Case Report:
A 2- years- old, 10kg, female child presented with bluish discolouration on crying since one and a half months of age. Transthoracic echocardiography (TTE) revealed a large OS-ASD with bidirectional shunt, supravalvular and infundibular PS with peak gradient of 120mmHg, mild right RV dysfunction (TAPSE- 13 mm). The patient underwent a balloon dilatation for infundibular and supravalvular PS at the age of 5 months, which provided some relief from the symptoms. However, when she again started having bluish discolouration of lips and fingertips on crying, she was referred to CTVS unit for intra-cardiac repair. Following confirmation of first and fifth day negative RTPCR reports for Corona Sars Virus 19, and obtaining the Informed consent from the parents, she was taken up for intra-cardiac surgical repair under cardiopulmonary bypass (CPB).
In the OR, her base line SpO2 was 92% on air. The usual opioids-based anesthesia technique was used similar to any high-risk patients with complex congenital heart disease. In addition to standard ASA monitoring, TEE was also performed using paediatric probe (Size- 11x8mm, Model- GE VIVID S60 N) ,which confirmed TTE findings, and severe tricuspid regurgitation (TR) with jet reaching to left atrium (LA) through large ASD suggestive of extremely high right atrial (RA) pressures (CVP =22 mmHg).(Fig. 1, Video 1) RV was severely dilated, almost akinetic, and interventricular septum was seen to be shifted towards the left ventricle (LV). In contrast, LV was D-shaped, exceedingly small and hyper-contractile (lateral wall was touching septum during systole) with LV ejection fraction of 80%. (Video 1,2) (Fig.1,2). However, the patient developed VF before applying the chest incision while the TEE probe was advanced from oesophagus to trans- gastric position. It was successfully managed with intravenous lignocaine (15 mg), external monophasic DC shock (50 Joules), and followed by open cardiac massage and with internal biphasic DC shock (7 Joules) after rapid sternotomy. Concomitantly, it was decided to go on CPB and so, heparin (300 units /kg) was used as an anticoagulant and after confirming of an ACT value > 480 seconds, standard CPB technique with moderate hypothermia (27.5 degrees C) and antegrade blood cardioplegic (Del Nido) myocardial protection was used for intra - cardiac repair. The cardiac repair involved as pericardial patch closure of OS -ASD, right ventricule outflow tract (RVOT) muscle bundle resection, non- transannular pericardial patch augmentation of RVOT and main pulmonary artery (MPA). In view of severe RV dysfunction, a PFO of 0.75 cm was also created.
After rewarming, the patient had a normal sinus rhythm (124 bpm) and was successfully weaned off CPB using infusion of Adrenaline (.05ug/kg/min), Milrinone(0.5ug/kg/min), NTG(1mic/kg/min) and Noradrenaline (.0 5ug/kg/min). However, during post- operative TEE evaluation patient again developed two episodes of VF, which were reverted to normal sinus rhythm without any residual ischemic changes (Normal ST, T waves) with the use of lignocaine and internal biphasic DC shock (7 J). In view of poor haemodynamic status [systolic blood pressure (SBP) - 68/27mmHg, CVP-14mmHg] and prolonged bypass time [CPB time 210 min, Aortic cross clamp time 83 minutes], Modified ultrafiltration [MUF] was performed and total 800 cc fluid was removed. However, once again she developed VF during MUF which was again managed successfully similar to the previous episodes. As the authors were not able to pin- point any other relevant cause for repeated VF [acidosis, electrolyte imbalance, ECG changes etc.], in the desperate scenario, the TEE probe was removed. Following this there occurred no repeated episode of VF. Following MUF there was a significant improvement in hemodynamics (BP -108/53mmHg, CVP-7mmHg, Hb- 16.6gm%) which were maintained with moderate doses of inodilators. Patient was weaned off CPB, heparin was neutralized with Protamine (1:1.3), and then the aorta was decannulated. Following adequate hemostasis, the chest was closed, and the patient was shifted to intensive care unit (ICU) for elective ventilation. During ICU stay, the patient required regular peritoneal dialysis for 3-4 days. Gradually, patient was weaned off inotropes and tracheal extubation was done on fifth post-operative day. Rest of the course was uneventful, and patient was shifted into the ward on 10th post -operative day.