Anomalous Systemic Veins & Unroofed Coronary Sinus

Seoul National University HospitalDepartment of Thoracic & Cardiovascular Surgery

Unroofed Coronary Sinus Syndrome

1. Definition A spectrum of cardiac anomalies in which part or all of the common wall between coronary sinus and the left atrium is absent. 2. History Winter ; Pathologic paper in 1954. Tuchman ; Isolated case report in 1956. Helseth ; The descriptive phrase in 1974.

Morphology of Unroofed Coronary Sinus

1. Unroofed coronary sinus with persistent left SVC Innominate vein is absent in 80-90%.

2. Unroofed coronary sinus without persistent left SVC A classical coronary sinus type of ASD.

3. Partially unroofed midportion of the coronary sinus Rare form, may be a large left-to-right shunt. Associated with TA, or in the presence of Lt SVC

4. Partially unroofed terminal portion of the coronary sinus. 5. Relationship to cor triatriatum and AV canal defects 6. Atrial isomerism The majority of such patients have an AV canal defects.

Diagnostic Criteria and Natural History

1. Diagnosis Echoardiography Catheter passage Cineangiogram Radionuclide angiography

2. Natural History 1) Cyanosis & polycythemia determines natural history. 2) Cerebral embolism & abscess complicate in 10-25%

Techniques of Operation

1. Isolated unroofed coronary sinus with left SVC 1) Reroofing the coronary sinus 2) Excision of atrial septum and atrial baffling 3) Rerouting coronary sinus and constructing atrial septum 4) Transfer of Lt SVC 5) Ligation of Lt SVC

2. Partially unroofed midportion of coronary sinus 3. Partially unroofed terminal portion of coronary sinus 4. Unroofed coronary sinus with Lt SVC and AV canal defect 5. Unroofed coronary sinus with other complex anomalies

Unroofed Coronary SinusOperation

Extracardiac Techniques

• A; LSVC to LA• B; LSVC to RA• C; LSVC to RA • D; LSVC to PA

Indications for Operation

1. Operation is advisable because of arterial desaturation, risk of cerebral emboli and the good results of operation.

2. When unroofed coronary sinus is associated with major cardiac anomalies, associated anomaly usually presents a clear indication.

Results and Functional Status

1. Early death Risk is low in isolated form. Higher in complex anomaly 2. Better understanding and methods Including avoidance of tunnel repair Improve this results considerably 3. Survival and functional status Good in uncomplicated Reoperation in tunnel technique

Anomalous Systemic Veins

1. Prevalence . 2% among operated cases . Common in atrial isomerism 2. Classification . Anomalies of cardinal venous system Both SVC, coronary sinus . Anomalies of IVC . Anomalies of valve of sinus venosus

Embryology of Systemic Vein

• Umbilical vein & vitelline vein (1st vein) Eventually form IVC,portal vein, ductus

venosus• Common cardinal vein by anterior and

posterior cardinal veins(2nd vein) Eventually form SVC, innominate vein,

coronary sinus, azygous vein

Anomalous Systemic Veins 1. Persistent left SVC draining into the coronary sinus . Most common . Cross anterior to Lt PA . 3-10% of CHD . 75% have a right SVC 2. Azygous continuation of IVC . 0.6% of CHD . rarely an isolated lesion . frequent with complex defects and atrial isomerism 3. Separate drainage of IVC and hepatic vein . rarely seen . 1% of venous anomalies