記述言語：日本語   出版者・発行元：日本肺高血圧・肺循環学会・日本小児肺循環研究会  

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掲載種別：研究論文（学術雑誌）   出版者・発行元：Japanese Circulation Society  

DOI： 10.1253/circrep.cr-21-0103

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

BACKGROUND: In the treatment of adult congenital heart disease (ACHD), the transfer of patients from pediatric cardiologists to ACHD cardiologists is of relevance. However, little is known about the clinical courses of ACHD patients that have been referred by non-CHD-specialized doctors (n-CSDs). METHODS: This retrospective cohort study included 230 patients (average age: 37 ± 15.2 years, male: 97) who were referred to a single specialized ACHD center between April 2016 and July 2019. We compared the characteristics and clinical courses between patients referred by n-CSDs and those referred by CHD-specialized-doctors (CSDs). RESULTS: Overall, 121 (53%) patients were referred by n-CSDs. Among them, 91 (75%) patients were referred by adult cardiologists. Univariate analysis showed that the patients referred by n-CSDs were older than those referred by CSDs (41.6 ± 16.3 vs. 32.0 ± 12.0 years, p <  0.01), were more likely to have simple CHD, and less likely to have severe CHD (27.0% vs. 12.8% and 16.5% vs. 40.4%, respectively, p <  0.01). Patients referred by n-CSDs were also more likely to have a history of loss of follow-up (16.5% vs. 3.7%, p <  0.01) and to require invasive treatments after referral, including cardiac surgeries and transcatheter interventions (47.9% vs. 26.6 %, p <  0.01). Notably, unintended invasive treatments that were not designated by the referring doctors were more frequently required in patients with moderate complexity referred by n-CSDs (50.0% vs. 23.3%, p =  0.02). CONCLUSIONS: Patients with moderate CHD complexity referred by n-CSDs are more likely to require unintended invasive treatments. Referrals to specialized ACHD centers may be most beneficial for these patients.

DOI： 10.1016/j.jjcc.2020.06.018

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記述言語：英語  

A 20-year-old male without any symptoms was referred for heart murmur on a medical examination. A thrill was palpable at the upper left sternal border. His cardiac murmur showed respiratory variation. The systolic murmur was louder (Levine grade IV/VI) during expiration and diminished during inspiration (Levine grade I/VI). He was thin and had a narrow thoracic cage in the anteroposterior direction due to straight back syndrome (SBS). An echocardiogram and a right ventriculogram showed changes in the diameter of the right ventricular outflow tract (RVOT) on respiration. During expiration, the RVOT was compressed and narrow, while it was expanded during inspiration. Cardiac catheterization demonstrated a 10-mmHg of pressure gradient across the RVOT during expiration but no pressure gradient during inspiration. Thus, respiratory compression to the RVOT by a narrow thoracic cage due to SBS was the cause of the cardiac murmur with respiratory alterations. Our case highlights the importance of physical examination, including an inspection of the patient's physique. <Learning objective: When examining a patient with a cardiac murmur, respiratory alterations of cardiac murmurs should be auscultated. In these cases, straight back syndrome would be one of the differential diagnoses and should be considered. During a physical examination, inspection of the patient's physique is also important.>.

DOI： 10.1016/j.jccase.2020.07.008

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担当区分：筆頭著者   記述言語：英語  

A 54-year-old male with a history of unrepaired ventricular septal defect (VSD) suffered from easy fatigability on exertion. A Levine grade V/VI continuous murmur was auscultated. Transthoracic echocardiogram showed a ruptured sinus of Valsalva aneurysm (SVA) and a significant left-to-right shunting from the ascending aorta to the right ventricle (RV). In addition, a 36 mmHg of pressure gradient was observed between the inflow and outflow tract in the RV, suggesting double-chambered RV (DCRV). Cardiac catheterization also revealed 33 mmHg of the pressure gradient in the mid-potion of the RV, which was coincident with DCRV. A calculated pulmonary-to-systemic flow ratio was 3.0. Therefore, the patient was offered surgical repair of the ruptured SVA and VSD, which was successfully performed. During the surgery, an anomalous muscle band, which is usually the cause of DCRV, was not found, instead, a thickened RV free-wall due to the exposure of the left-to-right shunt flow, so-named jet lesion, was found. Therefore, surgical resection of the anomalous muscle band was not required. The protruded SVA toward the RV, the jet lesion, and the increased RV stroke volume, which could induce relative stenosis, were the causes of the unusual DCRV. <Learning objective: A mechanism of an unusual double-chambered right ventricle induced by ruptured sinus of Valsalva aneurysm (SVA) is as follows. One is a morphological stenosis in the right ventricle (RV) due to a protruded SVA toward the RV. The second is a jet lesion; a thickened RV wall induced by the exposure of the shunt flow from the ascending aorta. The third is a relative stenosis due to the increased stroke volume of the RV.>.

