Large Tissue Debris Causing Cerebral Embolism After Transcatheter Aortic Valve Replacement.

Cerebral vascular embolism is one of the complications of transcatheter aortic valve replacement (TAVR). Thrombolytic therapy is not expected to be effective when embolic material consists of a large tissue fragment. Instead, mechanical aspiration may be more effective therapy for acute cerebral infarction after TAVR. Here, we describe the case of an 87-year-old woman with aortic valve stenosis and heart failure who underwent TAVR using a self-expandable valve. Acute cerebral infarction with left middle cerebral artery occlusion caused by a large tissue fragment developed after the procedure.

[Normal Anatomy and Variants of the Anterior Cerebral and Anterior Communicating Arteries].

The anterior cerebral artery(ACA)and anterior communicating artery(AcomA), two important arteries of the cerebral hemisphere, are involved in various disorders, including aneurysms, steno-occlusive diseases, and arteriovenous shunts, among other such conditions. Additionally, these vessels contain normal variants associated with their embryology and development. The ACA particularly shows characteristic variants such as a persistent primitive olfactory artery and an infraoptic course, among other such variations, which is attributable to the fact that it is embryologically older than the middle and posterior cerebral arteries. Aneurysm formation is associated with these variants. Therefore, knowledge of the normal anatomy and variants is important for diagnosis and treatment of conditions involving these arteries. In this article, we describe the normal anatomy and variants of the ACA and AcomA, focused on their embryological development.

[Venous Anatomy of the Superior and Inferior Sagittal Sinuses].

The superior sagittal sinus(SSS)is contained within the dura, which consists of the dura propria and osteal dura at the junction of the falx cerebri, in addition to the attachment of the falx to the cranial vault. The SSS extends anteriorly from the foramen cecum and posteriorly to the torcular Herophili. The superior cerebral veins flow into the SSS, coursing under the lateral venous lacunae via bridging veins. Most of the bridging veins reach the dura and empty directly into the SSS. However, some are attached to the dural or existed in it for some distance before their sinus entrance. The venous structures of the junctional zone between the bridging vein and the SSS existed in the dura are referred to as dural venous channels. The SSS communicates with the lateral venous lacunae connecting the meningeal and diploic veins, as well as the emissary veins. These anatomical variations of the SSS are defined by the embryological processes of fusion and withdrawal of the sagittal plexus and marginal sinus.

Location of embolization affects patency after coil embolization for pulmonary arteriovenous malformations: importance of time-resolved magnetic resonance angiography for diagnosis of patency.

OBJECTIVES: This study aimed to assess the diagnostic accuracy of computed tomography (CT) and time-resolved magnetic resonance angiography (TR-MRA) for patency after coil embolization of pulmonary arteriovenous malformations (PAVMs) and identify factors affecting patency. METHODS: Data from the records of 205 patients with 378 untreated PAVMs were retrospectively analyzed. Differences in proportional reduction of the sac or draining vein on CT between occluded and patent PAVMs were examined, and receiver operating characteristic analysis was performed to assess the accuracy of CT using digital subtraction angiography (DSA) as the definitive diagnostic modality. The accuracy of TR-MRA was also assessed in comparison to DSA. Potential factors affecting patency, including sex, age, number of PAVMs, location of PAVMs, type of PAVM, and location of embolization, were evaluated. RESULTS: The sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV), and accuracy of CT were 82%, 81%, 77%, 85%, and 82%, respectively, when the reduction rate threshold was set to 55%, which led to the highest diagnostic accuracy. The sensitivity, specificity, PPV, NPV, and accuracy of TR-MRA were 89%, 95%, 89%, 95%, and 93%, respectively. On both univariable and multivariable analyses, embolization of the distal position to the last normal branch of the pulmonary artery was a factor that significantly affected the prevention of patency. CONCLUSIONS: TR-MRA appears to be an appropriate method for follow-up examinations due to its high accuracy for the diagnosis of patency after coil embolization of PAVMs. The location of embolization is a factor affecting patency. KEY POINTS: • Diagnosis of patency after coil embolization for pulmonary arteriovenous malformations (PAVMs) is important because a patent PAVM can lead to neurologic complications. • The diagnostic accuracies of CT with a cutoff value of 55% and TR-MRA were 82% and 93%, respectively. • The positioning of the coils relative to the sac and the last normal branch of the artery was significant for preventing PAVM patency.

