Cor Vasa 2020, 62(5):513-516 | DOI: 10.33678/cor.2020.002

Isolated Unroofed Coronary Sinus without Persistent Left Superior Vena Cava

Tomáš Toporcer^a, Ján Luczy^a, Miroslav Gbur^b, Gabriel Valočík^b, Adrián Kolesár^a, František Sabol^a

^a Department of Heart Surgery, Medical Faculty, Pavol Jozef Šafárik University and the Eastern Slovak Institute for Cardiovascular Diseases Ltd., Košice, Slovakia

^b Department of Cardiology, Medical Faculty, Pavol Jozef Šafárik University and the Eastern Slovak Institute for Cardiovascular Diseases Ltd., Košice, Slovakia

Unroofed coronary sinus (UCS) represents the rarest type of atrial septal defect, with incidence less than 1% of all atrial septal defects, or less than 0.1% of all congenital heart anomalies. A case report of a 57-year-old man with four years of misdiagnosed UCS is presented. The patient showed dyspnoea, angina pectoris, and a new onset of atrial fibrillation. The diagnosis of UCS was finally established on the basis of right-sided cardiac catheterization, echocardiography, and computer tomography. Defect roofing with redirection of the coronary sinus into the left atrium was performed. UCS is in 75% of cases associated with other heart anomalies, especially persistent left superior vena cava. For isolated UCS, closing via the path from the side of right atrium is sufficient therapy with good long-term results. Persistence of left superior vena cava requires a technically serious occlusion of the defect from the left atrium or a redirection of the persistent vein itself.

Keywords: Atrial septal defect, Echocardiography, Heart surgery, Unroofed coronary sinus

Received: November 3, 2019; Revised: January 7, 2020; Accepted: January 8, 2020; Published: November 1, 2020  Show citation