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.

Unroofed coronary sinus (UCS) is a rare subtype of atrial septal defect. It is frequently associated with a persistent left superior vena cava and is often part of a more intricate cardiac malformation.

This report describes a rare case of an adolescent patient with UCS featuring atrial situs solitus, absence of the right superior vena cava and a persistent left superior vena cava draining into the left atrium consistent with total unroofing of the coronary sinus. This was associated with concurrent severe mitral insufficiency secondary to redundant and prolapsing leaflets, and a substantial left-to-right shunt across the coronary sinus orifice. A comprehensive examination of the existing literature is included, shedding light on the diagnostic challenges of UCS and describing the available surgical options within the context of mitral valve surgery.

UCS is a complex condition requiring careful consideration of associated anomalies and a tailored surgical approach.

Unroofed coronary sinus syndrome (UCSS) is a rare cardiovascular malformation with nonspecific clinical manifestations that easily causes misdiagnosis and missed diagnosis. The aim of this study is to present the different features of UCSS by various CCT (cardiac CT) postprocessing techniques and evaluate the diagnostic advantages of CCT.

9 UCSS patients who were diagnosed by imaging and undergone both CCT and transthoracic echocardiography (TTE) were included in this study, and their CCT images were reviewed. The UCSS images were classified by multiplanar reformations, maximum intensity projection, volume rendering and cinematic rendering. The size of CS roof defect was also measured.

Only 4 of 9 CCT confirmed UCSS patients were detected by TTE (4/9, 44.4%), the sensitivity of TTE was lower compared to CCT by Fisher's exact test (P < 0.05). UCSS was classified according to the Kirklin and Barratt Boyes's method, including 1 case was classified as type I, 4 cases as type II, 1 case as type III, 2 cases as type IV, 1 case as type V (variant type), and TTE was undiagnosed in all type III-V patients. Additionally, CCT showed 12 extra malformations in these patients, only 5 of them were found by TTE (5/12, 41.7%), and TTE missed all extracardiac malformations. The mean size of CS roof defect was 3.04 ± 1.57 cm.

CCT with various postprocessing technologies has excellent value in diagnosing and differentiating subtypes of UCSS, measuring size of coronary sinus defect, describing accompanying cardiovascular abnormalities.

• Isolated coronary sinus defect without persistent left superior vena cava is a rare cardiac defect. • Diagnosis can be difficult in the absence of other defects in an asymptomatic child. • Missed or difficult cases to diagnose may be found incidentally in adulthood. • Surgical outcome in the absence of any other defects can be very good.

Coronary sinus atrial septal defect (ASD) is a rare congenital cardiac anomaly, which might be difficult to diagnose. In this report, we describe a patient with small secundum ASDs and an associated large coronary sinus ASD, which had been missed at initial evaluation. The diagnosis of coronary sinus ASD was established by using transesophageal echocardiography after percutaneous closure of a small secundum ASD at another center. Patient underwent corrective surgery.

An unroofed coronary sinus (URCS) is a rare anomaly that produces communication between the left atrium (LA) and the coronary sinus (CS), resulting in a left-to-right shunt. Due to the lack of symptoms and particular anatomical characteristics, this disease is difficult to diagnose, and prone to be overlooked. An 85-year-old man was admitted to our hospital because of anorexia and shortness of breath. On physical examination, a systolic murmur was heard at the apex, and pitting edema was present in both legs. Transthoracic echocardiography showed severe regurgitation of the mitral valve and tricuspid valve. Transesophageal echocardiography confirmed a shunt between the LA and the CS. Because of uncontrolled heart failure, we performed surgical repair 50 days after admission. Under cardiopulmonary bypass and heart arrest, the URCS was detected in the LA and directly sutured. Repair of the mitral and tricuspid valves and the Maze procedure were also performed. The patient had a good postoperative course, and has been doing well for 2 years. Transesophageal echocardiography is helpful for diagnosis of URCS. Mitral regurgitation might raise the left atrial pressure and result in increase in shunt flow, causing left and right heart failure in elderly patients. <Learning objective: Diagnosis of an unroofed coronary sinus (URCS) is often overlooked in adult patients because of the lack of symptoms and/or particular anatomical characteristics. We herein describe an octogenarian patient who was diagnosed with URCS in association with severe mitral and tricuspid regurgitation. Transesophageal echocardiography helped to identify the location of the URCS. The cause of the heart failure was mitral regurgitation, which raised the left atrial pressure and increased shunt flow.>.

Mitral inflow obstruction caused by dilated coronary sinus is a rare congenital cardiac anomaly that must be differentiated from other causes of left ventricular inflow obstruction such as cor triatriatum, supramitral membrane, and supramitral ridge, because their surgical repair is different. Although preoperative transthoracic echocardiography is the primary imaging modality for diagnosis, intraoperative transesophageal echocardiography has a higher sensitivity and specificity over transthoracic echocardiography in differentiating these lesions. We report 2 cases of supramitral obstruction where the intraoperative transesophageal echocardiography has refined the preoperative diagnosis and changed the course of surgical repair.

We report a case of unroofed coronary sinus (URCS) in a 42-year-old female. At an outpatient clinic, she was found to have an atrioventricular septal defect and mitral regurgitation with pulmonary hypertension, and she was transferred to our institute for surgical treatment. Both atrioventricular valves were located at the same level, and both the right atrium (RA) and right ventricle were enlarged on two-dimensional transthoracic echocardiography. Color Doppler imaging demonstrated severe mitral and tricuspid regurgitation and a left-to-right shunt from the left atrium (LA) to the RA. Although an ostium primum defect of the atrial septum was suspected, the exact position of the shunt flow was unclear. Two-dimensional transesophageal echocardiography (2D-TEE) could visualize a direct communication between the LA and coronary sinus. Three-dimensional transesophageal echocardiography (3D-TEE) clearly visualized the entire route from the coronary sinus into the LA and RA. The utility of 3D-TEE as a modality complementary to 2D-TEE in diagnosis of URCS was confirmed.