IgG4-related disease is a systemic disease, characterized by elevation of serum IgG4 and, histopathologically, massive infiltration of IgG4+ lymphocyte and plasma cell infiltration, storiform fibrosis, causing enlargement, nodules or thickening. It may affect various organs simultaneously or metachronously. Here we analyzed the clinical and pathological characteristics of 99 patients diagnosed with IgG4-related periaortitis/periarteritis and retroperitoneal fibrosis. Of 99 patients (women/men, 15/84; mean age 67.3±9.5 years), 33 were diagnosed based on the histopathological findings of perivascular/retroperitoneal lesions, 50 were diagnosed based on the characteristic imaging findings of perivascular/retroperitoneal lesions and the presence of definitive IgG4-related disease in other organ(s), and the remaining 16 patients were diagnosed by experts based on the characteristic imaging findings of perivascular/retroperitoneal legions, serological findings, response to glucocorticoid treatment, and/or the presence of suspected IgG4-related disease in other organ(s). According to the new organ-specific criteria proposed by experts, 73 (73.7%) diagnoses were categorized to be definitive, and 6 (6.1%) and 17 (17.2%) diagnoses were categorized to be probable and possible, respectively. Further analyses are needed to clarify the optimal diagnostic and therapeutic strategy of IgG4-related periaortitis/periarteritis and retroperitoneal fibrosis.
The timing and choice of surgical method for type B aortic dissection, is still a topic of much debate. We performed Total arch replacement using Frozen elephant trunk (TAR-FET) as a means of preventing distant aortic events, such as retrograde type A aortic dissection (RTAD). We conducted analysis of 142 patients with acute type B dissection who were admitted between January of 2010 and July of 2017. Fifty-five cases required surgical intervention to treat enlargement of the false lumen diameter and ULP formation 2 weeks after the onset of symptoms. 17 TAR-FET were performed with a mean of 42±26 days period from onset to surgery. There were no complications of RTAD or paraplegic, and 90% of patient demonstrated aortic event free survival (5 years) and false lumen reduction ratio of 35%. Based on our analysis, using TAR-FET properly avoids serious complications like RTAD, and is a viable treatment option for type B dissection.
Spontaneous isolated dissection of the superior mesenteric artery (SMAD) is not still well known. We retrospectively analyzed our 30 patients with SMAD to elucidate the treatment strategy and long-term follow-up outcomes. Due to severe abdominal symptom we performed a stents deployment and surgical reconstructive surgery for each one case. Anerysmectomy and bypass surgery was performed for a patient with aneurysmal change. Other 27 patients were managed conservatively. SMAD patients had only two vascular events (renal infarction and graft occlusion), and showed good prognosis for 6–146 (mean 69) months follow-up. We found that there is a few SMAD patients necessary of invasive management at acute phase and that most patients are safely conservatively treated with good prognosis.
Kumamoto was hit by a series of strong earthquakes beginning on April 14, 2016. We treated many deep vein thrombosis (DVT) patients and pulmonary thrombosis (PTE) patients. We came up with a strategy for diagnosis and therapy of venous thromboembolism (VTE). For VTE patients, we prescribed anticoagulant drugs, mainly direct oral anticoagulant (DOAC). To evaluate the validity and safety of the medical strategy for VTE in disasters, we investigated the prognosis of VTE patients at 4 months after the initial quake. In the two months following the initial quake we attended to 43 VTE patients, 11 PTE patients (including 9 patients with both DVT and PTE) and 32 DVT patients. We prescribed DOAC to 34 patients and Warfarin to 4 patients. Based on the survey at 4 months after the first tremblor, the period of anticoagulation therapy was 95.0±17.2 days for PTE and 57.1±36.5 days for DVT and 12 patients were continuing to take anticoagulant drugs. There were no recurrent VTE or bleeding events. DOAC therapy of VTE is therefore considered effective and safe in the event of a natural disaster.
Immunoglobulin G4 (IgG4)-related disease, that is characterized by the elevation of circulating IgG4 level and the tissue-infiltration of IgG4-positive plasma cells, can target the cardiovascular tissue, although the diagnosis of IgG4-related cardiovascular lesion is not easy owing to the substantial risk for the tissue sampling. We herein examined the serum IgG4 levels among cardiac patients. In patients who were admitted to the cardiology department (n=477) and those who underwent computed tomography coronary artery angiography (n=401), elevated serum IgG4 level (≥135 mg/dL) was found 23 (4.8%) and 17 (4.2%), respectively. However, among those with elevated serum IgG4, only two patients could be clinicopathologically diagnosed with IgG4-related disease. Cardiovascular organ involvement may aggravate the prognosis of IgG4-related disease, which in general not life-threatening. Considering that the non-negligible prevalence of high IgG4 level among cardiac patients who were not diagnosed with IgG4-related disease, however, physicians should no count too much on the serum IgG4 levels for the diagnosis of IgG4-related cardiovascular lesions, especially when histopathologic findings are not available, or when other-tissue involvement of IgG4-related disease is not apparent.
