Can Takayasu arteritis cause a low left ventricular ejection fraction (LVEF)?

Can Takayasu Arteritis Cause Low LVEF?

Yes, Takayasu arteritis can cause reduced left ventricular ejection fraction (LVEF) through multiple mechanisms, though myocarditis as a direct cause is rare (occurring in only 2.8% of cases), while secondary causes such as uncontrolled hypertension, aortic regurgitation, myocardial ischemia, and pulmonary hypertension are more common. 1, 2

Mechanisms of LV Dysfunction in Takayasu Arteritis

Direct Myocardial Involvement (Rare)

• Myocarditis occurs in approximately 2.8% of Takayasu arteritis patients and represents a life-threatening presentation that can cause significant reduction in LVEF 1
• When myocarditis is present, aggressive immunosuppression with high-dose prednisone (40-60 mg daily) plus cyclophosphamide leads to significant improvement in LVEF and cardiac symptoms 1
• Cardiac magnetic resonance imaging (CMR) with late gadolinium enhancement (LGE) is the preferred diagnostic modality for detecting myocarditis and monitoring treatment response 1

Secondary Cardiac Complications (More Common)

• Cardiac failure in Takayasu arteritis is most commonly secondary to uncontrolled arterial hypertension or myocardial ischemia, not direct myocardial inflammation 1
• Aortic regurgitation develops in a significant proportion of patients due to ascending aortic dilatation and can lead to progressive LV dysfunction 2, 3
• Hypertensive cardiomyopathy secondary to renal artery stenosis is another mechanism causing reduced LVEF 1
• Pulmonary hypertension is associated with greater reduction in myocardial strain and can contribute to cardiac dysfunction 4

Subclinical LV Dysfunction with Preserved EF

• Even patients with preserved LVEF (≥50%) demonstrate subclinical myocardial dysfunction on advanced imaging, with reduced global longitudinal strain (GLS) and global circumferential strain (GCS) compared to healthy controls 4
• Patients with Takayasu arteritis and preserved LVEF show decreased GLS (-13.35% vs -14.77% in controls, p=0.021) and GCS (-21.46% vs -22.75% in controls, p=0.027) 4
• Risk factors for impaired LV strain include pulmonary hypertension, male gender, longer disease duration, elevated erythrocyte sedimentation rate (ESR), and presence of myocardial LGE on CMR 4

Reversibility with Treatment

• Timely intervention for aortic or renal artery stenosis can improve and even reverse LV dysfunction 5
• In patients with baseline reduced LVEF (<50%), mean EF improved from 24.62% to 45.6% (p=0.001) at 6 months after aortic/renal intervention 5
• In patients with preserved LVEF but reduced GLS, mean GLS improved from -8.80% to -16.3% (p<0.001) at 6 months following intervention 5
• NT-pro BNP levels decreased significantly in both groups, indicating improvement in cardiac function 5

Diagnostic Approach for Cardiac Involvement

Initial Evaluation

• Perform transthoracic echocardiography to assess LVEF, valvular function (particularly aortic regurgitation), and chamber dimensions 1, 3
• Measure four-extremity blood pressures to detect renal artery stenosis causing hypertension 6
• Obtain inflammatory markers (ESR, CRP), though these are elevated in only 50% of active cases 2, 6

Advanced Imaging When Indicated

• Cardiac MRI with LGE is essential when myocarditis is suspected (presenting with heart failure without ischemic coronary disease or hypertension) 1
• CMR feature tracking can detect subclinical LV dysfunction in patients with preserved LVEF 4
• Serial CMR can monitor treatment response in myocarditis cases 1

Management Strategy Based on Mechanism

For Myocarditis

• Initiate aggressive immunosuppression immediately with high-dose prednisone (40-60 mg daily) plus cyclophosphamide 1
• Consider tocilizumab if cyclophosphamide is contraindicated 1
• Monitor response with serial echocardiography and CMR 1

For Secondary Causes

• Control systemic hypertension aggressively, particularly when secondary to renal artery stenosis 2, 3
• Consider revascularization (surgical or endovascular) for critical renal artery stenosis causing refractory hypertension or worsening renal function, but only after achieving disease quiescence 6
• Beta-blockers may be useful in patients with severe aortic regurgitation to slow progression of LV dysfunction 3
• Delay elective revascularization until disease is quiescent, as outcomes are significantly worse during active inflammation 6, 7

Critical Pitfalls to Avoid

• Do not rely solely on inflammatory markers to assess disease activity, as they can be normal in 50% of active cases and do not correlate well with cardiac involvement 2, 6
• Do not assume all LV dysfunction in Takayasu arteritis is due to myocarditis—secondary causes (hypertension, aortic regurgitation, ischemia) are far more common 1
• Do not perform elective cardiac or vascular surgery during active inflammation, as observational studies show worse outcomes including higher restenosis rates 6, 7
• Do not overlook subclinical LV dysfunction in patients with preserved LVEF—consider advanced strain imaging with CMR or speckle tracking echocardiography 4, 5