Multimodality imaging including echocardiography and cardiac CT was essential for precise anatomical delineation of unroofed coronary sinus with CS orifice atresia and persistent left superior vena cava coexisting with severe rheumatic mitral stenosis, guiding safe surgical intervention.
Key Findings
Background
Unroofed coronary sinus with coronary sinus orifice atresia and persistent left superior vena cava (PLSVC) can coexist with acquired valvular disease such as severe rheumatic mitral stenosis.
This is described as an 'exceptionally rare congenital anomaly' in the context of coexisting acquired valvular disease.
The case represents a rare combination of congenital cardiac anomalies alongside rheumatic mitral stenosis.
The coexistence of these conditions presents unique diagnostic and surgical challenges.
Results
Multimodality imaging combining echocardiography and cardiac CT was necessary for precise anatomical delineation of this complex cardiac anatomy.
Echocardiography and cardiac CT were both utilized to characterize the anatomical abnormalities.
The imaging was described as 'essential for precise anatomical delineation' to guide safe surgical intervention.
Comprehensive preoperative imaging was highlighted as critical for managing combined congenital and acquired cardiac pathologies.
Discussion
Recognition of the complex anatomy involving PLSVC and coronary sinus atresia is critical to avoid inadvertent PLSVC ligation without ensuring adequate coronary sinus drainage.
Inadvertent PLSVC ligation without ensuring adequate CS drainage is identified as a potential 'catastrophic complication.'
The absence of a normal coronary sinus orifice (atresia) alters the venous drainage pathway and surgical risk profile.
Preoperative identification of this anatomy directly informed the surgical approach to avoid life-threatening complications.
Conclusions
Comprehensive preoperative imaging is essential in managing patients with combined congenital and acquired cardiac pathologies.
The case highlights that standard evaluation may be insufficient when rare congenital anomalies coexist with acquired conditions.
Multimodality imaging was required to safely plan surgical intervention in this complex case.
The authors emphasize the importance of this approach as a generalizable lesson for similar complex cardiac cases.