Surgical treatment of chronic aortic dissection with liver dysfunction due to constrictive pericarditis caused by IgG4-related disease: a case report.

Background: Severe liver failure with ascites may be associated with cardiac disease and may be the primary manifestation of constrictive pericarditis or aortic dissection. We report a case of a patient with a chief complaint of ascites for whom close examination revealed that the liver injury was attributed to constrictive pericarditis and chronic aortic dissection, with immunoglobulin G4 (IgG4)-related disease (IgG4-RD) as the primary cause. Case summary: A 72-year-old man presented to the emergency department with scrotal oedema and ascites. Initially, the patient was hospitalized in the Department of Hepatology. However, computed tomography (CT) revealed aortic dissection (DeBakey type II), pericardial thickening, and impaired right ventricular dilatation. Therefore, we performed an ascending aortic replacement. IgG4 staining of the aortic wall revealed an IgG4/IgG-positive cell ratio of 35%. Pathological examination did not confirm the diagnosis of IgG4-related aortitis; however, the patient was diagnosed with IgG4-RD because of decreased blood IgG4 levels in response to steroid medication and the presence of heterogeneous thickened lesions in the pericardium. The patient took prednisolone 5 mg/day for 1 month post-operatively. His IgG4 level decreased but re-elevated above the baseline value after discontinuation of oral medication. Discussion: Liver cirrhosis was suspected given the ascites, although a CT scan on admission confirmed insufficiency of systemic circulation due to cardiac constrictive pericarditis with aortic dissection. Despite the complexity of various pathologies in this patient, collaborative efforts and effective communication within the medical team enabled successful aortic surgery, averting life-threatening complications.

Endovascular treatment of contained ruptured internal thoracic artery aneurysm mimicking a tumor in a patient with neurofibromatosis type 1: a case report.

BACKGROUND: An internal thoracic artery aneurysm (ITAA) is an exceedingly rare condition, with approximately two-thirds of reported cases being iatrogenic pseudoaneurysms. The remainder are attributed to various causes, including vasculitis, connective tissue disease, and neurofibromatosis type 1 (NF-1). NF-1 is an autosomal dominant disorder characterized by distinct clinical manifestations that occasionally include life-threatening vascular complications. Although NF-1 patients may develop various vascular abnormalities, ruptured ITAA is rarely reported, with only seven published cases. CASE PRESENTATION: A 32-year-old man with NF-1 consulted for a three-day history of persistent left back and upper arm pain. Initial chest radiography indicated left pleural effusion and an opacity at the left lung apex. Computed tomography scan revealed a mass in the left upper mediastinum that was initially suspected to be a tumor. Subsequent contrast-enhanced computed tomography revealed the mass to be a subclavian artery aneurysm. Detailed contrast-enhanced computed tomography with 1-mm slices was performed for surgical planning, identifying the mass as a left ITAA with contained rupture. Given the risk of re-rupture, emergency angiography was performed, which confirmed rupture of the left ITAA without extravasation. The ITAA was successfully treated with multiple microcoils at the proximal and distal ends. The patient had an uneventful recovery and was discharged on the fourth postoperative day. CONCLUSIONS: This case highlights the importance of considering vascular lesions in NF-1 patients who present with pleural effusion. It also emphasizes the challenges in diagnosing ITAA and the effectiveness of thin-slice contrast-enhanced computed tomography scans and endovascular treatment.

Cool-Shot Technique to Protect Spinal Cord during Thoracoabdominal Aortic Replacement.

Deep hypothermia helps protect the spinal cord, but is invasive. Here, we present a method to avoid reperfusion injury by selectively circulating cold blood under high pressure to the intercostal artery during reperfusion after intercostal artery reconstruction. Of the 23 patients who underwent thoracoabdominal aortic aneurysm open repair, one died. The motor evoked potential disappeared during aortic clamping in nine patients. Six patients recovered completely from aortic clamping release, two showed recovery >50% and one achieved full recovery 3 months later. Permanent motor impairment did not occur. This method could prevent reperfusion injury and paraplegia following thoracoabdominal aortic aneurysm surgery.

Long-Term Outcomes of Coronary Artery Bypass Grafting Using an Internal Thoracic Artery versus a Radial Artery as a Second Arterial Graft.

