Ebstein’s Enigma: Managing Unforeseen Interventricular Septal Hematoma After One and Half Ventricular Repair for Ebstein’s Anomaly

ABSTRACT: Interventricular septal hematoma (IVSH) is an extremely rare complication reported after the congenital heart surgery. An IVSH is defined as a hemorrhagic dissection between muscle bundles of the interventricular septum. Herein, we discuss a case of IVSH, which developed following one and half ventricular repair for Ebstein's anomaly in a 47-year-old lady. It appeared initially as an intraventricular mass on postoperative echocardiography creating a diagnostic dilemma. It was confirmed to be an IVSH on intraoperative transesophageal echocardiography during surgical re-exploration indicated for increasing size of the lesion. The IVSH was not drained due to the possible risk of development of an iatrogenic ventricular septal defect and decided to be treated conservatively. It was followed up on the postoperative serial echocardiography. Frequent echocardiographic monitoring during the perioperative period is essential to detect this rare complication. The IVSH should be distinguished from an RV thrombus as the management strategy differs between them. The IVSH may be managed conservatively in a hemodynamically stable patient, whereas the RV thrombus requires immediate thrombolysis or surgical intervention. We also deliberate on the role of perioperative echocardiography in the diagnosis and decision making.