|Year : 2019 | Volume
| Issue : 1 | Page : 36-38
Successful Pregnancy Outcome in Eisenmenger Syndrome: A Case Report
Urvashi Chhikara, Latika Sahu, Asmita Rathore, Preeti Singh
Department of Obstetrics and Gynaecology, Maulana Azad Medical College, New Delhi, India
|Date of Web Publication||30-Apr-2019|
Dr. Urvashi Chhikara
Department of Obstetrics and Gynaecology, Maulana Azad Medical College, New Delhi–110002
Source of Support: None, Conflict of Interest: None
In patient with Eisenmenger syndrome, raised pulmonary artery pressure with decreased systemic resistance increases maternal and fetal mortality. Multidisciplinary approach is needed for the management of these patients. This case report shows successful feto-maternal outcome in a patient with Eisenmenger syndrome.
Keywords: Eisenmenger syndrome, pregnancy outcome
|How to cite this article:|
Chhikara U, Sahu L, Rathore A, Singh P. Successful Pregnancy Outcome in Eisenmenger Syndrome: A Case Report. MAMC J Med Sci 2019;5:36-8
|How to cite this URL:|
Chhikara U, Sahu L, Rathore A, Singh P. Successful Pregnancy Outcome in Eisenmenger Syndrome: A Case Report. MAMC J Med Sci [serial online] 2019 [cited 2020 Jan 24];5:36-8. Available from: http://www.mamcjms.in/text.asp?2019/5/1/36/257422
| Introduction|| |
Eisenmenger syndrome consists of pulmonary hypertension with bidirectional flow at atrioventricular or aortopulmonary level. The prognosis of pregnancy depends on the severity of pulmonary hypertension. High mortality risk is associated with pregnancy and peripartum period; even, in recent reports, mortality rate remains unacceptably high (25%–30%). Thus, these patients should be advised strongly against pregnancy. In this case report, we discuss a successful pregnancy outcome in a patient with Eisenmenger syndrome.
| Case Report|| |
A 26-year-old female was admitted to antenatal department at Lok Nayak hospital. She was diagnosed with congenital heart disease (ventricular septal defect) at 12 years of age. No corrective surgery was performed at that time. She was now admitted at 24 weeks of pregnancy with complaints of dyspnea on exertion for 2 months and cough for 2 days. No history suggestive of orthopnea, dizziness, or palpitations was present. On examination, her blood pressure was 110/80 mmHg; pulse rate was 86/min, regular and rhythmic; and chest was clear; cardiovascular examination showed systolic murmur on left sternal border with a loud P2; oxygen saturation at room air was 92%. Peripheral cyanosis and digital clubbing were present. No pedal edema was found. Electrocardiogram (ECG) showed right axis deviation. Blood tests showed polycythemia with hematocrit of 55%. Liver and kidney function tests were normal. Repeat echocardiography with contrast study showed perimembranous ventricular septal defect with pulmonary artery hypertension and bidirectional shunt suggestive of Eisenmenger syndrome [[Figure 1]] and [[Figure 2]].
|Figure 1: 2D Echo showing large perimembranous VSD of 18 mm size. Mean pulmonary artery pressure was 33 mmHg, suggestive of pulmonary artery hypertension. LV = left ventricle, RV = right ventricle, VSD = ventricular septal defect.|
Click here to view
|Figure 2: 2D Echo with Doppler revealed bidirectional flow across the ventricular septal defect (Eisenmenger syndrome).|
Click here to view
Cardiology consultation was sought. Medical approach included restricted physical activity, oxygen therapy, and calcium channel blocker (diltiazem tablet 30 mg three times a day). Pregnancy was uneventful till 28 weeks when she had worsening of her symptoms; diuretics were added and dose of diltiazem was increased. According to her blood reports, she had polycythemia with hematocrit of 55%. Risk assessment for venous thromboembolism was performed and she was started on subcutaneous enoxaparin 40 mg once daily from 28th week.
