Safety and outcomes of surgical treatment of atrial fibrillation in emergency surgery cases.

BACKGROUND: Recent developments in surgical devices, including left atrial appendage closure, have enabled surgeons to perform aggressive operations for atrial fibrillation (AF). However, the outcomes of AF surgery in emergent cases have not been extensively studied. OBJECTIVE AND METHODS: The present study aimed to investigate the effectiveness of AF surgery in emergency surgery cases associated with cardiovascular events. We enrolled 18 patients who underwent various types of AF surgery due to emergencies, including acute aortic dissection (n = 6), acute myocardial infarction (n = 5), bleeding due to perforation from radiofrequency catheter ablation (n = 4), acute mitral regurgitation (n = 2), and cardiac tumor (n = 1). Four and ten patients underwent the full maze procedure and pulmonary vein isolation, respectively. Ganglionated plexi ablation was also performed in three patients as part of a combined procedure. The left atrial appendage was solely closed in four patients. RESULTS: There was no surgical mortality or major adverse cardiac and cerebrovascular events in our patient series. The rates of freedom of recurrence of AF or atrial tachycardia at 1 and 3 years were 92.9% and 82.5%, respectively. After a mean follow-up period of 46.7 ± 25.8 months, no thromboembolism events were observed in the patients. Furthermore, no cardiovascular death was recorded. CONCLUSION: The surgical procedures for AF are safe and effective in cases requiring emergency surgery.

Nephrotic syndrome with acute kidney injury due to combination therapy of immune checkpoint inhibitors: a case report and review of the literature.

BACKGROUND: Recent studies have focused on immune checkpoint inhibitors. Renal complications associated with the use of immune checkpoint inhibitors are uncommon compared with other immune-related adverse events. Acute interstitial nephritis accounts for most of these renal complications, with nephrotic syndrome quite rare. We herein report a case of nephrotic syndrome associated with immune checkpoint inhibitors that was more severe than that in previous cases. By comparing this case with previous reports, the possible reasons for the particular severity of this case are discussed. CASE PRESENTATION: A 75-year-old man developed nephrotic syndrome with acute kidney injury after the first combination therapy of nivolumab and ipilimumab for malignant pleural mesothelioma. The results of a kidney biopsy indicated minimal change disease with mild atherosclerosis, acute interstitial nephritis, and fusion of nearly all podocyte foot processes. Nivolumab and ipilimumab therapy were stopped, and treatment with corticosteroids was initiated. We investigated previously reported cases of nephrotic syndrome using immune checkpoint inhibitors. Seventeen cases of immune checkpoint inhibitor-related nephrotic syndrome, including ours, have been reported. Two of the 17 patients with immune checkpoint inhibitor-related nephrotic syndrome required hemodialysis treatment for acute kidney injury. Unlike many previously reported cases, the present patient was administered two different immune checkpoint inhibitors, which may be one of the reasons for the development of severe nephrotic syndrome. CONCLUSIONS: In addition to previously reported risk factors, immune checkpoint inhibitor combination therapy can exacerbate nephrotic syndrome compared to immune checkpoint inhibitor monotherapy.

A case of carcinoid syndrome probably exacerbated by hemodialysis in which prochlorperazine maleate was effective.

Carcinoid syndrome is caused by the release of serotonin and other substances, which commonly occurs due to liver metastasis of neuroendocrine tumors. It rarely occurs due to liver metastasis of neuroendocrine carcinoma. We report the case of a patient with liver metastasis of neuroendocrine carcinoma who suffered from acute abdominal pain and diarrhea triggered by hemodialysis. Various differential diagnoses were considered, but we concluded these symptoms to be probably caused by exacerbation of carcinoid syndrome, as the serum 5HIAA level was markedly elevated, and a drug with anti-serotonin activity was effective. Prochlorperazine maleate, which has anti-serotonin activity, was effective for these symptoms, and the patient was able to continue maintenance hemodialysis, which contributed to his quality of life and prognosis. We speculated the mechanism of carcinoid exacerbation was that substances such as serotonin had entered the systemic circulation via the increased extrahepatic shunt of the portal venous blood flow, entering the inferior vena cava and that this condition had been triggered by hemodialysis via the same mechanism as portal systemic encephalopathy.

