The case presented is incredibly rare. To our knowledge, it is the first description of PLSVC draining to the LA without an ASD or right SVC. There are multiple reports of similar cases, known as Raghib Syndrome, where a persistent left superior vena cava (PLSVC) drains into the left atrium (LA). In these cases, however, there is an associated atrial septal defect and absent coronary sinus [1]. One case of a PLSVC draining to the LA without an ASD has been described, but this patient also had a right SVC which is not present in our case [3].
Hemodynamic effects due to PLSVC vary greatly based on coexistence of other congenital anomalies. Atypical venous return through the PLSVC can increase the risk of cardiac arrhythmias, conduction abnormalities, dyspnea, and fatigue. As such, differences in LA drainage may have implications on placement of central lines and pacemakers [4]. Procedures to correct PLSVC typically involve ligation if there is an adequate sized collateral vessel or anastomosis to the coronary sinus and closure of any septal defects. Surgical options differ significantly based on the presence or absence of an ASD.
While PLSVC can be clinically silent and discovered incidentally, variations in associated cardiac abnormalities can increase risk of developing heart failure, embolic cerebrovascular events, and hypoxemia. As such, it is important to classify these subgroups of PLSVC to help guide clinical and surgical management of these patients. We propose an updated classification system for PLSVC that includes the previously described presentation of Raghib’s syndrome but also encompasses other related defects such as the case at hand (Table 1).
Table 1: Proposed classification system for PLSVC.
Classification
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Clinical Presentation
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Citation
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PLSVC Type 1
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PLSVC draining to the LA with an associated ASD
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[1],[2]
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PLSVC Type 2
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PLSVC draining to the LA with no associated ASD and an absent right SVC
|
Novel finding
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PLSVC Type 3
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PLSVC draining to the LA with no associated ASD and a present right SVC
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[3]
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The case presented has not been previously described, and by relating previous literature to the management decisions made for this patient, we hope to provide a reference for other physicians confronted with similar abnormalities. This classification system is intended to streamline diagnoses and be used as a tool for clinicians to standardize care for this subset of congenital cardiac anomalies.