Address for
Correspondence: Dr. Priyanka Pavithran, Senior
Specialist, Dept. of Anaesthesiology, ASTER MIMS,
Calicut. E- mail: priyanka.pavithran@gmail.com
Keywords: Eisenmenger Syndrome, caesarian section
Introduction
Initially described by Victor Eisenmenger and redefined by
Wood [1], Eisenmenger syndrome is essentially presence of high
pulmonary vascular resistance associated with pulmonary artery
hypertension (PAH) at, or close to, systemic pressure and
associated with a reversed or bi-directional shunt at the
aortopulmonary, interatrial or interventricular level [2].
Pregnancy in a patient with this condition causes
deterioration due to the physiological cardiovascular changes
of pregnancy. Such patients presenting for caesarean section
is a major anaesthetic challenge which needs multidisciplinary
management. We describe our experience with such a case.
Case report
A young lady, a known case of Eisenmenger syndrome with large
ventricular septal defect (VSD), with 34 weeks gestation was
admitted for safe confinement. Her obstetric score was G3A2.
She developed pregnancy induced hypertension (PIH) at 20weeks
of gestation, which was managed with alpha methyl DOPA and
Labetalol. Her VSD was diagnosed at 12 years of age. She was
not on any cardiac medications and was advised to avoid
pregnancy. She was essentially asymptomatic, except for grade
2 dyspnoea on exertion during her third trimester of
pregnancy. Her clinical examination revealed central cyanosis,
Grade 2 clubbing and minimal bilateral pedal edema. Room air
SPO2 was 75 %. Laboratory investigations revealed Hb - 15.8
mg/dl, Hct - 53, Platelet count - 1.3 lakh /dl with all other
baseline parameters in normal limits. ABG showed a pH - 7.36,
PaCO2 54.5 mmHg, PaO2 54.5 mmHg and SPO2 - 82-88%. ECG showed
Right axis deviation with Right ventricular hypertrophy.
Echocardiography demonstrated a large VSD with bidirectional
shunt, Severe PAH, moderate coarctation of aorta, dilated
right atrium and ventricle with a good left ventricular
systolic function and ejection fraction of 60%. Cardiologist
evaluated her and categorised her as high risk with 50%
peripartum mortality. A multidisciplinary team involving
cardiologist, anaesthesiologist, obstetrician and
paediatrician was formed to decide on the management of this
patient and decision was made to monitor the mother and fetus
till term and to do an elective caesarean section.
At 36 weeks of gestation she was posted for emergency
caesarean section indicated by abnormal Doppler and severe
PIH. After appropriate evaluation and consideration of risks
and benefits of general and regional anaesthesia decision was
made to proceed with a graded epidural anaesthesia. On table,
patient was connected to the monitors including ECG, Pulse
oximetry. Right radial artery was cannulated to monitor
arterial blood pressure (ABP). Intravenous (IV) access was
established through two 18 G cannulae placed on dorsum of
right hand and in left external jugular vein. Cardiac
output monitoring was done through Flotrac. Mechanical
thromboprophylaxis was initiated. Extra precautions were taken
to avoid air bubbles in the IV lines and to limit fluid
administration. Infective endocarditis prophylaxis comprising
of ampicillin and gentamycin was administered as prescribed by
the cardiologist. Her baseline parameters were HR- 74/min, ABP
- 120/70 mmHg, SPO2 on room air of 76% improving to 89% on
oxygen mask. A phenylephrine infusion in a dose of 0.2-0.5
µg/kg/min was started.
An epidural catheter was placed in the L3 - L4 space and was
fixed at 8 cm. Incremental doses of 0.375% Bupivacaine
titrated against hemodynamic parameters was given. Over 30
minutes a total of 12 ml of 0.375% Bupivacaine and 100 µg
Fentanyl was administered, which resulted in a loss of cold
sensation up to T8 level. Surgery proceeded uneventfully and
was completed in 45 minutes. On delivery of baby 20 Units of
Pitocin diluted in 100 ml NS was given slowly. 600 ml of
crystalloid was used. After surgery epidural catheter was
removed, phenylephrine infusion was tapered and stopped and
patient was shifted to intensive care unit. Postoperative
analgesia was maintained with tramadol and morphine.
Intermittent pneumatic compressions and heparin was used for
thromboprophylaxis. After an uneventful postoperative period
she was discharged on eighth postoperative day.