DOI： 10.1016/j.jccase.2020.07.009

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記述言語：英語  

The patient was a 19-year-old woman who had experienced headache for 1 year. Soon after birth, ventricular septal defects were diagnosed, the size of which were small, therefore not requiring surgical repair. She also noticed hypertension, with up to 184/110 mmHg of blood pressure. Her physical examination revealed a difference in blood pressure between her upper and lower limbs (160/108 and 92/65 mmHg, respectively). A cardiac computed tomography image clearly demonstrated the narrowing of the aortic isthmus. Coarctation of the aorta (CoA) was definitively diagnosed and was the cause of the upper limb hypertension and headache. Cardiac catheterization revealed 3.8 mm of the aortic isthmus and 65 mmHg of the peak-to-peak pressure gradient across the CoA. The patient was offered endovascular therapy of the CoA. A non-covered stent implantation was successfully performed and the pressure gradient across the aortic isthmus disappeared. Her upper limb hypertension also improved. Aortic angioscopy revealed a yellow plaque on the aortic intima, located proximal to the coarctation site, which was exposed owing to high blood pressure. Our case highlights that an atherosclerotic change can develop even in young patients with hypertension. <Learning objective: An aortic angioscope can detect an early atherosclerotic change of aorta, which other imaging modalities such as computed tomography and intravascular ultrasonography cannot show. An early atherosclerosis can develop even in a young patient with hypertension; therefore, coarctation of the aorta should be diagnosed and treated appropriately as soon as possible.>.

DOI： 10.1016/j.jccase.2020.03.001

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記述言語：英語  

A 75-year-old male suffered from dyspnea on exertion. In a referring hospital, cardiac catheterization demonstrated a 25% increase in oxygen saturation between the high superior vena cava (SVC) and the right atrium, suggesting a pre-tricuspid left-to-right shunt. However, neither an intracardiac shunt nor a partial anomalous pulmonary venous connection was detected. Therefore, he was referred to our hospital for further evaluation. A transesophageal echocardiogram revealed a retrograde-dominant bidirectional flow in the right upper pulmonary vein (RUPV). A contrast agent injected via the left upper limb appeared in the SVC and thereafter some contrast entered into the RUPV. A three-dimensional reconstructed computed tomography showed a side-to-side communication between the RUPV and the SVC. A cavopulmonary window was definitively diagnosed, in which the RUPV not only drained into the left atrium but also connected to the SVC side-to-side. <Learning objective: In a suspected case of a pre-tricuspid left-to-right shunt without atrial septal defect and anomalous pulmonary venous connection, a cavopulmonary window would be another differential diagnosis. This rare cardiac anomaly should be taken into consideration in diagnosing a pre-tricuspid left-to-right shunt. Identifying the direction of pulmonary venous flow can be an opportunity to find it.>.

DOI： 10.1016/j.jccase.2020.01.004

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記述言語：日本語   出版者・発行元：(一社)日本成人先天性心疾患学会  

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記述言語：日本語   出版者・発行元：(一社)日本心臓病学会  

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記述言語：日本語   出版者・発行元：(一社)日本心臓病学会  

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記述言語：日本語   出版者・発行元：(一社)日本心臓病学会  

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記述言語：日本語   出版者・発行元：(一社)日本循環器学会  

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記述言語：英語   出版者・発行元：(一社)日本成人先天性心疾患学会  

