Total intravenous anesthesia for cesarean section in a patient with corrected

transposition of the great arteries: etomidate and remifentanil

Eisa Bilehjani MD, Assistant Professor in Aesthesia, Fellowship in Cardiovascular

Anesthesia, Madani Heart Hospital, Tabriz – Iran. (Corresponding Author)

Solmaz Fakhari MD, Resident in Anesthesiology. Madani Heart Hospital, Tabriz - Iran

Amir Abbas Kianfar MD, Assistant Professor in Aesthesia, Fellowship in Cardiovascular

Anesthesia. Madani Heart Hospital, Tabriz - Iran. Tabriz - Iran

Mehrnoosh Toofan MD, Assistant Professor in Cardiovascular disease, Fellowship in

Echocadiography. Madani Heart Hospital, Tabriz – Iran

Corresponding author: Dr. Eisa Bilehjani, Department of Cardiovascular Anesthesia,

Madani Heart Hospital, Tabriz University of Medical Sciences; Tabriz - Iran.

Tel: 0098 411 3360894, Fax:0098 411 3344021, E-mail: isa_bilehjani@yahoo.com

From: Department of Cardiovascular Anesthesia, Cardiovascular Research Center, Madani

Heart Hospital, Tabriz University of Medical Sciences: Tabriz – Iran

Short Title: TIVA for cesarean section in corrected TGA

Abstract:

We report anesthetic management of a 34 years old, term pregnant woman, for elective cesarean

section (C/S), who had congenitally corrected transposition of the great arteries (CCTGA), associated

with severe pulmonary stenosis and severe tricuspid regurgitation. She had a previous cardiac surgery

and C/S, 21 and 11 years ago, respectively. We used etomidate, remifentanil and cisatracurium as

TIVA* with invasive hemodynamic monitoring. Hemodynamic was very stable and event free, with

good newborn and mother outcome. Patient was awake extubated in the operating room. The

postoperative course was uneventful, and the patient was discharged from the hospital on the second

postoperative day.

Key words: TIVA, cesarean section, corrected transposition of the great arteries, etomidate, remifentanil

*; total intravenous anesthesia

Introduction:

There is an increase in the numbers of pregnant individual with serious congenital heart disease. In

such pregnancies, anesthetic management for labor or cesarean section (C/S) is very stressful.

Congenitally corrected transposition of the great arteries (CCTGA) is a rare congenital heart disease,

comprising less than 0.5-1% of all forms of congenital heart disease (1,2,3). Double discordance

(atrioventricular and ventriculoarterial discordance), results in a physiologically corrected circulation,

but the left ventricle supports the pulmonary circulation and the right ventricle supports the systemic

circulation. Isolated CCTGA without other cardiac malformations are rare, only about 1-15% of all

cases. Many kinds of other cardiac malformations are associated wit CCTGA (1,3,4). The natural

history and clinical presentation of CCTGA patients depends on the presence and severity of associated

cardiac lesions. However normal lifespan was reported (5), the ability of the right ventricle to sustain

the systemic circulation over time usually deteriorates, finally failure is happen (3,4). Pregnancy in

patients with CCTGA, carry high risk of morbidity and mortality, thus these patients usually were

encouraged to avoid becoming pregnant. Anesthetic management of the patient with CCTGA, depends

on the associated anatomical conditions and functional status of right ventricle. Anesthesiologist should

obtain information about a pregnant woman with CCTGA from cardiologists. In this report we present

a 34 years old, term pregnant woman with CCTGA associated with severe PS, severe tricuspid

regurgitation (TR) and reduced RV systolic function. She had a history of previous cardiac surgery

(atrioventricular septa defect repair) and C/S 21 and 11 years ago, respectively. She was referred to our

heart hospital for elective cesarean section. We managed her with total intravenous anesthesia (TIVA)

using etomidate and remifentanil. The TIVA method that we used produced a very safe and excellent

condition for C/S.

