemic desaturation and in patients with both left-to-right-shunt and right-to-left-shunt at atrial level.
冠状静脉窦缺损导致冠状静脉窦（CS）与左心房（LA）之间存在大小不一的交通。在大多数情况下，它与永存左上腔静脉（l-SVC）和冠状静脉窦型房间隔缺损相关，且常是更复杂心脏畸形的一部分。如果CS至LA的开窗是唯一的心脏缺损，这种异常构成了一种不寻常的房间交通形式的基础，可能导致诊断困难。报告了两例患有这种罕见异常的患者。一名9岁男孩自3岁起就有运动后轻度发绀的病史（动脉血氧饱和度[SaO2]<90%）。转诊医院的全面肺部和心脏诊断检查结果正常。最后，超声心动图显示一条l-SVC引流至轻度扩张的冠状静脉窦。对比超声心动图显示心房水平存在右向左分流。随后在心脏导管检查中证实了部分冠状静脉窦顶缺如的诊断。第二名患者在出生后第一年经超声心动图初步诊断为原发孔型房间隔缺损（ASD I）。胸部X线显示肺血管纹理增多后，她被转诊至我院进行心脏导管检查。由于一条大的l-SVC异常引流至LA且无名静脉缺如，存在轻度动脉血氧饱和度降低（SaO2 88%）。冠状静脉窦顶主要缺如。结合大的冠状静脉窦型房间隔缺损，由于单纯的冠状静脉窦顶缺如，导致心房水平出现明显的左向右分流和右向左分流。
在鉴别诊断原因不明的全身性血氧饱和度降低以及心房水平存在左向右分流和右向左分流的患者时，应考虑存在部分或完全冠状静脉窦顶缺如的冠状静脉窦缺损。
