Invasive management of unroofed coronary sinus: A systematic review

Abstract

Unroofed coronary sinus (UCS) is a rare congenital anomaly, constituting under 1 % of atrial septal defects. Caused by partial or complete deficiency of the coronary sinus roof, UCS is often difficult to diagnose. While surgery remains the standard treatment, transcatheter approaches are emerging. We conducted a systematic review to evaluate outcomes of surgical and transcatheter approaches to UCS repair. Studies from MEDLINE, Embase, SCOPUS, and Web of Science were screened and selected based on predefined criteria. We extracted data on patient demographics, operative techniques, associated defects, and outcomes. Thirteen studies involving 293 patients with UCS were included. Majority (66.4 %) were male, with a mean age of 17.5 ± 11.5 years. UCS type 1A, marked by the presence of a persistent left superior vena cava and absent coronary sinus, was the most common subtype (50.4 %). Surgical repair was performed in 95.2 % of patients, with intracardiac baffle (34.1 %) and patch repair (21.9 %) being the predominant techniques. The remaining 4.8 % of patients underwent transcatheter repair, typically using Amplatzer® Septal Occluder device, with no reported procedural complications. In-hospital mortality rate was 2.7 %, and 30-day mortality was 3.0 %. Postoperative complications were rare, including residual defects (0.42 %), atrial fibrillation (2.9 %), and complete heart block requiring pacemaker (1.2 %). There were no cases of ischemic stroke. Surgical repair of UCS is associated with low postoperative mortality and complication rates, affirming its status as the standard of care. Transcatheter techniques may be feasible for select patients, though further studies are needed to to assess long-term outcomes and optimize criteria for patient selection.

Keywords: Atrial septal defect; Coronary sinus; Surgery; Transcatheter repair; Unroofed.