Year : 2015 | Volume
: 5 | Issue : 1 | Page : 27--29
Anesthetic management of a patient with Eisenmenger's syndrome for an emergency caesarean section in an under resourced area
Joanna Samantha Rodrigues, Thrivikram Shenoy, Manasa Acharya
Department of Anaesthesiology, Kasturba Medical College, Mangalore, Karnataka, India
Dr. Joanna Samantha Rodrigues
Department of Anaesthesiology, Kasturba Medical College, Mangalore, Karnataka
Eisenmenger«SQ»s syndrome, although uncommon, has a plethora of literature available on its anesthetic management for caesarean sections. Options of management have varied from general anesthesia to continuous spinal to epidurals. However, management without the essential invasive monitoring devices poses a formidable challenge to the anesthetist. We present a case of a 26-year-old primigravida with Eisenmenger«SQ»s syndrome with pulmonary hypertension in labor who underwent an emergency cesarean section under epidural anesthesia in a hospital that lacked agents to maintain the cardiovascular stability as well as monitoring equipment - a situation not so uncommon in low resourced areas. The intra-operative course was uneventful but tragically she died on the 3 rd postoperative day.
|How to cite this article:|
Rodrigues JS, Shenoy T, Acharya M. Anesthetic management of a patient with Eisenmenger's syndrome for an emergency caesarean section in an under resourced area.J Obstet Anaesth Crit Care 2015;5:27-29
|How to cite this URL:|
Rodrigues JS, Shenoy T, Acharya M. Anesthetic management of a patient with Eisenmenger's syndrome for an emergency caesarean section in an under resourced area. J Obstet Anaesth Crit Care [serial online] 2015 [cited 2021 Jun 16 ];5:27-29
Available from: https://www.joacc.com/text.asp?2015/5/1/27/155196
Pulmonary hypertension, with congenital heart disease, is seen in large systemic-to-pulmonary communications, such as ventricular septal defect (VSD) and patent ductus arteriosus. On progression, it leads to shunt reversal a condition termed Eisenmenger syndrome. 
Pregnancy in this setting is associated with substantial maternofetal risk. Maternal mortality is high, with a cumulative risk of 30-70%. , The principle of any anesthetic technique chosen is to maintain systemic vascular resistance (SVR), avoiding its fall or increase in pulmonary vascular resistance (PVR). In a developing country like India, encountering a set up lacking modern facilities is common. Burdened financially, such a patient presents a herculean challenge.
A 26-year-old primigravida at 35 weeks of gestation, in labor, weighing 45 kg, known case of VSD with severe pulmonary arterial hypertension, diagnosed 5 years ago, who developed Eisenmenger's syndrome at the 2 nd month of gestation was referred to our hospital for emergency caesarean section.
Preoperatively, apart from exertional dyspnea, she was asymptomatic. Physical examination revealed Grade II clubbing, pulse rate of 82/min, blood pressure (BP) of 108/80 mmHg and room air oxygen saturation (SpO 2 ) of 80% with no rise in jugular venous pressure. Central cyanosis was observed. Lungs were clear to auscultate, and cardiac examination showed regular rate and rhythm with loud P 2 and pansystolic murmur over the lower left sternal border. Electrocardiography (ECG) showed right axis deviation, right ventricular hypertrophy and inverted P waves in lead II III and Lead avf. Two-dimensional echo color Doppler revealed a large subacute VSD, mild tricuspid regurgitation with a bidirectional shunt and an ejection fraction of 58% with severe pulmonary hypertension. Pulmonary artery pressure was not indicated. Hemoglobin (Hb) was 12.2 g%, and platelet count of 129,000/mm 3 . Arterial blood gas was unavailable. The cardiologist advised to undergo an emergency caesarean section under high risk. Peripheral venous access was secured. Meticulous attention was paid to the avoidance of bubbles in lines and syringes because of the risk of paradoxical embolus. She was premedicated with ranitidine 150 mg and metoclopramide 10 mg intravenous 20 min prior to surgery. In the operation theatre, standard monitors - ECG leads, noninvasive BP, saturation probe were connected. Oxygen via Hudson mask at 8 l/min was supplemented. SpO 2 increased to 88%. The preinduction BP and heart rate were 126/62 mmHg and 82/min respectively. Under all aseptic precautions with the patient in the left lateral position an epidural catheter was inserted at the L 3 -L 4 intervertebral space. Incremental doses of 3-5 mL of 2% lidocaine without epinephrine were administered every 5 min, and a sensory block to the level of T 8 was achieved with 12 mL lidocaine over 30 min. Adequate analgesia was achieved. No hypotension developed intra-operatively. No further top up doses of epidural were required. A live male baby with Apgar score of 5 at 1 min and 8 at 5 min was extracted. Intravenous infusion of oxytocin 15 units was administered slowly over 30 min. A volume of 800 mL Ringer's solution was administered during the 48 min surgery. Estimated blood loss was 200 mL. Urine volume was 200 mL. Patient was shifted to the intensive care unit. Unfortunately despite all our efforts, our patient developed respiratory distress and fever on the 3 rd postoperative day along with severe anemia (Hb 6 g%) and leukocytosis (25,000/cm 3 ) and she ultimately succumbed to her illness.
