[Downloaded free from http://www.ijms.in on Saturday, August 15, 2020, IP: 10.232.74.

26]

Case Report

Eisenmenger Syndrome with Pregnancy – Double Trouble

Juvva Kishan Srikanth, Nitesh Gupta, Shibdas Chakrabarti, Pranav Ish
Department of Pulmonary Critical Care and Sleep Medicine, Vardhman Mahavir Medical College and Safdarjung Hospital, New Delhi, India

Abstract
Eisenmenger syndrome is an absolute contraindication for pregnancy. However, those patients with a diagnosis in late 2nd or 3rd trimester need
to be carefully evaluated with a multidisciplinary team and strictly monitored by a critical care intensivist. We present a case of Eisenmenger
syndrome diagnosed late in pregnancy, managed conservatively in intensive care unit with positive airway pressure therapy.

Keywords: Eisenmenger syndrome, intensive care, pregnancy

Introduction auscultation. Bedside ultrasonography (USG) showed bilateral

Pregnancy should ideally be avoided in a woman with
Eisenmenger syndrome because of a high maternal mortality
rate and poor prognosis of the baby. The patients diagnosed late
should be assessed and closely monitored by an experienced
multidisciplinary team, including obstetric, critical care
and neonatal intensivists. We present a similar case with a
successful outcome.
Case Report
A 23‑year‑old primigravida, married for 3 years, with 30 weeks’
gestation presented with complaints of worsened dyspnea for
1 month associated with anasarca and cyanosis. She had dyspnea
on exertion since childhood for which she had taken oral drugs
with which she used to get partial relief. There was no history
of any hospital admissions in the past.
On examination, the patient was conscious, oriented, with
tachycardia and tachypnea. Her vital signs revealed a blood
pressure of 130/80 mmHg, a pulse of 120 beats/min, regular
and good volume, a respiratory rate of 40/min, and a room
air saturation of 80%. She had distended jugular veins with
elevated jugular venous pressure, pitting pedal edema,
cyanosis, and clubbing.
Respiratory system examination revealed vesicular breath
sounds with bilateral fine end‑inspiratory crepitations. She
had a right ventricular impulse and a palpable pulmonary
closure sound (P2) on precordial palpation with a loud P2 on
Access this article online
Quick Response Code:
Website:
www.ijms.in
B‑lines. Screening echocardiogram (ECHO) was suggestive of
the right atrium being grossly dilated [Figure 1a] with severe
tricuspid regurgitation  [Figure  1b]. Deep vein thrombosis
screen was negative.
A clinical diagnosis of right heart failure with pulmonary
hypertension was made. Electrocardiogram  (ECG) showed
right axis deviation. Review ECHO from cardiology was
suggestive of a large ostium secundum type of atrial septal
defect (ASD) with Eisenmenger syndrome [Figure 1c] with
severe pulmonary artery hypertension.
A continuous positive airway pressure (CPAP) therapy of 8 cm
of water [Figure 1d] along with oxygen was given in view of
heart failure and cardiogenic pulmonary edema. Cardiology
consultation was taken, and loop diuretics were given.
Fetal ultrasound was performed which showed a single live
intrauterine fetus of cephalic presentation with estimated
gestational age of 29  weeks and 6  days. Fetal growth
monitoring was performed using serial ultrasound. The patient
Address for correspondence: Dr. Pranav Ish,
Department of Pulmonary Critical Care and Sleep Medicine, Vardhman
Mahavir Medical College and Safdarjung Hospital, New Delhi, India.
E‑mail: pranavish2512@gmail.com
Received : 25‑10‑2019 Revised : 04-12-2019
Accepted : 23-12-2019 Published Online : 12-02-2020
This is an open access journal, and articles are distributed under the terms of the
Creative Commons Attribution‑NonCommercial‑ShareAlike 4.0 License, which
allows others to remix, tweak, and build upon the work non‑commercially, as long
as appropriate credit is given and the new creations are licensed under the identical
terms.
For reprints contact: reprints@medknow.com

DOI: How to cite this article: Srikanth JK, Gupta N, Chakrabarti S, Ish P.
10.4103/INJMS.INJMS_138_19 Eisenmenger syndrome with pregnancy – Double trouble. Indian J Med
Spec 2020;11:44-6.

44 © 2020 Indian Journal of Medical Specialities | Published by Wolters Kluwer ‑ Medknow

