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Objectives: This study aimed to evaluate the surgical outcomes of composite-valve root replacement with bioprosthesis (b-CVRR) after acute type A aortic dissection (AAAD) repair., Methods: We included 41 patients who underwent b-CVRR after surgery for AAAD from 2007 to 2022. We excluded seven patients with VSRR, three with mechanical valve use, one with mycotic aneurysm, and one with cardiopulmonary resuscitation., Results: The overlapping surgical indications for b-CVRR were pseudoaneurysm in 21 patients (51.2%), severe AI in 19 (46.3%), re-dissection in 15 (36.6%), root dilatation (> 50 mm) in 12 (41.5%), and rupture in 3 (7.3%). The coronary artery reconstruction methods were bilateral Carrel patch in 13 patients (32%), Carrel patch with Piehler in 16 patients (38%), bilateral Piehler in six patients (15%), and Piehler with coronary artery bypass (CABG) in six patients (15%). Four patients who underwent the Carrel patch technique required additional intraoperative CABG. Three hospital mortality occurred (7.3%; myonephropathic metabolic syndrome, heart failure, bleeding from the thyroid carotid artery without injury). No thrombosis of the reconstructed site was observed with the Piehler procedure., Conclusion: Surgical outcomes for patients treated with b-CVRR after AAAD repair were acceptable. Intraoperative coronary artery events occurred due to stenosis caused by traction on the reconstructed coronary artery due to adhesions., (© 2024. The Author(s), under exclusive licence to The Japanese Association for Thoracic Surgery.)

Plerixafor, a CXCR4 inhibitor, has the potential to mobilize leukemic cells, which may contribute to their chemosensitization. This phase 1 study evaluated the safety of myeloablative conditioning combined with plerixafor for allogeneic hematopoietic stem cell transplantation (HSCT). Patients with high-risk leukemia undergoing allogeneic HSCT after total body irradiation (TBI, 12 Gy)-based myeloablative conditioning were eligible; 9 patients were enrolled. The study was performed using a 3 + 3 design with an escalating total dose of plerixafor. Plerixafor was given subcutaneously 8 h before TBI and chemotherapeutic agents. Plerixafor was successfully escalated to the maximum dose (0.72 mg/kg) without dose-limiting toxicities. Underlying diseases were acute myelogenous and lymphoblastic leukemia, chronic myeloid leukemia, and myelodysplastic syndrome. As adverse events, plerixafor administration was associated with transient Grades 2-3 diarrhea (n = 7) and abdominal pain (n = 4). In 6 patients, leukemic cell mobilization into the peripheral blood by plerixafor was confirmed by a morphological or molecular method. All patients achieved neutrophil engraftment and 5 were alive in remission at a follow-up after 30-40 months. Plerixafor-combined myeloablative conditioning for allogeneic HSCT was well tolerated. Leukemic-cell mobilization into peripheral blood was observed in half of the patients. Further study is required to evaluate the efficacy and safety of this concept.

Background: We present a surgical technique for a reconstruction of a persistent left superior vena cava draining into the coronary sinus with orthotopic heart transplantation., Methods: Between January 2013 and July 2019, a total of 224 patients underwent orthotopic heart transplantation in our institution. Among them, three patients (3/224, 1.3%) had a persistent left superior vena cava draining into the coronary sinus. The persistent left superior vena cava and the coronary sinus was isolated and fully mobilized as an en bloc conduit at the time of explanting the heart. After the standard anastomoses using the bicaval technique, the conduit was routed anterior to the great vessels and anastomosed to the right atrial appendage. A smooth venous drainage into the right atrium from the rerouted persistent left superior vena cava was intraoperatively confirmed by transesophageal echocardiography., Results: The postoperative course was uneventful in all patients. There were no major postoperative complication and in-hospital mortality. The routed persistent left superior vena cava-coronary sinus conduits were confirmed to be patent without stenosis/obstruction by an enhanced computed tomography. Cardiac biopsies through the reconstructed persistent left superior vena cava were possible without any difficulty., Conclusion: The anterior rerouting technique for a reconstruction of a persistent left superior vena cava draining into the coronary sinus in an orthotopic heart transplantation was feasible and reproducible.