Prediction of postoperative cerebral infarction after cardiovascular surgery is difficult. The present study investigated whether quantitative evaluation of preoperative cerebral blood flow used in the Japanese EC-IC Bypass Trial (JET) study is useful for the prediction of postoperative cerebral infarction after cardiovascular surgery. First, patients were divided into two groups based on preoperative cerebral blood flow. In an evaluation using preoperative imaging, patients with good or mildly decreased preoperative cerebral blood flow, divided into clinical stage I or II by quantitative evaluation showed no postoperative cerebral infarction. However, 24% of patients with poor cerebral blood flow who were categorized as clinical stage II, experienced postoperative cerebral infarction. The incidence rate was not statistically significantly different when the groups were compared. Second, patients were divided into two groups based on the anatomical area of the brain affected corresponding to clinical stage II. Patients with a 10% and greater brain involvement had a significantly higher incidence of postoperative cerebral infarction (38%) compared to others (0%, p<0.01). This method may be useful for the prediction of postoperative cerebral infarction after cardiovascular surgery, but a further prospective study is needed.
Objectives: Although pulmonary hypertension (PH) caused by left heart disease (PH-LHD) is more common in PH, little is known about its properties of pulmonary artery (PA) in PH-LHD. The purpose of this study was to measure pulmonary regional pulse wave velocity (PWV) and to quantify the magnitude of reflected waves in patients with PH-LHD by the analysis of the pressure-velocity loops (PV-loop). Methods: High-fidelity PA pressure (Pm) and PA velocity (Vm) were measured in 11 subjects with PH-LHD (mean Pm>25 mmHg), 1 subject with atrial septal defect (ASD) without PH and 12 control subjects, using multisensor catheters. PWV was calculated as the slope of the initial part of the PV-loop in early systole. The similarity in the shapes of the pressure and flow velocity waveforms over one PV-loop was quantified as the magnitude of reflected wave by calculating the standard error of the estimate (Sy/x) from linear regression analysis between Pm and corresponding Vm. PWV and Sy/x during a Valsalva maneuver (VM) were also assessed in nine control subjects. Results: The contour of PV-loop was so characteristic between control and PH-LHD. Max. PWV (349 cm/s) was recorded in PH-LHD and min. PWV (111 cm/s) was recorded in ASD. VM increased Pm (12[7–15] mmHg vs 50[18–110] mmHg; p=0.009) and PWV (200[148–238] cm/s vs 260[192–306] cm/s; p=0.009) significantly without significant increase of Sy/x (19.6[12.7–28.9]% vs 28.2[19.3–40.7]%; p=0.079). Although Sy/x was significantly higher in PH-LHD than in control and ASD (17.5[8.4–28.9]% vs 31.0[14.3–36.3]%; p=0.009, ASD: 18.2%), no significant difference was found in PWV between PH-LHD and control (269[159–349] cm/s vs 203[154–289] cm/s; p=0.089). Conclusions: 1) The magnitude of wave reflection was elevated in PH-LHD significantly as compared with control and ASD. 2) Despite the significant increase in PA-PWV caused by abrupt elevation in Pm during Valsalva maneuver in control, chronic elevation in Pm did not increase PA-PWV in PH-LHD significantly. It was hypothesized that the pulmonary artery constituted a self-regulating system for maintaining the arterial stiffness stable against the chronic elevation in Pm in PH-LHD by a remodeling of increasing proximal pulmonary arterial cross-sectional area gradually, which was compatible with the Moens–Korteweg equation. The PV-loop could provide a new simple and conventional method for assessing the pulmonary arterial properties, clinically.
Surgical revascularization is performed to preserve limb and to maintain functional status of patients with critical limb ischemia (CLI). The PREVENT III risk score helps to predict the postoperative course of CLI. However, this score is not available to estimate the risk of amputation or death properly in patients with hemodialysis (HD) and tissue loss (HD: 4 points, Tissue loss: 3 points), because they are classified as a high-risk group. Therefore, we investigated 213 patients with revascularized HD for CLI and proposed prognosis amputation or death for patients with HD risk score (PAD for HD risk score). PAD for HD risk score (non-ambulation: 3 points, ulcer/gangrene: 2 points, GNRI<92: 2 points, CRP>0.3 mg/dl: 1 point, Age≥75: 1 point) is more accurate for the prediction of amputation or death than the PREVENT III risk score (area under the curve [AUC]: 0.79 [95% confidence interval: CI: 0.71–0.87], p<0.01 vs. AUC: 0.63 [95%CI: 0.56–0.71]). The patients were stratified into three groups by total score in ascending order. The rate of 1-year amputation-free survival and independent ambulatory status were significantly different among three groups. PAD for HD risk score is useful for rehabilitation planning in patients with HD and CLI.
Objective: To assess medical economic adequacy of endovascular aneurysm repair (EVAR) for abdominal aortic aneurysm (AAA). Methods: Cost-utility analysis. A total of 21 patients with AAA treated at Ibaraki Prefectural Central Hospital in 2014 were divided into non-ruptured EVAR (Group E) and open surgery (OS) (Group O), and ruptured OS (Group R) groups, and hospital costs were aggregated with a medical accounting system. Mid-level hospital costs were estimated by a diagnosis-procedure-combination analysis system. Incremental life years were extrapolated from the results of randomized controlled trials in the UK (EVAR Trial 1 and 2), a life table, and the Pancreas Cancer Registry in Japan. Quality-adjusted life years (QALY) were estimated under the assumption of a certain quality weight. Results: Incremental cost-effectiveness ratio (ICER) of EVAR compared with the OS was calculated to be 31.0 million yen/QALY, which is economically inadequate. ICER of EVAR compared with conservative treatment was inadequate in some subgroups of extremely old patients and in patients operated for far-advanced cancer. Conclusion: EVAR is inadequate with respect to medical economics as a substitute for OS for patients in whom both procedures are available. The indication for EVAR in patients ineligible for OS should be different from that for surgery in usual patients with AAA.