SHA24/025004

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

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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

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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

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Discharge on the thirteenth day

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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