Journal of Clinical and Diagnostic Research. 2023 Jan, Vol-17(1): UD01-UD03 1 1 DOI: 10.7860/JCDR/2023/58065.17254 Anaesthesia Section Unravelling the Convoluted Story of Perioperative Care in Three-year-old Child with Tetralogy of Fallot Undergoing Repair Surgery Case Report CASE REPORT A 3-year-old male child having congenital heart disease of tetralogy of fallot (diagnosed at birth) was posted for one step corrective surgery and preanesthetic check-up was requested. He had chief complaints of bluish discolouration of lips and nail beds, breathlessness, and intermittent loss of consciousness after crying or excessive playing. The child became limp off and on after any rigorous activity. These episodes lasted for 3-5 minutes and occurred 5-6 times in a month. Increased frequency of these episodes was observed since last 3-4 months which subsided after maintaining a knee-chest position or on reassurance by the mother. The child was born vaginally, at term gestation and was admitted in Neonatal Intensive Care Unit (NICU) for difficulty in breathing and had a birth weight of 1.5 kg. There was delay in achieving developmental milestones with a reported ability to sit at age of 1.5 years. The child is not able to stand and/or walk at the age of three years and can speak only in monosyllables. He was receiving tablet propranolol 5 mg twice daily since birth. The weight and height of the child was 6.1 kg and 77 cm respectively, with a body surface area of 0.36 m 2 . He was afebrile, with a pulse of 102 bpm, respiratory rate of 26/min. There was no pallor, icterus, lymphadenopathy, or oedema on the feet. Jugular venous pressure was normal. Cyanosis was present on lips, tongue, fingers and toes, there was grade 3 clubbing and oxygen saturation was 40% on room air. On inspection of the precordium, there was visible apical impulse, and thrill was palpable at second intercostal space along the sternal border. Parasternal heave was present. S1 and S2 heart sound were heard normally. Pansystolic murmur was present on lateral border of sternum in 2 nd intercostal space. There was no chest wall deformity and accessory muscle for respiration were not used. The ejection fraction was 65% on 2D echocardiography examination. There was a large, bidirectional, Ventricular Septal Defect (VSD) of 12 mm size. The pulmonary arteries were grossly hypoplastic with a severe infundibular pulmonary stenosis with pressure gradient of 68 mmHg. The pulmonary valve annulus was 6 mm. The superior vena cava was grossly dilated and Tetralogy of Fallot (TOF) was seen. Boot shaped heart was seen on chest X-ray [Table/Fig-1]. As a part of preoperative preparation, the baby was kept nil by mouth for 4 hours, intravenous (i.v.) cannula was secured, and i.v. fluids Dextrose Normal Saline (DNS) (2 mL/kg) was started to maintain adequate hydration. Adequate blood and blood products were cross matched and kept ready. Informed consent was obtained from patient’s father after counselling about risk of anaesthesia, postoperative complications of surgery and chance of prolonged Intensive Care Unit (ICU) stay. Precaution was taken to avoid hypothermia. Initially, Blalock-Taussig (BT) shunt was planned, but because of unfavourable anatomy, the surgical team had opted to perform Waterston’s shunt. During preinduction of anaesthesia, the child went into a cyanotic spell, blood pressure dropped to 60 mmHg and SpO 2 fell to 10%. To increase the systemic vascular resistance, the anaesthetic team tried to calm the child and make him comfortable in knee-chest position and 100% O 2 was given. A fluid bolus was given, and acidosis was corrected after injecting sodium bicarbonate in bolus dose as per body weight. Injection phenylephrine was given as per requirement to treat hypotension. Patient responded to the given treatment and became vitally stable. Anaesthesia was induced with inj. midazolam (0.05 mg/kg), inj. fentanyl (1 mcg/kg), inj. ketamine (2 mg/kg), inj. vecuronium (0.1 mg/kg) as per calculated dose per kg body weight in intravenous route and the child was intubated with No.5 uncuffed endotracheal tube and maintained on oxygen and air. Injection vecuronium was used for topping up of anaesthesia. Central Venous Pressure (CVP), Invasive Blood Pressure, temperature was monitored as per standard American Heart Association (AHA) monitoring. Normothermia and normocapnia was maintained. Intravascular fluid volume with attention to blood sugar monitoring was done. AISHWARYA NAYAK 1 , SANJOT NINAVE 2 , DHAWAL WADASKAR 3 , PRASAD PANBUDE 4 , AMOL BELE 5 Keywords: Anaesthesia, Congenital cyanotic heart disease, Cyanotic spell, Intracardiac repair, Waterston shunt ABSTRACT Tetralogy Of Fallot (TOF), one of the most prevalent cyanotic congenital heart diseases in children. Single step corrective surgery, early on in life, provides a fair expectation of favourable outcome in these individuals. However, complex, and skilled anaesthetic management is required by experienced team of healthcare providers. Preoperative surgical preparation, intraoperative key anaesthesia principles and postoperative care intensive care unit are all perioperative considerations in these patients. This article reports challenges faced in the perioperative anaesthesic management of a 3-year-old child having uncorrected TOF, who underwent Waterston shunt and later Intracardiac Repair (ICR). He presented with postoperative complication like cyanotic spell, gastrointenstinal bleeding, sepsis; but later recovered successfully. [Table/Fig-1]: Chest X-ray showing boot shaped heart.