MR findings of a rare defect, coronary sinus ASD

Abstract

The coronary sinus drains the cardiac veins into the right atrium. An unroofed coronary sinus is the least common type of atrial septal defect (<1%). An unroofed coronary sinus also communicates with the left atrium, in addition to its normal communication with the right atrium. This defect is difficult to diagnose. MR provides accurate anatomic details about the location and size of defect. Phase contrast velocity-encoded MR also helps towards quantifying the shunt volume. This case represents classical findings of this rare defect. Our patient first presented in the 8th decade with features of right heart failure.

Unroofed coronary sinus in a patient with neurofibromatosis type 1

“…Unroofed coronary sinus atrial septal defect is a rare cardiac malformation characterized by communication between the coronary sinus and the left atrium, resulting in partial or total absence of the coronary sinus roof ( 13 - 16 ) . This communication represents less than 1% of atrial septal defects, often from 0.2 to 0.6 cases by 1,000 live births ( 16 ) . The diagnosis of unroofed coronary sinus became possible after the advent of the echocardiography.…”

“…A comunicação interatrial do tipo seio coronário sem teto é uma malformação cardíaca rara, caracterizada pela comunicação entre o seio coronário e o átrio esquerdo, resultante da ausência parcial ou total do teto do seio coronário ( 13 - 16 ) . Essa comunicação representa menos de 1% dos defeitos do septo atrial, com frequência de 0,2 a 0,6 casos por 1.000 nascidos vivos ( 16 ) . O diagnóstico de seio coronário sem teto tornou-se possível após o advento da ecocardiografia.…”

Unroofed coronary sinus: An unusual interatrial communication and a rare childhood entity

“…It is not considered a true defect of the atrial septum and is characterized by interatrial shunting across the ostium of the coronary sinus. [123] It is in the majority of cases, associated with a persistent left superior vena cava, and is often a part of a more complex cardiac malformation. [123] An isolated coronary sinus to left atrial fenestration is extremely unusual.…”

“…[123] It is in the majority of cases, associated with a persistent left superior vena cava, and is often a part of a more complex cardiac malformation. [123] An isolated coronary sinus to left atrial fenestration is extremely unusual. [1234] It often poses diagnostic difficulties.…”

“…[123] An isolated coronary sinus to left atrial fenestration is extremely unusual. [1234] It often poses diagnostic difficulties. [234] We report a case of this unusual form of interatrial communication in a 7-year-old girl child who presented to us with breathlessness on exertion.…”