Bin Liu

Juan Gu

Sheng Lv

Yunxia Cai

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Anesthetic management for cesarean section in a patient with uncorrected double-outlet right ventricle

Gu et al. SpringerPlus  (2016) 5:415 
DOI 10.1186/s40064-016-2075-y
CASE STUDY
Anesthetic management for cesarean 
section in a patient with uncorrected 
double-outlet right ventricle
Juan Gu1, Yunxia Cai1, Bin Liu2 and Sheng Lv1*
Abstract 
Background: We describe the anesthetic management for cesarean section in a pregnant woman with uncorrected 
double-outlet right ventricle. The anesthetic method, treatment of complications and lessons are discussed.
Case presentation: A 28-year-old woman visited our emergency room for progressive dyspnea and recurrent hem-
optysis at 30 weeks’ gestation. Her New York Heart Association functional class was III–IV. Echocardiography indicated 
that she had congenital heart disease of double-outlet right ventricle. She hadn’t received any treatment. The obste-
trician decided to terminate the pregnancy by cesarean section. We chose epidural anesthesia and pumped phenyle-
phrine at the same time to minimize hemodynamic fluctuation. Just in the process of changing position to supine 
position with uterus displaced to the left, the patient coughed badly and complained about dyspnea. At the same 
time, the oxygen saturation decreased quickly. The symptoms were ameliorated soon by treating as heart failure. But 
the symptoms reappeared after oxytocin administration. At the end of surgery, the baby was sent to the Neonatal 
Intensive Care Unit for premature birth. The mother recovered successfully and discharged 7 days later.
Discussion and Evaluation: Double-outlet right ventricle is a seldom disease and pregnancy with uncor-
rected double-outlet right ventricle is rare. In this case, the patient belonged to the type of ventricular septal defect 
characterized by subaortic ventricular septal defect without pulmonary stenosis. Most of the aorta arises from the 
right ventricle, the volume of venous blood was injected from the right ventricle into the aorta, which decided the 
oxygen saturations. Compared to general anesthesia, epidural anesthesia reduces venous return and alleviates the 
cardiac burden. So, we pumped phenylephrine along with epidural anesthesia in case of critical cyanosis following 
significant blood pressure decrease. In the process of anesthesia, dyspnea, cough and cyanosis attacked the patient 
for two times. The most probable reason was heart failure which induced by the sudden increasing of returned blood. 
As a result of severe cough, pulmonary artery pressure increased rapidly. Then a great amount of venous blood was 
injected into the aorta and cyanosis became more serious as well as oxygen saturation declined quickly. 
Conclusion: Epidural anesthesia with continuous phenylephrine infusion is a preferable choice for parturient women 
with uncorrected double-outlet right ventricle for cesarean section. It is not the optimal choice for this type of 
patients to lie on the left. The dose of oxytocin should be reduced to avoid potential cardiovascular complications.
Keywords: Double-outlet right ventricle (DORV), Anesthesia, Cesarean section
© 2016 Gu et al. This article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://
creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided 
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if changes were made.
Background
The incidence of pregnancy with cardiac disease ranges 
from 0.4 to 4.1 %, and congenital heart disease takes up 
a large proportion (McFaul et  al. 1988). Double-outlet 
right ventricle (DORV) is a rare congenital heart dis-
ease. The incidence is approximately 0.5–0.8 per 10,000 
births (Pradat et al. 2003). DORV is a congenital cardiac 
malformation in which both pulmonary artery and aorta 
predominantly arise from the right ventricle, and ventric-
ular septal defect (VSD) always coexists (Lev et al. 1972). 
Open Access
*Correspondence:  lvshengmz@163.com 
1 Department of Anesthesiology, West China Second University Hospital, 
Sichuan University, 20#, Section 3 Renmin Nan Road, Chengdu 610041, 
Sichuan, China
Full list of author information is available at the end of the article
Page 2 of 4Gu et al. SpringerPlus  (2016) 5:415 
Signs of heart failure often appear when the patient 
is young, so this disease is often diagnosed and treated 
early (Lacour-Gayet 2008). Own to the improvement in 
surgery intervention, women who suffer from congeni-
tal heart anomalies are more likely to live to the age of 
child-bearing (Gianopoulos 1989). On the other hand, 
the heart is usually too fragile to endure the physiological 
changes during pregnancy, which will put them at risk. 
