01 February 2025: Articles
Emergency ECMO Deployment During Liver Transplantation in Portopulmonary Hypertension Patients
Unusual clinical course, Management of emergency care, Clinical situation which can not be reproduced for ethical reasons
Joao Da Costa Rodrigues1ABDEF, Corinne Gazarian1BDE, Julien Maillard


DOI: 10.12659/AJCR.946268
Am J Case Rep 2025; 26:e946268
Abstract
BACKGROUND: Portopulmonary hypertension (POPH) is part of Group 1 of the clinical classification of pulmonary hypertension and represents 5-15% of patients with pulmonary hypertension, with a 5-year mortality rate of 40%. The implementation of preoperative pulmonary antihypertensive treatment allows liver transplantation depending on clinical response, which constitutes potential curative treatment. Uncontrolled pulmonary hypertension is a major risk factor of perioperative morbimortality in the context of liver transplantation. In case of major hemodynamic instability, extracorporeal membrane oxygenation (ECMO) can be placed to manage circulatory failure. We describe a case of a patient with POPH in whom an emergency ECMO was implanted during liver transplantation complicated by an intraoperative worsening of pulmonary vascular resistances leading to cardiac arrest.
CASE REPORT: A 16-year-old patient with POPH had an orthotopic liver transplantation (OLT) after management of pulmonary hypertension with a triple antihypertensive therapy, which was complicated by hemorrhagic shock. Management of hemorrhagic shock led to greatly increased pulmonary vascular resistances, which led to a perioperative cardiac arrest, necessitating the implantation of a veno-arterial ECMO, allowing the completion of critical surgical steps before admission to the intensive care unit.
CONCLUSIONS: POPH is a challenge in the perioperative setting. OLT is a therapeutic option in that setting. ECMO may be necessary for patients with POPH in the perioperative hemodynamic management during OLT. In highly selected cases, VA-ECMO implantation and timing should be discussed by a multidisciplinary team before induction. The emergency perioperative implantation of ECMO is a realistic alternative.
Keywords: Hypertension, Pulmonary, Liver Transplantation, Extracorporeal Membrane Oxygenation, case reports, Perioperative Care
Introduction
Portopulmonary hypertension (POPH) is part of Group 1 of the clinical classification of pulmonary hypertension (PH) and is defined by pulmonary arterial hypertension (PAH) as a complication of portal hypertension with or without liver disease. Diagnosis is based on hemodynamic parameters obtained through right-side heart catheterization, and the combination of 2 criteria is required: a) portal hypertension (portosystemic gradient >5 mmHg) and b) pulmonary hypertension with a mean pulmonary arterial pressure (mPAP) >20 mmHg, pulmonary vascular resistance (PVR) >2 WU, and pulmonary capillary wedge pressure (PCWP) 15 mmHg [1].
POPH represents 5–15% of PAH, with a 5-year mortality rate of 40%. Treatment consists of medical therapies and orthotopic liver transplantation (OLT) in some patients [2]. However, POPH increases perioperative mortality for OLT and is associated with worse outcomes [3] three patients (50%.
Here, we present a case of a patient with treated POPH undergoing OLT, requiring urgent intraoperative implantation of veno-arterial extracorporeal membrane oxygenation (VAECMO) for cardiac arrest.