Impact of heart failure severity on bone mineral density among older patients with heart failure.

The study aimed to identify factors related to bone mineral density (BMD) among older patients with heart failure (HF). A total of 70 consecutive patients with HF aged 65 years or older who were admitted to an acute hospital due to worsening condition were enrolled before discharge. BMD of the femoral neck was evaluated using the DEXA method. Physical function, as well as echocardiographic and laboratory findings including biomarker of HF severity were collected. Bivariate and multiple regression analyses were employed to determine the association between BMD and the clinical variables. Bivariate analysis determined that age, grip strength, walking speed, serum albumin, and N-terminal pro B-type natriuretic peptide (NT-proBNP) were significantly correlated with BMD (P < 0.01), whereas other clinical parameters were not. The multiple regression analysis identified NT-proBNP as an independent related factor for BMD after adjusting with confounding clinical variables. NT-proBNP was independently related to BMD among older patients with HF. Our results suggest the inclusion of bone fracture prevention strategies in disease management programs, especially for older patients with HF.

Predictive impact of early mobilization on rehospitalization for elderly Japanese heart failure patients.

The aim of this study was to determine whether early mobilization was associated with rehospitalization among elderly heart failure patients. We measured the time from admission to mobilization and other clinical characteristics for 190 heart failure patients (mean age, 80.7 years). The primary outcome was heart failure rehospitalization. Kaplan-Meier survival curves were plotted and the hazard ratios for rehospitalization were determined using Cox proportional hazards regression models. During a median follow-up period of 750 days, 58 patients underwent rehospitalization. The time from admission to mobilization was significantly longer for these patients than for those who were not rehospitalized. Univariate and multivariate Cox proportional hazards analyses showed that the time from admission to mobilization was an independent predictor of rehospitalization, and receiver-operating characteristic analysis determined an optimal cutoff value of 3 days for differentiating the patients more likely to experience a subsequent cardiac event (sensitivity, 76%; specificity, 69%; area under the curve, 0.667). Kaplan-Meier survival curve analysis showed a significantly lower event rate in the ≤ 3-day group (p = 0.001, log-rank test). In conclusion, the time from admission to mobilization may be one of the strongest predictors of rehospitalization in elderly heart failure patients. Early mobilization within 3 days may be an initial target for the acute phase treatment of heart failure.

Angiographic and Clinical Characteristics of Thoracolumbar Spinal Epidural and Dural Arteriovenous Fistulas.

BACKGROUND AND PURPOSE: The purpose of this study is to compare the angiographic and clinical characteristics of spinal epidural arteriovenous fistulas (SEAVFs) and spinal dural arteriovenous fistulas (SDAVFs) of the thoracolumbar spine. METHODS: A total of 168 cases diagnosed as spinal dural or extradural arteriovenous fistulas of the thoracolumbar spine were collected from 31 centers. Angiography and clinical findings, including symptoms, sex, and history of spinal surgery/trauma, were retrospectively reviewed. Angiographic images were evaluated, with a special interest in spinal levels, feeders, shunt points, a shunted epidural pouch and its location, and drainage pattern, by 6 readers to reach a consensus. RESULTS: The consensus diagnoses by the 6 readers were SDAVFs in 108 cases, SEAVFs in 59 cases, and paravertebral arteriovenous fistulas in 1 case. Twenty-nine of 59 cases (49%) of SEAVFs were incorrectly diagnosed as SDAVFs at the individual centers. The thoracic spine was involved in SDAVFs (87%) more often than SEAVFs (17%). Both types of arteriovenous fistulas were predominant in men (82% and 73%) and frequently showed progressive myelopathy (97% and 92%). A history of spinal injury/surgery was more frequently found in SEAVFs (36%) than in SDAVFs (12%; P=0.001). The shunt points of SDAVFs were medial to the medial interpedicle line in 77%, suggesting that SDAVFs commonly shunt to the bridging vein. All SEAVFs formed an epidural shunted pouch, which was frequently located in the ventral epidural space (88%) and drained into the perimedullary vein (75%), the paravertebral veins (10%), or both (15%). CONCLUSIONS: SDAVFs and SEAVFs showed similar symptoms and male predominance. SDAVFs frequently involve the thoracic spine and shunt into the bridging vein. SEAVFs frequently involve the lumbar spine and form a shunted pouch in the ventral epidural space draining into the perimedullary vein.