The patient in this report was a pregnant woman with 
uncorrected DORV who was scheduled for cesarean sec-
tion. Because of complex pathologic changes, it is chal-
lenging for anesthesiologists to manage appropriately.
Case presentation
A 28-year-old pregnant woman (height 155  cm; weight 
55  kg) visited our emergency room complaining about 
progressive dyspnea and recurrent hemoptysis at 
30  weeks’ gestation. Two years ago, she was diagnosed 
to have congenital heart disease because of heart fail-
ure which occurred at her fist pregnancy. As a result, 
she had to have an abortion at 20  weeks’ gestation. But 
she refused to receive any treatment. She said that she 
was asymptomatic except for mild cyanosis before get-
ting pregnant. This was her second pregnancy. She said 
she was uncomfortable on the left lateral position during 
late pregnancy this time. Physical examination indicated 
cyanosis, orthopnea and acropachy. Temperature was 
36.6 centigrade, blood pressure was 115/60 mmHg, heart 
rate was 99 per minute, and pulse oxygen saturation 
(SpO2) was 75 % when she inhaled air. Electrocardiogram 
showed incomplete right bundle branch block. Chest 
radiograph revealed bilateral patch shadows, cardiac 
enlargement and bulging of pulmonary artery segment. 
Echocardiography of the mother showed: The diameter 
of the right atria and right ventricular were 55  cm and 
30  cm separately; the diameter of the left atria and left 
ventricular were normal; the aorta and pulmonary artery 
were parallel without enlargement; the aorta ridded on 
ventricular septum, 80 % of the aorta was from right ven-
tricular; there was a 26  mm ventricular septum defect 
under aorta; there was a left to right shunt between left 
and right ventricles was detected, the max velocity was 
1.6 m/s; there was absence of fibrous joint between ante-
rior mitral valve leaflet and posterior wall of aorta; the 
ejection fraction of the left ventricle was 58 %. The diag-
nosis of the echocardiography was DORV (subaortic ven-
tricular septal defect),mild reflux of pulmonary valve and 
contraction function of left ventricle was normal (Fig. 1). 
Echocardiography of the embryo indicated cor triatria-
tum of right atrium. The results of laboratory tests were 
normal except for platelet count which was 79 × 109/L. 
The hemoglobin concentration was 112  g/L. Her New 
York Heart Association (NYHA) functional class was 
III–IV. The patient was treated with oxygen, furosemide 
and dexamethasone when she arrived at our hospital. 
The dexamethasone was used to promote maturity of the 
feta’s lung. One day’s later, the symptoms of the patient 
were improved. The obstetrician decided to terminate 
pregnancy by cesarean section for deterioration of the 
heart function.
When she arrived at the operational room, oxygen 8 L/
min was provided with a mask. The operating table was 
turned to reverse trendelenburg position. She was moni-
tored with pulse oximetry, 5-lead ECG and invasive blood 
pressure Measures (SPACELAMBS, UltraviewSl2700). 
One 16-G access was placed in the peripheral venous. 