PURPOSE: To compare the outcomes of left circumflex artery (LCx) revascularization using an internal thoracic artery (ITA) or radial artery (RA) as the second arterial graft. METHODS: Patients who underwent primary isolated coronary artery bypass grafting with left anterior descending artery revascularization using an ITA and LCx revascularization using another bilateral ITA (BITA group) or an RA (ITA-RA group) were included. All-cause mortality (primary endpoint), cardiac death, major adverse cardiac events, in-hospital death, and deep sternal wound infection (secondary endpoints) were evaluated. RESULTS: Among 790 patients (BITA, n = 548 (69%); ITA-RA, n = 242 (31%)), no significant difference in all-cause mortality between the groups was observed (hazard ratio (HR): 0.87; 95% confidence interval (CI): 0.67-1.12; p = 0.27) during follow-up (mean, 10 years). Multivariate analysis revealed that the BITA group exhibited significantly lower rates of long-term all-cause mortality (HR: 0.63; 95% CI: 0.48-0.84; p = 0.01). In the propensity-matched cohort (n = 480, 240 pairs), significantly fewer all-cause deaths occurred in the BITA group (HR: 0.66; 95% CI 0.47-0.93; p = 0.02). There were no significant differences in secondary outcomes. CONCLUSIONS: When used as second grafts for LCx revascularization, ITA grafts may surpass RA grafts in reducing all-cause mortality 10 years postoperatively.

The Accessory Muscular Head of the Triceps Brachii Muscle with Tendinous Attachment / Cabeza Muscular Accesoria del Músculo Triceps Braquial con Inserción Tendinosa

SUMMARY: Variations in the triceps brachii muscle are uncommon, and especially limited reports exist on the accessory heads of tendinous origin that attach near the upper medial part of the humerus. During anatomical training at Nagasaki University School of Medicine, the accessory head of the triceps brachii muscle was observed on the right upper arm of a 72-year-old Japanese female. It arose tendinously from the medial side of the upper humerus, then formed a muscle belly and joined the distal side of the long head. This accessory head had independent nerve innervation, and the innervating nerve branched from a bundle of the radial nerve, which divided the nerve innervating the long head and the posterior brachial cutaneous nerve. The origin of the innervation of the accessory head was the basis for determining that this muscle head was an accessory muscle to the long head of the triceps brachii muscle. Embryologically, we discuss that part of the origin of the long head of the triceps brachii muscle was separated early in development by the axillary nerve and the posterior brachial circumflex artery, and it slipped into the surgical neck of the humerus and became fixed there. The accessory head crossed the radial nerve and deep brachial artery. When clinicians encounter compression of the radial nerve or profunda brachii artery, they should consider the presence of accessory muscles as a possible cause.

Las variaciones en el músculo tríceps braquial son poco comunes y existen informes especialmente limitados sobre las cabezas accesorias de origen tendinoso que se insertan cerca de la parte medial superior del húmero. Durante un entrenamiento anatómico en la Facultad de Medicina de la Universidad de Nagasaki, se observó la cabeza accesoria del músculo tríceps braquial en la parte superior del brazo derecho de una mujer japonesa de 72 años. Se originaba tendinosamente desde el lado medial de la parte superior del húmero, luego formaba un vientre muscular y se unía al lado distal de la cabeza larga. Esta cabeza accesoria tenía inervación nerviosa independiente, cuyo nervio se ramificaba a partir de un ramo del nervio radial, que dividía el nervio que inervaba la cabeza larga y el nervio cutáneo braquial posterior. El origen de la inervación de la cabeza accesoria fue la base para determinar que esta cabeza muscular era un músculo accesorio de la cabeza larga del músculo tríceps braquial. Embriológicamente, discutimos que parte del origen de la cabeza larga del músculo tríceps braquial se separó temprananamente en el desarrollo por el nervio axilar y la arteria circunfleja braquial posterior, y se deslizó hacia el cuello quirúrgico del húmero y quedó fijado allí. La cabeza accesoria cruzaba el nervio radial y la arteria braquial profunda. Cuando los médicos encuentran compresión del nervio radial o de la arteria braquial profunda, deben considerar la presencia de mús- culos accesorios como una posible causa.

