Validation of the Diagnostic Criteria for IgG4-Related Periaortitis/Periarteritis and Retroperitoneal Fibrosis (IgG4PA/RPF) 2018, and Proposal of a Revised 2023 Version for IgG4-Related Cardiovascular/Retroperitoneal Disease.

BACKGROUND: In 2018, diagnostic criteria were introduced for IgG4-related periaortitis/periarteritis and retroperitoneal fibrosis (PA/RPF). This study assessed the existing criteria and formulated an improved version.Methods and Results: Between August 2022 and January 2023, we retrospectively analyzed 110 Japanese patients diagnosed with IgG4-related disease (IgG4-RD) involving cardiovascular and/or retroperitoneal manifestations, along with 73 non-IgG4-RD patients ("mimickers") identified by experts. Patients were stratified into derivation (n=88) and validation (n=95) groups. Classification as IgG4-RD or non-IgG4-RD was based on the 2018 diagnostic criteria and various revised versions. Sensitivity and specificity were calculated using experts' diagnosis as the gold standard for the diagnosis of true IgG4-RD and mimickers. In the derivation group, the 2018 criteria showed 58.5% sensitivity and 100% specificity. The revised version, incorporating "radiologic findings of pericarditis", "eosinophilic infiltration or lymphoid follicles", and "probable diagnosis of extra-PA/-RPF lesions", improved sensitivity to 69.8% while maintaining 100% specificity. In the validation group, the original and revised criteria had sensitivities of 68.4% and 77.2%, respectively, and specificities of 97.4% and 94.7%, respectively. CONCLUSIONS: Proposed 2023 revised IgG4-related cardiovascular/retroperitoneal disease criteria show significantly enhanced sensitivity while preserving high specificity, achieved through the inclusion of new items in radiologic, pathological, and extra-cardiovascular/retroperitoneal organ categories.

Prognostic factors after open and endovascular repair for infected native aneurysms of the abdominal aorta and common iliac artery.

OBJECTIVE: Infected native aneurysms (INAs) of the abdominal aorta and iliac arteries are uncommon, but potentially fatal. Endovascular aneurysm repair (EVAR) has recently been introduced as a durable treatment option, with outcomes comparable to those yielded by conventional open repair. However, owing to the rarity of the disease, the strengths and limitations of each treatment remain uncertain. The present study aimed to separately assess post-open repair and post-EVAR outcomes and to clarify factors affecting the short-term and late prognosis after each treatment. METHODS: Using a nationwide clinical registry, we investigated 600 patients treated with open repair and 226 patients treated with EVAR for INAs of the abdominal aorta and/or common iliac artery. The relationships between preoperative or operative factors and postoperative outcomes, including 90-day and 3-year mortality and persistent or recurrent aneurysm-related infection, were examined. RESULTS: Prosthetic grafts were used in >90% of patients treated with open repair, and in situ and extra-anatomic arterial reconstruction was performed in 539 and 57 patients, respectively. Preoperative anemia and imaging findings suggestive of aneurysm-enteric fistula were independently associated with poor outcomes in terms of both 3-year mortality (hazard ratio [HR], 1.62; 95% confidence interval [CI], 1.01-2.62; P = .046, and HR, 2.24; 95% CI, 1.12-4.46; P = .022, respectively) and persistent or recurrent infection (odds ratio [OR], 2.16; 95% CI, 1.04-4.49; P = .039, and OR, 4.96; 95% CI, 1.81-13.55; P = .002, respectively) after open repair, whereas omental wrapping or packing and antibiotic impregnation of the prosthetic graft for in situ reconstruction contributed to improved 3-year survival (HR, 0.60; 95% CI, 0.39-0.92; P = .019, and HR, 0.53; 95% CI, 0.32-0.88; P = .014, respectively). Among patients treated with EVAR, abscess formation adjacent to the aneurysm was significantly associated with the occurrence of persistent or recurrent infection (OR, 2.24; 95% CI, 1.06-4.72; P = .034), whereas an elevated preoperative white blood cell count was predictive of 3-year mortality (HR, 1.77; 95% CI, 1.00-3.13; P = .048). CONCLUSIONS: Profiles of prognostic factors differed between open repair and EVAR in the treatment of INAs of the abdominal aorta and common iliac artery. Open repair may be more suitable than EVAR for patients with concurrent abscess formation.