Case Report
We present a case of a female patient aged 16 years 5 month, known to have POPH due to congenital mesenteric-portocaval shunt and absence of intrahepatic portal vein, who was initially diagnosed in the context of recurrent shortness of breath. Preliminary imaging assessment of liver structure highlighted 5 benign proliferative hepatic nodules and congenital porto-systemic shunt (mesenteric vein to vena cava). One year after the initial diagnosis, the patient benefited from a portocaval shunt closure by invasive intravascular radiologic approach. The severe pulmonary arterial hypertension (mPAP 55mmHg, PVR 15.4 WU) was managed by a sequential triple combination therapy of orally-administered endothelin receptor antagonist (Macitentan), phosphodiesterase 5 inhibitors (Tadalafil), and prostacyclin analogs (Epoprostenol) administered through a permanently implanted central venous line catheter. This treatment allowed satisfactory clinical and hemodynamic evolution with control of pulmonary arterial hypertension (mPAP of 36 mmHg and PVR of 4.4 WU). Five years after the initial diagnosis, she was placed on the waiting list for liver transplantation. Three months before OLT, a temporary contraindication to the procedure was established due to worsening of PAH, discovered during a routine follow-up right-heart catheterization, requiring an increase in the dose of prostacyclin. No worsening or new symptoms were reported. A month later, the right-heart catheterization was repeated, with a favorable response to this treatment. Evolution of the preoperative hemodynamic values measured during cardiac catheterizations and echocardiographic parameters related to the POPH treatments are shown in chronological order in Table 1. The trans-pulmonary gradient (mPAP-PCWP) was >12 mmHg, showcasing the lack of contribution from an increased cardiac output in the PAH. Figures 1 and 2 shows the pulsed Doppler waveforms of the tricuspid regurgitation before surgery and before hospital discharge. The last preoperative transthoracic echocardiogram was the one in which she presented a worsening of mPAP.
Three months after being placed on the waiting list, the patient was admitted to the hospital for an OLT the night before. Upon arrival, she was afebrile, with a blood pressure of 105/58 mmHg, a heart rate of 79 bpm, and an oxygen saturation of 98% on room air. Preoperative blood tests showed a hemoglobin level of 123 g/L, a white blood cell count of 3.9 G/L, a platelet count of 90 G/L, a BUN of 3.0 mmol/L, creatinine of 44 µmol/L, sodium at 136 mmol/L, and potassium at 3.8 mmol/L. The MELD score at admission was 7.
General anesthesia was induced following a standard sequence with sufentanil 0.2 mcg/kg, lidocaine 0.6 mg/kg, propofol 3 mg/kg, and atracurium 0.6 mg/kg. Intubation was performed via direct laryngoscopy and a size 6.5 endotracheal tube was placed without complications. Ventilation settings included a positive end-expiratory pressure of 7 cmH2O, a tidal volume of approximately 300 ml (9 ml/kg), and a respiratory rate of 18 breaths per minute to achieve normocapnia in a pressure-regulated volume-guaranteed mode. Dynamic compliance ranged from 45 to 61 ml/cm H2O. Anesthesia was maintained with continuous inhaled sevoflurane and intravenous midazolam, while neuromuscular blockade was sustained with continuous intravenous atracurium. Antibiotic prophylaxis was provided with piperacillin-tazobactam.
The perioperative hemodynamics monitoring included an arterial line, a central venous catheter, and a Swan-Ganz pulmonary artery catheter.
The first arterial blood gas results after induction of general anesthesia were a pH 7.31, PaCO2 6.31 kPa, PaO2 29.6 kPa, lactate 0.2 mmol/l, HCO3 24 mmol/l, and BE −2.4 mmol/l.
The Epoprostenol infusion was maintained through the dedicated permanently implanted venous catheter. During the whole surgical procedure, mPAP values were maintained at 41–54 mmHg; PCWP iterative measurements were 12 mmHg. The patient required noradrenalin support, which was adjusted to target a mean arterial pressure of 65 mmHg. The cold graft ischemia lasted 12 hours 2 minutes due to a long anhepatic phase and the warm ischemia lasted 38 minutes.
Intraoperatively, during the anhepatic phase, the surgical management was complicated by hemorrhagic shock due to bleeding at the hepatic hilum from portal hypertension varices. The situation deteriorated rapidly despite adequate support measures. The pulmonary hemodynamics also deteriorated, with an increase of mPAP to 83 mmHg, with a multifactorial etiology. Cardiac arrest was caused by adrenergic stress induced by the hemorrhagic shock, the subsequent massive fluid and blood transfusion resuscitation, the intravenous adrenaline boluses given for the profound hypotension, a lighter sedation (BIS value at 62), and the acidemic state with a pH of 7.05, requiring mechanical resuscitation by external cardiac massage and defibrillation. Implantation of a rescue veno-arterial (VA) femoro-femoral ECMO was performed at this point. The ECMO implantation was completed during external cardiac compressions, with a time from needle to ECMO of less than 10 minutes. The transesophageal echocardiography after the procedure showed favorable unloading of the right ventricle (RV). The total measurable blood loss was 1.4 liters, but this is an underestimate since an important quantity was lost during the hemorrhagic shock, with a urine output of 0.5 liters after 13 hours 44 minutes of anesthesia. The blood volume losses were compensated for with 23 units of concentrated red cells, 14 units of fresh frozen plasma, and 4 units of platelet concentrate. At the end of surgery, the visceral edema caused by the volume resuscitation did not allow abdominal wall closure, and placement of a vacuum-assisted closure system (VAC) was necessary, as well as catheterization of the bile duct via a ureteral probe. The patient was subsequently transferred to the intensive care unit (ICU) under mechanical ventilation for postoperative management.