Severe Left Ventricular Failure Unmanageable by Impella 5.0.

Mechanical circulatory support can be beneficial for patients with cardiogenic shock. Of these, the Impella has recently become the first-line device due to its feasibility, minimal invasiveness, and efficacy. We had a 58-year-old male with acute myocardial infarction followed by cardiogenic shock. We initially placed the patient on intra-aortic balloon pumping, which was switched to Impella 2.5 and could stabilize him. Unfortunately, the Impella 2.5 device suddenly stopped on the fifth day, thus, we tried to manage him by inotropes. However, his condition gradually deteriorated, so we applied Impella 5.0. Although his systemic circulation could be maintained, severe pulmonary hypertension persisted on Impella 5.0. He developed flash pulmonary edema, thus, we emergently added venoarterial extracorporeal membrane oxygenation on Impella 5.0 (ECPELLA). Then, we removed Impella 5.0 and changed peripheral venoarterial extracorporeal membrane oxygenation to central venoarterial extracorporeal membrane oxygenation. In this central venoarterial extracorporeal membrane oxygenation, we inserted the cannulas in the pulmonary artery and the left ventricle in addition to the usual cannulas in the ascending aorta and the right atrium. We aimed to control pulmonary arterial blood flow for lung protection as well as left ventricular unloading by this modification. However, his cardiac function showed no signs of recovery, and his lung condition showed further exacerbation. He was complicated by fungal sepsis and finally died of multi-organ failure. Although the Impella is an option, it is crucial to evaluate patients' condition carefully and to escalate the device, if needed, without delay.

High-flow arteriovenous fistula in X-linked Alport syndrome: a case report.

Most male X-linked Alport syndrome patients with COL4A5 nonsense mutations experience end-stage kidney failure by 30 years old. Although there is no definition of high-flow arteriovenous fistula, access blood flows greater than 2000 mL/min might predict the occurrence of high-output heart failure. A 50-year-old Japanese man had suffered from proteinuria at 4 years old and sensorineural hearing loss and a lenticular lens at 20 years old. He had started to receive hemodialysis treatment due to end-stage kidney disease at 22 years old. A genetic test confirmed a novel hemizygous nonsense variant COL4A5 c.2980G > T (p.Gly994Ter), and he was diagnosed with X-linked Alport syndrome. COL4A5 c.2980G > T was considered "pathogenic" according to the American College of Medical Genetics and Genomics guidelines and in vitro experiments. Shortness of breath on exertion was exaggerated, his brachial artery blood flow was over 4,236-4,353 mL/min, his cardiac output was 5,874 mL/min, and he needed radial artery banding at 51 years old. After radial artery banding surgery, the brachial artery blood flow decreased to 987-1,236 mL/min, and echocardiography showed a cardiac output at 5100 mL/min with improved E' and E/E'. His shortness of breath on exertion improved gradually. Although rare, high-output heart failure due to high-flow arteriovenous fistula should be kept in mind as a complication in X-linked Alport syndrome patients, and our patient was successfully treated with radial artery banding surgery.

Discordance of light chain isotypes between serum and glomerular deposits in proliferative glomerulonephritis with monoclonal IgG deposits: a case report and review of the literature.