Sternal closure with sandwiched three-piece bioresorbable mesh reduces postoperative hemorrhage: a retrospective study.

BACKGROUND: Median sternotomy is the most performed procedure in cardiac surgery; however, sternal displacement and bleeding remains a problem. This study aimed to investigate whether sternal reconstruction using a sandwiched three-piece bioresorbable mesh plate can prevent postoperative sternal displacement and bleeding more than a bioresorbable pin. METHODS: Patients (n = 218) who underwent median sternotomy were classified according to whether a sandwiched three-piece bioresorbable mesh plate and wire cerclage (group M, n = 109) or a bioresorbable pin and wire cerclage (group P, n = 109) were used during sternal reconstruction. The causes of postoperative sternal displacement and bleeding with computed tomography data were analyzed and compared between the groups. RESULTS: The preoperative patient characteristics did not significantly differ between the groups. However, the evaluation of sternal and substernal hematoma on postoperative day 5 using computed tomography showed sternal displacement in 4 (4%) and 22 (20%) patients, and substernal hematoma in 17 (16%) and 41 (38%) patients in groups M and P, respectively; this difference was significant. Furthermore, the amount of bleeding at 6 h postoperatively was lower in group M than in group P (235 ± 147 vs. 284 ± 175 mL, p = 0.0275). Chest reopening, intubation time, and length of intensive care unit and hospital stays did not differ between the groups. The evaluation of substernal hematoma based on computed tomography yielded a significantly lower for group M than for group P, revealing that the mesh plate was an independent predictor of substernal hematoma prevention. CONCLUSION: Sternal fixation with a three-piece bioresorbable mesh plate could prevent postoperative sternal displacement, bleeding, and substernal hematoma more than sternal fixation with a pin.

Left Atrial Appendage Closure During Coronary Bypass Surgery in Patients on Hemodialysis.

BACKGROUND: Patients with end-stage renal disease on hemodialysis (ESRD-HD) have a lifelong risk of atrial fibrillation-related stroke. We compared clinical outcomes in ESRD-HD patients undergoing coronary artery bypass grafting (CABG) with and without concomitant left atrial appendage (LAA) closure.Methods and Results: Of 2,783 consecutive patients undergoing isolated CABG between 2002 and 2020, 242 patients had ESRD-HD with sinus rhythm. The primary outcome was a composite of death and stroke. An inverse probability (IP)-weighted cohort was created based on the propensity score. The 2 IP-weighted groups had well-balanced baseline and surgical backgrounds, with an equivalent follow-up. Five-year stroke-free survival was significantly higher in patients with LAA closure (log-rank test, P=0.035). The adjusted hazard ratio of LAA closure for death and stroke was 0.43 (95% confidence interval [CI] 0.20-0.92; P=0.023). Competing risk analysis showed that LAA closure was significantly associated with a risk reduction of stroke (subhazard ratio 0.26; 95% CI 0.08-0.96; P=0.028). No significant difference was observed in adjusted risk ratios for reoperation for bleeding, new atrial fibrillation, 30-day mortality, and readmission for heart failure. CONCLUSIONS: Concomitant LAA closure during CABG can reduce the risk of death and stroke in ESRD-HD patients with normal sinus rhythm.

Management of cardiovascular surgery in patients with systemic lupus erythematosus including thromboembolism and multiple organ failure prevention: A retrospective observational study.

