木村 直行

Paroxysmal nocturnal haemoglobinuria is a rare disorder characterized by haemolytic anaemia and pancytopaenia. The use of cardiopulmonary bypass can lead to a haemolytic crisis in patients with paroxysmal nocturnal haemoglobinuria due to activation of complement-mediated haemolysis. We report the successful management of a 69-year-old man undergoing aortic valve replacement with standard heparin-protamine protocol by using eculizumab, a monoclonal antibody of complement factor C5. The surgery was performed without triggering a haemolytic crisis, and the patient was discharged from the hospital without major complications.

BACKGROUND: Acute type A aortic dissection (ATAAD) is relatively uncommon in dialysis patients, and characteristics and repair outcomes are not fully understood. PATIENTS AND METHODS: Patients with ATAAD (n = 960) were divided into a dialysis group (n = 19) and non-dialysis group (n = 941), depending on whether they required dialysis for preoperative end-stage renal disease (ESRD). Hospital charts and imaging data were reviewed, and characteristics and outcomes were compared between the groups. Segmental aortic wall or intima/media flap calcification in the thoracic and abdominal aorta was assessed in the dialysis patients. RESULTS: The leading primary causes of ESRD were polycystic kidney disease (n = 5) and chronic glomerulonephritis (n = 5). There were no significant differences (dialysis group vs. non-dialysis group) in age (60.5 vs. 64.5 years), preoperative hemodynamics, or organ ischemia. Dialysis patients were more likely to have an entry tear in the aortic arch (42% vs. 15%, p = 0.003). These patients showed moderate-to-severe calcification (multiple focal or single focal calcification > 10 mm) in the ascending aorta (17%), aortic arch (61%), descending aorta (67%), and abdominal aorta (83%). Arch replacement was common in this group (37% vs. 18%, p = 0.030). Although in-hospital mortality was increased in this group (21% vs. 7%, p = 0.059), morbidities did not differ significantly. Six-year survival was 60.3 ± 13.4% and 78.8 ± 1.6%, respectively (p = 0.01). CONCLUSIONS: Dialysis patients tend to have aortic calcification and a primary tear in the aortic arch. Outcomes are acceptable.

OBJECTIVE: Sarcopenia, age-related loss of muscle mass, is an objective and comprehensive marker of frailty. We aimed to clarify the influence of sarcopenia on the outcomes after heart valve surgery. METHODS: We retrospectively reviewed 1119 patients who underwent valve surgery via median sternotomy at our institution from June 2009 to December 2013. Patients aged <70 years, urgent/emergent cases, and patients without preoperative computed tomography of the abdomen were excluded. The remaining 428 patients were included in this study. Psoas muscle area, a validated measure of sarcopenia, was measured on preoperative computed tomography. Sarcopenia was defined as the lowest sex-specific quartile in psoas muscle area. The mean follow-up period was 3.4 years. RESULTS: Overall in-hospital mortality did not differ between the sarcopenia and nonsarcopenia patient groups. However, the incidence of stroke and intra-aortic balloon pump/percutaneous cardiopulmonary support use was greater in the sarcopenia group than in the nonsarcopenia group. The patients with sarcopenia had significantly decreased long-term survival and decreased freedom from major adverse cardiac and cerebrovascular events. Multivariable analysis and inverse probability weighting revealed that sarcopenia was an independent predictor for decreased survival (hazard ratio, 2.22; 95% confidence interval, 1.26-3.92; P = .006). CONCLUSIONS: Preoperative sarcopenia defined from the psoas muscle area was associated with long-term outcomes after valve surgery. Thus, the measurement of psoas muscle area can help facilitate more accurate risk scoring in elderly patients.

