We surveyed the working environment, the degree of satisfaction and the educational methods targeting under forty congenital cardiac surgeons in Japan. We herein report the results of this survey. Summary of this work was presented at the 2018 CHSS Japan Congress, Tokyo, Japan.

No abstract available.
Cost-Benefit Analysis ; Rituximab ; Waldenstrom Macroglobulinemia

No abstract available.
Lymphoma

BACKGROUND: Waldenström Macroglobulinemia (WM) is a rare subtype of indolent B-cell lymphoma, and prospective randomized studies on WM are scarce. The R-CHOP therapy [rituximab (R), cyclophosphamide, hydroxy-doxorubicin, vincristine, and prednisone] is a popular and recommended regimen for primary therapy, prescribed by several treatment guidelines for WM. However, treatment with R-CHOP is accompanied by severe myelosuppression and high rates of peripheral neuropathy. Therefore, we retrospectively evaluated the efficacy and toxicity of half-dose CHOP combined with R as a primary therapy for WM. METHODS: Patients with untreated symptomatic WM, treated at the Disaster Medical Center between April 2011 and September 2016, were retrospectively analyzed after administration of 6 cycles of half-dose R-CHOP for every 3 weeks. The response, median time to response, best response, progression-free survival, overall survival, and toxicities were evaluated. RESULTS: Of the 20 WM patients analyzed, 16 (80%) received half-dose R-CHOP without vincristine, and 13 (65%) responded to the treatment. With a median follow-up duration of 26.3 months, the 2-year progression-free survival and 2-year overall survival rates were 70 and 93.3%, respectively. The median time to response and best response were 6 and 9.9 weeks, respectively. Grade 3/4 leukocytopenia, neutropenia, febrile neutropenia, and Grade 1 peripheral neuropathy developed in 32, 37, 0, and 21% of patients, respectively. CONCLUSION: The half-dose R-CHOP is an effective and well-tolerated primary therapy for WM. To the best of our knowledge, this is the first study reporting the use of a reduced-dose R-CHOP regimen for the primary treatment of WM.
Cyclophosphamide ; Disasters ; Disease-Free Survival ; Febrile Neutropenia ; Follow-Up Studies ; Humans ; Leukopenia ; Lymphoma, B-Cell ; Neutropenia ; Peripheral Nervous System Diseases ; Prospective Studies ; Retrospective Studies ; Rituximab* ; Survival Rate ; Vincristine ; Waldenstrom Macroglobulinemia*

Ambroxol is used in COPD and asthma to increase mucociliary clearance and regulate surfactant levels, perhaps through anti-oxidant and anti-inflammatory activities. To determine the role and effect of ambroxol in an experimental model of asthma, BALB/c mice were sensitized to ovalbumin (OVA) followed by 3 days of challenge. Airway hyperresponsiveness (AHR), lung cell composition and histology, and cytokine and protein carbonyl levels in bronchoalveolar lavage (BAL) fluid were determined. Ambroxol was administered either before the first OVA challenge or was begun after the last allergen challenge. Cytokine production levels from lung mononuclear cells (Lung MNCs) or alveolar macrophages (AM) were also determined. Administration of ambroxol prior to challenge suppressed AHR, airway eosinophilia, goblet cell metaplasia, and reduced inflammation in subepithelial regions. When given after challenge, AHR was suppressed but without effects on eosinophil numbers. Levels of IL-5 and IL-13 in BAL fluid were decreased when the drug was given prior to challenge; when given after challenge, increased levels of IL-10 and IL-12 were detected. Decreased levels of protein carbonyls were detected in BAL fluid following ambroxol treatment after challenge. In vitro, ambroxol increased levels of IL-10, IFN-γ, and IL-12 from Lung MNCs and AM, whereas IL-4, IL-5, and IL-13 production was not altered. Taken together, ambroxol was effective in preventing AHR and airway inflammation through upregulation of Th1 cytokines and protection from oxidative stress in the airways.
Ambroxol* ; Animals ; Asthma ; Bronchoalveolar Lavage ; Cytokines ; Eosinophilia ; Eosinophils ; Goblet Cells ; In Vitro Techniques ; Inflammation* ; Interleukin-10 ; Interleukin-12 ; Interleukin-13 ; Interleukin-4 ; Interleukin-5 ; Lung ; Macrophages, Alveolar ; Metaplasia ; Mice ; Models, Theoretical ; Mucociliary Clearance ; Neutrophils ; Ovalbumin ; Ovum ; Oxidative Stress ; Pulmonary Disease, Chronic Obstructive ; Up-Regulation

