Patil YS*, Wajekar AS**, Garasia MB***.
(* Associate Professor, ** Assistant Professor, *** Professor and Head, Department of Anaesthesiology, Seth GS Medical College & KEM Hospital, Mumbai, India.)
Eisenmenger's syndrome is a cyanotic heart disease characterized by pulmonary hypertension with reversed or bidirectional shunt associated with septal defects or patent ductus arteriosus (PDA). Pregnancy associated decreased systemic vascular resistance increases the degree of right to left shunting, thereby increasing substantially both the maternal mortality and fetal wastage which is reported to be as high as 30–70%. We present a case of a 20 year old primigravida with Eisenmenger's syndrome who gave birth at 34 weeks of gestation via cesarean section to a live baby under combined spinal and epidural anaesthesia. On the second post-operative day, the patient had a fall in oxygen saturation despite supplemental oxygen, managed conservatively. We describe the successful anesthetic management for caesarean section and its complications in a patient with Eisenmenger's syndrome.
Eisenmenger’s syndrome is characterised by pulmonary hypertension with reversed or bidirectional shunt associated with septal defects or patent ductus arteriosus (PDA). Pregnancy can worsen Eisenmenger’s syndrome due to fall in systemic vascular resistance resulting in high maternal mortality and fetal wastage ranging from 30 to 70%.[1,2] Generally an anesthesiologist comes into picture for providing anesthesia for delivery of the fetus by vaginal or cesarean route. We present the anesthesia management of elective cesarean section in a patient with Eisenmenger’s syndrome.
A 20 year old unregistered primigravida, with 34 weeks of gestation, recently diagnosed with Eisenmenger’s syndrome, on oral sildenafil and furosemide since one week, presented for elective cesarean section in view of cephalopelvic disproportion. Her pulse was 72/min, blood pressure 120/72 mm Hg with room air saturation 98%. She had no cyanosis or clubbing. She had grade IV ejection systolic murmur at apex. Her haemoglobin was 12g% with hematocrit of 32. Electrocardiogram revealed right ventricular hypertrophy. Chest radiograph revealed cardiomegaly, prominent pulmonary conus and pulmonary vascular congestion. All other routine investigations were normal. Echocardiography revealed severe pulmonary artery hypertension (55-65 mm Hg), mildly dilated and thickened right ventricle (RV), normal RV systolic function and bidirectional shunt due to a large ventricular septal defect (VSD). Her arterial blood gases were normal.
Preoperative aspiration prophylaxis and infective endocarditis prophylaxis were given.
In the operating room, difficult intubation cart with necessary cardiac drugs was kept ready. Standard monitoring including electrocardiography, automated blood pressure, pulse oximetry and endtidal CO2 was instituted. De-airing of all the lines was done. Left radial artery and right internal jugular vein (IJV) were cannulated for invasive monitoring. Her central venous pressure (CVP) was 4-6 cm of water. The patient was preloaded with 300 ml Ringer’s lactate. Epidural and spinal anesthesia were performed in the L2-3 and L3-4 space respectively in sitting position. 25 mcg fentanyl was given intrathecally. Then she was made supine. Left uterine displacement was maintained throughout the perioperative period. T6 level of anesthesia was achieved using a titrated dose of 2% lignocaine in incremental doses over 20 minutes (Total 12 ml) with steady hemodynamic monitoring. There was a fall in systolic pressure up to 80 mm of Hg once, treated with phenylephrine 50 mcg. Oxygenation was instituted with face mask at 5 l/min.
A male child of 2 kg with good Apgar scores was delivered. Oxytocin infusion was started. Intraoperative period was uneventful. Total 1000 ml Ringer's lactate was administered intraoperatively to maintain a CVP of 6-8 cm of water. She had an episode of desaturation (SPO2 92%) on day 2 postoperatively which was treated with oxygenation, nebulizations and antibiotics. Furosemide and sildenafil were continued. Postoperative epidural analgesia with tramadol was continued. She was shifted to ward on day 7 and discharged after 15 days.
Conception is discouraged or early termination of pregnancy is advised in patients with Eisenmenger’s syndrome since the fall in systemic vascular resistance seen in pregnancy can worsen Eisenmengerization with a very high rate of mortality reaching 30-70%. Maternal mortality is higher when associated with VSD in such patients as compared to atrial septal defects or PDA. In spite of this, there are a few case reports of successful maternal and fetal outcomes. Sildenafil therapy produces better maternal outcome.
