Successful Endovascular Repair of an Aortobronchial Fistula due to Takayasu Arteritis.

Endovascular approaches to treating a diseased ascending aorta are challenging. We report the use of an endovascular occlusion device for successful closure of a ruptured penetrating atherosclerotic ulcer of an ascending aorta. A 47-year-old female patient with Takayasu arteritis complained of a worsening hemoptysis. She had a history of Bentall procedure for a sinus of Valsalva aneurysm and redo surgery for a ruptured penetrating atherosclerotic ulcer close to the distal anastomosis. She developed methicillin-resistant Staphylococcus aureus (MRSA) mediastinitis after the second procedure and required negative pressure wound therapy. Computed tomographic angiography revealed recurrence of a ruptured penetrating aortic ulcer and an aortobronchial fistula. Because of the high risk of redo sternotomy after MRSA mediastinitis, we used an endovascular occlusion device to achieve successful percutaneous closure. The patient was discharged without any complications. Postoperative computed tomography scans showed that the endovascular device was positioned without migration and that complete thrombosis of the penetrating atherosclerotic ulcer was achieved. This is the first report on endovascular repair of a ruptured penetrating atherosclerotic ulcer of the ascending aorta in Takayasu arteritis.

Acute left atrial dissection during redo mitral valve replacement.

Left atrial (LA) dissection is an extremely rare and life-threatening complication which is most commonly associated with mitral valve surgery. We report a case of LA dissection after a redo mitral valve replacement and discuss its etiology and management.

Debranch-First Technique with Zone 0 Elephant Trunk for Redo Operation of Residual Type B Aortic Dissection: The Completion of Aortic Proximalization.

This article presents a straightforward hybrid arch technique for treating residual type B aortic dissecting aneurysms following type A repair (replacement of the ascending aorta) that employs a frozen elephant trunk (FET) straight vascular prosthesis. The debranch-first method involves only cutting and sewing the previous ascending graft, inserting the FET from zone 0, and debranching the arch vessels using a trifurcated graft. This technique is less invasive as it eliminates the need to manipulate the dissected distal arch aneurysm. We successfully applied this technique to 3 patients, with no instances of in-hospital death, stroke, or paraplegia. The debranch-first technique, combined with zone-0 FET insertion, simplifies the redo repair of residual type B aortic dissection.

A rare case of lethal Najuta endograft collapse.

BACKGROUND: The development of fenestrated endograft, Najuta endograft Kawasumi Laboratories, Inc, Tokyo, Japan) in thoracic endovascular aortic repair (TEVAR) has enabled the treatment of aortic arch aneurysms approaching zone 0 without the need of supra-aortic vessel branch reconstruction. However, the indications of Najuta remain controversial due to complications such as endograft collapse, which is rare and lethal. CASE PRESENTATION: We here report a 75-year-old male patient with arch saccular aneurysm. Because of his liver cirrhosis, 2 debranching TEVAR has chosen as a treatment using Najuta. After extrathoracic bypass was performed, a CTAG stent graft was implanted distal to the LSCA in order to deliver Najuta stent graft steadily. Najuta stent graft was successfully positioned in zone 0. However, he was suffered from stent-graft collapse. After additional TEVAR to salvage stent-graft collapse to zone 0, he complicated type A dissection, which was treated by ascending aorta replacement. After salvage operation complicated multiorgan failure and he died.

Echo-guided seldinger technique facilitates ascending aorta cannulation in type A aortic dissection.

BACKGROUND: The ascending aorta is the most simple and rapid arterial access for the establishment of antegrade systemic perfusion. In acute type A aortic dissection, prompt establishment of antegrade central perfusion, especially in unstable hemodynamic emergency operations, help to diminish organ malperfusion and to prevent retrograde embolism. The effectiveness as well as the safety of antegrade perfusion under ultrasonographic guidance through the dissected ascending aorta was evaluated for the repair of type A aortic dissection utilizing a new echo stabilizer. RESULTS: Ascending aortic cannulation was successfully performed in 64 consecutive patients, using the Seldinger technique, with the hands-free continuous-echo monitoring, utilizing a new stabilizer. Epiaortic 2-Dimensional and color Doppler imaging provided real-time monitoring for the placement and proper perfusion of ascending aorta cannulation. CONLUSIONS: Ascending aorta can routinely provide a rapid and reliable route of antegrade central systemic perfusion in type A acute aortic dissection. The echo-guided stabilizer-assisted cannulation method can safely provide a rapid and reliable route for antegrade central perfusion during in type A dissections repair.