At the intensive care unit, the liver graft function was considered to be within normal limits in the early postoperative phase (Factor V at 100%), and ultrasound monitoring demonstrated normal echo structure and normal vascular resistance indices.
ECMO was initially weaned on postoperative day 10, with a subsequent increase in mPAP from 31 to 62 mmHg, an increase of CI from 2.8 to 3.92 l/min/m2, and a decrease of PVR from 7.5 to 6 WU after ECMO removal, partly due to a decrease in the hyperdynamic state and volume depletion. On postoperative day 28, the situation was complicated by a massive pulmonary embolism following thrombosis of the Swan-Ganz catheter, with a right ventricular dilatation, worsening of the mPAP interfering with the left ventricle filling, and affecting the liver graft, with the Factor V level declining to 68%. This situation required new circulatory assistance with a VA ECMO on day 28, which was subsequently weaned on day 56.
The patient remained intubated for 19 days following surgery and subsequently required a percutaneous tracheostomy for 57 days due to swallowing difficulties.
Throughout the entire 93-day ICU stay, the pulmonary antihypertensive treatment was adjusted to align with the patient’s complex clinical condition. This included managing drug interactions with other treatments and transitioning from oral therapies to intravenous preparations as needed, thus requiring maintenance of the Swan-Ganz catheter for a longer period than usually required for an OLT.
During her ICU stay, she had an initial cerebral CT scan and EEG showing lesions compatible with anoxic encephalopathy. The neuron-specific enolase (NSE) dosage was 119.7 (normal value <18.3). The sedation was eventually weaned off and the patient improved progressively, allowing for complete mechanical ventilation weaning and tracheotomy decannulation. After weeks of rehabilitation, she left the ICU with a GCS of 15 and a CPC score (cerebral performance categories) of 1. From a renal standpoint, she required continuous venovenous hemodiafiltration for 17 days to manage the initial postoperative hypervolemia and the subsequent acute renal failure (KDIGO 3). Diuresis and renal function improved during the ICU stay, with eventual complete recovery.
Discussion
This case report details the perioperative use of VA-ECMO as a rescue therapy during OLT in a patient with POPH. Diagnosed in 2–6% of OLT candidates, POPH comprises 5–15% of type 1 PAH, with complex pathophysiology involving pulmonary and portal circulation interactions, vasoconstriction, and exposure to harmful factors due to portosystemic shunts [4] a subset of group 1 pulmonary hypertension (PH.
A MELD score of 15 is required for OLT listing, but POPH patients can receive exception points if mPAP is below 35 mmHg, as higher values are linked to worse outcomes [5]. This case illustrates the challenge of patient selection based on mPAP. Prostacyclin treatment was maintained throughout, as mandated by Krowka et al [6]hepatopulmonary syndrome (HPS, although ESC/ERS guidelines do not explicitly mention POPH as an OLT indication. However, the literature shows better survival and reduced PAH treatment after OLT. ECMO use in OLT for POPH is documented in case reports and series. Instances include perioperative use for severe ARDS, right-heart failure, and to bridge patients to OLT [7–10].