We should had inserted the central venous catheter. But 
unfortunately, we failed after trying twice. Because the 
patient couldn’t lie down and the ultrasound machine 
was unavailable. Ringer’s lactate was administered slowly 
according to the heart rate and blood pressure. In order 
Fig. 1 Echocardiogram of the pregnant woman. Cardiac function: 
EF was 58 %, FS was 30 %. Dimensional and M echocardiograph: 
enlargement of right atria and ventricular (RA 55 cm, RV 30 cm); the 
aorta and pulmonary artery were parallel without enlargement; the 
aorta ridded on ventricular septum (80 % was from right ventricular); 
the defect of ventricular septum was 26 mm which was under aorta; 
there was absence of fibrous joint between anterior mitral valve 
leaflet and the posterior wall of aorta. Color Doppler ultrasound: there 
was a left to right shunt between ventricularis was detected (Vmax 
was 1.6 m/s)
Page 3 of 4Gu et al. SpringerPlus  (2016) 5:415 
to balance pulmonary circulation and systemic circula-
tion and in case of heart failure. We decided to perform 
epidural anesthesia and pump phenylephrine at the same 
time. She was kept on the left lateral position with hips 
and knees flexed to assist puncturing. One epidural cath-
eter was inserted into L3–L4 interspace with pointed 
toward caudal direction, another epidural catheter was 
inserted into L1–L2 interspace with pointed toward 
cephalic direction. Then the patient was placed on supine 
position with uterus pushed to the left. Just in the pro-
cess of changing position, the patient coughed badly as 
well as complained about chest congestion and dyspnea. 
At the same time, the SpO2 decreased to 50 %. Rale was 
auscultated on bilateral chest, the blood pressure was 
102/65 mmHg. The symptom improved soon after 10 mg 
furosemide was given intravenously and with the head of 
bed elevated. 2  % lidocaine 3  mL was injected into epi-
dural for testing. Then sensory block level of S5–T6 was 
achieved by titrating 15 mL chloroprocaine through the 
two catheters. Along with epidural anesthesia, phenyle-
phrine was pumped at a rate of 0.1–0.3 μg kg−1 min−1 to 
keep hemodynamics stable. During cesarean section, the 
mother was infused intravenously 5 IU dose of oxytocin 
after the baby was delivered. 3  min later, the symptoms 
as they occurred in position changing before reappeared 
unexpectedly. Then 10  mg furosemide, 3  mg morphine 
and 2  mg cediland were administered, then the patient 
felt better gradually. Apgar scores of the baby were 7 at 
1 min and 10 at 5 min. In the surgery, the patient received 
200 mL Ringer’s lactate, blood loss was about 200 mL and 
output of urine was 700 mL. The mother was transferred 
to intensive care unit, the baby was sent to the Neonatal 
Intensive Care Unit for premature birth postoperatively. 
7 days later, the patient recovered and was discharged.
Discussion
DORV has been divided into different types by the Soci-
ety of Thoracic Surgeons Database. In this case, the 
patient belonged to the VSD (ventricular septal defect) 
characterized by subaortic VSD without pulmonary ste-
nosis. Because the VSD is near to the aorta, the most of 
the oxygenated blood is ejected into the aorta. The oxy-
gen saturation rests upon the amount of deoxygenated 
blood ejected from the right ventricle into the aorta. As a 
patient with a large VSD, the pulmonary over circulation 
will induce pulmonary hypertension and heart failure. 
DORV of this type is often diagnosed in infancy for cya-
nosis and symptoms of congestive heart failure. DORV is 
a seldom disease and pregnancy with uncorrected DORV 
is rare. There are only few reports describing the anes-
thetic management of pregnant woman who suffered 
from DORV (Ito et  al. 2002; Andau et  al. 2004). Differ-
ent from the patients in the previous reports, this patient 
hadn’t received surgery repair and had more serious 
symptoms. In addition, poor NYHA functional class and 
cyanosis were risk factors for maternal or neonatal com-
plications (Siu et al. 2001; Pradat et al. 2003). So, proper 
anesthetic management was important.
The patient in this report didn’t have obvious symptom 
until at 30  weeks’ pregnancy. In addition, there was no 
pulmonary hypertension was indicated in the echocar-
diography. One possible reason was that the pulmonary 
circulation and systemic circulation of this patient were 
balanced very well. But following volume increasing in 
the third trimester of pregnancy, signs of heart failure 
appeared as it reported in early studies (Szekely et  al. 
1973). To this patient, progressive dyspnea and recurrent 
hemoptysis were the symptoms of cardiac decompensa-
tion. Compared to general anesthesia, epidural anesthesia 
reduces venous return and alleviates the cardiac burden. 