Actual incidence of cerebral infarction after thoracic endovascular aortic repair: a magnetic resonance imaging study.

OBJECTIVES: The actual incidence of cerebral infarction (CI), including asymptomatic infarction, owing to thoracic endovascular aortic repair (TEVAR) has not been reported in detail. This study was performed to investigate the incidence of post-TEVAR CI by using diffusion-weighted magnetic resonance imaging (DW-MRI) and to determine the risk factors for both symptomatic and asymptomatic CI. METHODS: We examined 64 patients undergoing TEVAR at our institute between April 2017 and November 2020. Aortic atheroma was graded from 1 to 5 by preoperative computed tomography. Cerebral DW-MRIs were conducted 2 days after the procedure to diagnose postoperative CI. RESULTS: A total of 44 new foci were detected by post-interventional cerebral DW-MRI in 22 patients (34.4%). Only one patient developed a symptomatic stroke (1.6%), and TEVAR was successfully completed in all cases. Debranching of the aortic arch and left subclavian artery occlusion with a vascular plug was performed in 19 (29.7%) and 12 (18.8%) patients, respectively. The number of patients with proximal landing zones 0-2 was significantly higher in the CI group than in the non-CI group (68.2% vs 11.9%; P < 0.001). The following risk factors were identified for asymptomatic CI: aortic arch debranching (P < 0.001), left subclavian artery occlusion (P = 0.001) and grade 4/5 aortic arch atheroma (P = 0.048). CONCLUSIONS: Over one-third of the patients examined by cerebral DW-MRI after TEVAR were diagnosed with CI. High-grade atheroma and TEVAR landing in zone 0-2 were found to be positively associated with asymptomatic CI. CLINICAL TRIAL REGISTRATION: 02-014.

Prenatal diagnosis of methylmalonic aciduria by measuring methylmalonic acid in dried amniotic fluid on filter paper using gas chromatography-mass spectrometry.

Methylmalonic aciduria is a common inherited metabolic disorder. Methylmalonic acid (MMA), a key indicator of methylmalonic aciduria, increases in the amniotic fluid of affected fetuses. For prenatal diagnosis, the MMA in amniotic fluid can be measured by stable-isotope dilution gas chromatography-mass spectrometry. Here, we quantified the MMA in cell-free amniotic fluid samples that had been dried on filter paper and transported at ambient temperatures, and compared the results with data obtained from the original amniotic fluid. Our results indicated that the filter paper method was reproducible and accurate enough to be applied to clinical analysis. We also used the filter paper method to screen at-risk fetuses and obtained a clear diagnosis in each case. We conclude that our method enables the prenatal diagnosis of methylmalonic aciduria using practical procedures and a simplified method for transporting the samples.

A GC/MS-based metabolomic approach for diagnosing citrin deficiency.

Citrin is the hepatic mitochondrial aspartate-glutamate carrier that is encoded by the gene SLC25A13. Citrin deficiency often leads to hyperammonemia, for which the current treatment concept is different from that for primary hyperammonemias. Metabolite level diagnosis, often referred to as chemical diagnosis, is not always successful in identifying citrin deficiency immediately or in a timely fashion. We previously made the chemical diagnosis of citrin deficiency in ten patients from nine families. In order to devise a more rapid and more accurate chemical diagnosis of this disorder than is currently available, we reinvestigated the gas chromatography/mass spectrometry-based urine metabolome in these patients. In patients aged 2 to 5 months, prominent biomarkers detected included one or more of the following metabolites: tyrosine, p-hydroxyphenyllactate, p-hydroxyphenylpyruvate, and N-acetyltyrosine, galactose, galactitol and galactonate, glucose, glucitol, and cystathionine. These biomarkers are less prominent in older patients, but are not increased in argininosuccinate synthetase deficiency or other hyperammonemias. α-Ketoglutaramate (KGM), a recently recognized urinary biomarker of primary hyperammonemias associated with defects of the urea cycle, was increased in most patients with citrin deficiency studied here in spite of normal urinary levels of glutamine (the immediate precursor of KGM), 5-oxoproline, glutamate, aspartate, and asparagine. Other important urinary biomarkers that should be measured for differential diagnosis of hyperammonemias, including orotate, uracil, and ß-ureidopropionate, were not increased. The presence of citrulline and citrulline-derived metabolites was noted in all cases. The present study shows that noninvasive urine metabolomics, together with an analysis of selected metabolites or groups of metabolites, provides a more reliable and rapid chemical diagnosis of citrin deficiency than was previously available and more readily differentiates this disorder from other hyperammonemic syndromes.

