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Table of Contents
CASE REPORT
Year : 2020  |  Volume : 11  |  Issue : 1  |  Page : 44-46

Eisenmenger syndrome with pregnancy – Double trouble


Department of Pulmonary Critical Care and Sleep Medicine, Vardhman Mahavir Medical College and Safdarjung Hospital, New Delhi, India

Date of Submission25-Oct-2019
Date of Decision04-Dec-2019
Date of Acceptance23-Dec-2019
Date of Web Publication12-Feb-2020

Correspondence Address:
Dr. Pranav Ish
Department of Pulmonary Critical Care and Sleep Medicine, Vardhman Mahavir Medical College and Safdarjung Hospital, New Delhi
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/INJMS.INJMS_138_19

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  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


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

How to cite this URL:
Srikanth JK, Gupta N, Chakrabarti S, Ish P. Eisenmenger syndrome with pregnancy – Double trouble. Indian J Med Spec [serial online] 2020 [cited 2020 Sep 24];11:44-6. Available from: http://www.ijms.in/text.asp?2020/11/1/44/278090




  Introduction Top


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 Top


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 auscultation. Bedside ultrasonography (USG) showed bilateral B-lines. Screening echocardiogram (ECHO) was suggestive of the right atrium being grossly dilated [Figure 1]a with severe tricuspid regurgitation [Figure 1]b. Deep vein thrombosis screen was negative.
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

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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 1]c with severe pulmonary artery hypertension.

A continuous positive airway pressure (CPAP) therapy of 8 cm of water [Figure 1]d 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 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 Top


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 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 Top


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 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 Top

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.  Back to cited text no. 1
    
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.  Back to cited text no. 2
    
3.
Mukhopadhyav P, Bhattacharya P, Begum N. Successful pregnancy outcome with eisenmenger syndrome. J Obstet Gynaecol India 2012;62:68-9.  Back to cited text no. 3
    
4.
Yuan SM. Eisenmenger syndrome in pregnancy. Braz J Cardiovasc Surg 2016;31:325-9.  Back to cited text no. 4
    
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.  Back to cited text no. 5
    
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.  Back to cited text no. 6
    
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.  Back to cited text no. 7
    
8.
Makaryus AN, Forouzesh A, Johnson M. Pregnancy in the patient with Eisenmenger's syndrome. Mt Sinai J Med 2006;73:1033-6.  Back to cited text no. 8
    
9.
Bazmi S, Malhotra S, Zaman F. A rare case of pregnancy with Eisenmenger syndrome. Int J Obstet Gynaecol Res 2015;2:151-4.  Back to cited text no. 9
    


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