Title Page

Case report:

Managing Anesthesia Care on Parturient with Uncorrected Pentalogy of Fallot Presenting

for Elective Cesarean Section

Suwarman, MD, PhD

Department of Anesthesiology and Intensive Care, Faculty of Medicine Padjadjaran University –
Hasan Sadikin General Hospital Bandung, Indonesia.

Sigit Sutanto, MD
Department of Anesthesiology and Intensive Care, Bhayangkara Anton Soedjarwo Hospital,
Pontianak, West Kalimantan, Indonesia

Correspondence :

Suwarman, MD, PhD

Address
Jalan Sukanagara No. 10, Bandung, 40291, Indonesia
Phone : +628122171673
Email : dr.suwarman@yahoo.co.id

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 Case report:

Managing Anesthesia Care on Parturient with Uncorrected Pentalogy of Fallot Presenting

for Elective Cesarean Section

Abstract
Cyanotic congenital heart diseases have unique and challenging clinical condition for anesthesiologist.

Tetralogy of Fallot (TOF) is one of the most common congenital heart diseases among adult population.

Pentalogy of Fallot (POF) is a variant of TOF with additional atrial septal defect (ASD). A

comprehensive preoperative assessment and thorough understanding of anatomical and physiological

changes is very important in ensuring safe anesthesia care.

We report a 29 years old primigravida with uncorrected POF pregnant at 36-37 weeks gestation

planned to have elective cesarean section. On physical examination we found perioral and peripheral

cyanosis with SpO2 84%-86% with pansistolic murmurs at auscultation. Transoesophageal

echocardiography revealed TOF and secundum ASD with left ventricular ejection fraction 57%. Patient

underwent cesarean section under general anesthesia. Midazolam, ketamine and rocuronium was chosen

as induction agents, and rapid sequence induction did not performed as prevention to hemodynamic

instability. During surgery, non-invasive monitoring was applied and the hemodynamic remains

relatively stable. The surgery was uneventful without complications.

In managing anesthesia care for parturient with POF, detailed preoperative assessment must be

made in order to understand the physiological changes and factors that may affecting anesthesia. A

multidisciplinary approach is required to ensure the good outcome for both patient and baby.

Keywords: cesarean section, congenital heart disease, general anesthesia, pentalogy of Fallot, tetralogy
of Fallot.

Introduction
Congenital heart disease occurs in 1% of the population. Cyanotic type congenital heart

disease usually has a worse clinical condition and become a challenge for anesthetists. Tetralogy

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of Fallot (TOF) is a cyanotic congenital heart disease that is most common in the adult

population. Classically TOF includes the following conditions: (1) large non-restrictive

ventricular septal defect (VSD), accompanied by (2) aortic overriding, (3) pulmonary

infundibular stenosis, and (4) consequently right ventricular hypertrophy. All of these conditions

result from embryonic anterocephalic deviation from the outlet septum. The spectrum of this

disease can expand with pulmonary valve stenosis, pulmonary valve stenosis or pulmonary

artery stenosis and hypoplasia in more severe conditions.1 Pentalogy of Fallot (POF) is a variant

of TOF with the addition of an atrial septal defect (ASD). Without correction, only 25% of

sufferers reach adolescence, after which the mortality rate reaches 6.6% per year of increasing

age. Only 3% survive to age 40. Unlike children, adults with TOF do not experience "tet spells".

Post correction, the 32-36 year survival rate is reported to be 85% -86%, although arrhythmia

symptoms and activity intolerance may occur in 10% -20% at 20 years post correction.1,2

Women with a completely corrected TOF with good operative results without residual

defects generally tolerate pregnancy. Women with untreated TOF, especially those with marked

cyanosis, have a high incidence of miscarriage (80% if the hematocrit > 65%) and a 10%

mortality rate. A stillbirth rate of up to 14% and fetal development disorders of 36% were

reported in pregnant women with cyanotic heart disease. The decreased systemic vascular

resistance (SVR) that occurs during pregnancy and delivery can exacerbate cyanosis, while

increasing physiological volume during pregnancy can exacerbate both ventricular failure. 2,3

