Abstract
BACKGROUND: Cor triatriatum and supramitral ring are congenital anomalies which result in formation of three chambers of atria. To the best of our knowledge, simultaneous presence of both entities in the same patient resulting in the formation of four chambers of atria has not been described in the literature. Here, we report a case of simultaneous presence of cor triatriatum and supramitral ring associated with Raghib syndrome and Eisenmenger syndrome. CASE PRESENTATION: We report the case of a middle-aged gentleman, who presented to us with features of atrial septal defect with Eisenmenger syndrome. Multimodality imaging confirmed the simultaneous presence of supramital ring and cor triatriatum resulting in "cor tetratriatum" along with Raghib syndrome. Presence of Eisenmenger syndrome compelled us to offer medical therapy for the patient. CONCLUSION: This is the first case report describing the simultaneous presence of supramitral ring and cor triatriatum resulting in a new entity-"cor tetratriatum".