Perioperative management of emergency non-cardiac surgery in a pediatric patient with Eisenmenger syndrome: a case report
Case Report

Perioperative management of emergency non-cardiac surgery in a pediatric patient with Eisenmenger syndrome: a case report

Chenjia Jiang1 ORCID logo, Jialian Zhao1 ORCID logo, Jiajia Zhao1 ORCID logo, Wenfang Huang1 ORCID logo, Chunxiu Li1, Yue Jin1,2 ORCID logo

1Department of Anesthesiology, Children’s Hospital, Zhejiang University School of Medicine, National Clinical Research Center for Children and Adolescent’s Health, Hangzhou, China; 2Perioperative and Systems Medicine Laboratory, Children’s Hospital, Zhejiang University School of Medicine, National Clinical Research Center for Children and Adolescent’s Health, Hangzhou, China

Contributions: (I) Conception and design: Y Jin, C Jiang; (II) Administrative support: Y Jin; (III) Provision of study materials or patients: All authors; (IV) Collection and assembly of data: C Jiang; (V) Data analysis and interpretation: All authors; (VI) Manuscript writing: All authors; (VII) Final approval of manuscript: All authors.

Correspondence to: Yue Jin, PhD. Department of Anesthesiology, Children’s Hospital, Zhejiang University School of Medicine, National Clinical Research Center for Children and Adolescent’s Health, Binsheng Road, Hangzhou 310052, China; Perioperative and Systems Medicine Laboratory, Children’s Hospital, Zhejiang University School of Medicine, National Clinical Research Center for Children and Adolescent’s Health, Hangzhou, China. Email: yue_jin@zju.edu.cn.

Background: Eisenmenger syndrome (ES) is a late-stage complication of untreated congenital heart disease. It is characterized by irreversible pulmonary arterial hypertension (PAH), right-to-left shunting, and cyanosis. Pediatrics with ES who undergo emergency non-cardiac surgery are at an extremely high perioperative risk. Potential complications include pulmonary hypertension crisis (PHC), right ventricular failure, and sudden cardiac death. There is limited evidence guiding perioperative management in this population.

Case Description: An 11-year-old girl required emergency surgery under general anesthesia for right ovarian torsion. She had a history of congenital heart disease, which was complicated by severe pulmonary hypertension (pulmonary artery pressure: 105/49 mmHg) and ES. Anesthesia was inducted with etomidate, midazolam, vecuronium, and sufentanil, and maintained with propofol, remifentanil and sevoflurane. Intraoperatively, hemodynamic parameters remained stable. However, the patient developed a postoperative PHC, which was characterized by ventricular tachycardia, hypotension, and hypoxemia. Prompt treatment with amiodarone, treprostinil, sodium bicarbonate, and supplemental oxygen successfully restored sinus rhythm and hemodynamic stability. The patient was discharged on postoperative day 11.

Conclusions: Emergency non-cardiac surgery poses an extremely high anesthetic risk for pediatrics with ES. It is critical to avoid factors that elevate pulmonary vascular resistance, ensure meticulous monitoring, and recognize and treat PHC early. This case underscores the importance of personalized anesthetic planning and multidisciplinary perioperative management for this vulnerable population.

Keywords: Eisenmenger syndrome (ES); pulmonary arterial hypertension crisis (PAH crisis); non-cardiac surgery; perioperative management; case report

Submitted Mar 24, 2026. Accepted for publication May 08, 2026. Published online Jun 26, 2026.

doi: 10.21037/tp-2026-0275

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Key findings

• We present a case of a pediatric patient with Eisenmenger syndrome (ES) who experienced a postoperative pulmonary hypertension crisis (PHC) following emergency non-cardiac surgery.

• Emergency non-cardiac surgery poses an extremely high risk for patients with ES and requires careful perioperative hemodynamic management.

What is known and what is new?

• ES is associated with a markedly increased risk of complications during surgery due to severe pulmonary hypertension and right-to-left shunting.

