Suspected Non-Infectious Prosthetic Valve Inflammatory Dehiscence In X-Linked Chronic Granulomatous Disease

Dr. Monica Bhagat, MD, Dr. Joshua A. Steinberg, MD, Dr. Frank Silvestry, MD, Dr. Lea Surrey, MD, Dr. Andrea J. Apter, MD, MA, MSc, FAAAAI, Dr. Patricia A. Takach, MD, FAAAAI, Dr. Benjamin P. Soule, MD

Abstract: 

Rationale

Chronic granulomatous disease (CGD) is characterized by severe recurrent bacterial and fungal infections. Hyper-inflammation leading to tissue dehiscence is observed. We present a case report of a patient with CGD diagnosed with a valvular abscess requiring mechanical prosthetic valve replacement, which was complicated by suspected sterile inflammation and recurrent valvular dehiscence.

 

Methods

Tissue culture, staining.

 

Results

This patient presented with Serratia osteomyelitis and pneumonia by 23 months of age and was diagnosed with X-linked CGD. At age 22 he was admitted with shortness of breath and chest imaging showed lung nodules. Biopsy of nodule positive for Philaphora richardsiae. Developed acute congestive heart failiure and echocardiogram showed an aortic perivalvular abscess. Treated with caspofungin and vancomycin. Underwent mechanical aortic valve replacement. Cardiac tissue cultures negative for infection; blood cultures negative. A new murmur was audible and patient diagnosed with aortic root abscess with partial dehiscence of prosthetic aortic valve. Underwent repeat aortic valve replacement and aortic root repair. Developed fevers and a new murmur on antibiotics. Echocardiogram demonstrated valvular and aortic root dehiscence. High-risk salvage surgery was indicated but declined by patient. Intensive consideration of corticosteroid therapy occurred but was declined. The patient passed away from septic shock.

 

Conclusions

We believe this is the first case report suggestive of recurrent endocarditis and peri-prosthetic valvular dehiscence in a XL-CGD patient. We suspect this outcome was due to CGD-related sterile hyper-inflammation, induced by surgical manipulation and/or the presence of prosthetic material. This case highlights the need to consider steroid therapy for such atypical sterile hyper-inflammatory pathology.

abstract No: 

873
    • AAAAI 2014 (70th)