Arch Iran Med
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Case Reports
Unusual Left Superior Vena Cava, Connected to the Left Atrium via the Left Superior Pulmonary Vein.
Persistent left superior vena cava (LSVC) that drains into the left atrium (LA) via the left superior pulmonary vein (LSPV) is a rare systemic venous drainage anomaly. It can cause cyanosis and unexplained recurrent strokes. Undiagnosed, it can seriously disrupt the conduct of the cardiopulmonary bypass (CPB), causing sudden air lock and/or flooding of the operative field with venous blood. ⋯ The LSVC was temporally clamped during the remainder of the surgical procedure, then ligated at both ends. The patient underwent mitral valve repair, closure of the infundibular septal defect, aortic valve replacement and tricuspid annuloplasty. He was discharged 10 days later.
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After ruling out the most common causes of severe hemolytic anemia by routine diagnostic tests, certain patients remain without a diagnosis. The aim of this study was to elucidate the genetic cause of the disease in these patients using next generation sequencing (NGS). ⋯ These findings show that PKD is among the rare blood disorders that could remain undiagnosed or even ruled out in Iranian population without performing NGS. This could be due to pitfalls in clinical, hematological or biochemical approaches in diagnosing PKD. Furthermore, genotyping PKD patients in Iran could reveal novel mutations in the PKLR gene.