Successful extracorporeal membrane oxygenation for cardiac arrest and cardiogenic shock due to lupus myocarditis: a case report

Introduction

Systemic lupus erythematosus (SLE) is a complex autoimmune disease characterized by multiorgan involvement and various clinical manifestations (1). SLE occurs primarily in women of childbearing age and presents with a variety of clinical manifestations, including cutaneous, renal, hematologic, and cardiovascular symptoms (1). Pericarditis is the most common cardiovascular complication in SLE (2). Lupus myocarditis is relatively rare but potentially life-threatening complication that can lead to fatal arrhythmias and cardiogenic shock that is reported in 3–9% of patients with SLE (3). There are very few reports of venous-arterial extracorporeal membrane oxygenation (VA-ECMO) being introduced in cases of cardiogenic shock or fatal arrhythmia caused by lupus myocarditis (3-8).

We report a case of cardiac arrest due to SLE-induced myocarditis, which was successfully resuscitated; however, prolonged cardiogenic shock resulted in the introduction of VA-ECMO. We present this article in accordance with the CARE reporting checklist (available at https://acr.amegroups.com/article/view/10.21037/acr-2025-83/rc).

Case presentation

A 47-year-old female presented with abdominal pain and was referred to our emergency department. Her medical history included SLE managed with prednisolone (7.5 mg). In the ambulance, she experienced cardiac arrest (asystole) but was successfully resuscitated with 1 min of chest compressions. On arrival at the hospital, her Glasgow Coma Scale score was E1V1M5 with her pupils dilated to 5 mm bilaterally. Her vital signs were as follows: blood pressure, 188/122 mmHg; heart rate, 140 beats per minute; blood oxygen saturation, 92% (O₂: 15 L/min); and body temperature, 36.1 ℃. An initial blood gas analysis showed severe acidosis (pH: 6.52), hypercapnia (CO₂: 116.6 mmHg), hypoxemia (O₂: 27.9 mmHg), with base excess, −31.6; bicarbonate, 9.3 mmol/L; and lactate, 165.4 mg/dL. The cause of initial cardiac arrest and elevated lactate levels were thought to be severely reduced cardiac output, we could not identify the cause of marked hypercapnia.

After intubation and initial management, due to protracted metabolic acidosis, the patient experienced pulseless electrical activity 12 min after hospital arrival and was resuscitated within 4 minutes with adrenaline. As the cause of the cardiac arrest was unclear cardiogenic shock, we performed coronary angiography (CAG) with no obvious stenosis. After CAG, the patient exhibited persistent cardiogenic shock, with a severely reduced left ventricular ejection fraction (LVEF) of 15%. Although blood pressure was maintained without vasopressors, the prolonged severe metabolic acidosis with pH: 7.03 and markedly elevated lactate levels of 193 mg/dL; thus, we judged the patient was hemodynamically unstable, and VA-ECMO was introduced for cardiogenic shock 76 min after arrival. Following a CT scan, which revealed only minor aspiration pneumonia, the patient was admitted to the intensive care unit (ICU).

After admission to the ICU, steroid therapy (prednisolone 40 mg + hydrocortisone 100 mg) was promptly started base on the patient’s known diagnosis of SLE, chronic steroid use, and the absence of other identifiable causes on initial investigations, which made lupus myocarditis the most likely diagnosis. The cardiac function gradually improved, and echocardiography showed an LVEF of 20% on day 3 and 40% on day 6. Although this recovery may have been partly due to post-resuscitation myocardial stunning and correction of acidosis, it is more likely that lupus myocarditis responded to steroid therapy, given the clinical context and exclusion of infection or coronary artery disease. After performing a myocardial biopsy on day 6, the patient was successfully weaned off VA-ECMO. Endomyocardial biopsy (EMB) revealed moderate endocardial thickening and interstitial fibrosis with slight infiltration of CD3-positive T-lymphocytes and CD68-positive macrophages in the interstitial tissues. No evidence of microthrombi or diffuse human leukocyte antigen-DR isotype positivity in the vascular endothelial cells was observed. (Figure 1).

Figure 1 Myocardial biopsy specimens indicating myocyte damage and inflammatory cell infiltration. (A) HE staining at ×400 magnification shows mononuclear cell infiltration into the stroma, possibly lymphocytes. (B) Immunohistochemical staining at ×400 magnification shows a slight infiltration of CD3-positive T lymphocytes in the interstitial tissues. (C) Immunohistochemical staining at ×200 magnification shows CD68-positive macrophages infiltrating the interstitial tissues. (D) Tenascin C (TC/4F10) staining at ×100 magnification is partially positive in the endocardium and interstitial tissues, indicating tissue remodeling. HE, hematoxylin and eosin.

She was extubated on day 8 and discharged from the ICU on day 13 with an LVEF of 53%. On day 48, the LVEF improved to 58%, and the patient was transferred for rehabilitation with prednisolone (10 mg). The trends in LVEF, steroid administration, and time flow after admission are shown in a time-course chart (Figure 2). A good neurological outcome was achieved with a cerebral performance category score of 1.