In Eisenmenger's syndrome, there is a reversal of a left to right shunt occurring due to high right-sided heart pressure as compared to the left side.
Pregnancy-induced systemic vasodilation is detrimental in parturients with Eisenmenger's syndrome. Reduced SVR may increase right-to-left shunting  and decrease pulmonary blood flow, leading to further hypoxemia with significant risks for both mother and fetus. Anesthetic management herein requires balancing SVR and PVR.
Regional and general anesthesia have been used. Inadvertent hypotension can occur with both techniques. The problems of general anesthesia are decrease in venous return and cardiac output. Many induction and maintenance agents depress myocardial function and reduce SVR. Drugs chosen should be those causing least hemodynamic disturbance, that is, opioid agonists or etomidate. Due to their unavailability, we opted for epidural anesthesia.
The hazards of general anesthesia are avoided by regional anesthesia, although the level of block required using a regional technique might produce excessive sympathetic block and an uncontrolled decrease in the SVR. 
Epidural anesthesia has been used successfully in this condition , and because of its slow onset; this technique has minimal precipitous hemodynamic changes. However, meta-analysis doesn't show a significant difference in perioperative mortality between general and regional anesthesia, and both approaches have significant morbidity and mortality. 
Argus eyed monitoring forms the mainstay of intra-operative management. Though our set up lacked invasive arterial BP and central venous pressure (CVP) monitoring, we relied immensely on the pulse oximetry to assess the degree of right to left shunt. We had no access to cardio stable drugs like etomidate or free supply of opioids. We could have referred the patient to our premiere, medical college hospital but the financial and time constraints prohibited us as our patient was in active labor.
Fluid management is a double edged sword. We provided adequate uterine tilt, monitored the urine output and dehydration status to judiciously administer fluids. Oxygen is a pulmonary vasodilator decreasing the blood flow across the right-to-left shunt thereby improving the saturation. So it should be provided throughout the perioperative period. At the end of the procedure with oxygen supplementation, our patient achieved a saturation of 98%. Thromboembolism prophylaxis should be encouraged by early ambulation and if prolonged immobilization is anticipated subcutaneous administration of heparin should be given. 
On the 3 rd postoperative day, our patient developed respiratory distress. We suspected either pulmonary embolism, right heart failure, transfusion-related acute lung injury, septicemia or disseminated intravascular coagulation. The most common cause of respiratory distress in such patients being right heart failure due to fluid shifts and iatrogenic fluid overload which can be detected by CVP monitoring or a pulmonary artery catheter. The blood cultures, however, revealed sepsis and was probably the cause of death. If this patient had received treatment in a tertiary carefully equipped hospital, which offered invasive intra- and post-operative monitoring modalities and essential drugs, we would have probably reaped a different outcome.
Although we were successfully able to anaesthetize a patient, with Eisenmenger's syndrome in an emergent situation, with minimal resources and financial restraints, the utility of an invasive monitoring device such as an arterial line or pulmonary artery catheter would have provided more information about the cardiac pressures intra-operatively and enabled vigilant monitoring in the postoperative period along with tactful management thereby reducing the mortality rate.
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