If general anesthesia was chosen, the drugs used for gen-
eral anesthesia might suppress the heart function which 
was already fragile. The mother was in the condition of 
hypoxia which would result in hypoxia of the baby. The 
drugs used in general anesthesia induction might worsen 
the hypoxia of the newborn. Hemoptysis occurred in 
the patient repeatedly, tracheal intubation might induce 
bucking and hemoptysis. Epidural anesthesia is easier 
to keep hemodynamic stability than spinal anesthesia. 
In addition, we were good at do epidural anesthesia for 
pregnant woman with heart disease. So we decided to 
perform epidural anesthesia. Catheters were placed both 
in L3–L4 and L1–L2 for ensuring adequate analgesia. 
Most of the aorta arises from the right ventricle, the vol-
ume of venous blood was injected from the right ventri-
cle into the aorta, which decided the oxygen saturations 
(Spaeth 2014). So, we pumped phenylephrine along with 
epidural anesthesia in case of critical cyanosis following 
significant blood pressure decrease.
In the process of anesthesia, dyspnea, cough and cya-
nosis attacked the patient for two times. The symptoms 
were relieved successfully and quickly by diuretic and 
cardiotonic. The most probable reason was heart fail-
ure which induced by the sudden increasing of returned 
blood. The first episode occurred when the position 
was changing. Before the process of epidural punctur-
ing, the patient was placed in position of side-lying with 
hips and knees flexing, the veins of low limbs were com-
pressed and the venous return were reduced. When 
the legs unbended and, most importantly, with uterus 
displaced to the left, the returned blood increased sud-
denly and heart failure occurred. Cough and dyspnea 
were the symptoms of pulmonary edema. As a result of 
severe cough, pulmonary artery pressure increased rap-
idly. Then a great amount of venous blood was injected 
into the aorta and cyanosis became more serious as well 
Page 4 of 4Gu et al. SpringerPlus  (2016) 5:415 
as oxygen saturation declined quickly. This hypothesis 
is consist with the patient’s complaint about discomfort 
when she lay on the left lateral in pregnant period. Car-
diac output was 5 % higher in the position of tilting ≥15° 
than in supine position (Lee et al. 2012). The symptoms 
recurred after oxytocin was given. Because with a uter-
ine contraction, additional 500  mL blood will return to 
heart (Maitra et  al. 2010). For women with cardiac dis-
ease, just 0.1  IU doses of oxytocin could induce adverse 
effects (Langesaeter et al. 2010). The patient in this report 
should have been given lower dose oxytocin that was 
both effective and leaded to fewer complications (Car-
valho et al. 2004).
Conclusions
In conclusion, a pregnant woman with uncorrected 
DORV for cesarean section was successfully managed by 
epidural anesthesia and continuous phenylephrine infu-
sion. The lessons we learned from this case were that it 
is not the optimal choice for this type of patients to lie on 
the left. The dose of oxytocin should be reduced to avoid 
potential cardiovascular complications. In addition, we 
should have placed the patient in sitting position when 
performing intrathecal anesthesia.
Abbreviations
DORV: double-outlet right ventricle; VSD: ventricular septal defect; SpO2: 
oxygen saturation; NYHA: New York Heart Association.
Authors’ contributions
JG, SL and YC managed the patient. JG wrote his case report with other 
coauthors. SL and BL reviewed the case. All authors read and approved the 
final manuscript.
Author details
1 Department of Anesthesiology, West China Second University Hospital, 
Sichuan University, 20#, Section 3 Renmin Nan Road, Chengdu 610041, 
Sichuan, China. 2 Department of Anesthesiology, West China Hospital, Sichuan 
University, 37#, Guo Xue Xiang, Chengdu 610041, Sichuan, China. 
Competing interests
The authors declare that they have no competing interests.
Consent statement 
Written informed consent was obtained from the patient for publication of 
this case report and any accompanying images.
Received: 11 February 2016   Accepted: 30 March 2016
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Anesthetic management for cesarean section in a patient with uncorrected double-outlet right ventricle

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