Meticulous de-airing of all the lines for prevention of paradoxical air embolism is of utmost importance. There exists a risk of introducing infection or air during IJV line catheterisation. But fluid therapy guided by CVP is essential in prevention of right heart failure. Infective endocarditis prophylaxis has been advised in all patients of cyanotic heart diseases.
The choice of anaesthesia either regional or general is guided by current pathophysiology, severity of disease, maintenance of haemodynamics, thromboprophylaxis and postoperative analgesia. Our aim was prevention of fall in SVR to avoid increase in right to left shunt.
Intermittent positive pressure ventilation can increase the PVR due to increase in intrathoracic pressure and resultant fall in venous return. Also the risk of failed intubation, aspiration and postoperative hypoxia is increased with general anesthesia. Single shot spinal anesthesia can produce a precipitous fall in blood pressure. Intrathecal administration of an opioid like morphine or fentanyl is not associated with hypotension. Titrated epidural anesthesia prevents hypotension. But only epidural anesthesia produces segmental action and may provide inadequate anesthesia with need to convert into general anesthesia. In this scenario, combination of intrathecal opioid and titrated epidural local anaesthetics helps to increase the safety and reliability of the neuraxial block. Also, postoperative epidural analgesia helps to reduce pulmonary hypertension. Early postoperative mobilization also helps to reduce other complications.
Polycythemia can be common in these patients secondary to chronic hypoxia. Our patient did not have any signs of hypoxia and polycythemia. This can be attributed to the bidirectional nature of the shunt and sildenafil. Hence thrombo-prophylaxis was not essential making neuraxial anaesthesia an attractive choice.
Oxytocin was used as slow infusion as a bolus dose can cause precipitous fall in SVR.
Postoperative cardiac complications can be common from day 2 up to day 30. Our patient had desaturation on day 2 which was managed conservatively.
We conclude that carefully titrated combined spinal epidural anesthesia is safe, appropriate and effective for patients with Eisenmenger's syndrome for cesarean section.
- Cole PJ, Cross MH, Dresner M. Incremental spinal anaesthesia for elective Caesarean section in a patient with Eisenmenger’s syndrome. Br J Anaesth [Internet] 2001 [cited 2015 Apr 13];86(5):723–6. Available from: http://www.ncbi.nlm.nih.gov/pubmed/11575352
- Gurumurthy T, Hegde R, Mohandas B. Anaesthesia for a patient with Eisenmenger’s syndrome undergoing caesarean section. Indian J Anaesth [Internet] 2012 [cited 2015 Apr 13];56(3):291–4. Available from: http://www.pubmedcentral.nih.gov/articlerender.fcgi?artid=3425292&tool=pmcentrez&rendertype=abstract
- Rathod S SS. Successful Pregnancy Outcome in A Case of Eisenmenger Syndrome: A Rare Case Report. J Clin Diagnostic Res 2014;8(10):OD08–OD09.
- Cartago RS, Alan PA BJ. Pregnancy outcomes in patients with severe pulmonary hypertension and Eisenmenger syndrome treated with Sildenafil monotherapy. Chest 2012;142(4):999.
- Wilson W, Taubert KA, Gewitz M, Lockhart PB, Baddour LM, Levison M, et al. Prevention of infective endocarditis: guidelines from the American Heart Association: a guideline from the American Heart Association Rheumatic Fever, Endocarditis, and Kawasaki Disease Committee, Council on Cardiovascular Disease in the Young, and the Co. Circulation [Internet] 2007 [cited 2015 Feb 20];116(15):1736–54. Available from: http://www.ncbi.nlm.nih.gov/pubmed/17446442
- Abboud TK, Raya J, Noueihed R, Daniel J. Intrathecal morphine for relief of labor pain in a parturient with severe pulmonary hypertension. Anesthesiology [Internet] 1983 [cited 2015 Apr 13];59(5):477–9. Available from: http://www.ncbi.nlm.nih.gov/pubmed/6638561
Patil YS, Wajekar AS, Garasia MB. Anaesthesia Management In A Patient Of Eisenmenger’s Syndrome For Caesarean Section. JPGO 2015. Volume 2 No. 6. Available from: http://www.jpgo.org/2015/06/anesthesia-management-in-patient-of.html