Successful pregnancy and delivery in a women with a single ventricle and Eisenmenger syndrome M. Boukhris , K. Hakim, F. Ouarda, H. M’saad, R. Boussaada
Cardiopediatrics department, Rabta hospital, Tunis Presented by :
Dr M’saad Hela
SHA conference 2013
A 27-year-old woman, gravida 1, para 0
Admitted at 19 weeks gestation
Dyspnea & palpitations
Effort intolerance since the childhood
Physical examination
Cyanosis : Sp02 = 70 %
Fingers clubbing
HR = 80 bpm , BP = 100/60 mmHg, RR =22 cpm
Cardiac auscultation : accentuated S2
Pulmonary auscultation : normal
EKG : 
Sinus rhythm with a right bundle branch block.
Laboratory analysis
Hb = 16.1 g/dl Ht = 49% Pt = 350 000/ mm3 Creat = 7 mg / l SGOT / SGPT = 36 / 44 UI/l
Echocardiography
Echocardiography
Echocardiography
Echocardiography
Echocardiography
Echocardiography
Abdominal sonogram

Normal living fetus.
Diagnosis


Single ventricle with normal positioned great vessels without pulmonary stenosis Eisenmenger syndrome
Medical abortion was indicated
But …
The patient refused the medical abortion.
She was lost to follow up.
She came in labor at 37 weeks gestation.
She was hemodynamically stable
The delivery was obtained by cesarean section with epidural anesthesia using bupivacaïne
The infant was a male weighing 3 kg
Apgar rating 7/9/10 after 1, 5 and 10 mn
He did not show any malformations. His echocardiography was normal
Treatment
O2 (6L/mn) Nitric oxide (0.8 PPM) during 48 hours. Antibiotics: Amoxicillin + Clavulinic acid for 5 days. Anticoagulation therapy as follows: unfractionated
heparin was introduced 8 hours after
the operation low molecular weight heparin 16 h later oral anticoagulants from the third day.

The post operative echocardiography noted the preservation of the left ventricular systolic function and the stability of pulmonary hypertension
The post-partum period was uneventful
Discharge on the thirteenth day
Three months later : Cath high PVR Eisenmenger
Endothelin receptors inhibitors (Bosentan) introduced. Mid-term outcome : good
Comments 
Single ventricle is a rare cyanotic abnormality found in 1% of patients with congenital heart diseases.
Comments
Different forms can be observed; those without a pulmonary protection may lead to an Eisenmenger syndrome.
Early diagnosis and banding of the pulmonary artery are able to prevent it.
In our patient, it was too late and the pulmonary hypertension was fixed.
Comments
Pregnancy makes dyspnea worse :
↑ the cardiac output Anemia
Delivery = risky event :
↑ right to left shunt : ↓ SVR & ↑ PVR
Distal microthrombosis Hypoxia
Comments
Severe complications may occur : Heart
failure Endocarditis Thromboembolic accidents
They must be prevented by: Nitric
oxide Antibiotics Anticoagulant therapy
Comments
Mode of delivery is controversial : Cesarean
section Vaginal delivery
Because of its shorter duration
Comment
Mode of anesthesia is controversial too :
General anesthesia Regional anesthesia
Regional anesthesia may ↓ SVR ↑ the R to L shunt ↑ hypoxemia
Comments 
The prognosis of such cases depends on the pulmonary hypertension which represents the major mortality and morbidity factor.

The excellent outcome of this pregnancy may be associated with the preserved systolic function of the single ventricle.
Conclusions
Patients with single ventricle and Eisenmenger syndrome rarely reach adult life.
In spite of the development of anesthesia and resuscitation, pregnancy with Eisenmenger syndrome is still contraindicated.
Combined obstetric, anesthetic and cardiology team is necessary.
Such case can represent a hope for women with congenital heart diseases.
Thank you for your attention