A Rare Case of an Unroofed Coronary Sinus With a Persistent Left Superior Vena Cava Diagnosed by Two-Dimensional Transthoracic Echocardiography

Abstract

An unroofed coronary sinus is a rare congenital anomaly in the roof of the coronary sinus causing a communication between the coronary sinus and the left atrium leading to a left to right shunt. It is often associated with a persistent left superior vena cava and other complex congenital lesions like anomalous pulmonary venous return and heterotaxy. Since it is a deep-seated defect, it is seldom diagnosed by transthoracic two-dimensional (2D) echocardiography and requires multimodal imaging for a diagnosis. Here, we present the case of a 27-year-old male in whom the defect was very apparent on standard 2D transthoracic echocardiography. Transthoracic 2D echocardiography revealed situs solitus, levocardia, and a dilated coronary sinus with unroofing which was most prominent in the standard parasternal long-axis view and the foreshortened apical four-chamber view. A color Doppler demonstrated a flow from the left atrium into the dilated coronary sinus. The right ventricle and atrium were dilated with mild pulmonary arterial hypertension. There was no right ventricular dysfunction. Examination with modified suprasternal views showed a left superior vena cava. All four pulmonary veins drained into the left atrium. Other chambers of the heart and great vessels were structurally normal without coarctation or patent ductus arteriosus. The interventricular septum was intact and atrioventricular and ventriculoatrial concordance was preserved. Detection of a dilated coronary sinus by transthoracic 2D echocardiography must be followed by multimodal imaging techniques like cardiac computed tomography and transesophageal echocardiography to detect and manage associated defects.

Simultaneous Identification of Unroofed Coronary Sinus Atrial Septal Defect and Atrial Septal Defect Secundum Using Cardiac Computed Tomography Angiography

“…However, CCTA appears to be superior to both TTE and TEE due to its high spatial and temporal resolutions, multiplanar reconstruction capabilities, and large field of view. CCTA can detect and characterize as well as identify related abnormalities of the heart and pulmonary vasculature [ 1 , 8 , 9 ]. The preferred treatment for UCS-ASD is believed to be surgery [ 1 ].…”

Case Report: Candidate Genes Associated With Prenatal Ultrasound Anomalies in a Fetus With Prenatally Detected 1q23.3q31.2 Deletion

“…Unroofed coronary sinus (UCS) refers to the communication between the coronary sinus wall and the left atrium, which is often associated with the persistent left superior vena cava (PLSVC). In fact, UCS is a special type of atrial septal defect, which is easy to be missed due to the lack of specific clinical features (Tonni and Grisolia, 2020 ; Khadkikar et al, 2021 ). The diagnosis of the lesion is highly significant for the patient's prognosis because it can result in pulmonary hypertension, brain abscess or cerebral embolism (Bonardi et al, 2012 ).…”

Raghib Syndrome and Pulmonary Arterial Hypertension in a Pediatric Patient: Case Report and Literature Review

“…Perinatal management of Raghib syndrome cases can be optimized in spite of the uncertainty surrounding the adequate timing of surgical intervention [ 8 ]. The left-to-right shunt will gradually lead to right heart insufficiency and eventually to pulmonary arterial hypertension, which usually develops late during the disease’s course [ 9 ].…”