• This case presents the successful management of a child with ES and offers practical strategies for intraoperative and postoperative care, including the choice of open laparotomy and the treatment of a PHC.

What is the implication, and what should change now?

• For patients with ES, perioperative care pathways should prioritize maintaining pulmonary vascular resistance/systemic vascular resistance balance and require postoperative intensive care unit care.

Introduction

Eisenmenger syndrome (ES) is an end-stage complication of uncorrected congenital heart disease involving systemic-to-pulmonary shunting (1). It is characterized by irreversible pulmonary arterial hypertension (PAH), elevated pulmonary vascular resistance (PVR), right-to-left shunting, and chronic hypoxemia (1,2). ES is s associated with significant morbidity and mortality (3).

Pediatric patients with ES undergoing non-cardiac surgery pose exceptional anesthetic and perioperative challenges. Any factors that increase PVR or decrease systemic vascular resistance (SVR) including hypoxia, hypercapnia, acidosis, pain, stress, or anesthetic-induced vasodilation may precipitate acute right ventricular failure, pulmonary hypertension crisis (PHC), malignant arrhythmias, and sudden cardiac death (3,4). Emergency surgical interventions further compound these risks due to limited time for optimization and the inability to defer surgery (4,5).

There is a lack of reports addressing perioperative management strategies for emergency non-cardiac surgery in pediatric patients with ES. Here, we present a case study of a pediatric patient with ES who underwent emergency surgery for ovarian torsion. The surgery was complicated by a postoperative PHC. We also discuss the perioperative anesthetic considerations and crisis management strategies. We present this article in accordance with the CARE reporting checklist (available at https://tp.amegroups.com/article/view/10.21037/tp-2026-0275/rc).

Case presentation

An 11-year-old girl (height: 150 cm; weight: 35 kg) presented with a two-day history of persistent lower abdominal pain, accompanied by vomiting and a fever reaching a maximum temperature of 38.5 ℃. An emergency abdominal ultrasound revealed a cystic mass in the right pelvis, consistent with an ovarian cyst suspected to be twisted, with minimal ascites. She was admitted for emergency surgery.

The patient had a history of known congenital heart disease, characterized by a secundum atrial septal defect and a ventricular septal defect. A cardiac catheterization performed five years earlier revealed right atrial and right ventricular enlargement, a secundum atrial septal defect measuring 14 mm with right-to-left shunting, and severe PAH. These findings led to a diagnosis of ES, which is characterized by markedly elevated PVR. The patient was treated with long-term medical therapy including bosentan, tadalafil, furosemide, and potassium chloride.

Upon admission, her vital signs were as follows: temperature, 36.4 ℃; blood pressure (BP), 96/62 mmHg; heart rate (HR), 124 breaths per minute (bpm); respiratory rate (RR), 24 bpm; and peripheral oxygen saturation (SpO2), 73%. Physical examination revealed central cyanosis of the lips, a grade 3/6 systolic murmur along the left sternal border, and lower abdominal tenderness without guarding. Laboratory evaluation showed elevated C-reactive protein (25.38 mg/L), and venous blood gas analysis demonstrated hypoxemia (partial pressure of oxygen: 48.4 mmHg) with mild metabolic compensation (Table 1).

Table 1

Results of point-of-care blood analysis

Parameters T1 (venous blood) T2 (arterial blood) T3 (arterial blood) T4 (arterial blood)
pH 7.417 7.464 7.357 7.286
PaCO2 (mmHg) 31.3 27 33.4 41.8
PaO2 (mmHg) 48.4 45.6 70.4 54.3
FiO2 (%) 21 21 50 45
Hb (g/L) 158 148 132 165
K+ (mmol/L) 4.7 4.1 4.0 4.2
Na+ (mmol/L) 137 138 138 137
Cl− (mmol/L) 112 114 115 116
Ca2+ 1.15 1.16 1.16 1.19
Glu (mmol/L) 5.6 5.7 5.2 9.8
Lac (mmol/L) 1.3 0.8 0.7 1.1
Hct (%) 48.2 45.5 40.6 50.4
HCO3− (mmol/L) 19.7 19.1 18.3 18.4
BE (mmol/L) −3.2 −2.8 −5.9 −8.0

