Koronarsinusdefekt (unroofed coronary sinus)
Minimally
invasive surgical repair for unroofed coronary sinus syndrome directed by three-dimensional transesophageal echocardiography. Preoperative cardiac computed tomography. Preoperative cardiac computed tomography showed extensive communication between the right and left atria caused by partial absence of the roof of the coronary sinus. LA left atrium, LV left ventricle, RA right atrium, RV right ventricle, CS coronary sinus
Minimally
invasive surgical repair for unroofed coronary sinus syndrome directed by three-dimensional transesophageal echocardiography. Preoperative three-dimensional transesophageal echocardiography. Preoperative three-dimensional transesophageal echocardiography showed there was an unroofed portion (20 × 10 mm) at the atrial side between segments P2 and P3 of the posterior mitral leaflet. P2 P2 segment of posterior mitral leaflet, P3 P3 segment of posterior mitral leaflet, A3 A3 segment of anterior mitral leaflet, URP unroofed portion
The value of
cardiac CT in the diagnosis of unroofed coronary sinus syndrome. Patient No.1, type I: A MPR axial view showed enlargement of the CS. B, C MPR and MIP showed ASD and PLSVC. The CS communicated LA to RA. D, E VR color rendering and CR showed complete absence of the parietal wall of the CS. PLSVC was connected to RSVC via Inn.V. F CR cross-sectional view visualized the spatial relationship between the ASD and the CS defect. RA right atrium, and the section of PLSVC(*) flowing into the left atrium also displayed. CS coronary sinus, LA left atrium, RSVC right superior vena cava, PLSVC persistent left superior vena cava, ASD atrial septal defect, Inn.V. innominate vein
The value of
cardiac CT in the diagnosis of unroofed coronary sinus syndrome. Patient No.3, type II: A MPR axial view showed enlargement of the CS. B, C MPR axial and coronal views showed L-CPV and R-CPV ectopically drained into the enlarged CS, and formed cor triatrium. D: MPR oblique view was a representation of the ASD and the defected CS, E, F VR and CR directly showed the UCSS and TAPVC, and SVC is displayed normally but no PLSVC exists. G: CR cross-sectional view showed the UCSS and ASD. RA right atrium, CS coronary sinus, GCV great cardiac vein, PE pericardial effusion, LA left atrium, L-CPV left common pulmonary vein, R-CPV right common pulmonary vein, TAPVC total anomalous pulmonary vena cava, SVC superior vena cava, LBCV left brachiocephalic vein, RBCV right brachiocephalic vein, MCV middle cardiac vein, GCV great cardiac vein, ASD atrial septal
The value of
cardiac CT in the diagnosis of unroofed coronary sinus syndrome. Patient No.6, type III: A, B MPR and MIP axial views showed an enlarged CS. C MIP coronal view showed the middle segment of the unroofed CS connecting to the LA. PLSVC converged into the LA. D MIP showed that variant great cardiac vein(GCV) drained into the LA. E, F VR color rendering and CR visualized the enlarged CS, PLSVC and Inn.V.. G CR cross-sectional view visualized the defect size of the CS. RA right atrium, CS coronary sinus, MCV middle cardiac vein, LA left atrium, RSVC right superior vena cava, PLSVC persistent left superior vena cava, Inn.V. innominate vein
The value of
cardiac CT in the diagnosis of unroofed coronary sinus syndrome. Patient No.8, type IV: A, B MPR axial and coronal views showed perforation of the parietal wall of the end segment of CS. Metal density (white arrowhead) at the atrial septum was a postoperative manifestation. C MIP axial view showed both the ASD and the CS wall defect. D, E VR color rendering and CR visualized the CS. F CR cross-sectional view provided the spatial location of the ASD and the CS defect. Postoperative metal density was visible (black arrowhead). RA right atrium, LA left atrium, CS coronary sinus, RSVC right superior vena cava, GCV great cardiac vein, ASD atrial septal defect
The value of
cardiac CT in the diagnosis of unroofed coronary sinus syndrome. Patient No. 9, type V: A, B: MPR and MIP showed the "Z"-shaped vessel communicated the LA to the CS. C MIP showed the convergence of the CS into the RA. D, E VR and CR showed the spatial relationship between the CS and the right and left atrium. LA left atrium, CS coronary sinus, GCV great cardiac vein, RA right atrium, MCV middle cardiac vein
An unroofed coronary sinus is a rare variant of atrial septal defect (ASD). The atrial wall between the coronary sinus and left atrium is either partially or completely absent, resulting in a left-to-right shunt.
It is associated with persistent left-sided SVC and heterotaxy syndromes.
Clinical presentation
Clinical presentation is variable and depends on the degree of left-to-right shunting. When there are associated symptoms it is then often termed unroofed coronary sinus syndrome (URCS).
Classification
Unroofed coronary sinus has been classified into 4 groups:
- type I: completely unroofed with persistent LSVC
- type II: completely unroofed without persistent LSVC
- type III: partially unroofed mid portion
- type IV: partially unroofed terminal portion
Radiographic features
The diagnosis is often made on transthoracic echocardiography, but because of the deep position of the anomaly within the chest, transesophageal echocardiography, cardiac CT, or cardiac MRI may also be useful.
Siehe auch:
Assoziationen und Differentialdiagnosen zu Koronarsinusdefekt (unroofed coronary sinus):
