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Unroofed coronary sinus syndrome: A case report
Cuihong Tian1, Wen Su1, Zhaowei Zhu1
1Department of Cardiology, Second Xiangya Hospital, Central South University, Changsha 410011, China.
Summary
Unroofed coronary sinus syndrome (UCSS) is a rare congenital heart defect. This case report details a 50-year-old patient diagnosed with UCSS and treated successfully.
Area of Science:
- Cardiology
- Congenital Heart Disease
- Embryonic Development
Background:
- Unroofed coronary sinus syndrome (UCSS), or coronary sinus septal defect, is a rare congenital heart anomaly, accounting for less than 1% of atrial septal defects.
- It results from incomplete formation of left atrial venous folds during embryonic development.
- UCSS can lead to significant clinical manifestations, including fatigue and dyspnea on exertion.
Observation:
- A 50-year-old patient presented with fatigue and shortness of breath after exercise.
- Color Doppler echocardiography revealed coronary sinus dilatation (17 mm×14 mm), suggesting a persistent left superior vena cava.
- Pulmonary angiography and cardiac computed tomographic angiography confirmed partial anomalous pulmonary venous drainage and absence of the coronary sinus septum.
Findings:
- The patient exhibited a persistent left superior vena cava draining into the left atrium.
- Four pulmonary veins were identified, with anomalous drainage into the left atrium.
- Absence of the coronary sinus septum was a key finding, confirming the diagnosis of UCSS.
Implications:
- Accurate diagnosis of UCSS is crucial for appropriate management.
- Surgical correction of UCSS can effectively alleviate symptoms and improve patient outcomes.
- This case highlights the importance of advanced imaging techniques in diagnosing rare congenital heart defects.