PURPOSE: To identify the perioperative factors associated with aneurysm size changes after endovascular aortic aneurysm repair (EVAR). METHODS: Between August, 2008 and December, 2014, 187 patients underwent EVAR treatment in our institution. The subjects of this study were 135 of these patients without peripheral artery disease, who were followed up with computed tomography (CT) for 3 years. Significant aneurysm size change was defined as sac size change of more than 5 mm from the baseline. RESULTS: Sac enlargement was identified in 25 patients (18.5%) and sac shrinkage was identified in 59 (43.7%) patients. The factors associated with sac enlargement were postoperative pulse wave velocity (OR: odds ratio 3.80, p = 0.047), prevalence of a type 2 endoleak 1 week after surgery (OR 4.26, p = 0.022), inner diameter (OR 1.10, p = 0.005), and distance from the lower renal artery to the terminal aorta (OR 1.05, p = 0.017). The factors associated with sac shrinkage were prevalence of a type 2 endoleak (OR 0.09, p < 0.001) and preoperative pulse wave velocity (OR 0.32, p = 0.022). The factors independently associated with type 2 endoleak were the use of an Excluder device (OR 3.99, p = 0.002) and the length of the aneurysm (OR 1.02, p = 0.027). CONCLUSION: Inner diameter, treatment length, perioperative pulse wave velocity, and type 2 endoleak were associated with sac size changes after EVAR.

BACKGROUND: Outcomes of early-onset acute type A aortic dissection (ATAAD) associated with Marfan syndrome (MFS) are known, but not with other etiologies. Methods and Results: ATAAD patients from 2 centers (n=1,001) were divided into 2 groups: age ≤45 years (n=93) and age >45 years (n=908). Although in-hospital death and 10-year survival were similar (12% vs. 7% and 62.6% vs. 67.3%), the 10-year aortic event-free survival differed (50.0% vs. 80.2%; P<0.01). ATAAD patients from 3 centers (n=132), all aged ≤45 years, were divided into 5 groups: lone hypertension (HTN, n=71), MFS (n=23), non-syndromic familial thoracic aortic aneurysm and dissection (NS-FTAAD, n=16), bicuspid aortic valve (BAV, n=11), and no known etiologic factor (n=11). The incidence of severe aortic insufficiency varied between groups (HTN: 11%, MFS: 39%, NS-FTAAD: 38%, BAV: 55%, no known factor: 46%; P<0.01), whereas in-hospital death did not (14%, 22%, 0%, 0%, and 9%; P=0.061). The 10-year survival was 52.2%, 64.7%, 83.6%, 100%, and 90.9%, respectively, and 10-year aortic event-free survival was 55.6%, 36.3%, 77.5%, 90.0%, and 30.0%. Median descending aorta growth (mm/year) was 1.1 (0.1-3.4), 2.3 (0.3-5.3), 1.9 (1.3-2.7), 0.9 (-0.1-2.0), and 1.0 (-0.2-2.9) (P=0.15), respectively. CONCLUSIONS: Late aortic events are common in young ATAAD patients. Known etiologic factors, though not BAV, negatively influence late outcomes in these patients.

Objective: To investigate the predictors of acute kidney injury (AKI) following surgery for abdominal aortic aneurysm. Materials and Methods: Subjects were 642 non-hemodialysis patients (open aortic repair [OAR] group, n=453; endovascular aortic repair [EVAR] group, n=189) who underwent elective surgery between 2009 and 2015. AKI was assessed according to the Kidney Disease Improving Global Outcomes criteria. In-hospital mortality and incidence of AKI were compared between the OAR and EVAR groups. The effect of AKI on outcomes and predictors of AKI were examined in both groups. Results: In-hospital mortalities were 0.7% (3/453) in the OAR group and 0.5% (1/189) in the EVAR group. The incidence of AKI increased in the OAR group (14.1% vs. 3.7%, P<0.01). In the OAR group, in-hospital mortality (0% vs. 4.7%, P<0.01) increased in patients with AKI. In the OAR group, hemoglobin level <10 g/dL, estimated glomerular filtration rate <60 mL/min/1.73 m2, operation time >300 min, history of ischemic heart disease, and amount of bleeding >1,000 mL were predictors of AKI. In the EVAR group, amount of transfusion>1,000 mL was a predictor of AKI, but AKI was not found to worsen outcomes. Conclusion: AKI affected outcomes of OAR. Knowledge of predictors may optimize perioperative care.