Systemic lupus erythematosus is a chronic autoimmune disease that affects most tissues. Cardiovascular events are critical, life-threatening, long-term complications of systemic lupus erythematosus (SLE). We report our single-center experience of performing cardiovascular surgery in patients with SLE while avoiding postoperative complications. We also suggest a new approach for cardiopulmonary bypass and perioperative management. We applied the antiphospholipid antibody syndrome (APS) severity classification published by the Japan Intractable Disease Information Center to patients with SLE for perioperative management. Patients with Grade III or higher severity are treated with a slightly relaxed version of catastrophic APS therapy. This treatment modality includes glucocorticoids, anticoagulation, intravenous immunoglobulin, and plasma exchange. Between April 2010 and January 2021, 26 patients (2 males, 24 females) with SLE underwent cardiovascular surgery. The mean age was 74.2 ± 13.0 years (38-84 years). The primary outcomes were in-hospital mortality and long-term results, and the secondary outcomes were related to bleeding/embolization and coagulation function/platelet count. A subset analysis was performed to examine treatment efficacy in the APS Grade III or higher group. Of the 26 patients, 17 underwent valve surgery, 4 underwent isolated coronary artery bypass grafting, and 5 underwent thoracic aortic aneurysm surgery. There were no in-hospital deaths or associated bleeding/embolic complications. Postoperative antithrombin III decreased in patients who underwent valvular and aortic surgery, and platelet counts recovered to preoperative levels within 7 to 10 days. The 5- and 10-year survival rates were 80.5% and 53.7%, respectively. In addition, there were 10 patients with APS Grade III or higher, but there was no significant difference in the frequency of complications other than platelet recovery after treatment. The surgical outcome of open-heart surgery in patients with SLE was good. Surgical treatment of cardiovascular disease in these patients is difficult and complex. We focused on blood coagulation abnormalities and treated each patient by selecting the best individual treatment protocol according to the severity of the disease, taking into account the risk of bleeding and thrombosis. Management of blood coagulation function in these patients is essential, and careful therapeutic management should be considered during open-heart surgery.

A case of Kleine-Levin syndrome arising during chemotherapy: efficacy of oral L-carnitine.

A woman in her 50 s with Kleine-Levin syndrome (KLS) was referred to our sleep clinic for recurrent episodes of sleep hypersomnia lasting for two to 3 days, despite attempts to remain awake. These episodes were unaccompanied by anxiety or depression, increased appetite, increased sex drive, irritability, or hallucinations, and had first appeared during chemotherapy for malignant lymphoma. Video polysomnography revealed mild obstructive sleep apnea syndrome (apnea-hypopnea index 7.9/h), but no other abnormalities. All blood tests and brain imaging investigations, including brain MRI and A 123I-ioflupane SPECT, yielded normal results. Oral L-carnitine was found to be effective for shortening the period of hypersomnolence, reducing the degree of hypersomnolence, and prolonging the inter-episode period. Since it has been reported that alkylating agents may induce carnitine deficiency, the present observations appear to support the involvement of carnitine in the onset of KLS.

Patterns and predictors of site-specific recurrence in cervical cancer after radical hysterectomy.

AIM: This study examines patterns and predictors of site-specific recurrence to explore the causes of local recurrence of cervical cancer. METHODS: Radical hysterectomy was performed in 121 patients (stage IB-IIB). Nerve-sparing was performed whenever possible. The first recurrence in local, regional, and distant areas was examined. We investigated the possibility of nerve involvement in local recurrence, focusing on paravaginal tissues containing the pelvic plexus. We provide Supporting Information on local recurrence in the paravaginal area. RESULTS: Local recurrence was an independent event from regional or distant recurrence. Local recurrence was seen only in high-risk patients, while regional and distant recurrences were not or less related to the risk category. The independent risk factors by logistic regression for local, regional, and distant recurrence were parametrial invasion, vaginal invasion, and lymph node metastasis, respectively. Local recurrence showed a comparable or more significant negative impact on survival than distant recurrence. Among seven patients with local recurrences, five had a recurrence in the paravagina. The rate of paravaginal recurrence was one in 76 early-stage and four in 45 locally advanced diseases. Four sites of paravaginal recurrence occurred on the nerve-sparing side and two on the non-nerve-sparing side. Supporting Information demonstrated histological evidence of perineural spread into the pelvic plexus and perineural invasion of the primary tumor. CONCLUSIONS: A high percentage of local recurrences are in paravaginal tissue containing the pelvic plexus. The causal association of nerve-sparing surgery and perineural invasion with local recurrence needs to be investigated in large prospective studies.

Two-stage aortic surgery for distal aortic arch and descending aorta aneurysms: A case report.