T1, after admission; T2, before anesthesia; T3, before abdominal closure; T4, before extubation. BE, base excess; Ca2+, calcium; Cl−, chloride; FiO2, fraction of inspired oxygen; Glu, glucose; Hb, hemoglobin; HCO3−, bicarbonate; Hct, hematocrit; K+, potassium; Lac, lactate; Na+, sodium; PaCO2, partial pressure of carbon dioxide; PaO2, partial pressure of oxygen; pH, potential of hydrogen.

Transthoracic echocardiography revealed the following: severe right ventricular enlargement (78 mm × 50 mm); right atrial dilation; interventricular septum flattening; a D-shaped left ventricle (Figure 1); a secundum atrial septal defect (12.1 mm) with bidirectional shunting; a small muscular ventricular septal defect (2.5 mm); severe PAH (pulmonary artery pressure: 105/49 mmHg); severe tricuspid regurgitation; moderate pulmonary regurgitation; and preserved left ventricular systolic function. Electrocardiography showed tall, peaked P waves, right ventricular hypertrophy, and conduction abnormalities.

Figure 1 Preoperative transthoracic echocardiography of the patient. The arrow indicates marked right ventricular enlargement with leftward displacement of the interventricular septum, resulting in a “D-shaped” left ventricle.

A multidisciplinary consultation involving pediatric cardiology, cardiac surgery, anesthesiology, and pediatric surgery determined that the patient was at an extremely high risk during the operation, with the potential for hypoxemia, a PHC, malignant arrhythmias, and cardiac arrest. Due to the patient’s severe cardiopulmonary status and the concern that carbon dioxide pneumoperitoneum could exacerbate hypercapnia and increase PVR, an open laparotomy was chosen over laparoscopy. Postoperative intensive care unit (ICU) monitoring was planned.

Upon arriving in the operating room, the patient’s vital signs were as follows: BP, 106/60 mmHg; HR, 80 bpm; RR, 16 bpm; and SpO2, 73%. Administering oxygen via a face mask increased the SpO2 to 85%. Invasive BP monitoring was established. Anesthesia was induced with the slow administration of midazolam (2 mg), etomidate (8 mg), sufentanil (20 µg), vecuronium (3 mg), and dexamethasone (4 mg) intravenously. Tracheal intubation was performed uneventfully under video laryngoscopy using a 6.0-mm cuffed endotracheal tube.

Bilateral transversus abdominis plane blocks were performed for analgesia. A 5 Fr, double lumen right internal jugular central venous catheter was placed, and the depth of anesthesia was monitored using a bispectral index (BIS). Hemodynamic parameters were continuously assessed using Pro-AQT monitoring. Anesthesia was maintained with propofol (50–100 µg·kg−1·min−1), remifentanil (0.2–0.5 µg·kg−1·min−1), and sevoflurane (1%). Mechanical ventilation was provided with an FiO2 of 40%. During the operation, SpO2 was kept between 83% and 92%, HR between 70 and 95 bpm, arterial BP between 90 and 110 mmHg/50 and 65 mmHg, cardiac index between 2.5 and 3.5 L·min−1·m−2, and SVR index between 23 and 30 Wood units. The operation lasted 52 minutes without any intraoperative complications. The patient was then transferred to the ICU.