PURPOSE: Thoracic dumbbell tumors are relatively rare, usually arising from neurogenic elements. Methods for surgical removal thereof remain controversial. The purpose of this study was to evaluate the surgical results of a single-stage posterior approach with laminectomy and costotransversectomy only for the management of thoracic dumbbell tumors. MATERIALS AND METHODS: Eight cases of thoracic large dumbbell tumor were analyzed retrospectively: seven men and one woman (mean age, 49 years). Pathologic findings included schwannoma in five patients, neurofibroma in two patients (Recklinghausen in one patient), and ganglioneuroma in one patient. All patients underwent single-stage removal of dumbbell tumors by a posterior approach followed by laminectomy and costotransversectomy combined with instrumentation. Clinical and radiologic outcomes were reviewed, thereafter. RESULTS: Operative time ranged from 185 to 420 minutes (mean, 313 minutes), with estimated blood loss ranging from 71 to 1830 mL (mean, 658 mL). Postoperative complications included atelectasis in one case. All patients had tumors successfully removed with no neurological deterioration. Spinal deformities were not observed in any patients at the last follow-up (mean, 52 months), with instrumentation. CONCLUSION: Single-stage surgery with laminectomy and costotransversectomy may be useful for removing thoracic dumbbell tumors without a combined anterior approach.
Adolescent ; Adult ; Aged ; Female ; Humans ; Laminectomy ; Male ; Retrospective Studies ; Thoracic Neoplasms/*surgery ; Thoracic Vertebrae/*surgery

We evaluated the indications and limitations of IABP support for weaning for pump and postoperative heart failure after artificial valve replacement. Driving IABP as cardiac support in cases of acute cardiac failure during and after operation, it is most effective for transient cardiac failure-associated coronary artery spasms during and post operation, but it is not effective and is indeed limited for patients who need long term extracorporeal circulation because of operative technical failure, insufficient cardioplegia and delayed right ventricular failure after operation. The latter groups, in which IABP is insufficiently effective need additional assist devices such as V-A bypass and ventricular assist device (VAD).

Cardiac cachexia is a terminal clinical stage of valvular heart disease, and there is high incidence of postoperative mortality and morbidity. Cardiac cachexia was considered to be present when patients with mitral lesions showed all of the following criteria; 1. mitral valve disease associated with relative tricuspid regurgitation, 2. lean body below 80% of %standard weight, 3. NYHA functional class IV, 4. marked hepatomegaly and congestive liver dysfunction (ICG retention rate over 30%.) Ten patients satisfying the criteria were divided into two groups according to the interval of postoperative respiratory care. Group 1 (n=5), patients necessitating mechanical ventilation for more than 5 days after operation, Group 2 (n=5), patients requiring ventilation up to 5 days after operation. Pre- and postoperative nutrition, respiratory and circulatory states were evaluated for these two groups. In pre- and postoperative periods, intravenous hyperalimentation was administed in two groups, during the postoperative period, two patients of group 1 required tube feeding. In the pre-operative period, three patients in group 1 needed respiratory care (1 intra-tracheal intubation and 2 oxygen mask inhalation). The results were as follows; 1. The duration of illness was longer in group 1 than in group 2. 2. In the postoperative period, there was no difference in the amount of catecholamine, postoperative course and prognosis between groups 1 and 2. Surgery for valvular disease is possible even in cases of cardiac cachexia, if sufficient management of nutritional state, respiration and circulation can be maintained.