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Prosthetic infective endocarditis with aortic root abscess in a patient with systemic lupus
erythematosus: a case report
A 35-year-old woman with poorly-controlled SLE for 10 years had an extensive medical history of
Raynaud’s phenomenon, livedo reticularis, sensorimotor polyneuropathy, lupus alveolitis, lupus
nephritis-associated nephrotic syndrome and chronic kidney disease. She was diagnosed with severe
aortic regurgitation and severe pulmonary hypertension by transthoracic echocardiogram (TTE) two
months prior to this admission. Afterwards, she presented to our hospital due to acute onset of hemoptysis
with tachypnea and desaturation. Physical examination revealed basilar rales with a grade III decrescendo
early diastolic murmur at left upper parasternal intercostals space, jugular vein engorgement and bilateral
pitting edema. Hemogram included a white cell count of 6700/mm3, hemoglobin 9.1 g/dl, platelet count
of 271K/mm3. Blood urea nitrogen was 100 mg/dl; serum creatinine was 4.09 mg/dl, which is similar to
the value in the past three months. Serum albumin was 3.1 g/dl and C-reactive protein (CRP) was 0.15
mg/dl. Chest X-ray disclosed cardiomegaly and bilateral increased interstitial infiltration. Serum
anti-double-stranded DNA was 174.4 WHO units/ml; C3 and C4 were 57.7 mg/dl and 16.3 mg/dl,
respectively. Anti-cardiolipin immunoglobulin (Ig)G, Anti-cardiolipin IgM, anti-beta 2-glycoprotein 1 IgG
and anti-beta 2-glycoprotein 1 IgM were all within normal range.
Under the impression of lupus alveolitis, congestive heart failure secondary to valvular heart disease
and chronic renal failure, she initiated on intravenous methylprednisolone (MTP), diuretics and oxygen
therapy with non-rebreathing mask. Two weeks after treatment, hemoptysis resolved but bilateral pitting
edema, jugular venous engorgement and basilar rales persisted. Therefore, she underwent aortic valve
replacement (AVR) with bioprothetic valve.
Two weeks after AVR, shaking chills with fever up to 38 Celcius degrees and leukocytosis
(24500/mm3) as well as elevated C-reactive protein (13.69 mg/dl) and procalcitonin (3.69 ng/ml) with
bilateral interstitial infiltration on chest X-ray were detected. Both sputum grain stain and urine analysis
yielded normal results. Four blood samples drawn from different sites at different time points all reported
methicillin-resistant CoNS (MR-CoNS). Intravenous daptomycin was administrated and meanwhile TTE
showed normal appearance and function of bioprosthetic aortic valve. Transesophageal echocardiogram
(TEE) was suggested for better inspection but patient refused due to its invasiveness. Follow-up TTE five
days after the fever attack disclosed dehiscence of bioprosthetic aortic valve and severe perivalvular leak
with left ventricular ejection fraction at 52% (Figure 1). Prosthetic valve endocarditis was impressed and
emergent aortic valve replacement was then immediately arranged three weeks after primary AVR.
During the operation, prosthetic valve dehiscence due to annular abscess at left and right coronary cusp
with the abscess extending to left ventricular outflow tract and lower portion of ascending aorta as well as
a perforation hole at the angle of previous aortotomy were noticed. Intra-aortic balloon pump (IABP) was
concomitantly inserted due to expectable poor heart function after the surgery. Tissue samples excised
from bioprosthetic valve and aortic root disclosed inflammatory exudates with neutrophils aggregation.
She required postoperative hemodynamic support with IABP and several vasopressors including
epinephrine, dopamine and milrinone, which gradually discontinued after stabilizing hemodynamic.
Post-operative TTE displayed normal function of the mechanical aortic valves and mild to moderate
pulmonary hypertension. Antibiotic treatment with daptomycin was completed and this patient was
discharged without hemodynamic compromise afterwards.
To our knowledge, the majority of early-onset CoNS PVE present between 2 months to one year after
valve surgery, rather than within the first 2 months. However, the patient in our case developed extensive
CoNS PVE with aortic root abscess merely three weeks after valve surgery, which was an unusual
presentation. Therefore, we should always keep in mind that patients with SLE and long-term use of
corticosteroid after valve surgery have the possibility of developing fulminant and extensive PVE. In
addition, Libman-Sacks endocarditis is an important differential diagnosis when a patient with SLE
presents IE-like symptoms and signs, particularly when verrucous lesions are detected by
echocardiography.
A
B
Fig. 1. (A) A diastolic parasternal long-axis tow-demensional transthoracic echocardiogram showing
dehiscence of the prosthetic aortic valve (arrow) (B) Doppler echocardiography of the same view as (A)
showed severe paravulvular leak (arrow). Note the mosaic jet between the prosthetic aortic valve and
aortic root.