BACKGROUND: Proliferative glomerulonephritis with monoclonal immunoglobulin G (IgG) deposits (PGNMID) is a disease entity with nonorganized granular glomerular deposition with monoclonal proteins of both heavy and light chains. Dysproteinemia was observed in only 30% of the patients with PGNMID. We herein report a case of PGNMID with discrepancy between serum and glomerular deposits. CASE PRESENTATION: The patient was a 50-year-old man who had been followed at a local clinic due to hypertension, type 2 diabetes, hyperlipidemia, hyperuricemia, fatty liver, and obesity. Proteinuria had been noted five years previously, and he had been referred to a hematology department due to hyperproteinemia, high gamma globulin, and κ Bence-Jones protein (BJP) positivity one year previously. Bone marrow aspiration showed 5% plasma cells, and he was referred to the nephrology department to evaluate persistent proteinuria. He was hypertensive, and his estimated glomerular filtration rate was 54.2 ml/min/1.73 m2. His urinary protein level was 0.84 g/g⋅Cr. Urine and serum immunofixation showed BJP-κ type and IgG-κ type, respectively. Kidney biopsy showed an increase in mesangial cells and matrix without nodular lesions under a light microscope. Immunofluorescence microscopy showed granular deposits of IgG and C3 on the capillary wall and weak positivity for C1q. IgG3 was predominant among the IgG subclasses, and intraglomerular κ and λ staining was negative for κ and positive for λ. Direct fast scarlet staining was negative. Electron microscopy showed lumpy deposits without a fibrillar structure in the subepithelial area. Based on the above findings, a diagnosis of membranous nephropathy-type PGNMID was made. Since proteinuria increased gradually after three years of treatment with valsartan (40 mg, daily), oral prednisolone (30 mg, daily) was initiated, which led to decreased proteinuria. The dose of oral prednisolone was gradually tapered to 10 mg per day. At that time, proteinuria was 0.88 g/g⋅Cr. We found 204 cases in 81 articles in the PubMed database, among which 8 showed discrepancy in the heavy and/or light chains between serum and kidney. CONCLUSIONS: We experienced a case of membranous nephropathy-type PGNMID with discrepancy in light chains between serum and kidney that was successfully treated with oral prednisolone.

Multiple cardiac surgical procedures in a case of an octogenarian with essential thrombocythemia.

BACKGROUND: Essential thrombocythemia (ET) is a chronic myeloproliferative disorder characterized by an elevation of platelet counts with a tendency for thrombosis and hemorrhage. The perioperative management of cardiovascular surgery of an ET patient is complicated. There is limited literature on the perioperative management of patients with ET undergoing cardiovascular surgery, particularly those requiring multiple procedures. CASE PRESENTATION: An 85-year-old woman with a history of essential thrombocythemia (ET), which resulted in an abnormally high platelet count, was diagnosed with aortic valve stenosis, ischemic heart disease and paroxysmal atrial fibrillation. She underwent aortic valve replacement, coronary artery bypass grafting, and pulmonary vein isolation. The postoperative course was uneventful, nor hemorrhage and thrombosis. CONCLUSIONS: We represent a case of perioperative management and successful treatment of three combined cardiac surgery for an octogenarian ET patient who is the oldest case ever reported.

A Case of Contained Rupture of the Common Iliac Artery with Idiopathic Retroperitoneal Fibrosis: Efficacy of Surgical Treatment and Immunosuppressive Therapy at 2-Year Follow-Up.

Rupture of inflammatory aortic aneurysm associated with retroperitoneal fibrosis (RF) is rare. We report a 62-year-old man with an inflammatory abdominal aortic aneurysm (IAAA) complicated with idiopathic RF, resulting in a contained rupture of the common iliac artery. The patient also presented with mild renal insufficiency due to urethral obstruction and left hydronephrosis. Surgical procedures including graft replacement and ureterolysis relieved the symptoms. Postoperative immunosuppressive treatment using corticosteroid and methotrexate successfully maintained clinical remission without signs of recurrence of RF and IAAA at the 2-year follow-up.

Mutation Analysis of Autosomal-Dominant Polycystic Kidney Disease Patients.

