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KMID : 1235920130050020104
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Medical Journal of Catholoc University of Daegu
2013 Volume.5 No. 2 p.104 ~ p.106
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A Case of Isolated Persistent Left Superior Vena Cava with Absent Right Superior Vena Cava in Prader Willi Syndrome
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Lee Hyun-Suk
Park Jin-Gyeong
Jung Ji-Eun
Kim Woo-Taek
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Abstract
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Persistent left superior vena cava (PLSVC) is the most common thoracic venous anomaly. However, the absence of
the right superior vena cava and PLSVC together is a very rare condition. Here, we report the first case of a patient
with Prader-Willi syndrome who had isolated persistent left superior vena cava but did not have the right superior
vena cava, and we also provide references to related literature. The patient was born at 36 weeks’ of gestation with a
birth weight of 2,280 g and was admitted to our neonatal intensive care unit owing to hypotonia and respiratory
difficulty. The results of a chromosomal study using fluorescent in situ hybridization showed 46 XX but
microdeletion at the 11p15.2 region, indicating a diagnosis of Prader-Willi syndrome. On echocardiography and 3D
computed tomography, the superior vena cava was found only on the left side and connected to right atrium through
cornonary sinus. Currently, the patient does not have any hemodynamic problems, but other complications such as
rhythm disturbance may occur. If central venus catheter insertion is needed in any case, be careful not to be right.
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KEYWORD
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Persistent isolated left superior vena cava , Prader Willi syndrome , Absent right superior vena cava
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