Busulfan (Bu) has been used in combination with fludarabine (Flu; BuFlu) or cyclophosphamide (Cy; BuCy) as conditioning for allogeneic hematopoietic stem cell transplantation (HSCT). This multi-institutional prospective study compared pharmacokinetic (PK) parameters of Bu between BuFlu and BuCy. Plasma Bu concentrations were measured by high-performance liquid chromatography at the first dose of the first and fourth days of intravenous Bu administrations (total of 16 doses of 0.8 mg/kg). Thirty-seven patients were evaluable (BuFlu, N = 18; BuCy, N = 19). The median age was significantly higher in BuFlu. In BuFlu, the median area under the blood concentration-time curve of Bu on the fourth day was 1183 μmol min/L (range 808-1509), which was significantly higher than that on the first day [1095 μmol min/L (range 822-1453), P < 0.01]. In contrast, such differences were not observed in BuCy. Consistently, there was a significant decrease in the clearance of Bu on the fourth day as compared with the first day in BuFlu. These results suggest that the PK of Bu was altered during the co-administration of Flu, which was not the case with Cy. A large-scale study is required to evaluate the significance of the differences in the PK of Bu between the conditionings on HSCT outcomes.

Plexiform neurofibroma is mainly associated with neurofibromatosis type 1 and is seldom observed in the liver. Its occurrence in the liver without neurofibromatosis type 1 is even rarer. We report an extremely rare case of plexiform neurofibroma of the liver diagnosed by laparoscopic biopsy in a patient without neurofibromatosis type 1. The patient was a 35-year-old man who had neither clinical signs nor any family history of neurofibromatosis type 1. Abdominal ultrasonography, as part of a health screening, had detected a hepatic tumor. Subsequent contrast ultrasonography, computed tomography, and magnetic resonance imaging showed the tumor extending from the retroperitoneal space around the aorta to the hepatic hilum and distal portal branches in the right hepatic lobe, gallbladder, and left hepatic lobe. 18 F-fluorodeoxyglucose positron emission tomography showed no abnormal accumulation. Histopathological examination of the tumor obtained laparoscopically led to a diagnosis of plexiform neurofibroma. Because the patient was asymptomatic with no features of malignancy, he was only monitored and managed. At follow-up 10 years later, computed tomography showed a decrease in tumor size. It is important to recognize that, while rare, plexiform neurofibroma can occur without neurofibromatosis type 1. We recommend follow-up instead of unreasonable surgery in such cases.

Objective: This study investigated the mid-term outcomes of simultaneous mitral valve repair in patients with mitral regurgitation (MR) and concomitant annulo-aortic ectasia., Methods: The study included 26 patients with MR and annulo-aortic ectasia [mean age 46.0 ± 19.9 (10-86) years] who underwent simultaneous mitral valve repair between January 2007 and March 2018. Of these 26 patients, 11 (42.3%) were diagnosed with Marfan syndrome and 10 (38.5%) with Barlow's disease. All patients underwent complete ring annuloplasty; a semi-rigid ring was used in 14 (53.8%) and a semi-flexible ring (anterior-flexible) in 12 patients (46.2%). All patients underwent valve-sparing root replacement using the reimplantation technique., Results: The overall 3-year survival rate was 95.7 ± 4.3%. The 3-year freedom from > moderate MR rate was 94.7 ± 5.1%, and the 3-year freedom from > moderate aortic regurgitation (AR) rate was 86.7 ± 7.3%. The 3-year freedom from reoperation rate was 100%. The 3-year freedom from > moderate MR rate was 100% in the semi-rigid ring group and 85.7 ± 13.2% in the semi-flexible ring group (log-rank test, p = 0.5371). The 3-year freedom from > moderate AR rate was 100% in the semi-rigid ring group and 72.9 ± 16.5% in the semi-flexible ring group (log-rank test, p = 0.0815)., Conclusions: Simultaneous mitral valve repair in patients with MR and concomitant annulo-aortic ectasia showed favorable mid-term outcomes.

