Key result
Safe anaesthetic management of a patient with Eisenmenger syndrome and β-thalassemia major undergoing splenectomy was successfully achieved using fentanyl, low-dose ketamine, and halothane.
Case Report (n=1)
No
Careful anesthetic management using fentanyl, low-dose ketamine, and halothane allowed successful splenectomy in a child with the rare combination of Eisenmenger syndrome and β-thalassemia major.
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May inform rare Eisenmenger anaesthetic choices; leaves open generalizability beyond this single case.
We describe for the first time, the perioperative care of a patient with a rare combination of Eisenmenger syndrome with β-thalassemia major presenting for splenectomy. Patients with Eisenmenger syndrome have polycythemia because of chronic hypoxia but our patient was anaemic and had thrombocytopenia because of thalassemia major. The management of such a case can be challenging for any anaesthesiologist because of severe V/Q mismatch (high shunt fraction and restrictive lung disease because of hypersplenism), decreased oxygen carrying capacity (anaemia) and increased risk of haemorrhage (thrombocytopenia), along with the potential increase in intracardiac shunt during anaesthesia.
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Gupta et al. (2011) conducted a case report in Eisenmenger syndrome and β-thalassemia major (n=1). Anaesthetic management (fentanyl, ketamine, halothane) was evaluated on Successful anaesthetic management and recovery. Safe anaesthetic management of a patient with Eisenmenger syndrome and β-thalassemia major undergoing splenectomy was successfully achieved using fentanyl, low-dose ketamine, and halothane.
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