Single-Stage Repair of Thoracic Aortic Aneurysm Associated with Aortic Stenosis and Pseudocoarctation by Means of the Clamshell Approach / 日本心臓血管外科学会雑誌

A 64-year old man was admitted to our hospital with a diagnosis of aortic stenosis. Pre-operative chest CT revealed pseudocoarctation of the aorta with a hypoplastic aortic arch, elongation and kinking of the aortic arch and proximal descending aorta. There was also a large aneurysm from the distal arch to descending aorta. We performed a single-stage repair of the aortic lesion from the ascending to the descending aorta with aortic valve replacement. For the surgical approach, transverse clamshell incision was applied safely. Concomitant aortic valve replacement in surgical repair of pseudocoarctation and thoracic aneurysm was rare, and clamshell incision seemed beneficial in such single-stage repair from the aortic root to the descending aorta.

A case report of efficiency of double filtration plasmapheresis in treatment of Goodpasture's syndrome.

Goodpasture's syndrome is characterized by pulmonary hemorrhage, rapid progressive glomerulonephritis and the presence of anti-glomerular basement membrane (anti-GBM) antibodies. Here, we report a case of Goodpasture's syndrome that we treated with double filtration plasmapheresis (DFPP) combined with immunosuppression therapy. The patient was a 32-year-old man with the main complaints of low-grade fever, general fatigue and dyspnea. The clinical diagnosis was renal-pulmonary syndrome based on pulmonary hemorrhage on chest X-ray, rapid progressive renal insufficiency, and elevated C-reactive protein (CRP). Goodpasture's syndrome was diagnosed because the patient was negative for MPO-ANCA and PR3-ANCA, and positive for anti-GBM antibodies. Renal biopsy showed crescentic glomerulonephritis. Hemodialysis, immunosuppression therapy (methylprednisolone and cyclophosphamide) and DFPP were performed. Anti-GBM antibodies were followed pre- and post-DFPP, and removal efficiency, cost performance and complications were evaluated. The antibody levels were 121 and 84 EU/mL before and after the first DFPP procedure, respectively, giving a removal efficiency of 24%. Subsequently, the removal efficiencies were 52%, 55% and 60% after the second, third and fourth DFPP procedures. For comparison, the immunoglobulin G (IgG) removal efficiencies were 53%, 57%, 60% and 55% after the four respective DFPP procedures; therefore, the removal efficiencies were similar for anti-GBM antibodies and IgG in all except the first DFPP procedure. The serum anti-GBM antibody and IgG concentrations decreased from pre- to post-DFPP, indicating that DFPP may be an effective therapeutic approach in Goodpasture's syndrome.