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Diagnosis of absent right superior vena cava with intraoperative transesophageal echocardiography in a child with Holt-Oram syndrome: Anesthetic and perfusion implications


1 Department of Anesthesiology and Critical Care, All India Institute of Medical Sciences (AIIMS) Bhubaneswar, Bhubaneswar, Odisha, India
2 Department of Cardiac Surgery, All India Institute of Medical Sciences (AIIMS) Bhubaneswar, Bhubaneswar, Odisha, India

Correspondence Address:
Satyajeet Misra
Department of Anesthesiology and Critical Care, AIIMS Bhubaneswar, Bhubaneswar - 751 019, Odisha
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/aca.ACA_198_20

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Year : 2021  |  Volume : 24  |  Issue : 3  |  Page : 358-361

 

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Holt-Oram syndrome is a rare autosomal disorder with cardiac, vascular, and upper limb anomalies. Previous reports have described anesthetic and perioperative challenges including difficulty in arterial and venous cannulations, airway management and rhythm, and temperature abnormalities. There are no previous reports of absent right superior vena cava (SVC) in children with Holt-Oram syndrome. We present images of a case where the diagnosis of absent right SVC with persistent left SVC was made with intraoperative transesophageal echocardiography and discuss the anesthetic and perfusion implications of such findings.






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1 Department of Anesthesiology and Critical Care, All India Institute of Medical Sciences (AIIMS) Bhubaneswar, Bhubaneswar, Odisha, India
2 Department of Cardiac Surgery, All India Institute of Medical Sciences (AIIMS) Bhubaneswar, Bhubaneswar, Odisha, India

Correspondence Address:
Satyajeet Misra
Department of Anesthesiology and Critical Care, AIIMS Bhubaneswar, Bhubaneswar - 751 019, Odisha
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/aca.ACA_198_20

Rights and Permissions

Holt-Oram syndrome is a rare autosomal disorder with cardiac, vascular, and upper limb anomalies. Previous reports have described anesthetic and perioperative challenges including difficulty in arterial and venous cannulations, airway management and rhythm, and temperature abnormalities. There are no previous reports of absent right superior vena cava (SVC) in children with Holt-Oram syndrome. We present images of a case where the diagnosis of absent right SVC with persistent left SVC was made with intraoperative transesophageal echocardiography and discuss the anesthetic and perfusion implications of such findings.






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