Case presentation:

Patient was a 34 years old, pregnant (38 weeks) woman, G2P1, with congenitally corrected

transposition of the great arteries (CCTGA), associated with severe PS, severe TR and systemic

ventricular dysfunction. She was scheduled and referred for elective cesarean section in our heart

hospital.She had a previous cardiac surgery (atrioventricular septal defect closure) and cesarean section

(C/S), 21 and 11 years ago respectively in history. Drug history was ferrous sulfate, folic acid, aspirin,

heparin and multivitamin. She was asyanotic, with moderate orthopenea and palpitation. Demographic

data and vital signs were as: weight=82kg, height=164cm, BP= 150/90mmHg, HR=80, RR=16. Fetus
was 38-39weak, in good condition. ECG was normal sinus rhythm (figure 1). Transthoracic

echocardiographic data were as: congenitally corrected TGA, severe valvar pulmonary stenosis,

reduced systemic ventricular function, moderate to severe left side AV valve regurgitation and

occasional right to left small shunt (figure 2).

There was not any abnormality in laboratory tests. Arterial and central venous catheters were inserted

with local anesthesia (BP=160/104, HR=74, CVP=9mmHg, Spo2= 92%). Anesthesia was induced with

etomidate 14mg, remifentanil 120μg and cisatracurium 14mg, applying Sellick's maneuver. After

tracheal intubation, anesthesia maintained with remifentanil infusion (2-3 μg/kg/min), with target of

maintaining SBP about 120-130mmHg. Operation performed with good newborn and patient outcome,

without any cardiac events during surgery. The newborn was a male, 3050g with 9/10 APGAR score in

first and fifth minutes, respectively. Post delivery midazolam 2mg, oxytocine 30mg and antibiotic were

administrated. Also tubal legation performed. At the end of operation incision was infiltrated with 12ml

of bupivacaine 0.25%. Patient was extubated when she was fully awake, in the operating room after

neuromuscular block reversing. Totally 1200ml crystalloid fluid was administrated during operation.

The postoperative course was uneventful, and the delayed post operative pain controlled with a single

dose morphine sulfate 10mg intramuscular. After a 14 hour CCU-stay, the patient was transferred to

ward and then discharged from the hospital on the second postoperative day (figure 3).

Discussion:

Statistics have shown a dramatic increase in survival of individuals with congenital heart disease.

Today it can be expected that most of children treated for congenital heart disease will survive to

adulthood and lead a full adult life. Thus, more women with congenital heart disease are interested in

pregnancy. Although most women with congenital heart disease can successfully carry a pregnancy,

some are at substantial risk, as anesthesia implication for labor or C/S is risk full. CCTGA is a rare

congenital heart disease affecting only 0.5-1% of patients with congenital heart disease (1,2,3,5). Few

patients with CCTGA live past 50 years, with the median age of death at 40 years (1,6-8). Ten-year

survival rates range from 64-83% from the time of diagnosis and depend on the associated anomalies

(1,3). The cause of death is usually heart failure, resulting from the inadequacy of right ventricle to

support the systemic circulation (3,4). Heart failure is often compounded by tricuspid valve

regurgitation. Isolated CCTGA without other cardiac malformations are rare, only about 1-15% of all

cases (1,7). Common associated intracardiac abnormalities are ventricular septal defect (VSD) (60-
74%), left ventricular outflow tract obstruction or pulmonary stenosis (PS) (30-74%), tricuspid valve

abnormalities (38-90%), atrial septal defect (ASD) (36%) and heart block (49%) (1,3,4). Extracardiac

anomalies are rare (1). Also CCTGA is usually diagnosed in late childhood or early adulthood, it can be

diagnosed prenatally (1). Patients traditionally present with heart failure or heart block (9), but other

common presenting characteristics include: single loud second heart sound, heart murmur, cyanosis,

bradycardia and tachyarrhythmia. The gold standard tool for diagnosis is transesophageal

echocardiography (TEE) (1). This is superior to transthoracic echocardiography (1). Chest X-ray, ECG

can help to diagnosis. Dextrocardia should also raise suspicion of CCTGA (1). The ECG may be

confused with an inferior myocardial infarction. Complete heart block is a common finding. Surgical

correction of associated abnormalities was performed safely but there is doubt about Surgical

correction of own CCTGA (double switch procedure) (3).

Our patient had a previous operation for atrioventricular septal defect closure, when she was 13years

old. During this long period she was symptom free and had a successful and event free pregnancy/C/S

(with spinal anesthesia), when she was 23years old.