Urinary 2-hydroxy-5-oxoproline, the lactam form of α-ketoglutaramate, is markedly increased in urea cycle disorders.

α-Ketoglutaramate (KGM) is the α-keto acid analogue of glutamine, which exists mostly in equilibrium with a lactam form (2-hydroxy-5-oxoproline) under physiological conditions. KGM was identified in human urine and its concentration quantified by gas chromatography/mass spectrometry (GC/MS). The keto acid was shown to be markedly elevated in urine obtained from patients with primary hyperammonemia due to an inherited metabolic defect in any one of the five enzymes of the urea cycle. Increased urinary KGM was also noted in other patients with primary hyperammonemia, including three patients with a defect resulting in lysinuric protein intolerance and one of two patients with a defect in the ornithine transporter I. These findings indicate disturbances in nitrogen metabolism, most probably at the level of glutamine metabolism in primary hyperammonemia diseases. Urinary KGM levels, however, were not well correlated with secondary hyperammonemia in patients with propionic acidemia or methylmalonic acidemia, possibly as a result, in part, of decreased glutamine levels. In conclusion, the GC/MS procedure has the required lower limit of quantification for analysis of urinary KGM, which is markedly increased in urea cycle disorders and other primary hyperammonemic diseases.

Free-floating thrombus of the aorta: 3 case reports.

BACKGROUND: Idiopathic free-floating thrombus (FFT) of the aorta is a rare occurrence, but it can lead to catastrophic consequences. The initial symptoms are typically cerebral or peripheral embolisms. Surgical thrombectomy and thrombolysis are two primary treatments for FFT. Here, we report three cases of patients with idiopathic FFT in the absence of coagulopathy who were treated successfully by surgery with no recurrent thrombi or relapse of symptoms. CASE PRESENTATION: Case 1 involved a 72-year-old male patient with a pedunculated thrombus in the distal aortic arch. Case 2 involved a 62-year-old female patient with a cylinder thrombus in the aortic arch and left common carotid artery. Case 3 involved a 65-year-old male patient with three pedunculated thrombi in the ascending aorta, aortic arch, and left subclavian artery. None of the patients had clinical signs of coagulopathy. Pedunculated or cylinder thrombi have a greater risk of breaking off, which can produce severe peripheral embolism in contrast with intramural thrombi (73% vs. 12%). Due to the high embolism risk for each patient, conservative medical treatment by heparinization was deemed inappropriate, so each patient underwent emergency surgical thrombus removal. After surgery, each of the three patients was treated with warfarin for secondary prevention of thromboembolism. At 7-month follow-up in outpatient practice, a computed tomography (CT) scan indicated that Patient 1 had no recurrent thrombus, and the patient has been symptom-free for 11 months. At 1-month follow-up in outpatient practice, a CT scan indicated that Patient 2 had no recurrent thrombus, and the patient has been symptom-free for 8 years. At 3-week follow-up in hospital, a CT scan indicated that Patient 3 had no recurrent thrombus, but he failed to follow-up after discharge, so his follow-up status is unknown. CONCLUSIONS: For a large pedunculated or cylinder thrombus located in the thoracic aorta, surgical thrombectomy should be performed. And, in surgical thrombectomy, the location of the cannulas and cross-clamp should be selected carefully according to the location of the thrombus. After surgery, anticoagulant is important to prevent recurrent idiopathic thrombi.

Current density distribution by ring and diagonally arranged half-ring electrodes in bipolar and overlapping biphasic impulse stimulation.

