Acute left heart failure is the initial manifestation of Takayasu arteritis: a case report

Abstract

Background

Takayasu arteritis, a chronic inflammatory disease predominantly affecting the aorta and major arterial branches, can lead to serious complications such as myocardial infarction and heart failure.

Case presentation

This case report details a rare initial presentation of a 24-year-old female with acute left heart failure, leading to a diagnosis of Takayasu arteritis. Initial misdiagnosis as viral myocarditis was reconsidered after comprehensive examinations, including echocardiography, vascular ultrasound, and imaging studies, which revealed the disease’s characteristic vascular involvement. The patient was treated with a combination of prednisone acetate, tocilizumab, and supportive therapies, resulting in symptomatic and laboratory improvement by the three-year follow-up.

Conclusions

The case emphasizes the need for clinicians to recognize atypical presentations of Takayasu arteritis to avoid misdiagnosis and to consider its multifactorial impacts on heart failure.

Supplementary Information

The online version contains supplementary material available at 10.1186/s12872-025-05074-z.

Background

Takayasu arteritis is an insidious inflammatory condition that primarily targets the aorta and its principal branches, with potential extensions to the pulmonary and coronary arteries. It may result in a spectrum of vascular complications, including stenosis, occlusion, dilatation, and aneurysm formation [1]. A subset of patients with this disease can develop aortic valve regurgitation. While Takayasu arteritis can progress to severe outcomes such as myocardial infarction and congestive heart failure, the initial presentation with acute heart failure is relatively rare. This case report focuses on a young female patient who experienced acute left heart failure as her first symptom of Takayasu arteritis, highlighting the importance for clinicians to be vigilant about the atypical presentations of this disease.

Case presentation

A 24-year-old female patient, admitted on September 16, 2021, presented with a history of chest tightness and exertional dyspnea for over a year. Her symptoms began abruptly with severe chest discomfort and difficulty breathing, to the point of being unable to lie flat at night (Fig. 1). Initial assessments at a local hospital revealed elevated cardiac troponin T (TnT) and NT-proBNP levels, along with ECG changes suggestive of ischemia and pleural effusion on chest X-ray. Echocardiography indicated diffuse left ventricular hypokinesis, left ventricular dilation, and severe aortic regurgitation, with a significantly reduced left ventricular ejection fraction (LVEF) of 33.9%. Considering the patient’s young age, elevated cardiac biomarkers, and echocardiographic findings of left ventricular dysfunction, the local hospital initially misdiagnosed “acute viral myocarditis” with a presumed viral etiology. However, definitive confirmation was limited by the absence of preceding viral prodrome and lack of serological or molecular testing for viral pathogens. Despite treatment aimed at managing heart failure, the patient continued to experience symptoms with minimal exertion, which resolved only after extended rest. Throughout her illness, she exhibited increased erythrocyte sedimentation rate and C-reactive protein levels. She was referred to our department for a more definitive diagnosis and treatment plan. Notably, she did not exhibit symptoms such as oral-genital ulcers, erythema nodosum, photosensitivity, joint pain, neck pain, or fever. Since the onset of her symptoms, her mental state and appetite have been satisfactory, with no significant changes in her bowel and bladder habits or weight. She has no history of tuberculosis, and there is no family or genetic predisposition to similar conditions

Fig. 1.

A timeline with the relevant data from the episode of care

Physical examination

Vital signs were within normal limits, with a blood pressure discrepancy between the left (133/57 mmHg) and right upper limbs (140/65 mmHg). Cardiac auscultation revealed an enlarged silhouette and a regular rhythm, with a 4/6 diastolic murmur heard at the aortic valve area and systolic murmurs over the carotid, subclavian, descending aorta, abdominal aorta, and renal arteries. Signs of arterial pulsation were present, including a capillary sign and water-hammer pulse, yet there was no edema in the lower limbs

Auxiliary examinations

Laboratory tests showed elevated NT-proBNP, ESR, CRP, Interleukin-6, and Tumor Necrosis Factor-α levels. Vascular ultrasound was performed because systolic murmurs were detected over the carotid, subclavian, descending aortic, abdominal aortic, and renal arteries during physical examination. Vascular ultrasound and contrast imaging revealed arterial wall thickening and stenosis in multiple vessels, along with neovascularization within the carotid artery walls. Advanced imaging with cardiac magnetic resonance (Fig. 2) and coronary artery CTA (Fig. 3) provided further insights into left ventricular dysfunction and near-total occlusion of the left main coronary artery, respectively. Echocardiography confirmed severe aortic regurgitation.

Fig. 2.