Hereditary hemorrhagic telangiectasia in Japanese patients.

To describe clinical presentations of hereditary hemorrhagic telangiectasia (HHT) patients in Japan. There were 80 patients (40 men and 40 women, age 2-78, mean 39.4 years old), who were either genetically verified or genetically not identifiable but clinically definite HHT patients. Clinical presentations of these HHT patients were analyzed retrospectively. Radiological examinations, which included at least brain magnetic resonance imaging and lung computed tomography, were performed when indicated. Seventy-eight patients had either endoglin (ENG) or activin A receptor type II-like 1 (ACVRL1) mutation. They were 53 HHT1 patients with ENG mutation in 27 families and 25 HHT2 patients with ACVRL1 mutation in 17 families. Two other female patients were clinically definite HHT, but genetic mutation could not be identified. Nosebleeds were noted in 53/53 (100%) HHT1 and 24/25 (96%) HHT2 patients. Telangiectases were observed in 34/53 (64%) HHT1 and 18/25 (72%) HHT2 patients. Pulmonary arteriovenous malformations (AVMs) were noted in 33/52 HHT1 (63%) and 5/25 HHT2 patients (20%). Brain AVMs were detected in 12/51 HHT1 (24%) and 1/25 HHT2 (4%) patients. Hepatic AVMs were noted in 7/29 (24%) HHT1 and 16/20 (80%) HHT2 patients. The number of HHT1 patients was roughly twice as many as that of HHT2 patients in Japan. Pulmonary and brain AVMs were predominantly observed in HHT1 while hepatic AVMs were detected in HHT2. It seemed that ethnicity and regionality had minimal roles in the clinical presentation of HHT.

Spinal arteriovenous metameric syndrome in a neonate presenting with congestive heart failure: case report.

BACKGROUND: Spinal arteriovenous metameric syndrome (SAMS) is a combination of more than two separate vascular malformations in the same embryonic metameres. This syndrome, also known as Cobb syndrome, is rare, especially in the neonate. CASE DESCRIPTION: A neonatal girl with a birthmark in the occipital and posterior nuchal regions presented with severe heart failure on the day of birth. The large arteriovenous fistulas in the left hypoglossal canal and in the posterior nuchal region were embolized with detachable coils on the postnatal days 5 and 18, which improved heart failure markedly. The associated intramuscular arteriovenous malformation in the posterior neck was left untreated because large arteriovenous fistulas had been occluded. She grew up without any neurological deficits and developed with normal milestones until the latest follow-up of 8 years old. CONCLUSION: To our knowledge, this is the first case with SAMS in a neonate presenting with congestive heart failure. Presence of a birthmark in a neonate presenting with congestive heart failure may suggest the possible underlying high-flow vascular malformations in the same metamere.

Predictors of in-hospital mortality in elderly patients with heart failure treated with tolvaptan.