Autosomal-dominant polycystic kidney disease (ADPKD) is characterized by bilateral kidney cysts that ultimately lead to end-stage kidney disease. While the major causative genes of ADPKD are PKD1 and PKD2, other genes are also thought to be involved. Fifty ADPKD patients were analyzed by exome sequencing or multiplex ligation-dependent probe amplification (MLPA), followed by long polymerase chain reaction and Sanger sequencing. Variants in PKD1 or PKD2 or GANAB were detected in 35 patients (70%). Exome sequencing identified 24, 7, and 1 variants in PKD1, PKD2, and GANAB, respectively, in 30 patients. MLPA analyses identified large deletions in PKD1 in three patients and PKD2 in two patients. We searched 90 cyst-associated genes in 15 patients who were negative by exome sequencing and MLPA analyses, and identified 17 rare variants. Four of them were considered "likely pathogenic" or "pathogenic" variants according to the American College of Medical Genetics and Genomics guidelines. Of the 11 patients without a family history, four, two, and four variants were found in PKD1, PKD2, and other genes, respectively, while no causative gene was identified in one patient. While the pathogenicity of each variant in these genes should be carefully assessed, a comprehensive genetic analysis may be useful in cases of atypical ADPKD.

Bullous Pemphigoid in X-linked Alport Syndrome.

Skin lesions in X-linked Alport syndrome (XLAS) are rarely observed. Bullous pemphigoid (BP) is caused by autoantibodies against BP180, also called α1 (XVII) chain, in the basement membrane zone (BMZ). A 48-year-old man with XLAS developed tense blisters. A skin biopsy showed a cleft between the basal cell layer and dermis, with the infiltration of neutrophils and eosinophils. α1 (XVII) staining was positive on the epidermal side of α2/5 (IV) staining. Oral prednisolone improved his symptoms gradually. Abundant tense blisters on the palms and soles might suggest an important role of the α5 (IV) chain in the integrity of BMZ.

Dexmedetomidine for dyspnoea.

Dexmedetomidine is a selective α2-adrenoreceptor agonist with a broad range of effects, including easily controllable sedation, analgesia and anxiolysis. Because of these favorable features, it has replaced traditional sedatives, such as benzodiazepines, and is becoming the first-line sedative for the patients in intensive care units. Terminally ill patients often need sedatives for symptom management, especially for dyspnoea. However, the use of dexmedetomidine in a palliative care setting has rarely been recognised to date. We experienced a patient nearing the end of life due to uncontrollable pulmonary haemorrhage on ventilator, whose dyspnoea was successfully managed by dexmedetomidine in addition to continuous intravenous infusion of oxycodone.

Three Weeks of Treatment Induced Long-term Remission in a Patient with Micrococcus luteus-related Peritonitis.

Micrococcus luteus can cause relapsing and refractory peritoneal dialysis infection because it leads to strong biofilm formation. A 69-year-old woman who had undergone peritoneal dialysis (PD) visited the emergency department complaining of cloudy peritoneal dialysate. She was initially given intraperitoneal cefazolin (1 g/day) and ceftazidime (1 g/day). Micrococcus luteus was detected in a culture test. Thus, ceftazidime was discontinued. She remained disease-free for 22 months until she developed PD-related peritonitis. We administered antibiotics for 21 days and thereafter identified 2 important clinical issues. Micrococcus species-related peritonitis can sometimes be cured without vancomycin. Furthermore, the provision of three weeks of sufficient treatment may be important.

Surgical Management of Cardiac Implantable Electronic Device Complications in Patients Unsuitable for Transvenous Lead Extraction.

BACKGROUND: Although surgical approaches for infected or failing cardiac implantable electronic device (CIED) leads are more invasive than transvenous approaches, they are still required for patients considered unsuitable for transvenous procedures. In this study, surgical management with transvenous equipment for CIED complications was examined in patients unsuitable for transvenous lead extraction.Methods and Results: We retrospectively examined 152 consecutive patients who underwent CIED extraction between April 2009 and December 2021 at the Department of Cardiovascular Surgery, Nippon Medical School. Nine patients (5.9%; mean [±SD] age 61.7±16.7 years) who underwent open heart surgery were identified as unsuitable for the isolated transvenous approach. CIED types included 5 pacemakers and 4 implantable cardioverter-defibrillators; the mean [±SD] lead age was 19.5±7.0 years. Indications for surgical management according to Heart Rhythm Society guidelines included failed prior to transvenous CIED extraction (n=6), intracardiac vegetation (n=2), and severe lead adhesion (n=1). Transvenous CIED extraction tools were used in all patients during or before surgery. Additional surgical procedures with CIED extraction included epicardial lead implantation (n=4) and tricuspid valve repair (n=3). All patients were discharged; during the follow-up period (mean 5.7±3.7 years), only 1 patient died (non-cardiac cause). CONCLUSIONS: Surgical procedures and transvenous extraction tools were combined in the removal strategy for efficacious surgical management of CIED leads. Intensive surgical procedures were safely performed in patients unsuitable for transvenous extraction.