Cardiac magnetic resonance imaging demonstrates diffuse subendocardial delayed enhancement of the left ventricular wall, with thinning and delayed enhancement in the anterior, anteroseptal, and anterolateral left ventricular walls, indicative of myocardial ischemia. A: Two-chamber short-axis late gadolinium enhancement (LGE) sequence showing subendocardial LGE in the anterior left ventricular (LV) wall, suggesting myocardial infarction. B: Two-chamber long-axis LGE sequence demonstrating diffuse subendocardial LGE in the anterior LV wall from the mid-level to the apex

Fig. 3.

Coronary artery CTA reveals near-total occlusion of the left main stem (LM), a critical finding in the context of ischemic heart disease. A: Axial CTA image demonstrating circumferential wall thickening of the aortic root extending to the left main coronary artery (LMCA) ostium, causing ostial stenosis. The left anterior descending artery (LAD) and its branches show normal. B: Curved planar reformation (CPR) image of the LMCA-LAD showing significant luminal stenosis of the LMCA with LAD normal. C: Volume-rendered (VR) coronary image revealing significant luminal stenosis of the LMCA. The right coronary artery (RCA), LAD, and its diagonal branch are well visualized

Diagnosis and differential diagnosis

The patient was diagnosed with Takayasu arteritis, old myocardial infarction, and severe aortic valve insufficiency (NYHA class III). The diagnosis was based on clinical presentation, imaging findings, and fulfillment of the 1990 American College of Rheumatology classification criteria for Takayasu arteritis. Our patient met three out of six diagnostic criteria of Takayasu arteritis (age < 40 years old, Bruit over subclavian arteries or aorta, Arteriographic abnormalities. The differential diagnosis included viral myocarditis and genetic cardiomyopathy, both of which were less likely given the lack of familial cardiomyopathy and no symptoms suggestive of viral infection. Autoimmune myocarditis was excluded based on negative serology (ANA/ANCA) and the absence of clinical manifestations suggestive of autoimmune disease. Takotsubo cardiomyopathy was unlikely given the absence of typical stressors and no transient apical ballooning on imaging. Behçet’s disease was excluded given the lack of diagnostic criteria (oral/genital ulcers), negative pathergy test, and absence of systemic manifestations.

Treatment and prognosis

After consultation with the cardiology and cardiac surgery departments, surgical intervention was recommended. However, the patient declined the surgery due to concerns about the high operative risk. The patient received prednisone 40 mg once daily (tapered to 5 mg once daily) and tocilizumab 560 mg via intravenous infusion every 4 weeks [2]. Due to the COVID-19 pandemic and epidemic containment measures, China maintained strict regional mobility restrictions. Tocilizumab was only available as an intravenous formulation requiring hospitalization; however, cross-regional medical travel was prohibited under epidemic prevention regulations. Subcutaneous administration enabled home-based therapy. So, tocilizumab was switched to adalimumab 40 mg administered subcutaneously every 2 weeks. The other treatment regimen included alongside antiplatelet and coronary dilation was performed to treat the LM stenosis. Diuretic therapy was used to manage cardiac dysfunction. After three years of follow-up, there was a significant improvement in symptoms and laboratory markers. NT-proBNP declined to a normal level (120 pg/mL) in September 2024. Coronary artery CTA conducted at an external facility indicated a marked improvement compared to previous assessments.

Discussion

The patient lacked the classic signs of large-vessel vasculitis, such as neck pain, absent pulses, unequal blood pressure, and limb claudication, and instead experienced acute left heart failure, which was mistakenly identified as “viral myocarditis” at another facility. However, upon meticulous physical examination and a battery of diagnostic tests including coronary CTA, cardiac MRI, echocardiography, and vascular ultrasound, a definitive diagnosis of large-vessel vasculitis was confirmed. In alignment with the 2018 EULAR management guidelines for Takayasu arteritis, the patient exhibited both symptomatic resolution and radiologically confirmed disease regression following combination therapy with glucocorticoids and biologic agents [3]. Beyond valvular affection, ischemic heart disease due to coronary artery stenosis was identified as the primary cause of the patient’s acute left heart failure.