Knowledge of the toxicity profile of long-term treatment with imatinib is limited. In the present study, we sought to evaluate renal function and hemoglobin levels during long-term imatinib treatment. Eighty-two patients with chronic myelogenous leukemia in chronic phase who had been on imatinib for over 5 years were retrospectively analyzed. The mean estimated glomerular filtration rate (eGFR) was significantly decreased over 5 years (77 ± 17 to 62 ± 14 ml/min/1.73m², P < 0.001). Higher age and lower eGFR value at initiation of imatinib were significantly associated with development of renal dysfunction by multivariate analyses. Mean hemoglobin levels also significantly decreased over the 5-year period (12.9 ± 1.7 to 12.4 ± 1.3 g/dl, P < 0.01). The rate of decrease in eGFR correlated significantly with hemoglobin levels (correlation coefficient = - 0.249, P < 0.05). Serum erythropoietin (EPO) levels did not increase in 16 patients with both renal dysfunction and anemia (median, 31.9 mIU/ml). In patients who participated in a clinical trial of imatinib discontinuation, mean eGFR (50.0 ± 6.5 to 56.0 ± 10.2 ml/min/1.73m², P < 0.05) and hemoglobin levels (12.0 ± 1.7 to 14.0 ± 1.6 g/dl, P < 0.01) improved significantly at 1 year after discontinuation. These findings suggest that long-term imatinib results in a partially reversible continuous decline in renal function and decreased hemoglobin levels.

Objective: Presenting our experience of direct perfusion of the carotid artery in patients with brain malperfusion secondary to acute aortic dissection., Patients: Among 381 patients who underwent aortic repair for acute type A aortic dissection from October 1999 to August 2017, brain malperfusion was recognized in 50 patients. Nine patients had direct perfusion of the right carotid artery in patients with brain malperfusion secondary to acute aortic dissection. Age at surgery was 65.7 ± 13.5 years and three patients were male. Preoperative consciousness level was alert in one patients, drowsy in six, and coma in two. Five patients had preoperative hemiplegia. All patients showed a blood pressure difference between the upper extremities and eight patients showed more than 15% difference of rSO 2 . Seven patients had a temporary external active shunt from the femoral artery to the right common carotid artery preoperatively. Two patients had direct perfusion to the right common carotid artery during cardiopulmonary bypass or in the intensive care unit after surgery because of a sudden decrease of rSO 2 and cessation of carotid artery flow. Antegrade cerebral perfusion was used in all patients. Total arch replacement was performed in six patients and hemiarch in three., Results: The hospital mortality was 33% (3 patients). Causes of death were huge hemispheric brain infarction or anoxic brain damage in two patients and myocardial infarction in one. The postoperative neurological outcome was alert in four, hemiplegia in two, and coma in three, but five patients showed some improvement of neurological signs., Conclusion: Aggressive direct reperfusion of the carotid artery before the aortic repair may reduce neurological complications in patients with preoperative brain malperfusion secondary to acute aortic dissection.

Fluorine-18 fluorodeoxyglucose (FDG) positron emission tomography (PET) has been recommended as a complementary tool for the staging of various malignancies, including malignant lymphoma. PET findings often shift patients to higher stages and may affect treatment outcomes. In this study, we retrospectively compared staging and treatment outcomes of newly diagnosed diffuse large B-cell lymphoma (DLBCL) assessed by PET (n = 153) or gallium-67 scintigraphy (Ga) (n = 95). In total, Ga upstaged two (2.1%) of 95 patients, whereas PET upstaged 13 (8.5%) of 153 patients. Bone/bone marrow (15 vs. 4%, P = 0.01) and muscle lesion (5 vs. 0%, P = 0.03) were identified more frequently in the PET group than in the Ga group. The estimated 3-year overall and progression-free survival rates did not differ significantly in the two groups at any stage. However, patients with stage III disease tended to have better progression-free survival in the PET group than in the Ga group [92.3 (95% CI 56.6-98.9%) vs. 58.3% (95% CI 27.0-80.1%), P = 0.086]. These results suggest that PET has a greater potential in detecting musculoskeletal lesions of DLBCL as extranodal lesions than Ga, and may contribute to the optimal staging.

Calcineurin inhibitors (CIs) such as cyclosporine A (CSA) and tacrolimus often cause renal dysfunction, resulting in increased serum creatinine, hyperkalemia, and hyperuricemia. However, the effects of CIs on sodium excretion have not been fully elucidated. We retrospectively evaluated the effects of CI administration on sodium excretion in recipients of allogeneic hematopoietic stem cell transplantation (HSCT). Fifty consecutive recipients each of allogeneic HSCT receiving either CSA or tacrolimus (100 patients in total) with available data for weekly fractional excretion of sodium (FENa) for a 4-week period after transplantation were enrolled in this retrospective analysis. No significant differences in patient characteristics were observed between CSA and tacrolimus groups except for the type of donor. FENa was significantly higher at the 3rd (1.25 ± 0.80) and 4th weeks (1.53 ± 1.06) after transplantation as compared with that at the 1st week (0.93 ± 0.51; P < 0.01, P < 0.001, respectively) in the tacrolimus group, but not at any time point in the CSA group. In addition, FENa was significantly higher in the tacrolimus group than the CSA group at the 4th week (1.53 ± 1.06 vs. 1.13 ± 0.80; P < 0.05). These results suggest that tacrolimus increases sodium excretion after allogeneic HSCT, and that this effect is minimal with CSA.