Detection of Trace Amounts of Water in Organic Solvents by DNA-Based Nanomechanical Sensors.

The detection of trace amounts of water in organic solvents is of great importance in the field of chemistry and in the industry. Karl Fischer titration is known as a classic method and is widely used for detecting trace amounts of water; however, it has some limitations in terms of rapid and direct detection because of its time-consuming sample preparation and specific equipment requirements. Here, we found that a DNA-based nanomechanical sensor exhibits high sensitivity and selectivity to water vapor, leading to the detection and quantification of trace amounts of water in organic solvents as low as 12 ppm in THF, with a ppb level of LoD through their vapors. Since the present method is simple and rapid, it can be an alternative technique to the conventional Karl Fischer titration.

Evaluation of bleeding and anticoagulation markers by edoxaban and low-dose cyclosporine: A case series study.

It was reported that high-dose cyclosporine at 500 mg daily increases edoxaban exposure. We investigated whether cyclosporine <500 mg daily leads to edoxaban-induced bleeding in the clinical setting. This case series study included patients receiving edoxaban and cyclosporine at Mie University Hospital. The outcomes were bleeding and anticoagulant markers, including activated partial thromboplastin time (APTT), prothrombin time (PT), and the international normalized ratio of prothrombin time (PT-INR). We examined the genotypes of cytochrome P450 3A5 (CYP3A5), multidrug resistance 1 (ABCB1), and solute carrier organic anion transporter 1B1 (SLCO1B1). Trends in anticoagulant markers were analyzed. Thirteen patients received edoxaban (standard dose; n = 3 and reduced dose; n = 10) and cyclosporine (1.94 ± 1.42 mg/kg). A bleeding event occurred in one patient receiving a standard dose of edoxaban plus cyclosporine of 25 mg daily (HAS-BLED score of 2 and genotypes; CYP3A5*3/*3, ABCB1 3435CT, and SLCO1B1*1a/*1b). After edoxaban treatment, anticoagulant markers were prolonged (APTT; 27.95 ± 3.64 s vs. 31.11 ± 3.90 s, p < 0.001, PT; 11.53 ± 1.01 s vs. 13.03 ± 0.98 s, p = 0.002, PT-INR; 0.98 ± 0.09 vs. 1.11 ± 0.11, p = 0.007). In summary, the genotypes of CYP3A5, ABCB1, and SLCO1B1 and the dosage of edoxaban may affect the risk of bleeding by edoxaban when co-administered with cyclosporine, even at low doses.

Mutation Analysis of Thin Basement Membrane Nephropathy.

Thin basement membrane nephropathy (TBMN) is characterized by the observation of microhematuria and a thin glomerular basement membrane on kidney biopsy specimens. Its main cause is heterozygous mutations of COL4A3 or COL4A4, which also cause late-onset focal segmental glomerulosclerosis (FSGS) or autosomal dominant Alport syndrome (ADAS). Thirteen TBMN cases were analyzed using Sanger sequencing, multiplex ligation-dependent probe amplification (MLPA), and exome sequencing. Ten heterozygous variants were detected in COL4A3 or COL4A4 in nine patients via Sanger sequencing, three of which were novel variants. The diagnostic rate of "likely pathogenic" or "pathogenic" under the American College of Medical Genetics and Genomics guidelines was 53.8% (7 out of 13 patients). There were eight single nucleotide variants, seven of which were glycine substitutions in the collagenous domain, one of which was a splice-site single nucleotide variant, and two of which were deletion variants. One patient had digenic variants in COL4A3 and COL4A4. While MLPA analyses showed negative results, exome sequencing identified three heterozygous variants in causative genes of FSGS in four patients with no apparent variants on Sanger sequencing. Since patients with heterozygous mutations of COL4A3 or COL4A4 showed a wide spectrum of disease from TBMN to ADAS, careful follow-up will be necessary for these patients.