Surgical Treatment for Popliteal Artery Entrapment Syndrome in Japan: a Retrospective, Multicentre Study Using a National Clinical Registry.

OBJECTIVE: Surgical treatment is an established method for popliteal artery entrapment syndrome (PAES), which, however, mainly derives from single centre experiences where PAES cases are centralised and treated periodically. This study evaluated clinical outcomes of surgical treatment for PAES in a clinical setting where PAES cases were not centralised. METHODS: Multicentre, retrospective cohort study using a national clinical registry. From a Japanese nationwide clinical registry, data for patients who underwent surgical treatment for PAES between 2013 and 2018, including 58 limbs from 41 institutes, were retrieved and evaluated. Patency was analysed using Kaplan-Meier curves. RESULTS: The mean patient age was 36 ± 19 years, 78% were male, and the incidence of PAES was 0.24 limbs/centre/year, reflecting a clinical setting where PAES cases are not centralised. The most frequent arterial symptom was intermittent claudication (90%). Computed tomography was performed in 57 limbs (98%) for the diagnosis, however active manoeuvres such as dorsiflexion and plantarflexion during the examination was performed in only 13 limbs (22%), and occlusion of the popliteal artery was present in 38 limbs (66%) at diagnosis. Regarding surgical treatment, myotomy alone was performed in only seven limbs (12%), and other limbs were revascularised. Mean follow up was 26 ± 20 months, and surgical treatment was effective as it relieved symptoms in > 96% of limbs, with five year primary and secondary patency of the surgical treatment for PAES of 72% and 93%, respectively. CONCLUSION: Results of surgical treatment were acceptable even in a clinical setting where PAES cases were not centralised. However, a low incidence of active manoeuvres performed during the examination and a high incidence of occlusion at diagnosis suggests there may be delayed or underdiagnosis of PAES in Japan, and increased awareness for PAES is warranted.

Concomitant carotid endarterectomy and aortic valve replacement in a patient with high risk of perioperative stroke.

Surgical strategy for significant carotid artery stenosis complicated with severe aortic valve stenosis is still controversial. Herein, we report a case of 80-year-old female in whom 78% stenosis by the NASCET criteria in left internal carotid artery was pointed out during preoperative checkup for symptomatic severe aortic stenosis. Carotid endarterectomy was done concomitantly with aortic valve replacement. No neurological complication occurred perioperatively.

Venous thromboembolism complications after endovenous laser ablation for varicose veins and role of duplex ultrasound scan.