Fetal echo was performed which showed normal study. Fetal growth monitoring was performed using serial ultrasound. Fetal growth restriction was found at 28–30 weeks, for which Doppler studies were performed biweekly. Antenatal corticosteroids were administered at 30 weeks for fetal lung maturity.
Elective cesarean section was planned at 34 weeks in view of absent end-diastolic flow in umbilical artery Doppler studies. Cesarean section was performed under epidural anesthesia; she delivered a baby boy, with a birth weight of 1355 g and Apgar score of 9,9,9 with average blood loss of 800 mL. Post-surgery, the patient was transferred to the intensive care unit (ICU), where she had repeated episodes of low oxygen saturation (80–85%). She stayed in the ICU for 10 days. Vasodilator agents, tadalafil tablet 20 mg three times a day, and ambrisentan 5 mg twice a day were added on day 6; warfarin tablet was started and the stitch were removed on day 10. Her baby was admitted in the neonatal ICU for 40 days in view of low birth weight and sepsis. The baby was discharged on day 45, with the weight of 1600 g. Both the mother and the baby were healthy on discharge. She was advised to follow-up in cardiology outpatient department.
| Discussion|| |
During pregnancy, normal blood volume increases up to 50% and during labor further increases with each uterine contraction. In patients with Eisenmenger syndrome, raised pulmonary artery pressure with decreased systemic resistance increases right to left shunt, worsening the hypoxia. Further, pregnancy is a pro-thrombotiv state, which can cause pulmonary embolism and sudden cardiac death in these patients. High maternal and fetal mortality was found in these patients according to different studies. Thus, pregnancy is not advisable to these patients.
Currently available drugs include potent vasodilator agents including endothelial receptor blockers like bosentan, ambrisentan, and macitentan. All these drugs are teratogenic (category X), thus discontinuation of these drugs is mandatory before patient becomes pregnant. Oxygen therapy should be given to patients and diuretics can be added if the patient has symptoms of cardiac failure.
Preferred mode of delivery is vaginal with adequate pain relief using intravenous analgesics or low-dose epidural anesthesia. Care must be taken during conduct of anesthesia as hypotension can be life threatening in these patients. Benefits of vaginal delivery include less blood loss, less risk of infection, and lesser risk of clot formation; but disadvantages include increased pain and stress and increased sympathetic flow, thus increasing load on the ventricles.
Most clinically significant congenital heart lesions can be repaired in the childhood. Surgically repaired structural anomalies have better pregnancy outcomes. Pregnancy carries a high risk for both the mother and the fetus in patients with Eisenmenger syndrome, and the outcome depends on the severity of pulmonary hypertension associated with the lesion. Pregnancy in these patients can be avoided by judicious use of contraceptives. But, if a patient with congenital heart disease and Eisenmenger syndrome comes with pregnancy, she should be managed in a tertiary care center with multidisciplinary approach, a team of obstetrician, cardiologist, skilled anesthetist and pediatrician.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts on interest
There are no conflicts of interest.
| References|| |
Cunningham FG. Cardiovascular disorders. In: Cunningham FG, Leveno KJ, Bloom SL, Spong CY, Dashe JS, Hoffman BL et al.
(editors). Williams Obstetrics; 24th ed. New York: McGraw-Hill Education, 2014 pp. 980-90.
Lane CR, Trow TK. Pregnancy and pulmonary hypertension. Clin Chest Med 2011;32:165-74.
Hegewald MJ, Crapo RO. Respiratory physiology in pregnancy. Clin Chest Med 2011;32:1-13.
Madden BP. Pulmonary hypertension and pregnancy. Int J Obstet Anesth 2009;18:156-64.
O’Callaghan DS, Savale L, Yaici A, Natali D, Jais X, Parent F et al.
Endothelin receptor antagonists for the treatment of pulmonary arterial hypertension. Expert Opin Pharmacother 2011;12:1585-96.
[Figure 1], [Figure 2]