Abstract Eisenmenger’s syndrome in pregnancy is
associated with a high maternal and fetal morbidity and
mortality. When it occurs with severe preeclampsia, the
morbidity and mortality are higher. We report the case of
a 30 weeks’ pregnant woman with Eisenmenger’s syn-
drome and severe preeclampsia. Cesarean section was
performed due to severe preeclampsia and an unfavor-
able cervix under general anesthesia. The intraoperative
period was uneventful and a healthy 1300 g male infant
was delivered, but the patient died on the second
postoperative day due to a pulmonary embolism. This
case confirms the frequently fatal maternal outcome of
Eisenmenger’s syndrome in pregnancy. Early termina-
tion of pregnancy is the treatment of choice.
Keywords Eisenmenger’s syndrome · Pregnancy ·
Severe preeclampsia · Cesarean section
Introduction
Eisenmenger’s syndrome was first described in 1897 [1].
In 1958, Wood defined Eisenmenger’s syndrome as an
elevated pulmonary artery pressure and pulmonary vas-
cular resistance, resulting in a reversed or bidirectional
shunt at any site between the two circulation systems [2].
It can occur with several congenital heart defects, i.e.
ventricular septal defect (VSD), atrial septal defect
(ASD) and patent ductus arteriosus (PDA) [3]. VSD is
the most frequent underlying shunt defect. The incidence
of Eisenmenger’s syndrome is approximately 3% of
patients with congenital heart defects [4]. Maternal mor-
tality in this situation is still high and maternal mortality
in association with VSD is higher (60%) than in associa-
tion with ASD and PDA [5].
Severe preeclampsia is one type of hypertension in
pregnancy and associated with high maternal and perina-
tal morbidity and mortality [6, 7]. There is no survival
case of Eisenmenger’s syndrome complicated by severe
preeclampsia reported in the literature [8].
Pulmonary embolism is one of the common complica-
tions in Eisenmenger’s syndrome with the incidence of
13.2% [9]. When this complication occurs in pregnant
women with Eisenmenger’s syndrome, the mortality is
high [5]. Herein, we report the fatal outcome of a preg-
nant woman with Eisenmenger’s syndrome associated
with VSD who underwent cesarean section because of
severe preeclampsia and an unfavorable cervix. She died
from pulmonary embolism in the postpartum period.
Case report
A 21-year-old Thai woman, primigravida, 30 weeks’ gestation,
came to the emergency room of King Chulalongkorn Memorial
Hospital, Bangkok, Thailand, because of progressive dyspnea. She
had been diagnosed as VSD with Eisenmenger’s syndrome based
on cardiac catheterization 8 years previously, and was awaiting
heart lung transplantation. She had been lost to follow up until this
admission. She had not received antenatal care until 3 weeks before
this admission, when she developed progressive dyspnea, and was
managed at a private hospital. There was no sign of pulmonary em-
bolism, such as dyspnea, tachypnea, cough, hemoptysis, pleuritic
chest pain with splinting, and friction rub, at that time [10]. The
doctor referred her to the tertiary-care hospital but she refused and
went back home. Her past medical history was otherwise normal.
On physical assessment the pulse rate was 96/min, respiratory
rate 24/min and blood pressure 170/110 mmHg. There was
marked cyanosis and clubbing of fingers. A right ventricular
heave was noted, and there was a fixed split second heart sound
with an increased pulmonic component. There was grade II/VI
systolic murmur at the left parasternal border. Breath sounds were
normal. The size of the uterus was consistent with 30 weeks’ ges-
tation, with a fetal heart rate of 152/min. Both legs showed pitting
edema and hyperreflexia of the deep tendon reflexes.
V. Phupong (
✉
) · C. Charakorn · K. Prammanee
Department of Obstetrics and Gynecology, Faculty of Medicine,
Chulalongkorn University, Rama IV Road, Pathumwan,
Bangkok 10330, Thailand
e-mail: vorapong.p@chula.ac.th
Tel.: +66-2-2564241, Fax:+66-2-2549292
P. Ultchaswadi · S. Prasertsri · S. Charuluxananan
Department of Anesthesiology, Faculty of Medicine,
Chulalongkorn University, Rama IV Road, Pathumwan,
Bangkok 10330, Thailand
Arch Gynecol Obstet (2003) 267:163–166 © Springer-Verlag 2003
CASE REPORT
Vorapong Phupong · Pornthip Ultchaswadi
Chuenkamon Charakorn · Kongkwan Prammanee
Suthisinee Prasertsri · Somrat Charuluxananan
Fatal maternal outcome of a parturient with Eisenmenger’s syndrome
and severe preeclampsia
Received: 5 October 2001 / Accepted: 3 December 2001