OBJECTIVE: A nationwide survey was conducted in Japan to determine the incidence of venous thromboembolism (VTE) after endovenous thermal ablation for varicose veins and to investigate its pathogenic background. METHODS: The survey targeted all cases of endovenous thermal ablation between January 2011 and December 2013. Based on this survey, a retrospective study of patients who developed endovenous heat-induced thrombosis (EHIT) of classes 2 to 4, no EHIT-related deep venous thrombosis (DVT), and pulmonary embolism (PE) was conducted. Lower extremity venous ultrasound was performed within 72 hours and at 1 to 3 months postoperatively in all institutions. We investigated factors associated with the occurrence of complications and the usefulness of postoperative ultrasound. RESULTS: Survey responses were collected from 213 institutions. Endovenous laser ablation was performed for 43,203 patients (EHIT 2 in 318 patients, EHIT 3 in 50 patients, EHIT 4 in 7 patients, other DVTs in 24 patients, and PE in 3 patients). The incidence of VTE complications was 1.0% for EHIT 2, 0.11% for EHIT 3, 0.013% for EHIT 4, 0.063% for other DVTs, and 0.0067% for PE based on the adjusted population. Sex, age, obesity, origin of the varicose vein, vein diameter, and preoperative Caprini score were not strong indicators of VTE complications. Of 50 patients with EHIT 3, there were 35 patients who had EHIT 3 during the first postoperative ultrasound session. In one patient, EHIT 3 progressed to EHIT 4 despite initiation of anticoagulant therapy. Of seven patients with EHIT 4, only one patient had EHIT 4 at the first postoperative ultrasound examination. Because ultrasound performed before the occurrence of EHIT 4 revealed that three patients had EHIT 2 or EHIT 3, EHIT 4 could have possibly been predicted by ultrasound. Of three patients with PE, two developed PE before ultrasound, and EHIT was not detected by ultrasound in one of those patients before PE developed. Anticoagulant therapy was administered in most patients with EHIT 3 and all patients with EHIT 4 and PE, with favorable outcomes. CONCLUSIONS: The incidence of VTE complications after endovenous laser ablation was low. Furthermore, the value of performing postoperative ultrasound for VTE management seems to be low because the occurrence of severe VTE complications could not be predicted by lower extremity venous ultrasound. Although the perioperative detection of VTE complications by this modality resulted in the early administration of anticoagulant therapy and may have contributed to improved prognosis, the number of those patients was limited.

Increased Incidence of Cancer in Japanese Patients with Critical Limb Ischemia.

Objective: This multicenter observational study was conducted in order to investigate the incidence of cancer in patients with critical limb ischemia. Materials and Methods: We prospectively investigated the incidence of cancer in 68 patients with critical limb ischemia over a two-year period. Patients underwent an intensive examination at enrollment, which included tumor marker levels and chest and abdominal computed tomography, as well as one- and two-year follow-up examinations. We compared the observed incidence of cancer with the expected incidence calculated from national cancer rates by the standardized incidence ratio (SIR). Results: The majority (83.6%) of the patients were men, and 92.5% of the patients had a peripheral arterial disease that was classified as Fontaine stage III or IV. During enrollment, newly diagnosed cancers were detected in seven patients. Four additional cancers were detected during the follow-up period. All of the detected cancers were asymptomatic. We observed an increased risk of cancer (SIR, 4.04; 95% confidence interval, 1.31-9.42) in patients with critical limb ischemia. Conclusion: This study suggests that critical limb ischemia is associated with an increased risk of cancer. Our findings should be taken into serious consideration by future investigators considering the use of therapeutic angiogenesis.

Family history of aortic aneurysm is an independent risk factor for more rapid growth of small abdominal aortic aneurysms in Japan.

OBJECTIVE: We aimed to investigate risk factors associated with more rapid growth of abdominal aortic aneurysms (AAA) <50 mm (small AAAs) in Japan. METHODS: We retrospectively investigated the clinical data of 374 patients with small AAAs (maximum diameter, ≤50 mm) who were referred to The University of Tokyo Hospital, Tokyo Medical University Hospital, or Saitama Medical Center, between 1995 and 2008. RESULTS: A total of 374 patients (321 men and 53 women) were followed up for a median of 66 months. The median diameter on initial examination was 40 mm, and the median growth rate of the AAAs was 2.1 mm/y. The growth rate of AAAs with an initial diameter ≥45 mm was significantly greater than those with an initial diameter <45 mm (3.3 mm/y vs 2.0 mm/y, respectively; P = .007). The growth rate of AAAs was significantly greater in patients with hypertension than in those without (2.3 mm/y vs 1.7 mm/y, respectively; P = .006) and in patients with a family history of aortic aneurysm than in those without (4.2 mm/y vs 2.0 mm/y, respectively; P = .009). Logistic regression analysis revealed that a large initial diameter and family history of aortic aneurysm were independent predictors of accelerated growth rate of small AAAs in Japan. CONCLUSIONS: In the present study, a large initial diameter and family history of aortic aneurysm were independent risk factors for more rapid growth of small AAAs. Although few studies have reported similar findings thus far, family history of aortic aneurysm should be carefully considered during follow-up of patients with small AAAs.

