A 44-year female, presented with chief complaints of blurring of vision (bitemporal site) and supraorbital pain for one month. She was diagnosed with pituitary macroadenoma and posted for transnasal trans-sphenoidal removal of the tumour. Previously, this patient underwent an extracardiac fontan procedure for Double Inlet Left Ventricle at the age of 19 years. Since then the patient was on the tablet acenocoumarol 2 mg regularly and with no signs and symptoms of cyanotic heart disease at present. Dyspnea (NYHA class II) was present and her metabolic equivalent of the task score (METS) was 4 with moderate perioperative cardiac risk.

General physical examination was unremarkable. Cadiovascular system examination showed a surgical scar. On auscultation, grade 2 murmur in the pulmonary area was present. Electrocardiogram (ECG) showed right ventricular hypertrophy with right axis deviation and non-specific ST-T changes. Echocardiography (ECHO) showed post-op extracardiac Fontan status for single ventricle physiology-Double inlet left ventricle with- severe pulmonary stenosis (SV-DILV-PS) with normal left ventricular function at present but raised pulmonary artery pressure. Moderate degree of thoracic kyphoscoliosis (Cobb’s angle 28 degree) was present. Chest x-ray showed cardiomegaly, sternal wiring, thoracolumbar kyphoscoliosis with mediastinal shift to the right (Figure 1). Preoperative pulmonary function tests revealed moderate restrictive pattern. Complete blood count, liver, kidney function test, urine routine, blood sugar, serum cortisol and thyroid function tests were normal. Prothrombin Time (PT)/International normalised ratio (INR) was 2.57. The patient was advised to repeat INR daily and switch to low molecular weight heparin from warfarin when INR<= 1.5. Magnetic Resonace imaging (MRI) scan has been done for pituitary macroadenoma localisation (Figure 2A,B).

Patient was adviced nill per oral 8 hours prior to surgery. Incentive spirometry encouaraged. Nebulisation with ipratropium, budesonide and milrinone at 1mg/ml (to reduce pulmonary artery pressure) was done. The morning dose of low molecular heparin was omitted and INR repeated on the day of surgery. The patient was counselled about postoperative nasal packing and mouth breathing.

Patient was brought to the operation theatre. Antibiotic prophylaxis was given to prevent infective endocarditis and systemic infection. Standard ASA monitors were attached. Right radial artery cannulated for intra-arterial blood pressure monitoring. In addition Bispectral Index (BIS), urine, temperature and CVP monitoring were used. Premedication was done with injection glycopyrrolate 0.2 mg, midazolam 1 mg, palonosetron 0.075 mg. Patient was induced with injection. fentanyl 150 mcg, etomidate 25 mg, cisatracurium 12 mg and intubated with endotracheal tube size 7.5 mm. The oropharynx was packed with saline-soaked gauze. After intubation, lumbar drain was inserted in the lateral position. Anaesthesia was maintained with oxygen and air (50:50) mixture along with desflurane to maintain BIS between 40 and 60. Infusion of propofol (25-75mcg/kg/min), cisatracurium (2 mcg/kg/min), fentanyl(0.5-1mcg/kg/min) and hydrocortisone (50mg eight hourly) was maintained throughout the surgery. CVP was maintained between 12-16cm of water by intraoperative fluid therapy and urine output was also adequate. Injection paracetamol (1 gram) and continuous fentanyl infusion used for analgesia. Arterial blood gas done towards end of surgery and were within normal limits. At the end of the surgery, the patient has reversed with glycopyrrolate 0.4 mg and neostigmine 2.5 mg and extubated smoothly ( esmolol bolus 90 sec before extubation) when fully awake. Post operativelythe patient was shifted to the neuro ICU. Repeat ECHO and cardiac evaluation was done. Early ambulation and resumption of anticoagulation therapy was advised. Post operative course remained uneventful and patient was discharged successfully on the post-op day seven.