BACKGROUND: We have studied the acute and long-term efficacy of overlapping biphasic impulse (OLBI) stimulation for atrial pacing with VDD pacemakers and demonstrated the feasibility of DDD pacing in OLBI with diagonally arranged half-ring (Half-Ring) electrodes. We made two three-dimensional computational analysis models to verify our clinical studies. METHODS AND RESULTS: Model I was composed of a heart, a pacemaker, and a human body. Model II was a cube with dimensions of 20 by 20 by 20 mm quarried from Model I for the detailed study of current density distributions. Laplace's equation was solved using the finite element method and the current density J was calculated. For Model I, the distal and proximal voltages were -10 V, 0 V in bipolar and -5 V, +5 V in OLBI, using Ring electrodes. In Model II, the actual measurements of electrode impedances obtained from the clinical study (1,180 Ω for Ring and 630 Ω for Half-Ring) were added to the analysis conditions. Model I showed that OLBI produced more concentrated current density distributions than those by bipolar. According to Model II, at the atrial myocardium position current density produced by Half-Ring was larger than that by Ring electrodes, 70 µA/mm(2) versus 30 µA/mm(2) in OLBI configuration. It also indicated that even if electrode impedances were equal between Half-Ring and Ring electrodes, the maximum current density produced by Half-Ring would be greater than that by Ring electrodes. CONCLUSIONS: It was considered that OLBI configuration with Half-Ring electrodes provides more effective current density distributions.

Non-ischaemic intramyocardial dissection following total arch replacement.

Intramyocardial dissection (ID) is a rare left ventricular (LV) disorder characterized by myocardial fibre dissection and neocavitation. In this study, we present a rare case of a 66-year-old woman who had a history of sarcoidosis with non-ischaemic ID following total arch replacement. ID developed suddenly in the free wall of the LV and expanded rapidly to form an LV aneurysm. We successfully performed LV reconstructive surgery to prevent ID rupture.

Urinary metabolic profile of phenylketonuria in patients receiving total parenteral nutrition and medication.

Nutrition and drugs are main environmental factors that affect metabolism. We performed metabolomics of urine from an 8-year-old patient (case 1) with epilepsy and an 11-year-old patient (case 2) with malignant lymphoma who was being treated with methotrexate. Both patients were receiving total parenteral nutrition (TPN). We used our diagnostic procedure consisting of urease pretreatment, partial adoption of stable isotope dilution, gas chromatography/mass spectrometry (GC/MS) measurement and target analysis for 200 analytes including organic acids and amino acids. Surprisingly, their metabolic profiles were identical to that of phenylketonuria. The neopterin level was markedly above normal in case 1, and both neopterin and biopterin were significantly above normal in case 2. Mutation analysis of genomic DNA from case 1 showed neither homozygosity nor heterozygosity for phenylalanine hydroxylase deficiency. The metabolic profiles of both cases were normal when they were not receiving TPN. TPN is presently prohibited for individuals who have inherited disorders that affect amino acid metabolism. Although the Phe content of the TPN was not the sole cause of the PKU profile, its effect, combined with other factors, e.g. specific medication or possibly underlying diseases, led to this metabolic abnormality. The present study suggests that GC/MS-based metabolomics by target analysis could be important for assuring the safety of the treatments for patients receiving both TPN and methotrexate. Metabolomic profiling, both before and during TPN, is useful for determining the optimal nutritional formula not only for neonates, but also for young children who are known heterozygotes for metabolic disorders or whose status is unknown.

Emergency surgery for left main disease: with and without cardioplegic arrest.

BACKGROUND: The aims of this study were to evaluate the on-pump beating-heart technique of coronary artery bypass in patients with acute myocardial infarction and left main disease, and to retrospectively compare the early postoperative results with those of conventional on-pump arrested-heart coronary surgery. METHODS: Eighty-five patients with acute myocardial infarction caused by left main disease, who underwent emergency surgery between January 1998 and April 2017 at Saiseikai Utsunomiya Hospital, were enrolled in this study. Of these patients, 56 were evaluated using propensity-matched analysis. The patients were divided into two groups according to the surgical procedure: group A ( n = 28) had on-pump surgery on the arrested heart, and group B ( n = 28) had on-pump surgery on the beating heart. Early postoperative results were compared between the two groups. RESULTS: Preoperative and intraoperative characteristics showed no significant differences between the two groups. The peak creatine kinase myocardial band level was significantly lower in group B (group A 151 vs. group B 91 IU·L-1, p = 0.01). The early mortality rate was higher in group A than group B, but the difference was not significant (group A 28.6% vs. group B 17.9%, p = 0.53). CONCLUSIONS: There was no significant advantage based on surgical procedure between on-pump beating-heart surgery and on-pump surgery on the arrested heart. On-pump beating-heart coronary artery bypass grafting significantly reduced the peak creatine kinase myocardial band level, but there were no significant differences in the early postoperative data, including the mortality rate and left ventricular function.