Discussion
Pregnancy imposes multiple physiological changes on the
maternal body in order to compensate for the increased oxygen
demands. This includes a fall in pulmonary vascular resistance
(PVR) to increase pulmonary blood flow which fails to happen
due to the fixed pulmonary vascular resistance in these
patients. Further, the decrease in systemic vascular
resistance (SVR) seen in pregnancy further decreases
oxygenation by increasing the right to left shunt. In
combination all these act to reduce maternal oxygenation
further leading to fetal compromise. Maternal mortality in
patients with Eisenmenger syndrome has been noted to be as
high as 30%-50% [3]. Auto transfusion during labor and
delivery can increase the volume load on the heart. Major
causes of death in postoperative period are hypovolemia,
thromboembolism and preeclampsia [4]. Eisenmenger syndrome
with severe PIH is associated with extremely high risk.
Perioperative management of pregnant patients with Eisenmenger
syndrome needs a multidisciplinary team approach with
intensive monitoring extending well into the postoperative
period since majority of maternal deaths happen in postpartum
period.
Supplemental oxygen should be provided at all stages of labor.
Intensive monitoring in the perioperative period is mandatory.
Invasive monitors like pulmonary artery catheter have been at
disadvantage due to the vascular complications in these
patients with abnormal pulmonary vasculature. Pulse oximetry
is the best monitor for assessing the right to left shunt [5].
IBP can demonstrate any sudden change in blood pressure.
Adequate analgesia should be given to avoid sympathetic
stimulation.
For patients presenting for caesarean section, the main
anaesthetic goal would be to maintain the balance between SVR
and PVR. The anaesthesiologist should focus on maintaining SVR
and intravascular volume, avoiding vena caval compression,
myocardial depression and an increase in PVR. Both regional
and general anaesthetic techniques have been used, both with
benefits and risks associated with each technique. Studies
have shown that no single anaesthetic technique has been shown
to be superior [6].
General anaesthesia can decrease the venous return due to IPPV
and the inhalational agents can cause myocardial depression
and a fall in SVR. Performing a rapid sequence induction will
cause further harm by the sudden hemodynamic effects caused by
the fast administration of the induction drugs. Regional
techniques can cause a precipitous decrease in SVR, though a
graded epidural produces a gradual fall in SVR [7]. Epidural
anaesthesia has now been widely reported in the management of
these cases [8,9]. Several of these patients are on
anticoagulants which may prove to be a hindrance to choosing a
regional technique.
Irrespective of the anaesthetic technique chosen the intensive
monitoring should extend into the postoperative period because
of the high maternal mortality in the postoperative period.
Meticulous fluid management is needed, replacing any blood
loss with blood products if needed.
The role of anticoagulation is controversial. Adverse outcomes
have been seen with administration of heparin. Judicious
pharmacotherapy, mechanical thromboprophylaxis and early
ambulation can decrease the chances of thromboembolism and
improve the maternal outcome [5].
Conclusion
Successful perioperative management of patients with
Eisenmenger syndrome needs a multidisciplinary approach with
intensive monitoring. The key to the anaesthetic management
would be appropriately balancing the SVR and PVR. The high
maternal mortality in the postoperative should always be kept
in mind while maintaining an argus eye monitoring in the
postoperative period as well.
References
1. Puri GD, Pradhan A, Kumar B, Hegde H V, Singh A,
Prasad GR V. Anaesthetic management of a patient with
Eisenmenger syndrome for lower abdominal surgery. Trends
Anaesth Crit Care. 2011;1:51-3.
2. Gupta N, Kaur S, Golla A PM. Anaesthetic management of a
patient with Eisenmenger syndrome and β -thalassemia major for
splenectomy. Indian J Anaesth. 2011;55:187-9.
3. Mishra L, Pani N, Samantaray R NK. Case Report
Eisenmenger’s syndrome in pregnancy : Use of epidural
anesthesia and analgesia for elective cesarean section. J
Anaesthesiol Clin Pharmacol. 2014;30:425-6.
4. Yuan S. Eisenmenger Syndrome in Pregnancy. Braz J
Cardiovasc Surg. 2016;31:325-9.
5. Fang G, Tian YK, Mei W, Presentation C. Case Report
Anaesthesia Management of Caesarean Section in Two Patients
with Eisenmenger’s Syndrome. Anesthesiol Res Pract. 2011;1-5.
6. Ammash NM, Connolly HM, Abel MD WC. Noncardiac Surgery in
Eisenmenger Syndrome. J Am Coll Cardiol. 1999;33:222-7.
7. Chestnut DH, Polley LS, Tsen LC WC. Obstetric anesthesia
principles and practices. In 2009. p. 884-5.
8. Rodrigues JS, Shenoy T AM. Case Report Anesthetic
management of a patient with Eisenmenger's syndrome for an
emergency caesarean section in an under resourced area. J Obs
Anaesth Crit Care. 2015;5:27-9.
9. Senthilnathan M, Velayudhan S, Cherian A KP. Anaesthetic
management of a pregnant woman with preeclampsia and
Eisenmenger ’ s syndrome : Role of advanced haemodynamic
monitoring. Indian J Anaesth. 2017;61:97-8.