The Egyptian Journal of Cardiothoracic Anesthesia

: 2019  |  Volume : 13  |  Issue : 2  |  Page : 35--37

Eisenmenger syndrome and emergency laparotomy: a case report

Neeraj Kumar1, Amarjeet Kumar1, Kumari Sneha2, Mukta Agarwal2, Sanjeev Kumar3,  
1 Department of Trauma & Emergency, All India Institute of Medical Sciences, Patna, Bihar, India
2 Department of Anaesthesia, All India Institute of Medical Sciences, Patna, Bihar, India
3 Department of CTVS, All India Institute of Medical Sciences, Patna, Bihar, India

Correspondence Address:
Amarjeet Kumar
All India Institute of Medical Sciences Campus, Patna, Bihar, 801507


The ES (Eisenmenger syndrome) consists of pulmonary hypertension with a reversed or bidirectional shunt at the atrio-ventricular or aorto-pulmonary level. Anaesthetic management for these patients is always challenging. So careful circulatory and respiratory managements is required to avoid various factors related to surgery and anesthesia that can potentially increase right to left shunt. So primary goal is to maintain cardiac output to prevent a decrease in systemic vascular resistance and an increase in pulmonary vascular resistance. Here we describe an anaesthetic management of a 15-year-old lady,diagnosed with ES posted for emergency removal of left adnexal mass. In this case we have used general anaesthesia with patient controlled epidural analgesia for abolishing postoperative pain.

How to cite this article:
Kumar N, Kumar A, Sneha K, Agarwal M, Kumar S. Eisenmenger syndrome and emergency laparotomy: a case report.Egypt J Cardiothorac Anesth 2019;13:35-37

How to cite this URL:
Kumar N, Kumar A, Sneha K, Agarwal M, Kumar S. Eisenmenger syndrome and emergency laparotomy: a case report. Egypt J Cardiothorac Anesth [serial online] 2019 [cited 2020 Feb 18 ];13:35-37
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The Eisenmenger syndrome (ES) consists of pulmonary hypertension with a reversed or bidirectional shunt at the atrioventricular or aortopulmonary level [1]. The rate of incidental surgeries is increasing day by day only because these patients may survive into adulthood. Their compromised cardiovascular system always poses a huge challenge and changes the hemodynamics in the presence of anesthetic agents or surgery because of the fixed pulmonary vascular beds. Maternal mortality in the presence of ES is reported as 30–50% and even up to 65% in those with a cesarean section [2].

 Case report

A 15-year-old female patient with 30 kg body weight and American Society of Anesthesiologists class III was admitted for emergency exploratory laparotomy. She was a known case of ES diagnosed 5 years back and was on tablet sildenafil 50 mg eight hourly. She presented with dyspnea on exertion [New York Heart Association (NYHA) Class II], and the metabolic equivalent task score was 3. On physical examination, we noticed clubbing (grade II) and cyanosis of her fingers. Jugular venous pressure was not raised, and there was no pedal edema. At the time of admission, vital signs were as follows: temperature of 39.8°C, pulse rate of 142/min, blood pressure of 140/85 mmHg, a respiratory rate of 26 breaths/min, and oxygen saturation of 75% on 6 l/min of oxygen by facemask. On auscultation, the lungs were clear, and there was loud P2 with pan-systolic murmur heard over the lower left sternal border. Preoperative arterial blood gas (ABG) analysis on room air showed: pH of 7.42, PaO2 of 36 mmHg, PaCO2 of 34 mmHg, HCO3 of 18 mEq/l, BE of −4, and SaO2 of 72%. ECG showed right-axis deviation, right ventricular hypertrophy, and inverted P waves in leads II, III, and AVF. Transthoracic echocardiographic findings were large ventricular septal defect (VSD) 16 mm, with bilateral shunt predominantly right to left (the ratio of total pulmonary blood flow to total systemic blood flow is Qp/Qs ratio and if it is 1: 1 it indicates no intrapulmonary shunting and if the VSD>1–1.5 cm then it limits Qp : Qs ratio to three to four folds) [3], dilated right atrium (58 mm) and right ventricle (46 mm), moderate tricuspid regurgitation, moderate pulmonary regurgitation, estimated systolic pulmonary artery pressure of 107 mmHg and estimated left ventricular ejection fraction of 55%, and no clots. Her chest radiography showed left atrial dilatation and mild cardiomegaly. Ultrasonography whole abdomen showed a left adnexal mass (with no pus or fluid collection). Uterus was not seen. Both ovaries and kidneys were normal. Mild pelvic free fluid or collection was seen. MRI pelvis showed cervicovaginal atresia with unicornuate uterus and a noncommunicating rudimentary horn. Her preoperative hemoglobin was 16 g/dl with hematocrit of 48% (possibly due to adaptation to the low level of circulating oxyhemoglobin), and the rest of the hematological and biochemical investigations were unremarkable.

