Eisenmenger syndrome in pregnancy.JJ Kansaria, VS Salvi
Department of Obstetrics & Gynaecology, Seth G. S. Medical College, K. E. M. Hospital, Parel, Mumbai - 400 012, India., India
Correspondence Address: Source of Support: None, Conflict of Interest: None PMID: 0011013476
Source of Support: None, Conflict of Interest: None
Maternal mortality in the presence of Eisenmenger syndrome is reported to be 30 to 50% & increases further with associated complications. A case of Eisenmenger syndrome in pregnancy where the patient progressively deteriorated postpartum & expired 3 weeks later is reported.
Keywords: Adult, Case Report, Eisenmenger Complex, physiopathology,Fatal Outcome, Female, Human, Pregnancy, Pregnancy Complications, Cardiovascular, physiopathology,Pregnancy Outcome,
Eisenmenger syndrome in pregnancy is associated with a high maternal mortality (30-50%), and overall foetal wastage is reported to be up to 75%. Medical termination of pregnancy is the preferred management for women with Eisenmenger syndrome who present early in pregnancy.
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.
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.
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. 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%,. In a review of the subject, Gleicher et al 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.
Prophylactic anticoagulation probably with subcutaneous heparin, should be offered, because of the risk of thromboembolism, both systemic and pulmonary. However, Pitts et al, 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. 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. Third trimester foetal surveillance with ultrasound and ante-partum testing is important because at least 30% of the foetuses will be growth retarded. 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,. 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,,. The use of epidural or intrathecal morphine sulphate, a technique devoid of effect on systemic blood pressure has been described by Abboud et al and represents perhaps the best approach to anaesthestic management of these difficult patients.