cyanotic congenital heart disease is still a common occurrence and many patients with uncorrected cardiac lesions present for various non - cardiac surgery .
this case report is aimed at presenting a case of labor epidural analgesia and subsequently epidural anaesthesia in a patient with uncorrected pentalogy of fallot .
a 22-year - old primigravida was admitted to the labour ward with history of 8-months amenorrhoea .
she was known to have cyanotic congenital heart disease since childhood , and was not on any regular medications or follow - up .
she also gave a history of palpitations , giddiness and blurring of vision , which improved over the course of pregnancy .
echocardiogram [ figure 1 ] at the 4 month revealed pentology of fallot ( pof ) and hence she was advised therapeutic termination of pregnancy , for which she had refused .
echocardiography image of the patient with pentology of fallot showing atrial and ventricular septal defect on pre - anesthetic check up , grade ii clubbing and peripheral cyanosis were present .
electrocardiogram ( ecg ) showed normal sinus rhythm , right axis deviation and right bundle branch block .
echocardiography revealed malpositioned great vessels , double - outlet right ventricle with severe pulmonary stenosis , large subaortic ventricular septal defect with significant overriding of aorta , moderate - sized atrial septal defect with left 
arterial blood gas showed ph 7.38 , pao2 48 mmhg , paco2 34 mmhg and sao2 of 84% .
as the patient was getting frequent painful contractions that were associated with tachycardia , the obstetricians requested for labour analgesia . with a strict aseptic technique , in sitting posture at l3/4 interspace , 25 g fentanyl was injected intrathecally using a 25 g bevel cut spinal needle .
this was followed by placement of an 18 g epidural catheter at the l2/3 interspace .
the patient had excellent pain relief immediately , with a visual analogue score of 0 .
continuous electrocardiography ( ecg ) , pulse oximetry and non - invasive blood pressure monitoring were performed during the labour .
labour progressed normally with an outlet forceps - assisted vaginal delivery of a healthy male baby within 2 h. apgar at birth was 8/10 and 10/10 after 5 min .
five hours following delivery , the patient started complaining of severe pain at the episiotomy site .
examination revealed a huge vulval haematoma that required immediate drainage . in the operating theatre , under full cardiac monitoring ,
epidural analgesia was provided with 10 ml of 0.125% bupivcaine and 50 g of fentanyl .
the patient was haemodynamically stable , with the oxygen saturation between 80 and 85% throughout the surgical procedure .
the rest of the post - partum period was uneventful and the patient was discharged on the 7 day of admission to the hospital .
tetralogy of fallot ( tof ) is the most common cyanotic congenital heart defect seen in around 56% of congenital heart malformations .
it is characterized by anterior and superior infundibular septal displacement , ventricular septal defect , aortic override , infundibular obstruction and right ventricular hypertrophy .
when the tof is associated with atrial septal defect , it is commonly referred to as pof .
there is an association between tof and chromosomal defects seen in 15% of the population , with the possibility of genetic transmission to the offsprings .
there is fall in systemic vascular resistance ( svr ) , increase in blood volume and increase in cardiac output secondary to increased heart rate and stroke volume , and a physiological left ventricular hypertrophy .
the pathophysiology of tof depends on the level of right ventricular outflow obstruction . when the obstruction is mild , the patients present with increased pulmonary blood flow ( acyanotic fallot or rose tetrology ) .
acyanotic fallot is more commonly seen in adults , while children present with significant right ventricular outflow obstruction , right  left shunt and cyanosis .
maternal cyanotic cardiac lesions lead to an impaired uteroplacental perfusion and diminished foetal oxygenation , leading to a higher incidence of miscarriages , premature births and low birth weight babies .
anaesthetic management for patients with cyanotic heart disease requires a thorough understanding of the pathophysiology , events and medications , which alters the magnitude of the right - to - left intracardiac shunt . decreased svr , increased pulmonary vascular resistance and increased myocardial contractility increase the magnitude of the right - to - left shunt .
the choice of anaeshetic technique is crucial in the management of patients with cyanotic heart disease .
sympathetic blockade seen in regional techniques may increase shunt by decreasing svr , leading to decreased ventricular filling and cardiac output .
pharmacological agents that decrease svr increase the magnitude of the right - to - left shunt and accentuate arterial hypoxemia .
also , intermittent positive pressure ventilation and positive end expiratory pressure cause increases in pulmonary vascular resistance and decrease pulmonary blood flow . providing adequate analgesia , maintenance of uterine 
placental perfusion , preventing aortocaval compression and minimization of sympathetic block by maintaining intravascular volume are the goals of anaesthetic management
. in cyanotic congenital heart diseases ( tof , pof ) , administration of labour analgesia during early labour is advisable to limit increases in pulmonary vascular resistance and consequent right - to - left shunting .
small incremental doses of local anaesthetic drugs with opioids are preferred over large bolus doses to avoid abrupt reduction in svr , which may cause reversal of shunt flow and hypoxemia .
intravenous fluids should be carefully monitored to prevent deficits or excesses , leading to shunt worsening and patient 's decompensation .
adequate pain relief with minor haemodynamic changes should be aimed when regional anaesthesia is considered .
shrivastava et al . reported administration of epidural block to a patient with uncorrected tof for caesarean section .
mendes et al . described a case where labour analgesia was administered in uncorrected tof that was diagnosed during gestation .
as an institutional practice , we employ combined spinal epidural labour analgesia , which is an effective and safe technique .
it allows the use of subarachnoid opioids , such as fentanyl or sufentanil , leading to faster analgesic effect , lower sympathetic and motor block .
placing an epidural catheter allows administration of low concentration of local anaesthetic with minimal haemodynamic changes .
the pathophysiology and the haemodynamic derangements of pof are more or less similar to tof , eventually depending on the magnitude of shunt .
the anaesthetic management of patients with pof is not commonly reported in the literature owing to the rarity of occurrence .
recent advances in cardiac interventions and surgery have enabled more number of women with congenital heart disease to thrive well into their childbearing years .
although pregnant women with congenital heart disease are rarely reported around the world , a thorough understanding of the pathophysiology and anaesthetic management of these patients is essential .
the choice of anaesthesia in these patients requires thorough understanding of problems , careful titration of drugs and maintaining haemodynamic stability .
our patient with pof was managed with labour analgesia and subsequently epidural anaesthesia for drainage of vulval haematoma with successful outcome .
 tetraology of fallot is the most common congenital heart disease causing intracardiac right - left shunts . 
 it is characterized by presence of ventricular septal defect , aortic overriding , pulmonary artery outflow obstruction and right ventricular hypertrophy . 
 when these features are associated with atrial septal defect , it is often referred to as pentology of fallot ( pof ) . if the lesions remain uncorrected , they can cause significant morbidity and mortality to the patient . pregnancy and labour in such a patient present with significant haemodynamic changes , which can be challenging to the anaesthesiologist . 
 our patient with pof was managed with labour analgesia and subsequently epidural anaesthesia for drainage of vulval haematoma with successful outcome . 
INTRODUCTION
CASE REPORT
DISCUSSION
CONCLUSION