Spontaneous rupture of autogenous saphenous vein graft in bypass surgery for peripheral arterial disease possibly associated with collagen disease.

We encountered two cases of spontaneous rupture of a saphenous vein bypass graft for lower-leg peripheral arterial disease possibly associated with collagen disease. Rupture occurred 5 and 14 days postoperatively. Neither case had any signs of infection, graft degeneration, or evident injury, but both were associated with collagen disease diagnosed at another hospital. We believe that an association with collagen disease might have contributed to vein wall fragility in the present cases.

Use of side branch of total arch replacement graft as bypass inflow to prevent visceral ischemia with type B aortic dissection.

When operating on patients with type B aortic dissection, the preoperative hemodynamics and malperfusion of visceral organs should be considered. We report a 70-year-old man with dissecting distal arch aneurysm following type B aortic dissection, whose celiac artery arose from a false lumen and who was successfully treated with total arch replacement and ascending graft-celiac artery bypass.

Obstructive sleep apnea as a potential risk factor for aortic disease.

Obstructive sleep apnea (OSA) is not only a cause of hypertension; it also possibly affects the pathogenesis and progression of aortic disease because an inspiratory effort-induced increase in negative intrathoracic pressure generates mechanical stress on the aortic wall. The objective of the present study was to examine the incidence by location of OSA as a complication in patients with aortic aneurysm and patients with aortic dissection (AD). An overnight sleep study was conducted in the following study groups: the aortic disease group (n = 95) consisting of patients with thoracic aortic aneurysm (TAA, n = 32), patients with abdominal aortic aneurysm (AAA, n = 36), and patients with AD (n = 27); and a control group (n = 32), consisting of patients with coronary risk factors who were matched with the aortic disease group for age, gender, and body mass index (BMI). The 3% oxygen desaturation index (ODI) was significantly higher in all the TAA, AAA, and AD groups (P = 0.045, P = 0.003, and P = 0.005, respectively) than in the control group. The incidence of moderate to severe OSA [apnea hypopnea index (AHI) ≥15 events/h] was significantly higher in the first three groups (P = 0.026, P = 0.001, P = 0.003, respectively) than in the control group, while no significant difference was found between the TAA group and the AAA group with respect to these variables. Furthermore, no significant differences were found between the thoracic AD subgroup and the abdominal AD subgroup with respect to AHI and 3% ODI, as well as with respect to the incidences of moderate to severe OSA. Patients with TAA, patients with AAA, and patients with AD showed high incidences of moderate to severe OSA. Although this result suggests that OSA may be one of risks for aortic disease, unelucidated mechanism(s) other than negative intrathoracic pressure may be involved in the pathogenesis of aortic disease.

Diabetes and old age could affect long-term patency of paramalleolar distal bypass for peripheral arterial disease in Japanese patients.

BACKGROUND: The results of paramalleolar distal bypass for critical limb ischemia in patients with peripheral arterial disease were reviewed to determine the factors affecting the long-term patency of this procedure in Japanese subjects. METHODS AND RESULTS: A total of 65 legs from 60 consecutive Japanese patients with critical limb ischemia who underwent distal bypass to the ankle were retrospectively reviewed. Postoperative graft patency, morbidity and mortality were analyzed. All patients were monitored during a mean follow-up period of 2.2 ± 1.7 years (median, 1.7 years). The accumulated primary and secondary patency rates were both 81.0% at 1 year and 78.7% at 3 and 5 years. The amputation-free rates and survival rates at 1 year, and 3 and 5 years were 94.5% and 82.6%, 82.6% and 88.1%, and 76.7% and 69.7%, respectively. The Cox proportional hazard model was used to determine factors contributing to long-term results. Age (odds ratio, 1.1; P<0.05) and diabetes (odds ratio, 18.0; P<0.05) were found to be the significant determinant factors of graft occlusion. No variables were found to be significant determinant factors of amputation-free or survival rates. CONCLUSIONS: Distal bypass to the paramalleolar tibial artery is an effective strategy for peripheral arterial disease with reasonable long-term reliability. Diabetes and old age were found to be the possible determinant factors of graft failure in Japanese patients.

