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Anomalous Systemic Veins & Unroofed Coronary Sinus. Seoul National University Hospital Department of Thoracic & Cardiovascular Surgery. Unroofed Coronary Sinus Syndrome. 1. Definition A spectrum of cardiac anomalies in which part or
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Anomalous Systemic Veins & Unroofed Coronary Sinus Seoul National University Hospital Department 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%
Techniquesof 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
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