Several case reports reported management of anesthesia care in pregnancy with uncorrected

cyanotic congenital heart disease using general or regional anesthesia with good results both for

mother and the baby.4,5,6

Case Report

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 A 29-year-old woman, 36-37 weeks pregnant with Pentalogy of Fallot was scheduled for

elective cesarean section. The patient who was previously admitted to a cardiology clinic early in

pregnancy was known to have uncorrected POF from birth. The patient received 1x30 mg of

digoxin therapy, 1x30 mg of beraprost, and 1x25 mg of spironolactone since the beginning of

pregnancy. Patients complained of fatigue and blueness around the mouth and fingertips,

especially when doing activities, complaints are increased along with gestational age.

On preoperative examination, the patient appeared to be seriously ill with level of

consciousness compos mentis. She had a body weight of 52 kg and a height of 148 cm. The

patient prefers a side sleeping position because it minimize shortness of breath. Vital signs show

blood pressure of 110/70 mmHg, pulse rate 90 x/min, breath rate 18-22 x/min with a peripheral

oxygen saturation of 84% -86% free air, 93% using nasal canule. There were perioral and

peripheral cyanosis, clubbing fingers and leg edema. On auscultation there was a pansystolic

murmur. Laboratory results Hb 10.7 g / dl, Ht 32.7%, leukocytes 3800 / uL, platelets 229000 /

uL, with electrolytes, liver function, kidney function and clotting factors are within normal

limits. X-ray was not performed on patient because of fetal welfare considerations.

Transesophageal echocardiography showed dilatation of the right atrium, right ventricle,

Pentalogy of Fallot (ToF + ASD secundum), mild TR, mild PR, infundibular pulmonary stenosis

with a PS gradient of 103 mmHg, normal left and right ventricular systolic function, with left

ventricular ejection fraction up to 57%.

The patient was planned for cesarean section under general anesthesia. After sufficient

fasting and installation of an intravenous line, ranitidine and metoclopramide 30 minutes before

surgery were administered. Antibiotics were given as prophylaxis. Non-invasive monitors with

ECG, NIBP and SpO2 were placed on the patient, given 500 ml of Ringers lactate and

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preoxygenated. Induction was performed with 2.5 mg midazolam, 75 mg ketamine, and 50 mg

rocuronium, the patient was subjected to endotracheal intubation. Maintenance with sevoflurane

1-2 vol%, oxygen and air with 50% oxygen fraction. During surgery blood pressure ranges from

90/55 mmHg to 142/95 mmHg, with extrasystolic ventricular waves frequently occurring. After

intubation, the oxygen saturation reaches 95%. When the baby was born the oxygen saturation

had decreased to 85%, then 250 ml of Ringer's lactate and 10 mg of ephedrine were given to

increase the SVR, saturation then returned to improve. Alive baby boy with a good APGAR

score was born, then he was given fentanyl 50 mcg. Oxytocin was given slowly drip, uterine

contractions looked good. The estimated amount of bleeding is 500 ml. After the surgery was

completed, reversal muscle relaxants were given with neostigmine 0.04 mg / kg and atropine

0.01 mg / kg. Adequate spontaneous breathing was achieved after 30 minutes and the patient was

extubated fully awake. Postoperatively, hemodynamically stable with an oxygen saturation of

85-90% using oxygen simple mask. The patient was given postoperative analgesics with

intravenous morphine 10 mcg / kg / hour, the patient was admitted to the high care unit. Until the

third postoperative day, the patient was hemodynamically stable and the patient was transferred

to a normal ward. The patient was discharged on the 5th day of care.