BACKGROUND: Clinical characteristics and treatment outcomes of acute type A aortic dissection with D-dimer elevation have not been clarified. METHODS AND RESULTS: D-dimer was measured preoperatively within 24 hours of symptom onset in 262 patients with acute type A aortic dissection. The median (and interquartile range) admission D-dimer concentration in our total patient group was 26.7 (8.3-85.9) μg/mL. Median (interquartile range) D-dimer concentrations were 5.0 (2.6-18.0) μg/mL for complete false lumen thrombosis (n=33), 60.9 (19.4-160.4) μg/mL for partial thrombosis (n=81), 26.5 (10.0-70.6) μg/mL for a patent false lumen (n=131), and 8.7 (3.2-26.9) μg/mL for ulcerlike projection (n=17) (P<0.01). With a D-dimer concentration of ≤8.3 μg/mL representing the lower quartile, we then investigated predictors of a low D-dimer level. Multivariate analysis showed dissection limited to the ascending aorta (P<0.01; odds ratio, 9.81) or descending aorta (P<0.01; odds ratio, 7.68), a completely thrombosed false lumen (P<0.01; odds ratio, 4.02), and absence of brain ischemia (P=0.013; odds ratio, 4.74) to be predictors of the lower D-dimer concentration. Compared with patients with a low D-dimer concentration (≤8.3 μg/mL, n=66), patients with a D-dimer concentration >8.3 μg/mL (n=196) had a reduced preoperative platelet count and increased operation time and transfusion volume. In-hospital mortality was elevated in this group (1.5% versus 11.2%; P=0.031), although 7-year survival did not differ for hospital survivors (lower versus higher, 93.1% versus 79.1%; P=0.21). CONCLUSIONS: D-dimer concentrations are strongly influenced by the extent of dissection and false lumen status. Operative risks are increased in patients with a relatively high D-dimer concentration.

Giant coronary artery aneurysm (GCAA) combined with coronary artery fistula to the pulmonary artery (PA) is rare. A 79-year-old man was accidentally discovered with GCAA. He was operated on by use of aneurysmorrhaphy, and closure of the fistulae was performed. Because ischemic changes appeared, coronary artery bypass grafting was done. The postoperative course was uneventful, and the patient was discharged on postoperative day 14. We report here a case of GCAA with a size of 66 × 52 mm in diameter associated with a fistula formation into the PA. It is one of the largest sizes of GCAA that has occurred after fistula formation.

Objective: We investigated the characteristics and surgical outcomes of abdominal aortic aneurysm (AAA), which typically occurs in elderly persons, in Japanese patients aged 50 years or younger. Materials and Methods: Clinical records of 999 patients who underwent open or endovascular repair for AAA at our hospital between 2007 and 2015 were reviewed to identify the clinical characteristics and surgical outcomes of young patients with AAA. The cohort included 14 patients aged 50 years or younger (mean, 40.4 years; young group) and 985 patients aged older than 50 years (mean, 72.8 years; old group). Results: Marfan syndrome, prior aortic dissection, and a history of aortic surgery were more prevalent in the young group, and 50% of the patients in the young group had dissecting aneurysms. All patients in the young group underwent open repair. Overall in-hospital mortality rates were 7.1% (1/14) and 1.9% (19/985) in the young and old groups, respectively (P=0.67). Seven-year survival and aortic event-free survival rates in the young group were 82.5%±11.5%, and 71.2±14.5%, respectively. Conclusion: AAA in patients aged 50 years or younger tended to be associated with Marfan syndrome, a history of aortic surgery, and prior aortic dissection. Early outcomes of AAA among young patients are acceptable, but close postoperative monitoring is important.

<p>A 76-year-old woman with a 2-week history of dyspnea on exertion was admitted to our hospital. A computed tomography scan showed a 70-mm diameter aortic arch aneurysm containing a large thrombus that was compressing the pulmonary artery. Echocardiography showed severe pulmonary stenosis and no shunt flow. Operative findings revealed an aneurysmal thrombus protruding into the lumen of the pulmonary artery through a foramen. A ductus arteriosus aneurysm was diagnosed. After the thrombus removal, arch replacement and ductus closure with a prosthetic patch were performed. Histological examination showed that the thrombus had no vascular components. The patient's symptoms were relieved, and she was discharged.</p>

Standard full median sternotomy for total aortic arch replacement in patients with tracheostomy has higher risks for mediastinitis and graft infection. To avoid surgical site infection, it is necessary to keep a sufficient distance between the tracheostomy and the site of surgical skin incision. We herein report a case of a 74-year-old man with permanent tracheostomy after total laryngectomy, who underwent total aortic arch replacement for an aneurysm. Antero-lateral thoracotomy in the 2nd intercostal space with lower partial sternotomy( ALPS approach) provided an enough distance between the tracheostomy and the surgical field. It also provided a good view for surgical procedure and enabled the standard setup of cardiopulmonary bypass with ascending aortic cannulation, venous drainage from the right atrium and the left ventricular venting through the upper right pulmonary vein. The operation was completed in 345 minutes and the patient was discharged on the 11th postoperative day without any complications.