Outcomes vary, highlighting the need for careful consideration of ECMO’s risks and benefits. In this case, VA-ECMO was used to manage a refractory increase in mPAP leading to cardiac arrest complicated by hemorrhagic shock, bridging the patient to OLT and supporting multi-organ recovery. Other authors reported rescue ECMO for a perioperatively diagnosed POPH, where the patient benefited from inhaled nitric oxide (NO) and intravenous Epoprostenol. In a particular case, ECMO was implanted postoperatively for severe ARDS (PaO2/FiO2 40), with a VV configuration for lung support, correction of pulmonary hypoxic vasoconstriction, and optimization of gas exchange [11]it represents a contraindication to the procedure until pulmonary vasodilatative therapy has been optimized. We report the case of a 43-year-old man, scheduled for OLT due to alcoholic cirrhosis with hemosiderosis. His Model for End-Stage Liver Disease was 25 at that time. The preoperative evaluation showed a severe alteration of diffusion (pO2 68 mmHg. Barbas et al reported the case of a patient with a MELD score of 30 and severe POPH (mPAP 60 mmHg, PVR 4.5 WU) in which triple achieved near target values and obtained a mPAP of 38 mmHg and a PVR of 1.8 WU, leading to placement of the patient on the OLT waiting list. Due to 2 admissions to the operating room with postponement of OLT due to crippling mPAP values of 60 mmHg, refractory to medical management after induction of anesthesia, and with an expected resolution of POPH after OLT, a third attempt was made with VA ECMO support, weaned at day 1 after surgery [12]. Braun et al reported on 8 cases of ECMO use during perioperative OLT, including 1 for a massive pulmonary embolism with cardiorespiratory arrest. Three patients required VA-ECMO for right-heart failure. One patient needed ECMO 6 months after OLT due to severe pulmonary hypertension and right-heart failure, but this was unsuccessful, leading to death [13]. Finally, Lee et al documented 2 cases of planned VA-ECMO use during OLT for POPH. In the first case, ECMO was needed due to worsening of respiratory symptoms and RV dysfunction, but the patient died postoperatively from hemodynamic instability and coagulopathy. In the second case, ECMO was used preemptively for high right-heart failure risk, and the patient successfully recovered with good graft function 1 year after surgery. The authors emphasize that VA-ECMO can be a viable strategy but must be used cautiously due to potential complications like vascular cannula interference, major bleeding, and thrombotic issues related to the lack of anticoagulation [13]. The neurological outcome warrants consideration. Despite a significantly elevated NSE, our patient achieved a favorable neurological recovery. It is important to note that NSE is present not only in neurons but also in neuroendocrine cells, red blood cells, and platelets. Any hemolytic processes, such as those that can occur during ECMO, might lead to false-positive NSE results. Research by Okubo et al demonstrated a strong correlation between hemolysis markers (lactate dehydrogenase and free hemoglobin) and NSE levels in patients who regained consciousness after VA-ECMO; however, no such correlation was found in patients who did not regain consciousness [14]. Additionally, some degree of hemolysis occurs in packed red blood cells, and Czimmeck et al reported on blood transfusion as a potential confounding factor for elevated NSE levels, which could also apply to our case [15].
Key management aspects include pre-emptive risk assessment, consideration of the timing of ECMO implantation, duration of support, weaning complexity, and vigilant postoperative care to prevent complications [16] severe pulmonary hypertension is regarded as a contraindication to liver transplantation (LT. In conclusion, while POPH complicates OLT outcomes, VA-ECMO can be a viable option for right-heart support and circulatory stabilization in selected cases. However, clear guidelines are lacking, and patient selection is critical.
Conclusions
This report demonstrates the feasibility of intraoperative ECMO in managing perioperative instability in patients with POPH during OLT. POPH represents a challenge that can worsen the postoperative outcome of an OLT. The benefits of OLT for POPH patients are clearly documented, with progressive improvement in hemodynamics, enabling complete cessation of antihypertensive therapy in some patients. The use of perioperative VA ECMO support, with its ability to unload the right-heart function and to stabilize the circulation, may be an option in highly selected cases, supported by several published case reports. However, defining a clear role for ECMO is not possible based on the literature. Careful patient selection is mandatory in elective implantation. For emergency implantation, during surgery or postoperatively, one should follow the actual recommendations as in any patient presenting with cardiogenic shock or ARDS.