Human herpesvirus-6 (HHV-6) encephalitis and myelitis following allogeneic hematopoietic stem cell transplantation (HSCT) is frequently life-threatening. We retrospectively evaluated the clinical significance of hyponatremia in cases of HHV-6 encephalitis/myelitis. Using an institutional database and medical records, we identified and retrospectively analyzed 16 cases of HHV-6 encephalitis and/or myelitis after allogeneic HSCT. HHV-6 encephalitis and myelitis were defined as the symptoms/signs with HHV-6-DNA in the cerebrospinal fluid. Seizure and memory disorder were defined as symptoms/signs of encephalitis, and dysesthesia and vesicorectal disorder as those of myelitis. Five patients developed encephalitis with or without myelitis, and 11 patients developed myelitis alone. Hyponatremia (median 129.1 mEq/L; range 125.9-130.1) was observed in all five patients with HHV-6 encephalitis at diagnosis, and values were significantly lower than those in patients with HHV-6 myelitis alone (median 137.6; range 134.0-142.2; P < 0.01). In three of the five patients with encephalitis, the decrease in sodium level preceded the clinical onset of encephalitis by one or two days. These results suggest that hyponatremia may be an important manifestation of HHV-6 encephalitis, but not of myelitis, and could be a useful tool for the early prediction or diagnosis of HHV-6 encephalitis.

We retrospectively evaluated single-institute outcomes of allogeneic hematopoietic stem cell transplantation (allo-HSCT) with a reduced-intensity conditioning regimen consisting of fludarabine (125 mg/m²) and melphalan (140 mg/m²) for multiple myeloma. Twenty-three patients (median age: 46 years) were evaluated. Stem cell sources were bone marrow or peripheral blood stem cells from siblings (n = 4) and bone marrow from unrelated donors (n = 19). For graft-versus-host disease prophylaxis, cyclosporine A or tacrolimus with short-term methotrexate was given. Disease status at time of transplant was complete response in four patients, very good partial or partial response in 13, and stable or progressive disease in six. The median follow-up period of 7 survivors at analysis was 73.2 months (range 46.0-158.9 months). During the follow-up, disease recurrence or progression was observed in 21 patients, and was primary causes of death in 88% of the patients. The 5-year overall survival and progression-free survival rates were 38.6% (95% CI 19.3-57.7%) and 5.4% (95% CI 0.4-21.6%), respectively. Although allo-HSCT with this conditioning could be safely performed, further refinement of this approach aiming at more effective eradication of myeloma cells is clearly warranted.

High-dose melphalan has been gaining recognition as a highly emetogenic agent used in hematopoietic stem cell transplantation (HSCT). The aim of this retrospective study was to elucidate the efficacy of aprepitant in preventing high-dose melphalan-induced emesis. Sixty patients who received melphalan (70 mg/m(2)/day, 2 days) and fludarabine (125 mg/m(2)/day, 5 days) as conditioning for allogeneic HSCT for hematological malignancies, and who received ondansetron and methylprednisolone as an antiemetic prophylaxis, were eligible. Twenty of these 60 patients also received aprepitant for 5 days (aprepitant group); the remaining 40 patients served as a control. The rates of complete response (CR), defined as no emesis without rescue medications, and complete protection (CP), defined as no emesis with or without rescue medications, were assessed between the two groups. The observation period was 12 days from the first day of melphalan administration. The CR and CP rates were significantly higher in the aprepitant group than in the control group during the observation period (35 % versus 10 %, P < 0.05; 85 % versus 33 %, P < 0.001; respectively). These results suggest that aprepitant in combination with ondansetron and steroid effectively ameliorates nausea and vomiting caused by the high-dose melphalan-based conditioning for allogeneic HSCT.