RATIONALE: Although surgical treatment strategies for patients with extensive thoracic aortic disease involving the aortic arch have improved considerably, the impact of stent graft length and placement site on aortic remodeling at long-term follow-up is not fully understood, and the protection of the Adamkiewicz artery (AKA) using the frozen elephant trunk (FET) method is also unclear. PATIENT CONCERNS: The patient was a 69-year-old man with diabetic nephropathy who became increasingly fatigued and started maintenance hemodialysis 6 months prior to admission. At 64 years, he underwent clipping of a right cerebellar artery aneurysm. In addition, a 1.8 cm aneurysm was found in the contralateral extracranial internal carotid artery. He also had an atrial septal defect and moderate aortic regurgitation and was receiving continuous positive airway pressure therapy for sleep apnoea syndrome. DIAGNOSIS: He had aneurysms in the aortic arch (4.8 cm in diameter) and descending aorta (6 cm in diameter), which was located at T6-9. Preoperative 3-dimensional computed tomography showed that the (AKA) bifurcated at T10-11. INTERVENTIONS: Considering the patient's several comorbidities and frailty, we planned to perform 1-stage extended aortic arch repair using the FET procedure. However, we performed 2-stage aortic surgery to prevent spinal ischemia, anticipating substantial cardiac enlargement and blood pressure instability due to dialysis treatment. Aortic valve replacement, atrial septal defect patch closure, and aortic arch surgery were performed. A 7-cm elephant trunk was inserted in the descending aorta. Postoperatively, the patient continued rehabilitation until his blood pressure stabilized during dialysis therapy. At postoperative week 4, he underwent thoracic endovascular aortic repair for a descending aortic aneurysm. OUTCOMES: After surgery, his physical strength decreased; however, he recovered and was discharged 1 month later without any complications. One year after the second operation, he is living a healthy life. LESSONS: Extensive aortic arch surgery using the FET procedure is effective for distal aortic arch and descending aortic aneurysms. Nevertheless, in cases in which the position of the AKA is close to the aortic aneurysm and blood pressure control is difficult, a 2-stage procedure and accurate positioning of thoracic endovascular aortic repair are both desirable.

Coronary Artery Bypass Grafting in Patients with Chronic Kidney Disease: Chronic Kidney Disease Has an Independent Adverse Effect on the Long-Term Outcome of Coronary Artery Bypass Grafting.

We examined short- and long-term outcomes of coronary artery bypass grafting (CABG) in patients with ischemic heart disease and the effect of renal function on these outcomes. We included 2783 patients who underwent primary elective CABG at a single institution between 2002 and 2020 (age: 67.6 ± 10.2 years; male: 2281 male). They were stratified based on their preoperative estimated glomerular filtration rate and underwent off-pump CABG (completion rate, 98.1%); 57.6% cases used bilateral internal thoracic arteries (BITA). In-hospital mortality rate was 1.0%. Logistic regression analysis revealed that low left ventricular function (<40%), but not chronic kidney disease (CKD) severity, was an independent predictive risk factor for postoperative hospital mortality. Significant differences existed in respiratory complications, infections, and hospitalization duration according to CKD severity. Deep sternal wound infection rate was 0.5%. The mean follow-up period was 7.1 (0-18.5) years. Estimated 10-year survival rates were negatively correlated with CKD severity; in the Cox hazard model, severe CKD was an independent predictor of long-term survival. We examined the relationship between preoperative and intraoperative factors and their effects on long-term survival using propensity score matching by dividing the renal function severity into G1-2 and G3-5. Renal function severity, age, and operative time were independent risk factors. No prognostic improvement was observed with BITA grafts; graft patency was superior in the right internal thoracic artery (52/52; 100%) than in the great saphenous vein (48/59; 81.4%) in G5. Post-CABG in-hospital mortality was unrelated to renal function, but CKD severity strongly influenced long-term survival. Operation time was an important predictor of long-term prognosis in patients with impaired renal function. Treatment plans, including graft and anastomosis-site selections, should be designed to shorten the operation time. In conclusion, using the right internal thoracic artery in CABG is more beneficial in patients with CKD and cardiovascular comorbidities.

Surgical treatment of acute aortic dissection in a patient with SLE and prior antiphospholipid syndrome on therapy for over 30 years: a case report.