References:
1.. Humbert M, Kovacs G, Hoeper MM, 2022 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension: Eur Heart J, 2022; 43(38); 3618-731
2.. McLin VA, Franchi-Abella S, Brütsch T, Expert management of congenital portosystemic shunts and their complications: JHEP Rep, 2024; 6(1); 100933
3.. Fukazawa K, Pretto EA, Poor outcome following aborted orthotopic liver transplantation due to severe porto pulmonary hypertension: J Hepato-Biliary-Pancreat Sci, 2010; 17(4); 505-8
4.. Jasso-Baltazar EA, Peña-Arellano GA, Aguirre-Valadez J, Portopulmonary hypertension: An updated review: Transplant Direct, 2023; 9(8); e1517
5.. Savale L, Sattler C, Coilly A, Long term outcome in liver transplantation candidates with portopulmonary hypertension: Hepatology, 2017; 65(5); 1683-92
6.. Krowka MJ, Fallon MB, Kawut SM, International Liver Transplant Society practice guidelines: Diagnosis and management of hepatopulmonary syndrome and portopulmonary hypertension: Transplantation, 2016; 100(7); 1440-52
7.. Savale L, Guimas M, Ebstein N, Portopulmonary hypertension in the current era of pulmonary hypertension management: J Hepatol, 2020; 73(1); 130-39
8.. Ashfaq M, Chinnakotla S, Rogers L, The impact of treatment of portopulmonary hypertension on survival following liver transplantation: Am J Transplant, 2007; 7(5); 1258-64
9.. Cartin-Ceba R, Burger C, Swanson K, Clinical outcomes after liver transplantation in patients with portopulmonary hypertension: Transplantation, 2021; 105(10); 2283-90
10.. Salgia RJ, Goodrich NP, Simpson H, Outcomes of liver transplantation for porto-pulmonary hypertension in model for end-stage liver disease era: Dig Dis Sci, 2014; 59(8); 1976-82
11.. Stratta C, Lavezzo B, Ballaris MA, Extracorporeal membrane oxygenation rescue therapy in a case of portopulmonary hypertension during liver transplantation: A case report: Transplant Proc, 2013; 45(7); 2774-75
12.. Barbas AS, Schroder JN, Borle DP, Planned initiation of venoarterial extracorporeal membrane oxygenation prior to liver transplantation in a patient with severe portopulmonary hypertension: Liver Transpl, 2021; 27(5); 760-62
13.. Braun HJ, Pulcrano ME, Weber DJ, The utility of ECMO after liver transplantation: Experience at a high-volume transplant center and review of the literature: Transplantation Aug, 2019; 103(8); 1568-73
14.. Okubo R, Shirasaka T, Ushioda R, Relationships among hemolysis indicators and neuron-specific-enolase in patients undergoing veno-arterial extracorporeal membrane oxygenation: J Artif Organs, 2024 [Online ahead of print]
15.. Czimmeck C, Kenda M, Aalberts N, Confounders for prognostic accuracy of neuron-specific enolase after cardiac arrest: A retrospective cohort study: Resuscitation, 2023; 192; 109964
16.. Lee J, Allen WL, Scott CL, Preemptive venoarterial extracorporeal membrane oxygenation for liver transplantation – judicious candidate selection: J Clin Med, 2023; 12(15); 4965
Figures
In Press
Case report
Am J Case Rep In Press; DOI: 10.12659/AJCR.945539
Case report
Am J Case Rep In Press; DOI: 10.12659/AJCR.945795
Case report
Am J Case Rep In Press; DOI: 10.12659/AJCR.946588
Case report
Am J Case Rep In Press; DOI: 10.12659/AJCR.946011
Most Viewed Current Articles
21 Jun 2024 : Case report
88,675
DOI :10.12659/AJCR.944371
Am J Case Rep 2024; 25:e944371
07 Mar 2024 : Case report
50,044
DOI :10.12659/AJCR.943133
Am J Case Rep 2024; 25:e943133
20 Nov 2023 : Case report
25,247
DOI :10.12659/AJCR.941424
Am J Case Rep 2023; 24:e941424
18 Feb 2024 : Case report
22,651
DOI :10.12659/AJCR.943030
Am J Case Rep 2024; 25:e943030