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So as to produce totally anomalous systemic venous connection, all of the systemic venous tributaries, along with the coronary sinus, should be connected with the morphologically left atrium. Previous descriptions of this rare constellation of anomalous connections of the systemic venous tributaries of the heart have been compromised by the inclusion of individuals having isomeric atrial appendages. In these settings, most frequently, the totally, or almost totally, anomalous systemic venous connections are associated with a sinus venosus defect. It is the anomalous pulmonary venous connections that then create a venovenous bridge, which permits the systemic venous tributaries to drain into the morphologically left atrium, even though they may be predominantly connected to the right atrium. More rarely, it is feasible for the primary atrial septum to develop so as to leave the systemic venous sinus in direct connection with the body of the morphologically left, rather than the morphologically right, atrium. We report a series of patients potentially falling into the category of anomalous systemic venous connections. The findings show a spectrum from partially to totally anomalous connections, with some better interpreted on the basis of anomalous drainage. Included in our cases, nonetheless, is an autopsied example of totally anomalous systemic venous connection produced by an abnormal location of the primary atrial septum. We discuss the potential morphogenesis for this finding. We emphasize the distinction that needs to be made between anomalous systemic venous connections and anomalous systemic venous drainage.

The Warden procedure for the correction of a right-sided partial anomalous pulmonary venous connection to the high superior vena cava is well established. It has the advantages of avoiding sinoatrial node dysfunction and pulmonary and systemic venous obstruction. In the case related here, a 3-year-old girl presented with a superior vena cava type of sinus venosus atrial septal defect and an anomalously draining right upper pulmonary vein, with bilateral superior venae cavae. Our approach to the Warden procedure was through a right posterolateral thoracotomy, which provided additional advantages. [ABSTRACT FROM AUTHOR]

We present a case of persistent left superior vena cava, anomalous right pulmonary venous connection to the right-sided superior vena cava and sinus venosus atrial septal defect detected by computed tomography (CT) pulmonary angiography and echocardiography. These defects were surgically corrected using a double-patch technique. In fact, CT can provide anatomical information about a complex anomaly in the systemic and pulmonary vasculatures. Though modern imaging techniques are useful for detecting complex cardiovascular disease, careful echocardiographic examination should be performed to diagnose complex cardiac anomalies. [ABSTRACT FROM AUTHOR]

Balloon atrial septostomy is ordinarily a safe palliative procedure for cyanotic congenital heart disease; however, if echocardiographic guidance is unavailable and fluoroscopy is used, distortions in the cardiac anatomy can invalidate the usual landmarks. Herein, we report iatrogenic mitral papillary muscle rupture during balloon atrial septostomy in a 4-day-old male neonate with total anomalous connection of the pulmonary veins. The anomalous connection and severe mitral regurgitation were emergently corrected, and the patient grew and developed normally. At age 24 years, he had only mild residual mitral regurgitation and was in New York Heart Association functional class I. In addition to describing the surgical treatment and positive late outcome of a rare complication, we highlight the importance of accurately evaluating balloon catheter location during atrial septostomy, especially in patients with a small left atrium. [ABSTRACT FROM AUTHOR]

Abstract: This report describes a rare case of anomalous systemic and pulmonary venous return that was surgically corrected. It consisted of left inferior vena cava with hemiazygous continuation into the persistent left superior vena cava, partial anomalous pulmonary venous return from the right lung and sinus venosus atrial septal defect. [Copyright &y& Elsevier]

We report a case of an anomalous pulmonary vein on chest X-ray resembling a scimitar sign in an 80-year-old female undergoing investigation of syncope. Multislice computed tomography (CT) with multiplanar reformatting and maximum intensity projections demonstrated an aberrant right inferior pulmonary vein coursing inferomedially towards the diaphragm before turning superiorly and draining normally into the left atrium. The diagnosis of an incidental meandering right pulmonary vein was established. The case is used to review the literature on this rare pulmonary anomaly, including pathogenesis, its relationship with scimitar syndrome and scimitar variant, and diagnosis, with an emphasis on the role modern CT techniques can play in non-invasive diagnosis. A revision to the nomenclature of pulmonary vascular anomalies is proposed to help reduce confusion in the literature.