BACKGROUND: In patients with systemic lupus erythematosus (SLE), lengthy treatment and long-term steroid use are the main risk factors for developing aortic aneurysms or aortic dissections. In patients with cardiac tamponade, hemodynamic collapse may lead to acute renal and hepatic failure. CASE PRESENTATION: We report the successful treatment of a 55-year-old woman with SLE since the age of 21. She suddenly felt chest pain approximately 2 weeks before developing fever and vomiting and was admitted to our hospital. Initially, she had severe liver dysfunction and was admitted to the hepatology department, where treatment for fulminant hepatitis was initiated. However, computed tomography (CT) showed an acute aortic dissection (DeBakey type II) and severe bloody pericardial effusion. Therefore, we performed emergency pericardial drainage. Plasma exchange therapy was initiated as emergency aortic surgery was deemed impossible due to impaired liver function tests and coagulation. Ten days later, the patient developed peritonitis due to small bowel perforation, and laparotomy was performed for abscess drainage and perforation closure. She had received steroid pulse therapy at the age of 21. At 40 years of age, she developed deep vein thrombosis due to antiphospholipid antibodies and was prescribed prednisolone. She was ambulatory at 3 months after the onset of acute aortic dissection, and CT revealed a rapidly enlarging true aneurysm in the distal arch. We performed elective aortic surgery. Although there were no antiphospholipid antibodies, surgery could have led to a devastating antiphospholipid syndrome. Therefore, we decided to treat the patient with triple therapy. Methylprednisolone was intravenously administered intraoperatively and at 1 day postoperatively. The patient was discharged without complications after returning to her usual oral prednisolone regimen. CONCLUSIONS: The patient described herein had a systemic circulatory failure due to cardiac tamponade, accompanied by liver failure. This condition is a significant cause of death in patients with aortic dissection-associated SLE and is extremely dangerous. However, multi-specialty intervention helped the patient recover, and she has been attending the outpatient clinic. Aortic surgery requiring hypothermia in SLE patients with antiphospholipid syndrome and a history of thrombocytopenia or thrombosis requires a multi-disciplinary treatment team, including cardiac surgeons and medical experts.

Pseudoaneurysm with a fistula to the right ventricle late after surgical repair of type A aortic dissection in a patient with systemic lupus erythematosus.

BACKGROUND: Pseudoaneurysm with a shunt to the right ventricle after aortic repair for acute aortic dissection is an extremely rare and life-threatening condition. Surgical treatment is unavoidable, but surgery is complicated, and there are some pitfalls. This study describes the reoperation performed in a patient at a high surgical risk by clarifying the shunt site using multimodality imaging before surgery. CASE PRESENTATION: A 69-year-old woman with a history of systemic lupus erythematosus (SLE) and Sjogren's syndrome presented with a pseudoaneurysm 1 year after emergency surgery for acute type A aortic dissection. Eight years after the first surgery, she experienced sudden chest pain and presented to the emergency department. Her dyspnea worsened; therefore, echocardiography and three-dimensional computed tomography (3DCT) were performed, and a pseudoaneurysm and shunt to the right ventricle were identified. The medical team attempted to close the shunt with a percutaneous catheter but was unsuccessful, and she was referred to our department for surgical treatment. The pseudoaneurysm originating from the proximal side of the aorta was large (diameter = 55 mm), and echocardiography-gated 3DCT identified the shunt from the pseudoaneurysm to the right ventricle. First, extracorporeal circulation was initiated, and resternotomy was performed. We could not insert the left ventricular venting tube from the right side because of the pseudoaneurysm size. Instead, the tube was inserted from the left atrial appendage. We found a half-circumferential disengaged anastomosis around the proximal anastomosis, which formed the large pseudoaneurysm leading to a fistula in the right ventricle. We closed the fistula and performed a Bentall operation. The patient had a good postoperative course and was discharged on postoperative day 21. She continued treatment for SLE and Sjogren's syndrome, and her inflammatory reaction improved. CONCLUSIONS: We performed a Bentall operation and fistula closure with resternotomy in a patient with type A aortic dissection with SLE and Sjogren's syndrome. Multimodal imaging is essential in defining the pseudoaneurysm and the fistula surrounding the anatomy while ensuring their resolution and guiding the approach for operation.

