Irshad Basheer1, Giridharan Sridharan2, Ajaykumar Anandh3, Prakash Venkatesan4, Senthil Kumar V. S5

24 years old female, deaf from childhood, primigravida, presented for elective caesarean section. She had prolonged QT interval in ECG. Further history and examination revealed that she had been hospitalised for repeated syncope. She was diagnosed to have congenital long QT syndrome after Holter monitoring. Familial history of long QT syndrome was present. She was started on Tab. Propranolol 20 mg twice daily. She was also advised by cardiologist to undergo procedure for implantable cardiac defibrillator, but the patient did not undergo the procedure due to personal reasons.
She conceived spontaneously and was on regular follow up with the obstetric and cardiology teams. She was scheduled for elective caesarean section in view of underlying condition. All routine investigations were done and found to be normal. Echocardiogram was also done and it revealed normal study with ejection fraction of 69%. Electrocardiogram - sinus rhythm with QT prolongation. She was advised nil per oral as per fasting guidelines. She was premedicated with Inj. Ranitidine 50 mg, which was given slowly in intravenous line on morning of procedure and intravenous fluids with 0.9% normal saline of 100 mL per hour was started from morning. She was also advised to take morning dose of Tab. Propranolol with sips of water.
After obtaining consent from the patient, the patient was shifted to operating room. Subarachnoid blockade was planned. Monitors such as 5 lead ECG, noninvasive blood pressure and pulse oximetry were connected. Biphasic defibrillator ECG cables were also connected and kept ready. Patency of existing large bore intravenous line was checked and another large bore intravenous line secured. Baseline vitals were recorded. Goals were to maintain sinus rhythm with heart rate ranging from 60-80 beats per minute and maintaining mean arterial pressure of 70 mmHg. Under aseptic precautions, subarachnoid blockade was performed and 10 mg of 0.5% heavy bupivacaine was given and level achieved was T4. Caesarean section was done and boy weighing 2.1 kg with 8/10 and 9/10 Apgar at 0 and 5 minutes were extracted. Post extraction of baby, 10 units of Inj. Oxytocin was given as slow infusion. Blood pressure fell to mean arterial pressure of 62 mmHg and maintained with help of Inj. Ephedrine 3 mg and intravenous fluids. Post procedure, the patient was shifted to high dependency unit for observation and the period was uneventful. Paracetamol 1 gm intravenous was given in our case for management of pain. Cardiology review was obtained and suggested to continue same line of management as she was on and added implantable cardiac defibrillator device at the earliest. Both mother and baby was fine and discharged.