Clarifying the anatomy and physiology of totally anomalous systemic venous connection.

ABSTRACT: The description of totally anomalous systemic venous connection is limited to case reports. In this review, we seek to clarify anatomic, physiologic, and hemodynamic aspects of this extremely rare anomaly. We also present findings of two patients in whom connection of all the systemic veins was anomalous. In the first patient, with usual atrial arrangement, all systemic veins, including the coronary sinus, were connected anomalously to the morphologically left atrium. Limited left-to-right shunt across an atrial septal defect provided the only source of blood flow to the lungs. The diagnosis was established by saline contrast echocardiography and cardiac catheterization. Extreme hypoplasia of the right ventricle precluded corrective surgery, so we performed a bidirectional Glenn operation,

SUBMITTER: Gupta SK 

PROVIDER: S-EPMC5594938 | biostudies-literature | 2017 Sep-Dec

REPOSITORIES: biostudies-literature