A Rare Solitary Fibrous Tumor Originating From the Distal Aortic Arch.

We report a 68-year-old man who presented with a well-circumscribed 6.9 × 6.5 × 3.6-cm tumor with a feeding vessel from the thyrocervical trunk in his left pleural cavity. The tumor was attached to the distal aortic arch, so he underwent a tumor resection with prosthetic graft replacement of the distal aortic arch. Pathological examination revealed a solitary fibrous tumor with potentially malignant features. To our knowledge, this is the first case of a solitary fibrous tumor arising from the aortic adventitia reported in the literature.

Prenatal diagnosis of propionic acidemia by measuring methylcitric acid in dried amniotic fluid on filter paper using GC/MS.

Propionic acidemia is a frequent inborn error of metabolism. Methylcitric acid, a key indicator of propionic acidemia, increases in the amniotic fluid of affected fetuses. For prenatal diagnosis, the methylcitric acid in amniotic fluid can be measured by stable-isotope dilution GC/MS. Here, we quantified this indicator in samples of amniotic fluid that had been dried on filter paper and transported at ambient temperatures, and compared the results with data obtained from the original amniotic fluid. We then used the filter-paper method to screen at-risk fetuses and obtained a clear-cut diagnosis in each case.

Open Conversion for Type A Ascending Aortic Dissection after Thoracic Endovascular Aortic Repair by the Chimney Graft Technique.

An 83-year-old man with aortic arch aneurysm underwent zone 0 thoracic endovascular aortic repair (TEVAR) by the chimney graft technique with two supra-aortic arch debranching grafts and developed subacute type A ascending aortic dissection. We performed emergency open conversion with circulatory arrest under deep hypothermia. The tip of the chimney graft (around the sino-tubular junction in the ascending aorta) was stiff, making it difficult to inspect the lumen and perform anastomosis. Deep hypothermic circulatory arrest and cutting the endograft stents to mobilize the graft were necessary for secure anastomosis.

Aortic valve reconstruction using autologous pericardium (Ozaki procedure) for active infective endocarditis: a case report.

Approximately one-third of patients with infective endocarditis require surgical treatment, but the ideal procedure that prevents infection ensures long durability and maintains quality of life remains unclear. A 21-year-old man who was diagnosed with aortic active infective endocarditis was referred to our hospital for surgical treatment. Echocardiography showed bicuspid aortic valve, severe aortic regurgitation, a large vegetation, and a paravalvular abscess. We planned to perform elective surgical treatment after antibiotic therapy; however, progression to heart failure required urgent operation. Aortic valve reconstruction (AVr) using autologous pericardium was performed. Perioperative and postoperative courses were uneventful. No recurrence of infection or adverse events were observed 4 years postoperatively. Considering prosthetic valve infection and redo operation, AVr may be considered among young patients.

Simple and quantitative analysis of urinary sulfated tauro- and glycodihydroxycholic acids in infant with cholestasis by electrospray ionization mass spectrometry.

Here we report a simple, sensitive, and accurate method for detecting urinary sulfated tauro- and glyco-bile acids that uses electrospray ionization mass spectrometry. The sulfated tauro- and glycodihydroxycholic acids mainly generated [M-2H](2-) negative ions at m/z 288.6 and m/z 263.6, respectively. These doubly charged ions appeared primarily in samples prepared from the urine of patients with cholestasis and were detected quantitatively. Cholestatic jaundice is the primary clinical sign of biliary atresia. The measurement of doubly charged negative ions, especially of sulfated taurodihydroxycholic acid (principally taurochenodeoxycholate-3-sulfate), is a useful screening modality for biliary atresia in neonates.