Discussion
In normal pregnancy, there are physiological changes, including an increase in cardiac

output since the 5th week of pregnancy and reaches a peak at the 32nd week. At the 8th week of

pregnancy, there is an increase in cardiac output by as much as 50%. This increase occurs due to

an increase in stroke volume and heart rate. There was a decrease in SVR and PVR in 70% at the

beginning of pregnancy until the 8th week. This decrease is thought to be due to an increase in

the concentration of estrogen, NO, and vasodilator peptides which can lower blood pressure due

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to a vasodilating vascular state. Blood pressure will drop at its nadir point at 20 weeks of

gestation and return to normal at term. Patients with uncorrected congenital heart disease have a

high risk of cardiovascular complications during pregnancy and delivery. The hemodynamic load

in pregnancy is exacerbated by differences in anatomical structures causing cardiovascular

complications. Patients are also at high risk of experiencing miscarriage.1, 2

Managing anesthesia care in patients with POF requires an understanding of the

anatomical defects, physiological adaptations, and the selection of drugs that can affect

intracardiac shunting changes. There is no specific anesthetic technique recommended in patients

with cyanotic congenital heart disease. The considerations of anesthesia in these patients include

preventing an increase in PVR, maintaining normal SVR, cardiac contractility, preload, and

preventing reversal of shunting. It is also important to prevent hypercarbia, hypoxemia, acidosis

and pain, all of which will increase PVR and affect reversal of shunting. Chronic hypoxemia in

these patients triggers an adaptation mechanism to meet tissue oxygenation characterized by

polycythemia, increased blood viscosity, vasodilation, hyperventilation and chronic respiratory

alkalosis. This adaptive mechanism can limit cardiac and oxygen reserves in the face of stress

during labor. Dehydration and hypovolemia should be avoided to improve circulating volume

and decrease blood viscosity.2,3

General and regional anesthesia techniques with epidural or combined spinal epidural

dose titrations have been reported with good results. Single shot spinal anesthesia is

contraindicated in this group of patients because it can cause a rapid decrease in SVR which can

lead to reversed shunting. With general anesthesia, the risk of hemodynamic fluctuation during

laryngoscopy and airway manipulation should be considered.4,5,6

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 In our patients, general anesthesia was chosen because of the consideration of ensuring

adequate oxygenation with better control of ventilation and hemodynamic stability. As induction

agent, ketamine was chosen because of its effect on maintaining SVR. 7 The cardio stimulating

effect of ketamine is balanced with the sympathoinhibitoric effect of midazolam to produce good

cardiovascular stability.8 The muscle relaxant was chosen for rocuronium because it is more

cardiovascular stable and does not release histamine. 9 Patients are not subjected to rapid

sequence intubation due to avoiding hemodynamic fluctuations and consideration of sufficient

fasting as well as efforts to prevent aspirations have been made. N2O was not used during the

operation, since it can increase PVR. 10 Slow drip oxytocin is used to prevent a rapid decrease in

SVR. Opioids are given after the baby is born to minimize the risk of the drug's effects on the

baby. Postoperatively, pain is prevented by providing adequate analgesics with intravenous

morphine. Oxygen supplementation with a simple mask and sufficient fluids are given and in

order to prevent hypovolemia. Patient is admitted to the high care unit under intercooperative

care between intensive care, cardiology and obstetrics. During the postoperative period the

patient and baby were in a stable state, the patient was discharged after the 5th day of treatment.

Conclusion
Patient with cyanotic congenital heart disease like pentalogy of fallot has risk of

cardiovascular complication during pregnancy and labor. There is no specific anesthesia

technique to date which is recommended in patient with cyanotic congenital heart disease

presenting cesarean section. Managing anesthesia care in this certain patient group requires an

understanding of the anatomical defects, physiological adaptations, and the selection of drugs

that can affect intracardiac shunting changes. A multidisciplinary approach is required to ensure

the good outcome for both patient and baby.

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 Acknowledgement

 All persons who have made some contribution to the publication, but convince only one or two of

the three authorship criteria (each author must have made significant scientific contribution to the

work; each author must be familiar with the content; each author must be willing to take

responsibility for the completeness and accuracy of the content) should be mentioned in the

Acknowledgment segment.

 All sources of support such as financial and material should also be revealed in the

Acknowledgment section. The roles of each sponsor in data achievement or other responsibilities

must be informed.

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 FIGURES

Figure 1. Patient prefer lying to her side in order to minimize shortness of breath

Figure 2 Echocardiography

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