Three hours after the surgery, the patient was fully awake, hemodynamically stable, and extubated. Chronic PAH medications were resumed. Approximately 9 hours later, she developed ventricular tachycardia (HR: 162 bpm) accompanied by hypotension (80/48 mmHg) and hypoxemia (SpO2: 70%), consistent with a PHC. Arterial blood gas analysis revealed worsening hypoxemia (PaO2: 35.7 mmHg) and metabolic acidosis. Immediate treatment was initiated with an intravenous infusion of amiodarone (loading dose of 5 mg/kg, followed by a continuous infusion of 5 µg·kg−1·min−1), treprostinil (10 ng·kg−1·min−1), sodium bicarbonate, fluid resuscitation, and supplemental oxygen. Subsequently, her BP increased to 90/67 mmHg, and her SpO2 improved to 75%. Thirty minutes later, sinus rhythm was restored (110–115 bpm), with BP of 82/58 mmHg and SpO2 of 75%. Her chest tightness also markedly improved. The amiodarone infusion (5 µg·kg−1·min−1) was maintained for three days, then transitioned to oral therapy (0.05 g t.i.d.) and discontinued on postoperative day 5. The treprostinil infusion (10 ng·kg−1·min−1) was maintained for three days, then reduced to 5 ng·kg−1·min−1 and discontinued on postoperative day eight. SpO2 remained at approximately 80% during this period. The patient was discharged on postoperative day 11 with an SpO2 of 84–87% on room air and remained clinically stable at 1-year follow-up, although severe PAH persisted.

All procedures performed in this study were in accordance with the ethical standards of the institutional and/or national research committee(s) and with the Declaration of Helsinki and its subsequent amendments. Written informed consent was obtained from patient’s parents for the publication of this case report and accompanying images. A copy of the written consent is available for review by the editorial office of this journal.

Discussion

This report outlines the perioperative management of an 11-year-old girl with ES resulting from a large atrial septal defect and a small ventricular septal defect. She underwent an emergency laparotomy for ovarian torsion, which was successfully performed. The case highlights key anesthetic and surgical considerations involved in managing patients with severe PAH and right-to-left shunting. It also documents the successful treatment of a postoperative PHC.

ES is characterized by a persistently high level of PVR and severely limited cardiopulmonary reserve (1,2). Pediatric patients with ES who undergo emergency non-cardiac surgery face an exceptionally high perioperative risk, particularly of PHC, malignant arrhythmias, and circulatory collapse (3,4). This case study illustrates the challenges of managing these patients perioperatively and highlights key principles for mitigating risk. The fundamental perioperative goal of management in ES is to maintain a delicate balance between PVR and SVR (5,6). Any increase in PVR or decrease in SVR can exacerbate right-to-left shunting, resulting in acute hypoxemia and hemodynamic deterioration. The reported perioperative mortality rate in patients with ES ranges from 3.8% to 7% (3,7). Hypoxia, hypercapnia, acidosis, pain, stress, anesthetic-induced vasodilation are well-recognized triggers of pulmonary hypertensive crises and must be avoided (8,9).

For ES patients, the induction of general anesthesia is one of the most vulnerable periods (10). Inadequate anesthetic depth, airway manipulation, or improper ventilation can trigger sympathetic activation and sudden increases in PVR. Therefore, induction strategies should prioritize hemodynamic stability, avoiding hypoxia and hypercapnia, minimizing sympathetic stimulation, and carefully selecting anesthetic agents that preserve SVR (3,11). In this case, etomidate-based induction combined with opioid supplementation enabled a smooth transition to anesthesia without significant hemodynamic disturbance (12).

Currently, there is no consensus on the optimal anesthetic technique for patients with ES. While both neuraxial and general anesthesia have been used successfully, general anesthesia with tracheal intubation provides reliable control of oxygenation and ventilation, as well as the ability to deliver inhaled pulmonary vasodilators (3,10,13). However, reductions in SVR resistance induced by anesthesia and airway manipulation during intubation or extubation may exacerbate right-to-left shunting and hemodynamic instability (6,9). The use of neuraxial anesthesia in pediatric patients is limited due to poor cooperation and the restricted application of advanced monitoring techniques. Extracorporeal membrane oxygenation (ECMO) is reserved as a rescue therapy for refractory hypoxemia and pulmonary hypertensive crisis (14). During a pulmonary hypertensive crisis, the prompt administration of pulmonary vasodilators to reduce PVR and vasopressors to maintain SVR is essential (6). However, the measures cannot reverse established pulmonary vascular remodeling.