Double-sided superior vena cava: developmental considerations associated with the thymic veins / Vena cava superior de doble cara: consideraciones de desarrollo asociadas con las venas tímicas

SUMMARY: The superior vena cava is usually located only on the right side, but persistence of the left superior vena cavais observed in about 0.3 to 0.5 % of adults. A routine dissection of the cadaver of a 91-year-old Japanese female, whose cause of death was sepsis due to cholecystitis, was performed at Nagasaki University and revealed a double-sided superior vena cava. On the right side, the superior vena cava opened to the right atrium, while on the left, it opened into the extended coronary sinus. Veins in the left head, neck and upper limb regions joined to form the persistent left superior vena cava, with eventual drainage into the expanded coronary vein. An anastomosing branch occurred between each superior vena cava, and two thymic veins opened to the anastomosing branch. The azygos vein in the azygos venous system opened into the right superior vena cava, whereas a hemi-azygos vein opened into the azygos vein. The accessory hemi-azygos vein also opened into the azygos vein and opened cranially into the left superior vena cava. The left supreme intercostal vein also opened into the left superior vena cava. Several studies have reported a persistent left superior vena cava and the various considerations for its occurrence. Here, we propose a new hypothesis for the embryonic development of the persistent left superior vena cava with the thymic vein. This hypothesis essentially states that the left brachiocephalic vein fails to mature due to inadequate venous return from the thymic vein during the embryonic period, and the left superior vena cava then remains to maintain venous return from the left head, neck and upper limb. We also discuss the clinical significance of the persistent left superior vena cava.

RESUMEN: Usualmente la vena cava superior se localiza solo en el lado derecho, sin embargo en aproximadamente 0,3 a 0,5 % de los adultos se observa la persistencia de la vena cava superior izquierda. En la Universidad de Nagasaki se realizó una disección de rutina del cadáver de una mujer japonesa de 91 años, cuya causa de muerte fue sepsis debido a una colecistitis. El cuerpo presentaba una vena cava superior doble. En el lado derecho, la vena cava superior llegaba al atrio derecho, mientras que en el lado izquierdo drenaba al seno coronario. Las venas de las regiones de la cabeza, el cuello y del miembro superior izquierdo formaban la vena cava superior izquierda persistente, con drenaje hacia la vena coronaria. Se observó una rama anastomótica entre cada vena cava superior y dos venas tímicas drenaban a la rama anastomótica. La vena ácigos drenaba a la vena cava superior derecha, mientras que una vena hemiácigos drenaba a la vena ácigos. La vena hemiácigos accesoria también drnaba en la vena ácigos y cranealmente lo hacia la vena cava superior izquierda. La vena intercostal suprema izquierda drenaba en la vena cava superior izquierda. Varios estudios han informado una vena cava superior izquierda persistente y las diversas consideraciones para su aparición. Aquí, proponemos una nueva hipótesis para el desarrollo embrionario de la vena cava superior izquierda persistente con la vena tímica, que esencialmente establece que la vena braquiocefálica izquierda no se dearrolla debido a un retorno venoso inadecuado de la vena tímica durante el período embrionario, y se mantiene la vena cava superior izquierda para el retorno venoso de la cabeza, el cuello y el miembro superior izquierdo. Además se informa de la importancia clínica de la persistencia de la vena cava superior izquierda.

Evidence and Challenges in Left Atrial Appendage Management.

This review aimed to discuss the anatomical properties of the left atrial appendage (LAA), its relationship with atrial fibrillation (AF), effectiveness of LAA occlusion (LAAO), techniques, and new devices used to perform this procedure. An electronic search was performed to identify studies, in the English language, on LAA management. Searches were performed on PubMed Central, Scopus, and Medline from the dates of database inception to February 2020. For the assessed papers, data were extracted from the reviewed text, tables, and figures, by two independent authors. Anticoagulant therapy for patients with AF has proven beneficial and is highly recommended, but it is challenging for many patients to maintain optimal treatment. Surgery is the most cost-effective option; surgical methods include simple LAA resection, thoracoscopic surgery, and catheter treatment. Each procedure has its advantages and disadvantages, and many prospective studies have been conducted to evaluate various treatment methods. In managing the LAA, dissection of the LAA, such as changes in its shape and size due to remodeling during AF, changes in autonomic nerve function, and thrombosis, must be understood anatomically and physiologically. We believe that early treatment intervention for the LAA should be considered particularly in cases of recurrent AF.