The pathogenesis of acute left heart failure in the context of large-vessel vasculitis is intricate and linked to several factors: (1) Hypertensive heart disease: Large-vessel vasculitis can lead to renal artery stenosis, as well as narrowing of the thoracic or abdominal aorta, resulting in hypertension. Persistent hypertension can cause heart damage, particularly affecting the left ventricle, which may progress to cardiac hypertrophy and heart failure—this is the most frequent cause of heart failure associated with large-vessel vasculitis. (2) Cardiac valvular disease: The inflammation can extend to the cardiac valves, notably the aortic valve, causing regurgitation. Aortic regurgitation increases the workload on the left ventricle, and chronic leakage can result in ventricular dilation and heart failure. (3) Coronary artery involvement: Large-vessel vasculitis can impact the coronary arteries, precipitating myocardial ischemia. Imaging studies indicate that the prevalence of coronary artery involvement may be as high as 50–60% [4]. Myocardial ischemia compromises the heart’s pumping ability, potentially leading to heart failure. Myocardial damage: This form of damage, associated with large-vessel vasculitis, is more prevalent in women and tends to occur at a younger age. The primary symptoms are chest pain and heart failure. Myocardial damage can directly impair the heart’s contractile function, contributing to heart failure. The complex interplay between hypertension, valve disease, and coronary artery involvement in the development of acute left heart failure highlights the multifactorial nature of this condition.

While large-vessel vasculitis (LVV) is a critical etiology, heart failure (HF) in young adults (< 40 years) demands a broader diagnostic framework. Young HF patients (representing ~ 10% of all HF cases) exhibit distinct etiologies compared to older populations, including [5]:

• Genetic cardiomyopathies: Hypertrophic cardiomyopathy, arrhythmogenic right ventricular dysplasia, and sickle cell disease.

• Premature vascular dysfunction: Early-onset atherosclerosis or coronary anomalies. Metabolic disorders: Fabry disease or mitochondrial cardiomyopathies.

• Autoimmune-mediated damage: Systemic lupus erythematosus (SLE) or vasculitis can cause myocarditis, valvulitis, or coronary arteritis.

• Cardiotoxic exposures: (1) Chemotherapy (e.g., anthracyclines), antipsychotics (e.g., clozapine), or immunotherapies. (2) Vaccine-associated myocarditis.

• Post-COVID-19 sequelae: HF was frequently reported as a long-term sequela of COVID-19, appearing in 11.17% of documented cases across 42 studies [6].

For this patient, clinical evaluation revealed no history of SARS-CoV-2 infection, pre-symptomatic COVID-19 vaccination, substance abuse (alcohol/stimulants), or metabolic diseases. Given the absence of autoimmune markers (ANA/ANCA negative), we excluded autoimmune conditions as potential contributors to this patient’s heart failure.

While Takayasu arteritis presenting with acute heart failure has been reported (attributed to hypertension, myocarditis, valvulopathy, or coronary disease) [6–9], this case details Takayasu arteritis initially misdiagnosed as acute myocarditis-related heart failure. Its educational value lies in two key insights: (1) In young women presenting with acute heart failure, meticulous physical examination combined with inflammatory markers and vascular imaging (CTA/MRA) is essential to consider Takayasu arteritis. (2) While hypertension-induced increased afterload is the most common cause of Takayasu arteritis-related heart failure, comprehensive assessment of coronary arteries, valves, and myocardium is imperative to identify the etiology. In this patient, coronary stenosis—causing ischemic cardiomyopathy—constituted the primary cause of acute heart failure, alongside significant valvular disease. Clinicians must heighten their vigilance regarding the potential for large-vessel vasculitis to complicate heart failure, and to meticulously differentiate the etiologies of heart failure to prevent misdiagnosis and ensure appropriate management.

Limitations

In this case, details about comprehensive viral serology, endomyocardial biopsy, or cardiac PET/FDG imaging are lacking. The diagnosis is primarily based on clinical and radiological findings. Absence of histological evidence (e.g., from artery biopsy, myocardial biopsy, or vascular specimen) weakens diagnostic certainty. As a single-case report, this study carries intrinsic constraints regarding population-level inferences. Findings regarding diagnostic protocols, therapeutic efficacy, and disease progression should be interpreted as hypothesis-generating rather than definitive clinical guidance.

Authors’ contributions

Study design: Na Gao. Collection and interpretation of data: Taotao Li and Jingxuan Liu. Manuscript writing: Taotao Li and Jingxuan Liu.Imaging data collectionin the text: Nan Zhang.All authors have read and approved the final manuscript.

Funding

The present study was supported by grants from the National Natural Science Foundation of China (No. 82270427), the Beijing Natural Science Foundation of China (No. 7232038, No. 7222043), and Beijing Anzhen Hospital High Level Research Funding (2024AZC3005).

Data availability

The datasets used and/or analysed during the current study are available from the corresponding author on reasonable request.

Declarations

Ethics approval and consent to participate

Not applicable.

Consent for publication

Written informed consent for publication of the clinical details was obtained from the patient. The consent form is available for review by the Editor of this journal.

Competing interests

The authors declare no competing interests.