We investigated the clinical picture of non-traumatic acute aortic dissection (AAD) occurring behind the wheel. Between 1990 and 2014, AAD had occurred in 11 patients while driving (nine men, mean age; 58.3 years, seven commercial drivers). The symptoms included chest and/or back pain (n = 9) and syncope (n = 2). One patient with syncope caused a traffic accident. Ten patients had type A dissection (DeBakey type I) and 1 type B dissection. In-hospital mortality was 9.9% (1/11). Our data showed if affected drivers are transported to a hospital in a timely fashion, a good surgical outcome can be expected.

A 74-year-old female patient experienced sudden and severe pain in her lower back and both legs. Upon examination, her femoral pulses were weak, and her legs were pale. Computed tomography revealed a 66-mm thoracic aneurysm in the distal arch and type B aortic dissection. Stenosis was present from the terminal aorta to the iliac arteries. The left common iliac artery was occluded at its bifurcation, and both lower limbs were ischemic. We performed bilateral axillary-femoral artery bypass, which improved blood flow to both limbs. The next day, it was apparent that compartment syndrome had developed in the patient's left leg. Rehabilitation therapy was effective for the compartment syndrome, the patient's symptoms resolved, and she was discharged. We later performed two-stage total arch replacement, and the subsequent clinical course has been without incident.

A 71-year-old woman presented with heart failure due to aortic and mitral valve regurgitation. She had developed midiastinitis and graft infection, 15 months before, following replacement of the ascending aorta for acute aortic dissection. Omentum flap operation had been performed and the infection had been controlled. This time, she underwent re-thoracotomy, and replacement of ascending aorta, aortic valve replacement and mitral valve plasty were performed. The omenal tissue was exfoliated without any damage to the heart or the great vessels by using an ultrasonic scalpel. As the omental tissue was viable, it was placed back in the mediastinal space.

We report a case of deep femoral artery (DFA) aneurysm associated with pre-vasculo-Behcet status. A 34-year-old man with a history of recurring oral and genital ulcers was admitted complaining of worsening left thigh pain over the previous 30 days. Computed tomography showed a left DFA aneurysm (60 mm × 70 mm), concomitant aneurysms in the popliteal and carotid arteries, and deep vein thrombosis. Active pre-vasculo-Behcet status was diagnosed, and DFA ligation was performed urgently. Remission was achieved with postoperative prednisolone and colchicine without vascular complications. DFA aneurysm and vascular pathologies were successfully managed by ligation surgery and medical therapy.

要旨：77 歳男性．急性A 型大動脈解離を認め，術前心タンポナーデに伴うショック状態であった．造影CT では，偽腔開存の大動脈解離を認め腹部分枝まで解離が及んでいた．緊急上行大動脈置換術を施行し，術中腸管虚血を疑う所見なく手術を終了した．術後10 時間で下血を認め緊急試験開腹手術を施行した．小腸に虚血を認めず，S 状結腸壊死を認め結腸切除を施行した．帰室後よりアシドーシスの進行と膀胱内圧軽度高値を認め，再度腸管虚血を疑い，2 回目の緊急試験開腹術を施行した．広範な小腸虚血を認めたが，切除範囲が広く切除を断念した．しかし保存的治療で腸管虚血・全身状態の改善を認め退院となった．可逆的な腸管虚血の診断は難しいが，広範な虚血のため虚血部腸管が切除できない症例でもその後虚血が回復することがあり，腸管虚血に対し試験開腹による腹腔内圧の減圧効果が治療に奏功した可能性がある．

We report a case of total occlusion of a Zenith bifurcated stent graft 16 months after implantation. A 72-year-old man was admitted to our hospital complaining of bilateral lower extremity numbness, followed by severe rest pain 4 h after sudden onset of symptoms. Computed tomography showed total occlusion of the endograft at the mid-portion of the main body. He underwent left axillobifemoral bypass using a reinforced polytetrafluoroethylene T-shaped graft, leading to resolution of symptoms 7 h after onset. Axillobifemoral bypass successfully relieved acute lower extremity ischemia caused by total occlusion of the abdominal aortic endograft.