Hybrid procedures combining conventional and thoracic endovascular aortic repair for thoracic aortic aneurysms.

PURPOSE: To minimize surgical invasiveness for extensive aortic aneurysms and expand the indications for thoracic endovascular aortic repair (TEVAR), we evaluated outcomes of hybrid procedures combining conventional surgical aortic repair and TEVAR for thoracic aortic aneurysms. METHODS: The following hybrid procedures were performed: second-stage TEVAR after total aortic arch replacement using the elephant trunk as the landing zone in 17 patients; and for multiple aortic aneurysms, vascular graft replacement and TEVAR in 13 patients, vascular graft replacement and TEVAR with bypass in 2 patients, and TEVAR with bypass in 23 patients. RESULTS: There were three (5.3%) hospital deaths, from serious complications including stroke, paraplegia, paraparesis, and aspiration pneumonia; and eight late deaths. There was only one aneurysm-related death, of a patient who underwent emergency surgery for an esophageal fistula resulting from enlargement of a residual false lumen of a thoracoabdominal aorta after second-stage TEVAR. CONCLUSION: Hybrid procedures minimize surgical invasiveness in thoracic aortic aneurysm repair, but further evaluation of a larger number of patients is necessary.

Intraoperative evaluation of blood perfusion by laser-assisted indocyanine green angiography after ex vivo vascular reconstruction of intrahilar renal artery aneurysm.

The surgical reconstruction of intrahilar renal artery aneurysms (RAAs) is a difficult surgery because of complex anatomy. We present a case of right intrahilar RAA diagnosed in a 67-year-old man. We performed ex vivo reconstruction using an organ preservation solution to prevent postoperative renal failure. We assessed graft patency and blood perfusion was assessed by laser-assisted indocyanine green angiography using the SPY system after autotransplantation. Postoperative renal insufficiency was not observed. The results demonstrate that ex vivo reconstruction of intrahilar RAAs using an organ preservation solution, and graft patency and blood perfusion evaluation using the SPY system are effective methods for preserving renal function.

Thoracic endovascular aortic repair with aortic arch vessel revascularization.

BACKGROUND: Revascularization of aortic arch vessels was performed with thoracic endovascular aortic repair (TEVAR) to preserve the endoprosthesis landing zone in 19 high-risk patients. METHODS: The operative procedure used was a bypass or transposition involving the common carotid and subclavian arteries. Homemade fenestrated stent-grafts, deployed in landing zone 0, were used for TEVAR. RESULTS: All lesions resolved without endoleaks. No perioperative deaths occurred; seven patients had postoperative complications. One patient with acute respiratory distress syndrome required reoperation to change the bypass route and permit tracheostomy. One patient died of pneumonia 2 months after treatment, after an anastomotic pseudoaneurysm and cerebral infarction developed and an operation was performed to obtain hemostasis. The procedure-related mortality was 5.3%. CONCLUSION: Aortic arch vessel revascularization before TEVAR may permit less invasive surgery, although careful patient selection is essential.

Staged hybrid repair using telescoped stent graft fixation for aortic arch and descending aortic aneurysms.