Surgical 5-year Outcomes of Extra-Anatomical Bypass for Middle Aortic Syndrome: A Case Series.

BACKGROUND: Middle aortic syndrome is a rare disease. Several surgical treatments are available; however, the optimal treatment strategy and long-term outcomes remain unelucidated. We herein report the 5-year outcomes of six patients treated with extra-anatomical bypass surgery for middle aortic syndrome. CASE PRESENTATIONS: Between 2013 and 2016, six patients underwent extra-anatomical bypass for middle aortic syndrome at our institute: three had Takayasu's arteritis, one had vessel vasculitis, and two had middle aortic hypoplastic syndrome of unknown origin. The patients included five women and one man, with a mean age of 59.7 years. Four patients had uncontrolled hypertension and were receiving antihypertensive medications. The mean ankle-brachial pressure index was .61. The three patients with Takayasu's arteritis were hospitalized for congestive heart failure. These patients underwent bypass surgery from the descending aorta to the infrarenal abdominal aorta, and one also underwent concomitant heart surgery. The patient with microscopic polyangiitis underwent Y-grafting with an aortic aneurysmectomy. Subsequently, bypass surgery was performed from the descending aorta to the graft via the diaphragm. The two patients with unknown causes underwent bypass surgery from the proximal descending aorta to the distal descending thoracic aorta. There were no early or late deaths at the 5-year follow-up. We did not observe any changes in anastomotic site stenosis or new aneurysmal changes during the follow-up period. The number of antihypertensive medications was reduced in all cases, and critical symptoms, including headache, severe abdominal pain, claudication, and heart failure, improved in all patients. The ankle-brachial pressure index increased to 1.11 and did not change for five years. Renal function remained stable, and the brain natriuretic peptide level decreased from 302.8 to 74.5 pg/mL at follow-up. CONCLUSION: Extra-anatomical bypass for middle aortic syndrome is safe and effective, and can help prevent renal failure, and relieve critical ischemic symptoms.

Atypical Shone's Complex Diagnosed at 70 Years Old: Presenting with Double-orifice Mitral Valve, Bicuspid Aortic Valve, and Aortic Coarctation.

Atypical Shone's complex is a rare congenital anomaly involving a left-sided obstructive lesion of two or three cardiovascular levels. A 70-year-old man with dyspnea on exertion was diagnosed with severe aortic stenosis (AS) with a bicuspid valve, complicated by severe aortic coarctation (CoA) and a double-orifice mitral valve. He underwent surgery for AS and CoA in one session. It is important to search for complicated malformations, even in cases of bicuspid aortic valve found in old age.

Significant Asymmetry of the Bilateral Upper Extremities of a Skeleton Excavated from the Mashiki-Azamabaru Site, Okinawa Island, Japan.

The human skeleton of a young adult male with marked asymmetry of the bilateral upper extremities was excavated from the Mashiki-Azamabaru site (3000-2000 BCE) on the main island of Okinawa in the southwestern archipelago of Japan. The skeleton was buried alone in a corner of the cemetery. In this study, morphological and radiographic observations were made on this skeleton, and the pathogenesis of the bone growth disorder observed in the left upper limb was discussed. The maximum diameter of the midshaft of the humerus was 13.8 mm on the left and 21.2 mm on the right. The long bones comprising the left upper extremity lost the structure of the muscle attachments except for the deltoid tubercle of the humerus. The bone morphology of the right upper extremity and the bilateral lower extremities was maintained and was close to the mean value of females from the Ohtomo site in northwestern Kyushu, Japan, during the Yayoi period. It is assumed that the anomalous bone morphology confined to the left upper extremity was secondary to the prolonged loss of function of the muscles attached to left extremity bones. In this case, birth palsy, brachial plexus injury in childhood, and acute grey matter myelitis were diagnosed. It was suggested that this person had survived into young adulthood with severe paralysis of the left upper extremity due to injury or disease at an early age.