Anaesthetic considerations in a patient with Uncorrected Pentology Of Fallot presenting for

IntraCardiac Repair - A Case Report

Introduction:

Pentalogy of Fallot is a rare form of cyanotic congenital heart disease, characterized by an association of
atrial septal defect (ASD) with tetralogy of Fallot (TOF)

Case History :

A 24 year female presented to the cardiology department with complaints of increased breathlessness ,
palpitations and easy fatiguability for the past 2 weeks . She is a known case of Cyanotic Congenital
Heart Disease - Pentology of Fallot diagnosed during a routine health check up 5 years ago but was not
on regular followup.

O/E :

Pt had central cyanosis , pandigital clubbing. Room air saturation was 70 percent in all 4 limbs JVP was
not elevated .Left Parasternal heave was present. Echo revealed a Restrictive Perimembranous VSD
with R to L shunt , Large OS ASD , severe valvular pulmonary stenosis and a RVOT gradient of 160 mm
hg and PA pressure of 40 mm hg . Cardiac Catherization revealed a raised RV pressure of 170/54 mm hg
and post stenotic dilatation of pulmonary arteries

Anaesthetic concerns :

The main goal is to avoid any further increase in Pulmonary vascular resistance which would worsen the
situation and precipitate cardiac failure. The most common triggers include hypoxia , hypercarbia ,
acidosis , lighter plane of anaesthesia and systemic vasodilation

Anaesthetic management :

The patient was induced with titrated doses of ketamine and fentanyl , Paralyzed with vecuronium 5
mg and maintained on O2 - air and desflurane mixture. Vasopressors - phenylephrine and noradrenaline
and inodilator Milrinone were kept ready. Fall in BP was immediately corrected with boluses of
Phenyephrine.The patient was taken on cardiopulmonary bypass and the main pulmonary artery was
opened , pulmonary valve was replaced and sutured to the main pulmonary artery and rvot , Vsd was
closed with gortex patch , and asd was closed with pericardial patch . Weaning was done using milrinone
and noradrenaline and patient was shifted to ICU for elective postop ventilation and extubated the next
day.

Conclusion :

Carefully administered anesthesia with meticulous planning, judicious use of drugs, combined with strict
monitoring, and vigilance can make a safe outcome even in difficult cases.