Staged repair of extensive thoracic aortic aneurysms is complicated, with a high incidence of interval rupture between stages. We describe the systematic staged hybrid procedure of a previous endovascular repair of a descending aortic aneurysm and open surgical repair of an aortic arch aneurysm. In the second-stage arch repair, the stent graft was easily retracted and fixed, without dissection, around the aortic arch aneurysm distal side. Extensive thoracic aortic aneurysms were managed without interim rupture or neurologic deficits. This approach avoided the potential for interim rupture because recovery from the first-stage endovascular repair was shorter than that from open repair.

Skin perfusion pressure measurement to assess improvement in peripheral circulation after arterial reconstruction for critical limb ischemia.

AIM: To assess the utility of skin perfusion pressure (SPP) measurement in evaluating the outcome of vascular constructions for critical limb ischemia (CLI) patients. METHODS: We retrospectively studied 19 lower limbs in 18 patients who underwent arterial reconstruction for CLI from whom SPP measurements had been obtained pre- and postoperatively between 2008 and 2010. Six limbs whose ulcers had healed postoperatively were classified into group H, 7 limbs whose ulcers had not healed into group U, and 6 limbs without ulcers into group N. SPP values were compared among these groups. RESULTS: The preoperative SPP values in all groups were <30 mmHg, without significant differences among the groups. The SPP values in groups H and N significantly improved after operation, and those in group U were significantly lower than those in the other groups. CONCLUSIONS: SPP measurement before and after arterial reconstruction is useful to assess improvement in tissue circulation and to predict the likelihood of wound healing. An SPP value ≥30 mmHg was considered necessary for wound healing, supporting the findings of the few reports in the literature on the usefulness of SPP for assessing vascular reconstruction effects on ulcer wound healing.

A case of abdominal aortic aneurysm associated with L-shaped crossed-fused renal ectopia.

Genitourinary anomalies are a tremendous challenge for the vascular surgeon, especially when dealing with an abdominal aortic aneurysm. We report a case of crossed-fused renal ectopia, a rare anomaly accompanied by abdominal aortic aneurysm. Bilateral renal arteries and one aberrant artery from the right common iliac artery supply the ectopic kidney. Because renal ischemia during aortic reconstruction can be a serious problem, we reconstructed a temporary right axillo-left renal artery bypass graft first, then reimplanted the aberrant renal artery. When choosing the procedure for renal preservation, preoperative multidetector-row computed tomography was useful to plan the operative strategy.

Long-term results of second-stage thoracic endovascular aortic repair following total aortic arch replacement.

PURPOSE: We investigated the surgical results of secondary thoracic endovascular aneurysm repair (TEVAR) using the elephant trunk graft after total aortic arch replacement (TAR) for extensive thoracic aortic lesions. METHODS: The subjects comprised 16 patients who underwent TEVAR as a staged procedure following TAR at our institution between 1997 and 2007. Long-term results were retrospectively surveyed (mean observation period 68.4 months). We performed TEVAR with the elephant trunk graft as a proximal landing zone for the descending thoracic repair, the mean duration between TAR and TEVAR was 4.7 weeks for the staged operations and 18.3 months for the nonstaged operations. RESULTS: Early results were good in all cases, with no deaths and no noteworthy complications. For the seven patients without dissection, long-term results were also good. Among the nine patients with dissection, the false lumen in the thoracoabdominal area enlarged in three during follow-up. We performed thoracoabdominal repair in two, but one died of an aneurysm-esophageal fistula. There was only one long-term aneurysm-related death. CONCLUSION: Second-stage TEVAR using the elephant trunk graft after TAR allows less invasive surgery for extensive aortic lesions and achieves good long-term results. However, enlargement of the false lumen was a long-term concern in patients with aortic dissection, and careful follow-up is essential.

Surgical technique of total arch replacement for failed thoracic endovascular aortic repair.

Since its introduction in the 1990s, the number of thoracic endovascular aortic repairs is increasing rapidly. However, data on the operative method for reoperation after failed thoracic endovascular aortic repairs are still scanty. We describe the surgical technique of total arch replacement with partial stent removal and the elephant trunk procedure for failed thoracic endovascular aortic repair.