Objectives: We conducted an analysis of first-time tolvaptan users (≥80 years old) to determine the factors associated with the prognosis of elderly patients with heart failure. Methods: We retrospectively analyzed 66 consecutive patients with worsening heart failure (aged ≥80 years) who were admitted to Fujita Health University Bantane Hospital from 2011 to 2016 and treated with tolvaptan. Differences between the in-hospital death and survival groups were evaluated. Multivariate logistic regression analysis was also performed to identify the risk factors for mortality. Results: Sixty-six patients were included, and 26 patients died during the index hospitalization. The patients who died had a significantly higher prevalence of ischemic heart disease; a higher heart rate; higher levels of plasma C-reactive protein, blood urea nitrogen (BUN), and creatinine; a lower serum albumin level; and a lower estimated glomerular filtration rate than surviving patients. The proportion of patients requiring early initiation of tolvaptan treatment (within 3 days of admission) was significantly higher in surviving patients. On the basis of multivariate logistic regression analysis, although a high heart rate and high BUN levels were independent factors for in-hospital prognosis, they were not significantly associated with the early use of tolvaptan (≤3 days vs. ≥4 days; odds ratio=0.39; 95% confidence interval=0.07-2.21; p=0.29). Conclusions: This study revealed that a higher heart rate and higher BUN levels were independent factors for in-hospital prognosis in elderly patients who received tolvaptan and that early tolvaptan use may not always be effective in elderly patients.

[Spontaneous cerebrospinal fluid rhinorrhea associated with long-standing overt ventriculomegaly in adults (LOVA)].

OBJECTIVE: Spontaneous cerebrospinal fluid rhinorrhea associated with aqueductal stenosis is rare. CSF diversion is reported to be a failure in the majority of cases. The combination of the repair of the skull base and CSF diversion is reported to be successful. We describe a case successfully treated by intradural repair with ventricular drainage followed by endoscopic third ventriculostomy. CLINICAL PRESENTATION: A 28-year-old woman presented with rhinorrhea, and occasional attacks of headache, vomiting, and unconsciousness for two years. She had been diagnosed as arrested hydrocephalus for 10 years. Magnetic resonance imaging revealed triventriculomegaly with ballooning of the floor of the third ventricle, tonsilar herniation, right anterior horn herniation into the cribriform plate, and bilateral temporal lobe herniation into the temporal base. INTERVENTION: A ventricular drain was inserted followed by dissection of the herniated brain and repair of the enlarged cribriform foramen with periosteal flap. Make sure that the bacterial culture negative, endoscopic third ventriculostomy has been performed. There is no recurrence of hydrocephalus and rhinorrhea for two years. CONCLUSION: Direct communication between the lateral ventricle and the nasal/paranasal sinus is a rare complication of aqueductal stenosis and LOVA. Surgical repair of the skull base followed by cerebrospinal fluid diversion with endoscopic third ventriculostomy was a safe and reliable method.

A simple proteinuria-based risk score predicts contrast-associated acute kidney injury after percutaneous coronary intervention.

Contrast-associated acute kidney injury (CA-AKI) is a complication of percutaneous coronary intervention (PCI). Because proteinuria is a sentinel marker of renal dysfunction, we assessed its role in predicting CA-AKI in patients undergoing PCI. A total of 1,254 patients undergoing PCI were randomly assigned to a derivation (n = 840) and validation (n = 414) dataset. We identified the independent predictors of CA-AKI where CA-AKI was defined by the new criteria issued in 2020, by a multivariate logistic regression in the derivation dataset. We created a risk score from the remaining predictors. The discrimination and calibration of the risk score in the validation dataset were assessed by the area under the receiver-operating characteristic curves (AUC) and Hosmer-Lemeshow test, respectively. A total of 64 (5.1%) patients developed CA-AKI. The 3 variables of the risk score were emergency procedures, serum creatinine, and proteinuria, which were assigned 1 point each based on the correlation coefficient. The risk score demonstrated a good discriminative power (AUC 0.789, 95% CI 0.766-0.912) and significant calibration. It was strongly associated with the onset of CA-AKI (Cochran-Armitage test, p < 0.0001). Our risk score that included proteinuria was simple to obtain and calculate, and may be useful in assessing the CA-AKI risk before PCI.

Mutational and clinical spectrum of Japanese patients with hereditary hemorrhagic telangiectasia.

