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.