Echocardiogrphically, our patient had a severe valvar PS, moderate to severe TR, reduced systemic

ventricular function. This may means that the patient needed all cautions must be considered in PS, left

ventricular dysfunction and mitral valve regurgitation as in other non-CCTGA patients. Pregnant

patients with CCTGA have right ventricles that are already stressed due to the physiological changes of

pregnancy and any additional stress may precipitate to heart failure.

Different anesthetic methods were used for labor or cesarean section in these patients. Epidural

analgesia (low dose of local anesthetic augmented with opioid, Opioid-only) and continuous spinal

analgesia also were used in labor successfully (10,11). Epidural blockade can provides a more gradual

onset of block, thus allowing more precise control over the patient’s blood pressure while providing

adequate pain relief in C/S. Continuous epidural anesthesia was usually considered the best anesthetic

method for complicated patients and it was also used safely for C/S in CCTGA (12). General anesthesia

also used safely. Takasusuki and et al used general anesthesia (using anesthesia induction with

thiamylal, vecuronium and fentanyl, and maintenance with oxygen-air-isoflurane), for elective cesarean

section in CCTGA with mitral insufficiency (13). Tsuda and et al used midazolam and fentanyl for

induction and sevoflurane and fentnyli for maintainece of anesthesia, in emergency C/S, but dopamine

and dobutamine were needed to support blood pressure and cardiac function (14). Kawano and et al
used combined general anesthesia (induction with thiamylal and maintenance with propofol infusion

that supplemented by isoflurane) and continuous epidural anesthesia for elective C/S (15). Patients with

decreased cardiac function dependent on an optimum preload in order to maintain an adequate cardiac

output and they have a decreased tolerance for any changes in volume status. Thus we tried to maintain

the patient in a euvolemic status. Using direct arterial, central venous pressure monitoring and arterial

blood gas/electrolites with other basic monitoring (continuous pulse oximetry, ECG and ETco2) could

help us to provide an optimal fluid therapy. The patient’s arterial blood pressure never fell below

120mmHg or exceed over 140mmHg.

The largest increase of cardiac output occurs during and immediately after delivery, as much as 60%

above pre-labor values. Invasive hemodynamic monitoring in operative room and ICU helped us to

control that definitely. However pulmonary artery catheter was used for monitoring cardiac

performance (13), but result interpretation in the setting of CCTGA is difficult, that we decided don’t

place that.

Using etomidate/remifentanil for induction and titrating remifentanil infusion, we could control BP

excellently stable. Etomidate is an old anesthetic agent. Hemodynamic stability of etomidate is unique

among anesthetics. After early widespread use of etomidate, its use was limited significantly, because

of reports of temporary adrenal steroid synthesis inhibition. But the unique properties of etomidate did

not change over time, (hemodynamic stability, cerebral protection and a rapid recovery after either a

single dose or a continuous infusion). Because of these beneficial properties and lock of any recent

reports of clinical adrenocortical suppression (after a single dose or brief infusion), its clinical use was

came popular for anesthesia induction again. Recent studies showed that etomidate is still very safe for

major surgeries (16). Remifentanil is a titratable ultra short half-life opioid that has minimal side effects

on mother or newborn. It was used safely, for induction and maintenance of anesthesia in C/S. Peak

effect of remifentanil appears at 45-60 second and its half-life is about 5-10 minutes (17). Respect to

these properties of remifentanil, an arterial line can be very helpful to more precise real-time control of

arterial blood pressure. There are controversies about effectiveness of incision infiltration with

bupivacaine (18,19). In our case it seems it was very effective.

In summary as advances in medicine allow for the improvement in diagnosis and treatment of the

congenital heart diseases, the number of patients needs labor or C/S with these complicated diseases

will be increasing. It is imperative that anesthesiologists should understand the pathophysiological

aspects of these anomalies and how anesthesia may affect them. The maternal mortality associated with

C/S in the presence of congenital heart disease may be high. Anesthetic management of the patients

with CCTGA, basically depends on the associated intracardiac anomalies. Thus anesthesiologist should

obtain definitive information about a pregnant woman with CCTGA, from cardiologists.

We suggest that TIVA with etomidate and remifentanil together and using invasive hemodynamic

monitoring can provide a very safe anesthesia method for C/S in complicated patients.

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