BACKGROUND: Hereditary hemorrhagic telangiectasia (HHT) is a dominantly inherited vascular disorder characterized by recurrent epistaxis, skin/mucocutaneous telangiectasia, and organ/visceral arteriovenous malformations (AVM). HHT is mostly caused by mutations either in the ENG or ACVRL1 genes, and there are regional differences in the breakdown of causative genes. The clinical presentation is also variable between populations suggesting the influence of environmental or genetic backgrounds. In this study, we report the largest series of mutational and clinical analyses for East Asians. METHODS: Using DNAs derived from peripheral blood leukocytes of 281 Japanese HHT patients from 150 families, all exons and exon-intron boundaries of the ENG, ACVRL1, and SMAD4 genes were sequenced either by Sanger sequencing or by the next-generation sequencing. Deletions/amplifications were analyzed by the multiplex ligation-dependent probe amplification analyses. Clinical information was obtained by chart review. RESULTS: In total, 80 and 59 pathogenic/likely pathogenic variants were identified in the ENG and ACVRL1 genes, respectively. No pathogenic variants were identified in the SMAD4 gene. In the ENG gene, the majority (60/80) of the pathogenic variants were private mutations unique to a single family, and the variants were widely distributed without any distinct hot spots. In the ACVRL1 gene, the variants were more commonly found in exons 5-10 which encompasses the serine/threonine kinase domain. Of these, 25/59 variants were unique to a single family while those in exons 8-10 tended to be shared by multiple (2-7) families. Pulmonary and cerebral AVMs were more commonly found in ENG-HHT (69.1 vs. 14.4%, 34.0 vs. 5.2%) while hepatic AVM was more common in ACVRL1-HHT (31.5 vs. 73.2%). Notable differences include an increased incidence of cerebral (34.0% in ENG-HHT and 5.2% in ACVRL1-HHT), spinal (2.5% in ENG-HHT and 1.0% in ACVL1-HHT), and gastric AVM (13.0% in ENG-HHT, 26.8% in ACVRL1-HHT) in our cohort. Intrafamilial phenotypic heterogeneity not related to the age of examination was observed in 71.4% and 24.1% of ENG- and ACVRL1-HHT, respectively. CONCLUSIONS: In a large Japanese cohort, ENG-HHT was 1.35 times more common than ACVRL1-HHT. The phenotypic presentations were similar to the previous reports although the cerebral, spinal, and gastric AVMs were more common.

Circulating miR-489 as a potential new biomarker for idiopathic dilated cardiomyopathy.

OBJECTIVES: MicroRNAs (miRNA) are functional RNAs that have emerged as pivotal gene expression regulators in cardiac disease. Although several cardiomyocyte miRNAs have been reported to play roles in heart failure progression among patients with idiopathic dilated cardiomyopathy (DCM), the role of circulating miRNAs has not yet been well-examined. METHODS: After total RNA extraction from the peripheral blood samples of three control participants and six patients with DCM, miRNA profiling was performed using miRNA arrays. Based on the results of this initial screening, real-time polymerase chain reaction (RT-PCR) was used to perform a quantitative analysis of blood samples from a larger number of matched patients (DCM, n=20; controls, n=5). Finally, the correlations between specific miRNA expression levels and hemodynamic parameters were analyzed. RESULTS: A primary screening of 2,565 miRNAs resulted in the identification of nine miRNA candidates. Quantitative RT-PCR results revealed significantly increased miR-489 expression levels in the DCM group. Moreover, there was a significant positive correlation between miR-489 expression level and left ventricular ejection fraction. CONCLUSIONS: Our results suggest that circulating miR-489 could be a potential noninvasive diagnostic biomarker for DCM. Additionally, the quantification of circulating miR-489 may have value as a potential prognostic marker for patients with DCM.

Impact of physical function on indeterminable anaerobic threshold in patients with heart failure.

BACKGROUND: Anaerobic threshold (AT) during cardiopulmonary exercise testing (CPET) is not always determinable in patients with heart failure (HF). However, little is known about the clinical features of patients with HF who have indeterminable AT. Therefore, the present study aimed to clarify the clinical features of such patients. METHODS: A total of 70 patients with HF (58 males; age: 68±12 years) who underwent CPET during hospitalization were divided into two groups: determinable AT (n=50) and indeterminable AT (n=20). Physical function, echocardiographic results, and laboratory findings were subsequently determined. RESULTS: Univariate analyses showed that the indeterminable AT group had significantly higher age and left ventricular ejection fraction, and significantly lower body mass index, calf circumference, handgrip strength, walking speed, serum hemoglobin, and serum albumin than the determinable AT group. Multiple logistic regression analysis identified handgrip strength and walking speed as independent predictive factors for indeterminable AT. Receiver-operating characteristic analyses revealed that handgrip strength of 21.2 kg and walking speed of 0.97 m/s were optimal cutoff values for differentiating patients who were likely to experience indeterminable AT. CONCLUSIONS: The present study identified handgrip strength and walking speed as powerful predictors for indeterminable AT with HF.

