A 33 years old woman with Eisenmenger syndrome, presented at 27.

4 weeks amenorrhoea
with class III dyspnoea on exertion and occasional cyanotic spells. She was a gravida 2 with
a past history of one abortion.
On examination, she was short statured (height of 132 cm) and weighed 30 kg. She also
had central and peripheral cyanosis, polycythaemia (haematocrit of 62 %.), and clubbing
Grade III. The foetal size corresponded to 24 weeks (lag of four weeks).
The 2-Dimensional echocardiography (2D echo) and Doppler report showed an
arteriovenous shunt at the level of the great vessels, with pulmonary hypertension and right
ventricular hypertrophy. The exact site of the shunt could not be localised. There was no
evidence of an intracardiac shunt. She was advised cardiac catheterisation post delivery to
identify the exact site of the arterio-venous shunt. Foetal 2D echo was normal.
She was advised complete bed rest and continuous oxygen by mask. She was started on
digoxin, hydrochlorthiazide and mist potassium chloride. Her course in the antenatal period
was uneventful. Monitoring of foetal growth by serial obstetric scans showed a constant lag
of 4 weeks.
She went into spontaneous labour at 37 weeks amenorrhoea. Central venous pressure was
maintained at 8cm H2O. She had a term vaginal delivery of a growth retarded, live born
female baby (weight -1.55 kg). In the immediate post partum period, she developed a large
episiotomy haematoma, which was evacuated, and the episiotomy resutured. The patient
was shifted to intensive care unit and started on a dopamine drip in view of persistent
hypotension and low central venous pressure and transferred back to post natal ward after
48 hours, after stabilization of vital parameters.
Over the next two weeks the patient had frequent episodes of cyanotic spells and dyspnoea,
which were relieved with nasal oxygen. The patient took discharge against medical advice
on day 17 post-delivery. She was readmitted in intensive care unit on day 21 post-delivery
with class IV dyspnoea, central cyanosis and altered sensorium. She was diagnosed to be in
congestive cardiac failure and severe hypoxia. Arterial blood gas analysis showed severe
respiratory acidosis with a pH of 7.05, PaCO2 44.2%, PaO2 39%, SaO2 43.7%. The patient
progressively deteriorated and expired on day 24 post partum. An autopsy was not done.

:: Discussion

In the presence of a congenital left to right shunt, progressive pulmonary hypertension

leads to shunt reversal or bi-directional shunting and the development of Eisenmenger
syndrome. Whatever the aetiology, pulmonary hypertension carries a grave prognosis
during pregnancy[3].
During the antepartum period, the decreased systemic vascular resistance associated with
pregnancy increases the likelihood and the degree of right to left shunting. The pulmonary
perfusion then decreases; this decrease results in hypoxaemia and deterioration of the
maternal and foetal condition. In such a patient, systemic hypotension leads to decreased
right ventricular filling pressure and in the presence of fixed pulmonary hypertension, such
decreased right heart pressure may be insufficient to perfuse the pulmonary arterial bed.
This insufficiency may result in sudden profound hypoxemia and death. Such hypotension
can result from haemorrhage or complications of conduction anaesthesia and can lead to