In the present case, emergency surgery intervention was unavoidable, making the choice of surgical approach critical. Carbon dioxide pneumoperitoneum during laparoscopic surgery may aggravate hypercapnia and increase PVR, thereby worsening right ventricular afterload (3,15). Due to the patient’s severe PAH and Eisenmenger physiology, open surgery was deemed safer than laparoscopy (16). Comprehensive preoperative assessment defined cardiac anatomy, shunt direction, pulmonary arterial pressure, and ventricular function. Intraoperatively, advanced hemodynamic monitoring, including invasive arterial pressure and cardiac output monitoring, together with transesophageal echocardiography and BIS monitoring, provided a continuous assessment of circulatory status and guided anesthetic titration (3,10,17). Throughout the procedure, HR and BP remained close to preoperative baseline levels, SpO2 ranged from 83% to 92%, and BIS values were maintained between 45 and 55, a finding that is consistent with the recommended management strategies (15,18).

Importantly, this case highlights that perioperative risk extends beyond the operating room. The patient experienced a PHC after surgery, which was characterized by ventricular tachycardia, hypotension, and hypoxemia. Postoperative factors, such as residual anesthetic effects, pain, stress, changes in oxygenation and ventilation, and fluctuations in SVR, can cause abrupt increases in PVR (3,15). This makes the postoperative period particularly vulnerable. The management of a PHC requires the rapid recognition of the crisis and immediate intervention (19,20). This intervention includes correcting the precipitating factors, optimizing oxygenation and ventilation, maintaining SVR, and initiating selective pulmonary vasodilator therapy early on, with inotropic support when indicated (18,21). In the present case, early and aggressive intervention successfully reversed the crisis and prevented further clinical deterioration. This case underscores the importance of mandatory postoperative intensive care monitoring and preparedness for pulmonary hypertension crises in pediatric patients with ES.

Conclusions

In summary, emergency non-cardiac surgery in pediatric patients with ES is still an extremely challenging procedure. Successful outcomes depend on a thorough preoperative assessment, personalized anesthetic planning, multidisciplinary collaboration, vigilant perioperative monitoring, and the ability to prompt the management of pulmonary hypertensive crisis. Further case experience is needed to refine perioperative strategies and improve outcomes in this vulnerable population.

Acknowledgments

None.

Footnote

Reporting Checklist: The authors have completed the CARE reporting checklist. Available at https://tp.amegroups.com/article/view/10.21037/tp-2026-0275/rc

Peer Review File: Available at https://tp.amegroups.com/article/view/10.21037/tp-2026-0275/prf

Funding: This work was supported by National Natural Science Foundation of China (No. 82372159).

Conflicts of Interest: All authors have completed the ICMJE uniform disclosure form (available at https://tp.amegroups.com/article/view/10.21037/tp-2026-0275/coif). The authors have no conflicts of interest to declare.

Ethical Statement: The authors are accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved. All procedures performed in this study were in accordance with the ethical standards of the institutional and/or national research committee(s) and with the Declaration of Helsinki and its subsequent amendments. Written informed consent was obtained from patient’s parents for the publication of this case report and accompanying images. A copy of the written consent is available for review by the editorial office of this journal.

Open Access Statement: This is an Open Access article distributed in accordance with the Creative Commons Attribution-NonCommercial-NoDerivs 4.0 International License (CC BY-NC-ND 4.0), which permits the non-commercial replication and distribution of the article with the strict proviso that no changes or edits are made and the original work is properly cited (including links to both the formal publication through the relevant DOI and the license). See: https://creativecommons.org/licenses/by-nc-nd/4.0/.

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Cite this article as: Jiang C, Zhao J, Zhao J, Huang W, Li C, Jin Y. Perioperative management of emergency non-cardiac surgery in a pediatric patient with Eisenmenger syndrome: a case report. Transl Pediatr 2026;15(6):254. doi: 10.21037/tp-2026-0275

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