Effect of cardiac rehabilitation on circulating microRNA expression in heart failure: a preliminary study.

OBJECTIVES: There are benefits of exercise-based cardiac rehabilitation (CR) in patients with heart failure (HF), but their underlying molecular mechanisms remain elusive. The effect of CR on the expression profile of circulating microRNAs (miRNAs), which are short noncoding RNAs that regulate posttranscriptional expression of target genes, is unknown. If miRNAs respond to changes following CR for HF, then serum profiling of miRNAs may reveal cardioprotective mechanisms of CR. METHODS: This study enrolled three hospitalized patients with progressed systolic HF and three normal volunteer controls. In patients, CR was initiated after improvement of HF, which included 2 weeks of bicycle ergometer and resistance exercises. Genome-wide expression profiling of circulating miRNAs was performed using microarrays for the patients (mean±SD age, 60.0±12.2 years) and controls (58.7±0.58 years). Circulating miRNA expression profiles were compared between patients with HF before and after CR and the controls. RESULTS: Expression levels of two miRNAs were significantly different in patients before CR compared with controls and patients after CR. The expression of hsa-miR-125b-1-3p was significantly downregulated and that of hsa-miR-1290 was significantly upregulated in patients before CR. CONCLUSIONS: When performing CR, expression of certain circulating miRNAs in patients with HF is restored to nonpathological levels. The benefits of CR for HF may result from regulation of miRNAs through multiple effects of gene expression.

[A Suspected Case of Hemorrhagic Hereditary Telangiectasia Presented with Cerebral Hemorrhage in Infancy].

Hereditary hemorrhagic telangiectasia (HHT) is characterized by telangiectasias in multiple organs. We experienced an infant with cerebral hemorrhaging and suspected HHT based on his family history of HHT. Computed tomography angiography confirmed a cerebral arteriovenous fistula. The onset of cerebral arteriovenous fistulas associated with HHT is relatively early, and the incidence of bleeding is relatively common. When HHT is suspected based on a family history, early imaging screening is recommended to improve the neurological prognosis, even in asymptomatic cases. (Received April 7, 2020; Accepted May 7, 2020; Published August 1, 2020).

[Experience in the treatment for intracranial arteriovenous shunts in children].

PURPOSE: To report our experiences in the treatment for intracranial arteriovenous shunts (AV shunts) in the pediatric population. MATERIAL AND METHODS: Twelve children with intracranial AV shunts were treated with endovascular embolization between December 1993 and March 2008. These comprised two cases of vein of Galen aneurysmal malformation, three of dural sinus malformation, two of infantile dural AV shunt, five of pial AV fistula including two of vein of Galen aneurysmal dilatation. There were eleven boys and one girl. The age at the first embolization ranged from day 0 to 9 years. We reviewed their clinical features and outcomes. RESULTS: Six patients including four neonates presented with congestive heart failure, one infant with macrocrania and three children with headache, seizure or ataxia. The number of endovascular embolization ranged from one to five per patient. These included eighteen transarterial embolizations and ten transvenous embolizations. All patients except for one who died eventually from pulmonary hemorrhage showed improvement in their symptoms. Although only five patients achieved complete occlusion of AV shunts, six patients including them developed normally. CONCLUSION: Intracranial AV shunts in the pediatric population present characteristic clinical features depending on the age of the presentation. Endovascular embolization is currently the treatment of choice for them. It is more important to obtain normal development than to achieve normal morphological appearance. It is also important to understand the difference of pathophysiological features of these diseases in the pediatric and adult population.