Figure 2 The trend in the LVEF and the steroid dose (equivalent to prednisolone). LVEF, left ventricular ejection fraction.

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 Helsinki Declaration and its subsequent amendments. Written informed consent was obtained from the patient 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

SLE is a systemic autoimmune disease that predominantly affects women of childbearing age. It is characterized by multi-organ involvement, including the skin, kidneys, hematologic systems, and cardiovascular system (1). SLE can present with multiple cardiovascular complications, with pericarditis being the most common. Lupus myocarditis, which occurs in approximately 9% of patients with SLE, remains rare but life-threatening (3). The condition often progresses to arrhythmia, heart failure, and cardiogenic shock, requiring urgent intervention. Notably, myocarditis was the first manifestation of SLE in 59% of the cases in a multicenter study, indicating the importance of early suspicion and a prompt diagnosis in patients with SLE presenting with cardiac symptoms (8).

Thomas et al. reported that of 29 cases of lupus myocarditis, 81% achieved full cardiac recovery with an LVEF ≥55% during a median follow-up period of 37 months (8). To the best of our knowledge, there have only been 5 case reports and one case series of VA-ECMO for acute myocarditis due to SLE (3-8). Table 1 summarizes the clinical course of the 9 cases, including our case. One patient underwent a heart transplant and one died, but in the remaining 7 patients, the cardiac function recovered and they were successfully weaned off ECMO within a relatively short period of time. Therefore, although lupus myocarditis often takes a serious course in the acute phase, most survivors experience a good long-term prognosis with appropriate treatment.

Although echocardiography and cardiac magnetic resonance imaging (MRI) are often used in the diagnostic process, an EMB remains the gold standard for a definitive diagnosis (9). However, EMBs are rarely performed because of their invasive nature and limited diagnostic yield, as most samples are taken from the right ventricle, whereas lupus myocarditis predominantly affects the left ventricle, which reduces sensitivity (10). In a series of cardiac biopsies of patients suspected of having SLE myocarditis, nonspecific interstitial fibrosis and myocyte hypertrophy were the most common findings. Evidence of inflammatory myocardial infiltration was found in only 27% of cases, and these were considered non-specific for myocarditis. Similarly, in our case, biopsy findings were non-specific; however, based on the clinical course, a diagnosis of lupus myocarditis was made. These findings suggest that myocardial biopsy has limited value in the diagnosis of SLE myocarditis and that it is more useful for ruling out other diagnoses.

To address these limitations, non-invasive imaging modalities such as cardiac MRI and fluorodeoxyglucose positron emission tomography (FDG-PET) have emerged as valuable diagnostic tools. Cardiac MRI provides detailed structural and functional insights, including the presence of myocardial edema, inflammation, and fibrosis, through techniques such as T2-weighted imaging and late gadolinium enhancement. These features are highly sensitive for myocarditis and can help confirm the diagnosis in cases where EMB results are inconclusive (11). Furthermore, MRI allows for repeated evaluations to monitor treatment response without procedural risks, making it an essential tool in managing lupus myocarditis. FDG-PET, on the other hand, detects active myocardial inflammation by identifying areas of increased glucose uptake by inflammatory cells. This imaging modality is particularly useful for differentiating lupus myocarditis from other causes of cardiogenic shock, such as ischemic heart disease, and for assessing the extent of inflammation in systemic autoimmune conditions (10). By integrating findings from MRI and FDG-PET with clinical and laboratory data, clinicians can achieve a more comprehensive and accurate diagnosis while avoiding the risks associated with invasive procedures.

This case represents a valuable example of achieving full cardiac recovery in a patient with severe myocarditis due to SLE that progressed to acute cardiogenic shock and cardiac arrest. Prompt initiation of VA-ECMO and steroid therapy played a critical role in the successful outcome. It highlights the importance of early intervention and a multidisciplinary approach in managing severe cardiac complications associated with SLE. This case contributes to the limited pool of reported instances of lupus myocarditis treated with VA-ECMO. Sharing such detailed clinical experiences is vital for expanding the collective understanding of rare but severe manifestations of SLE and refining future treatment guidelines.

Conclusions

This was a rare case of myocarditis due to SLE that progressed to cardiogenic shock and cardiac arrest, requiring VA-ECMO support. Although the patient was initially unstable, with timely steroid treatment and VA-ECMO support, her cardiac function completely recovered, and she had a good neurological outcome. This case emphasizes the importance of prompt intervention for severe cardiac symptoms in SLE patients.

Acknowledgments

None.

Footnote

Reporting Checklist: The authors have completed the CARE reporting checklist. Available at https://acr.amegroups.com/article/view/10.21037/acr-2025-83/rc

Peer Review File: Available at https://acr.amegroups.com/article/view/10.21037/acr-2025-83/prf

Funding: None.

Conflicts of Interest: All authors have completed the ICMJE uniform disclosure form (available at https://acr.amegroups.com/article/view/10.21037/acr-2025-83/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 Helsinki Declaration and its subsequent amendments. Written informed consent was obtained from the patient 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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