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Anesthetic management for cesarean delivery in a parturient with repaired pulmonary atresia, ventricular septal defect and major aortopulmonary collateral arteries


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A Case Report: Anesthetic Management for Cesarean Delivery in a Parturient with Repaired Pulmonary Atresia, Ventricular Septal Defect and Major AortoPulmonary Collateral Arteries

Sun-Kyung Park, MD, Seokha Yoo, MD, Jin-Tae Kim, MD, PhD, Young-Jin Lim, MD, PhD, Jae-Hyon Bahk, MD, PhD

Department of Anesthesiology and Pain Medicine, Seoul National University Hospital, Seoul, Republic of Korea


Pulmonary atresia with a ventricular septal defect and major aortopulmonary collateral arteries (PA/VSD/MAPCAs) is an extremely rare congenital malformation characterized by lack of a pulmonary valve and a pulmonary vascular bed supplied by aortopulmonary collateral arteries.

Anesthetic management for cesarean delivery in these patients poses unique challenge to anesthesiologist, due to physiologic changes during pregnancy.

We present a case of a parturient with corrected PA/VSD/MAPCAs who had undergone cesarean delivery under epidural anesthesia.

Case Report

A 33-year-old primigravida

Height 149.7 cm , weight 48 kg

35+6 weeks’ gestation

She admitted for cesarean delivery due to fetal breech presentation.


She was diagnosed with PA/VSD/MAPCAs and underwent surgical corrections during childhood.

s/p left modified Blalock-Taussig shunt & left unifocalization (1989)

s/p right ventricle (RV) to pulmonary artery (PA) conduit interposition(2001)

s/p palliative right ventricular outflow tract (RVOT) reconstruction (2002)


CATCH 22 syndrome

- FISH (2014): 22q11.2 deletion confirmed

Subclinical hypothyroidism on Levothyroxine


NYHA class I-II

Exertional dyspnea

SpO2 85% on room air

Her echocardiography revealed a large VSD with bidirectional shunt, moderate RV-PA conduit stenosis with peak velocity of 2.7 m/sec, mild to moderate pulmonary regurgitation.

Her estimated left ventricular ejection fraction was 69%.


Fetal Echocardiography (at 27 weeks’ gestation)

  : No definite intracardiac anomaly , wide PFO, PDA

Prenatal FISH

  : No 22q11.2 deletion

Multidisciplinary plan for Safe Delivery
Consultation with Primary Physician (Pediatric cardiologist)
Elective cesarean delivery at 35 weeks
Minimization of hemorrhage
Recommend to use diuretics and to avoid volume overload.
Anesthetic technique
: Epidural anesthesia with careful titration & close hemodynamic monitoring
Neonatologist’s attendance
Postoperative intensive care

Antibiotics was administered 1 hour prior to surgery as prophylaxis against infective endocarditis

Oxygen was supplied in order to maintain SpO2 more than 85%.

A radial arterial line was placed.

An epidural catheter was inserted at the L3-4 intervertebral space.

3 mL of lidocaine 2% was given as a test dose.

After 10 minutes, 2% lidocaine 140 mg and 0.75% ropivacaine 75mg was administered in gradual manner.

•A sensory block to the T6 was achieved bilaterally.
•The onset of regional blockade was not associated with any changes in BP, HR
•A female baby was delivered with Apgar scores 9 at 1 minute and 9 at 5 minutes.
•Umbilical cord pH 7.264
• Hemodynamic parameters were stable throughout the surgery.
•The mother was transferred to the intensive care unit for close monitoring after delivery.
•She remained stable and was transferred to the general ward 24 hours after delivery.
•She was discharged without any complications on the postoperative day 5.
•One month after delivery, she was examined in the cardiologic outpatient clinic, and she was doing well.


The physiologic changes associated with pregnancy can compromise parturients with PA/VSD/MAPCAs.

The stress and pain during labor and delivery can increase PVR, thereby worsening right-to-left shunt.

SVR is reduced throughout pregnancy and may lead to worsening of a right-to-left shunt.

Surgically corrected patients may have various types of residual abnormality.

Careful review of the history and consultation with the primary care physician will help understand the residual pathophysiology and plan the perioperative management.

Careful titration of epidural anesthesia with the invasive arterial monitoring can be safely used in these patients. 

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