[Downloaded free from http://www.ijms.in on Saturday, August 15, 2020, IP: 10.232.74.26]
Srikanth, et al.: Eisenmenger syndrome with pregnancy
a b
c d
Figure 1: (a) Screening two‑dimensional echocardiography done by
intensivist; subcostal view showing dilated right atrium marked with a
white dot. (b) Screening two‑dimensional echocardiography done by
intensivist; apical 4 chamber view showing severe tricuspid regurgitation
jet with Doppler flow showing the regurgitant jet. (c) Two‑dimensional
echocardiography done by cardiologist; apical 4 chamber view showing
atrial septal defect with right to left atrial jet in color Doppler. (d) The
patient on positive airway pressure therapy
was continued on conservative management in intensive care
unit with oxygen, CPAP, and diuretics for 8  days. Antenatal
corticosteroids were administered at 31  weeks for fetal lung
maturity since termination of pregnancy was planned in view
of impending cardiac failure. Normal vaginal delivery was done
at 31 weeks, a healthy baby weighing 2.1 kg and Apgar score
of 9, 9, 9 was delivered while the mother was on CPAP therapy.
The patient improved gradually and was discharged on day 7
postpartum on the advice of oxygen therapy at home.
Discussion
Eisenmenger syndrome is rare in pregnant women with an
incidence of about 3%.[1] The underlying etiology is usually
ventricular septal defect  (VSD), followed by ASD and
patent ductus arteriosus (PDA).[2] Our patient had ASD with
Eisenmenger syndrome.
Eisenmenger syndrome patients are particularly vulnerable to
hemodynamic changes and even minor decrease in systemic
Indian Journal of Medical Specialities ¦ Volume 11 ¦ Issue 1 ¦ January-March 2020
vascular resistance  (SVR) may increase the right‑to‑left
shunting and possibly induce circulatory collapse. Decreased
SVR during pregnancy increases the right‑to‑left shunting,
subsequently leading to a reduced pulmonary perfusion
and hypoxia and further deterioration of mother and baby.[3]
Moreover, straining during delivery may result in an increased
right ventricular pressure, which may cause fatal arrhythmia
and even sudden death.[4] Regardless of etiology, pregnancy in
the Eisenmenger syndrome is associated with major morbidity
and mortality. Because of the high risk of maternal mortality,
pregnancy is contraindicated in women with Eisenmenger
syndrome.[5]
The management of patients with Eisenmenger syndrome
includes oxygen therapy, diuretics, vasodilators, and
anticoagulants.[6] Oxygen is a pulmonary vasodilator, which
decreases the blood flow across the right‑to‑left shunt and
thereby improving oxygen saturation.[4] Diuretics will be
useful to relieve hepatic congestion and increased intravascular
volume. Pulmonary vasodilators, phosphodiesterase
inhibitors  (sildenafil and tadalafil), and endothelin receptor
antagonists (bosentan, etc.,) are not recommended in pregnancy
because of possible teratogenicity and adverse effects on
uterine circulation.[6]
The greatest risk lies in the periods of delivery and early
postpartum due to large hemodynamic changes. The major
causes of death could be hypovolemia, thromboembolism,
and preeclampsia.[3]
Despite risk, vaginal delivery is a preferred delivery mode.
Cesarean section is reserved for conditions such as severe
intrauterine growth retardation.[7] Maternal mortality in
Eisenmenger syndrome is reported to be 30%–50% and up
to 65% in those with cesarean section.[8] It is higher when
associated with VSD  (60%) than with ASD  (44%) or with
PDA (41.7%).[9] For decreasing future morbidity and mortality,
women with Eisenmenger syndrome ideally should avoid
pregnancy or an early pregnancy interruption should be
considered within 10th gestational week.
Conclusion
Thus, to conclude, pregnancy should be avoided in a woman
with Eisenmenger syndrome because of a high maternal
mortality rate. Those patients with continuing pregnancy
should be assessed by an experienced multidisciplinary
team, including obstetric critical care specialist, intensivist,
cardiologist, and a neonatal physician. Training of the
intensivist for advances in obstetric critical care, including
critical care USG, ECHO, CPAP, continuous monitoring, and
a controlled vaginal delivery under epidural analgesia can help
to achieve a successful pregnancy outcome.
Declaration of patient consent
The authors certify that they have obtained all appropriate
patient consent forms. In the form, the patient has given
her consent for her images and other clinical information
45
[Downloaded free from http://www.ijms.in on Saturday, August 15, 2020, IP: 10.232.74.26]

Srikanth, et al.: Eisenmenger syndrome with pregnancy

to be reported in the journal. The patient understands that
name and initials will not be published, and due efforts
will be made to conceal identity, but anonymity cannot be
guaranteed.
Financial support and sponsorship
None.
Conflicts of interest
There are no conflicts of interest.
References
1. Rathod  S, Samal  SK. Successful pregnancy outcome in a case
of Eisenmenger syndrome: A  rare case report. J  Clin Diagn Res
2014;8:OD08‑9.
2. Wang  L, Liu  YN, Zhang  J. Analysis of the pregnancy outcome of 7
pregnant women with Eisenmenger’s syndrome. Clin Med 2010;30:3‑5.
3. Mukhopadhyav  P, Bhattacharya  P, Begum  N. Successful pregnancy
outcome with eisenmenger syndrome. J  Obstet Gynaecol India
2012;62:68‑9.
4. Yuan SM. Eisenmenger syndrome in pregnancy. Braz J Cardiovasc Surg
2016;31:325‑9.
5. Warnes  CA, Williams  RG, Bashore  TM, Child  JS, Connolly  HM,
Dearani JA, et al. ACC/AHA 2008 Guidelines for the Management of
Adults with Congenital Heart Disease: A report of the American College
of Cardiology/American Heart Association task force on practice
guidelines (writing committee to develop guidelines on the management
of adults with congenital heart disease). Circulation 2008;118:e714‑833.
6. Fang G, Tian YK, Mei W. Anaesthesia management of caesarean section
in two patients with Eisenmenger’s syndrome. Anesthesiol Res Pract
2011;2011:972671.
7. Kandasamy R, Koh KF, Tham SL, Reddy S. Anaesthesia for caesarean
section in a patient with Eisenmenger’s syndrome. Singapore Med J
2000;41:356‑8.
8. Makaryus AN, Forouzesh A, Johnson M. Pregnancy in the patient with
Eisenmenger’s syndrome. Mt Sinai J Med 2006;73:1033‑6.
9. Bazmi  S, Malhotra  S, Zaman  F. A  rare case of pregnancy with
Eisenmenger syndrome. Int J Obstet Gynaecol Res 2015;2:151‑4.

46 Indian Journal of Medical Specialities ¦ Volume 11 ¦ Issue 1 ¦ January-March 2020