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.
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
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
: No 22q11.2 deletion
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.
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.