After explaining the anesthetic procedure and risks involved, we obtained written informed consent, and an emergency laparotomy under general anesthesia with lumbar epidural anesthesia was planned. We also planned for patient-controlled epidural analgesia (PCEA) for abolishing postoperative pain. In the operating room, standard ECG, non-invasive blood pressure (NIBP), and pulse oximeter were attached. We secured intravenous access using an 18 G intravenous cannula in the left upper limb. Preoperative blood pressure was 138/78 mmHg, pulse rate was 125 beats /min, respiratory rate was 24 breaths/min and oxygen saturation was 77% on 100% oxygen. Under full aseptic precautions and after adequate local infiltration, epidural anesthesia was given in the left lateral position in the L3–L4 space with midline approach by Tuohy 18 G epidural needle using loss of resistance technique. A 20 G epidural catheter was inserted and fixed at 8 cm from the skin, and thereafter the test dose was given. The patient was induced with etomidate 0.3 mg/kg and fentanyl 2 μg/kg. Endotracheal intubation was facilitated with vecuronium 0.1 mg/kg. We performed right-sided internal jugular vein cannulation for central venous pressure monitoring, and the right radial artery was cannulated anticipating large fluid shift and for measurement of continuous invasive blood pressure monitoring. We have taken the utmost precautions to avoid the risk of any systemic air embolism. Anesthesia was maintained with sevoflurane 1–1.5% in 50% oxygen and 50% air with controlled mode of ventilation. Intraoperatively, only one episode of hypotension 88/58 mmHg was observed, which was managed with phenylephrine. Total duration of surgery lasted for 120 min, and the estimated blood loss was ∼930 ml; a total volume of 1500 ml of ringer lactate and one unit packed red blood cells was infused, as the maximum allowable blood loss was calculated as 900 ml. The rest of the intraoperative period and recovery was uneventful. Intra-operative ABG analysis on 50% oxygen showed a pH of 7.39, PaO2 of 96 mmHg, PaCO2 of 32 mmHg, HCO3 of 16 mEq/l, BE of −4, SaO2 of 95%, and Hct of 26. Our goal was to keep ETCO2 between 30 and 35 mmHg mainly to prevent hypercarbia and respiratory acidosis. The patient was then kept in the high dependency unit overnight for observation. We started PCEA infusion pump by using 0.125% levobupivacaine and 2 μg/ml fentanyl at a rate of 5 ml/h for the next 24 h as background infusion without lockout time through a lumbar epidural catheter. She was discharged asymptomatic on the fourth postoperative day. We planned for early mobilization to prevent the risk for thromboembolism.


The anesthetic management of patients with ES poses a unique and difficult challenge for the anesthesiologist. The main anesthetic goal remains the avoidance of fall in arterial blood pressure by maintaining both the cardiac output and systemic vascular resistance [4].

Various studies concluded that no anesthetic technique, either general or regional anesthesia, is superior over an other in providing myocardial protection to ES patients mostly because their fixed pulmonary vascular bed, which makes these patients unable to adapt to sudden changes in hemodynamics [5],[6]. As per our institutional experience in treating these ES patients, we may use the lumbar epidural anesthesia technique (in our case we used 0.125% levobupivacaine 8 ml with fentanyl 25 μg) successfully without any abrupt fall in systemic vascular resistance and cardiac output. However, epidural anesthesia has been successfully used for minor surgeries such as tubal ligation and cesarean section [6],[7]. This may be achieved by combining a short-acting, intravenous narcotic such as fentanyl, which is usually well-tolerated, in addition to a low-dose induction agent such as etomidate or ketamine or an inhalational agent, for example, isoflurane [5]. Atracurium and vecuronium can be used as muscle relaxants because of their minimal effects on the cardiovascular system [7].

We have carried out periodic arterial blood gas analysis for assessment of acidosis, hypercarbia, and hypoxia, as it can lead to an increase in pulmonary vascular resistance, which further causes more right to left shunting. Continuous intra-arterial monitoring of the blood pressure is a safe and reliable means of early recognition of sudden alterations in intravascular volume and hemodynamics, and, simultaneously, it also determines periodic arterial blood gas. Eisenmenger patients should be observed on a monitored bed because of their predisposition to develop ventricular and supraventricular tachycardia.Precautions must be taken to prevent venous stasis by early ambulation and by applying effective elastic stocking or periodic pneumatic compression. In addition, every attempt should be made to prevent hypovolemia; thus, meticulous attention to fluid balance is essential.

Furthermore, adequate pain management is crucial because the stress of postoperative pain can result in adverse hemodynamics and possibly a hypercoagulable state [8]. Hence, in our case, we have kept her on continuous PCA pump infusion targeting a visual analog scale of less than 5.

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Conflicts of interest

There are no conflicts of interest.


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