In India, approximately 2 lakh children per year are born with congenital heart disease. Among the cyanotic heart disease, most common being tetralogy of fallot. The survival 

Cyanotic heart disease patients into the fourth decade are extremely rare. There is a tendency of cyanotic heart disease women reaching the third or fourth decade to become pregnant. TOF leads to increase in maternal morbidity and even mortality [1].

We report a case of 20-year-old primigravida belonging to low socioeconomic status with uncorrected TOF managed with General Anesthesia.

Case Report

A 20 year old primigravida at 33 weeks 2 days presented in emergency with chief complaint of difficulty in breathing (NHYA III) since 3-4 days. She dated back the event of shortness of breath and cyanotic changes of lips and fingers since the fourth month of pregnancy which used to get relieved after rest. She also gave a history of similar episodes in childhood which used to get relieved after rest. No medical aid was taken previously. There was no history of orthopnea, paroxysmal nocturnal dyspnoea, chest pain, syncope, cough or wheezing. Her vitals were: PR: 88/MIN, SPO2: 86% RA (92% WITH O2), BP: 112/70 mm Hg, RR: 16/MIN, BMI: 20.6 kg/m2, MMS: II, TMD: 6.5, Hb 16g%, HCT: 47%, ABG> pH: 7.35, pO2: 46, pCO2: 40. ECHO: EF: 50% VSD: 15mm, CCHD with TOF, confluent PAs, severe infundibular PS, and normal RV/LV function.

Patient was shifted to OT, drip on a nondependent arm with 18G and crystalloid fluid started. Intra Arterial cannula in the left radial artery and invasive BP monitoring started. Preoxygenation with 100% oxygen done. General anesthesia was given with titrated dose of ketamine, etomidate and muscle relaxant and intubated with 7.5 cuffed ETT and maintained with isoflurane. For pain relief inj fentanyl, paracetamol was given. Inj Syntocin 10 U given slowly after delivery of baby. Reversal with inj neostigmine and glycopyrrolate done. The entire perioperative period was uneventful with postoperative vitals wese: HR: 100/min BP: 120/70 mmHg, SpO2: 95%.

Unrepaired TOF consists of a VSD, overriding of aorta, right ventricular outflow tract obstruction, right ventricular hypertrophy. Unrepaired TOF is associated with significant risk with poor fetomaternal outcome [2]. It increases chances of miscarriages, fetal or maternal death,  so in unrepaired defect pregnancy is not In unrepaired TOF, one should avoid decrease in SVR, which worsen the severity of right to left shunt. Maintain adequate intravascular volume and venous return. In the presence of right ventricular compromise, high filling pressures are required to enhance right ventricular performance and increased pulmonary blood flow. Avoid aortocaval compression and prevent pain, hypoxemia, hypercarbia and acidosis which may increase PVR [3]. During general anesthesia, avoid myocardial depression. Although use of either general anesthesia or combined epidural spinal anesthesia has been reported but considering the severity of the lesion we opted for general anesthesia with constant monitoring and maintaining adequate volume status and avoiding positive pressure ventilation [4]. Phenylephrine is a vasopressor of choice as it is a pure alpha 1 agonist. It Increases SVR without tachycardia. It also increases pulmonary blood flow and improves oxygenation. Oxytocin should be given slowly to avoid hypotension and tachycardia.

Although combined spinal epidural or general anesthesia for TOF has been reported. Considering the severity of the case, General anesthesia was preferred and during cesarean, perioperative period was uneventful. 

1. Saxena, A. "Congenital heart disease in India: A status report." Indian Pediatr, vol. 55, no. 12, 2018, pp. 1075–1082.

2. Badhan, A. et al. "Anesthetic management of a pregnant woman with uncorrected tetralogy of Fallot for cesarean section." Int J Res Med Sci, vol. 7, 2019, pp. 2835–2836.

3. Warnes, C.A. et al. "ACC/AHA 2008 guidelines for the management of adults with congenital heart diseases: A report of the American College of Cardiology/American Heart Association." Circulation, vol. 118, 2008, pp. e714–e833.

4. Bremerich, D.H. et al. "Comparison of continuous background infusion plus demand dose and demand-only parturient-controlled epidural analgesia using ropivacaine with sufentanyl for labor and delivery." Int J Obstet Anesth, vol. 14, 2005, pp. 114–120.