Perforation of the right atrial appendage during implantation of a leadless pacemaker.

We report a case of perforation of the right atrial appendage during implantation of a leadless pacemaker in a 94 years old woman. We performed emergency surgery to repair the perforation site. To our konwledge, there are few reports of right atrial perforation during a leadless pacemaker indwelling.

Disruption of the glomerular basement membrane associated with nutcracker syndrome and double inferior vena cava in Noonan syndrome: a case report.

BACKGROUND: Nutcracker syndrome (NCS) is characterized by compression of the left renal vein (LRV) between the aorta and the superior mesenteric artery. While rare, NCS was reported to be accompanied by double inferior vena cava (IVC). We herein report a case of Noonan syndrome (NS) with double IVC who presented with macrohematuria and proteinuria. CASE PRESENTATION: The patient was a 23-year-old man, who had been diagnosed with NS due to RIT1 mutation, after showing foamy macrohematuria 3 weeks previously. A physical examination revealed low-set ears and a webbed neck. A urinalysis showed hematuria and proteinuria, and urinary sediments showed more than 100 isomorphic red blood cells per high-power field. His proteinuria and albuminuria concentrations were 7.1 and 4.5 g/g⋅Cr, respectively. Three-dimensional contrast-enhanced computed tomography (CT) showed double IVC and narrowing of the LRV after interflow of the left IVC. The aortomesenteric angle on a sagittal reconstruction of the CT image was 14.7°. Cystoscopy revealed a flow of macrohematuria from the left ureteral opening. On Doppler ultrasonography, there was scant evidence to raise the suspicion of the nutcracker phenomenon. Since severe albuminuria continued, a left kidney biopsy was performed. Light microscopy showed red blood cells in Bowman's space and the tubular lumen. Electron microscopy revealed disruption of the glomerular basement membrane (GBM). Vulnerability of the GBM was suspected and a genetic analysis revealed a heterozygous mutation at c.4793 T > G (p.L1598R) in the COL4A3 gene. Screening for coagulation disorders revealed the factor VIII and von Willebrand factor (vWF) values were low, at 47.6 and 23%, respectively. A multimer analysis of vWF showed a normal multimer pattern and he was diagnosed with von Willebrand disease type 1. As the bleeding tendency was mild, replacement of factor VIII was not performed. His macrohematuria and proteinuria improved gradually without treatment, and his urinalysis results have been normal for more than 6 months. CONCLUSIONS: The present case showed macrohematuria and proteinuria due to NCS in NS with double IVC and von Willebrand disease type 1. The macrohematuria and proteinuria originated from glomerular hemorrhage because of vulnerability of the GBM due to COL4A3 mutation.

Usefulness of hybrid veno-arterial extracorporeal membrane oxygenation for lung ischemia-reperfusion injury after biventricular assist device implantation.

We experienced a case of fulminant myocarditis complicated by severe lung ischemia-reperfusion injury after switching from veno-arterial extracorporeal membrane oxygenation to biventricular assist device. We controlled lung blood flow by hybrid veno-arterial extracorporeal membrane oxygenation, which was established by modifying the biventricular assist device circuit without resternotomy, blood delivery to the pulmonary artery and blood removal from the left ventricle in addition to central veno-arterial extracorporeal membrane oxygenation, and accelerated lung recovery. The patient's lung damage and cardiac function were restored, and she completely recovered and was discharged without any complications. Regulation of lung blood flow is important and effective for lung ischemia-reperfusion injury after biventricular assist device implantation.