Abstract
BACKGROUND Granulomatosis with polyangiitis (GPA)/Wegener's granulomatosis (WG) and eosinophilic granulomatosis with polyangiitis (EGPA)/Churg-Strauss' syndrome (CSS) are ANCA (antineutrophil cytoplasmic antibodies) associated vasculitides that can affect the heart, predominantly the myocardium. Valvular affection is rare and is described anecdotally. The purpose of this case report was to present aortic valve affection of an ANCA positive vasculitis. CASE REPORT We present the case with a 56-year-old male diagnosed with ANCA associated vasculitis, who began experiencing respiratory symptoms primarily thought to be respiratory tract affection. These symptoms worsened, and an echocardiography revealed heart failure with decreased left ventricular ejections fraction (EF=30-35%) and a severe insufficiency of the aortic valve. The patient underwent aortic valve replacement with symptomatic relief. Pathological examination of aortic valve resectates revealed inflammation and thickening of the aortic cusps. CONCLUSIONS Patients with ANCA associated vasculitis can rarely present with valvular inflammation causing severe regurgitation. The aortic valve can be involved, although cases have also described mitral valve involvement and both valves simultaneously. In patients with ANCA associated vasculitis a severe worsening of dyspnea can be caused by exacerbation of pulmonary involvement, but severe valvular disease should also be considered.
| Original language | English |
|---|---|
| Journal | The American journal of case reports |
| Volume | 20 |
| Pages (from-to) | 423-429 |
| ISSN | 1941-5923 |
| DOIs | |
| Publication status | Published - 1. Apr 2019 |
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Keywords
- Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis
- Aortic Valve Insufficiency
- Heart Failure
Cite this
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Anti-Neutrophil Cytoplasmic Antibody (ANCA) Associated Vasculitis Causes Aortic Valve Degeneration and Severe Aortic Regurgitation. / Guterbaum, Thomas Jeremy; Husic, Mirza; Voss, Anne; Dahl, Jordi Sanchez.
In: The American journal of case reports, Vol. 20, 01.04.2019, p. 423-429.Research output: Contribution to journal › Journal article › Research › peer-review
TY - JOUR
T1 - Anti-Neutrophil Cytoplasmic Antibody (ANCA) Associated Vasculitis Causes Aortic Valve Degeneration and Severe Aortic Regurgitation
AU - Guterbaum, Thomas Jeremy
AU - Husic, Mirza
AU - Voss, Anne
AU - Dahl, Jordi Sanchez
PY - 2019/4/1
Y1 - 2019/4/1
N2 - BACKGROUND Granulomatosis with polyangiitis (GPA)/Wegener's granulomatosis (WG) and eosinophilic granulomatosis with polyangiitis (EGPA)/Churg-Strauss' syndrome (CSS) are ANCA (antineutrophil cytoplasmic antibodies) associated vasculitides that can affect the heart, predominantly the myocardium. Valvular affection is rare and is described anecdotally. The purpose of this case report was to present aortic valve affection of an ANCA positive vasculitis. CASE REPORT We present the case with a 56-year-old male diagnosed with ANCA associated vasculitis, who began experiencing respiratory symptoms primarily thought to be respiratory tract affection. These symptoms worsened, and an echocardiography revealed heart failure with decreased left ventricular ejections fraction (EF=30-35%) and a severe insufficiency of the aortic valve. The patient underwent aortic valve replacement with symptomatic relief. Pathological examination of aortic valve resectates revealed inflammation and thickening of the aortic cusps. CONCLUSIONS Patients with ANCA associated vasculitis can rarely present with valvular inflammation causing severe regurgitation. The aortic valve can be involved, although cases have also described mitral valve involvement and both valves simultaneously. In patients with ANCA associated vasculitis a severe worsening of dyspnea can be caused by exacerbation of pulmonary involvement, but severe valvular disease should also be considered.
AB - BACKGROUND Granulomatosis with polyangiitis (GPA)/Wegener's granulomatosis (WG) and eosinophilic granulomatosis with polyangiitis (EGPA)/Churg-Strauss' syndrome (CSS) are ANCA (antineutrophil cytoplasmic antibodies) associated vasculitides that can affect the heart, predominantly the myocardium. Valvular affection is rare and is described anecdotally. The purpose of this case report was to present aortic valve affection of an ANCA positive vasculitis. CASE REPORT We present the case with a 56-year-old male diagnosed with ANCA associated vasculitis, who began experiencing respiratory symptoms primarily thought to be respiratory tract affection. These symptoms worsened, and an echocardiography revealed heart failure with decreased left ventricular ejections fraction (EF=30-35%) and a severe insufficiency of the aortic valve. The patient underwent aortic valve replacement with symptomatic relief. Pathological examination of aortic valve resectates revealed inflammation and thickening of the aortic cusps. CONCLUSIONS Patients with ANCA associated vasculitis can rarely present with valvular inflammation causing severe regurgitation. The aortic valve can be involved, although cases have also described mitral valve involvement and both valves simultaneously. In patients with ANCA associated vasculitis a severe worsening of dyspnea can be caused by exacerbation of pulmonary involvement, but severe valvular disease should also be considered.
KW - Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis
KW - Aortic Valve Insufficiency
KW - Heart Failure
U2 - 10.12659/AJCR.912693
DO - 10.12659/AJCR.912693
M3 - Journal article
VL - 20
SP - 423
EP - 429
JO - American Journal of Case Reports
JF - American Journal of Case Reports
SN - 1941-5923
ER -