sudden death[4]. Such an occurrence is the principal clinical concern in the intrapartum
management of patients with pulmonary hypertension.
Maternal mortality in the presence of Eisenmenger syndrome is reported as 30 to 50% [1],[2].
In a review of the subject, Gleicher et al [1] reported a 34% mortality associated with vaginal
delivery and a 75% mortality associated with caesarean section. In addition to the
previously discussed problems associated with haemorrhage and hypovolaemia,
thromboembolic phenomena occur, and they have been associated with up to 43% of all
maternal deaths in Eisenmenger syndrome[1].
Prophylactic anticoagulation probably with subcutaneous heparin, should be offered,
because of the risk of thromboembolism, both systemic and pulmonary. However, Pitts et
al[5], reported an increased mortality associated with prophylactic peri-partum heparin
therapy. Sudden delayed postpartum death occurring 4-6 weeks after delivery, also has
been reported on several occasions[1]. Although the pathophysiology of this condition is
unknown, such deaths may involve a rebound worsening of pulmonary hypertension
associated with the loss of pregnancy associated hormones. Because of the high mortality
associated with continuing pregnancy, medical termination of pregnancy is the preferred
management for women with pulmonary hypertension of any aetiology.
For a patient who continues her pregnancy, hospitalisation for the duration of pregnancy is
often appropriate. Continuous administration of oxygen, the pulmonary vasodilator of choice
is mandatory. In cyanotic heart disease of any aetiology, foetal outcome correlates well with
maternal haematocrit and successful pregnancy is unlikely with a haematocrit >65%.
Maternal arterial partial pressure of oxygen should be maintained at a level of 70 mmHg or
above[6]. Third trimester foetal surveillance with ultrasound and ante-partum testing is
important because at least 30% of the foetuses will be growth retarded [1]. The overall foetal
wastage with Eisenmenger syndrome is reported to be up to 75%.
Pulmonary artery catheterisation is recommended during the intra-partum period. Besides
high inspired oxygen, efforts should be made to avoid central hypovolaemia. This includes
the use of uterine displacement to ensure adequate venous return to the heart. In addition,
alpha sympathomimetic agents such as phenylephrine, methoxamine and nor-adrenaline
will increase systemic resistance and thus increase pulmonary blood flow. Recently, the use
of inhaled nitric oxide, during labour, has been recommended since it is a specific
pulmonary vasodilator[7],[8]. Both the patients in these case reports, however, expired in the
post-partum period.
Anaesthesia for patients with pulmonary hypertension is controversial. Theoretically,
conduction anaesthesia, with its accompanying risk of hypotension, should be avoided.
However, there are several reports of its successful use in patients with pulmonary
hypertension of different aetiologies[4],[9],[10]. The use of epidural or intrathecal morphine
sulphate, a technique devoid of effect on systemic blood pressure has been described by
Abboud et al[11] and represents perhaps the best approach to anaesthestic management of
these difficult patients.

:: References
1.

Gleicher N, Midwall J, Hochberger D, Jaffin H. Eisenmengers syndrome and pregnancy.

Obstet Gynecol Surg 1979; 34:721-741.

2.

Daliento L, Somerville J, Presbitero P, Menti L, Brach-Prever S, Rizzoli G, et al.

Eisenmenger syndrome. Factors relating to deterioration and death. Eur Heart J 1998;
19:1845-1855.

3.

Clark SL, Cotton DB, Hankins GDV, Phelan JP. Critical Care Obstetrics. 2nd ed.
Cambridge: Blackwell Sciene Ltd.; 1991. pp 114-135.

4.

Knapp RC, Arditi LI. Pregnancy complicated by patent ductus arteriosus with reversal of
flow. N Y State J Med 1967; 67:573-577.

5.

Pitts JA, Crasby WM, Basta LL. Eisenmenger's syndrome in pregnancy: does heparin
prophylaxis improve the maternal mortality rate? Am Heart J 1977; 93:321-326.

6.

Sobrevilla LA, Cassinelli MT, Carcelan A, Malaga JM. Human fetal and maternal oxygen
tension and acid base status during delivery at high altitude. Am J Obstet Gynecol
1971; 111:1111-1118.

7.

Lust KM, Boots RJ, Dooris M, Wilson J. Management of labor in Eisenmenger syndrome
with inhaled nitric oxide. Am J Obstet Gynecol 1999; 181:419-423.

8.

Goodwin TM, Gherman RB, Hameed A, Elkayam U. Favourable response of

Eisenmenger syndrome to inhaled nitric oxide during pregnancy. Am J Obstet Gynecol
1999; 180:64-67.

9.

Midwall J, Jaffin H, Herman MV, Kupersmith J. Shunt flow and pulmonary

hemodynamics during labour and delivery in the Eisenmenger Syndrome. Am J Cardiol
1978; 42: 299-303.

10.

Spinnato JA, Kraynack BJ, Cooper MW. Eisenmenger's syndrome in pregnancy: epidural
anaesthesia for elective caesarean section. N Engl J Med 1981; 304:1215-1217.

11.

Abboud TK , Raya J, Noueihed R, Daniel J. Intrathecal Morphine for relief of labor pain
in a parturient with severe pulmonary hypertension. Anaesthesiology 1983; 59:477479.