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担当区分：筆頭著者   掲載種別：研究論文（学術雑誌）  

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

The interaction between atherosclerosis and commensal microbes through leaky gut syndrome (LGS), which is characterized by impaired intestinal permeability and the introduction of undesired pathogens into the body, has not been fully elucidated. Our aim was to investigate the potential role of a ClC-2 chloride channel activator, lubiprostone, which is reported to have beneficial effects on LGS, in the development of atherosclerosis in apolipoprotein E-deficient (ApoE-/-) mice. After a 15-week feeding period of a Western diet (WD), ApoE-/- mice were treated with a Western-type diet (WD) alone or WD with oral supplementation of lubiprostone for 10 weeks. This feeding protocol was followed by experimental evaluation of LGS and atherosclerotic lesions in the aorta. In mice with lubiprostone, in vivo translocation of orally administered 4-kDa FITC-dextran was significantly improved, and RNA expression of the epithelial tight junction proteins, Zo-1 and occludin, was significantly up-regulated in the ileum, compared to the WD alone group, suggesting a possible reversal of WD-induced intestinal barrier dysfunction. As a result, WD-induced exacerbation of atherosclerotic lesion formation was reduced by 69% in longitudinally opened aortas and 26% in aortic root regions. In addition, there was a significant decrease in circulating immunoglobulin level, followed by an attenuation of inflammatory responses in the perivascular adipose tissue, as evidenced by reduced expression of pro-inflammatory cytokines and chemokines. Lubiprostone attenuates atherosclerosis by ameliorating LGS-induced inflammation through the restoration of the intestinal barrier. These findings raise the possibility of targeting LGS for the treatment of atherosclerosis.

DOI： 10.1371/journal.pone.0218096

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記述言語：日本語   出版者・発行元：(一社)日本心臓病学会  

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記述言語：日本語   出版者・発行元：(NPO)日本小児循環器学会  

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

BACKGROUND: Endothelial dysfunction and increased arterial stiffness gradually develop before the manifestation of catastrophic cardiovascular events. Therefore, detection and assessment of vascular function are required to address pre-existing pathological conditions. However, the currently available diagnostic devices and methods are insufficient due to variability among investigators and the time-consuming nature of manual procedures. METHODS: Recently, novel devices were developed for the detection of both arterial stiffness and endothelial dysfunction in a single blood pressure measurement using a cuff-oscillometric technique (AVE-1500, Shisei Datum, Japan). API (arterial pressure volume index) is defined as the reciprocal of the slope of the tangent of the brachial artery pressure-volume curve, and AVI (arterial velocity pulse index) is defined as the ratio of the difference between the ejection and reflection waves. In the present study, we performed retrospective, cross-sectional analyses of subjects (n = 102; mean age = 70.5 ± 10.4 years) with detailed coronary angiographic examinations and clinical background parameters. RESULTS: After adjusting for various variables using multiple linear regression analyses, we found that API, but not AVI, was significantly correlated with coronary artery severity and complexity scores. CONCLUSIONS: We propose that API may be a new vascular index useful for monitoring and assessing the severity and complexity of atherosclerosis in subjects with coronary artery disease and for evaluating atherosclerotic diseases.

DOI： 10.1080/10641963.2018.1465072

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

We have previously shown that neural precursor cell-expressed developmentally downregulated gene 4-2 (Nedd4-2) isoforms with a C2 domain are closely related to ubiquitination of epithelial sodium channel (ENaC), resulting in salt-sensitive hypertension by Nedd4-2 C2 targeting in mice. The sodium voltage-gated channel alpha subunit 5 (SCN5A) gene encodes the α subunit of the human cardiac voltage-gated sodium channel (I Na), and the potassium voltage-gated channel subfamily H member 2 (KCNH2) gene encodes rapidly activating delayed rectifier K channels (I Kr). Both ion channels have also been shown to bind to Nedd4-2 via a conserved Proline-Tyrosine (PY) motif in C-terminal with subsequent ubiquitination and degradation by proteasome. Therefore, loss of Nedd4-2 C2 isoform might be involved in electrophysiological impairment under various conditions. We demonstrate here that Nedd4-2 C2 isoform causes cardiac conduction change in resting condition as well as proarrhythmic change after acute myocardial infarction (MI). The Nedd4-2 C2 knockout (KO) mice showed bradycardia, prolonged QRS, QT intervals, and suppressed PR interval in resting condition. In addition, enhancement of T peak/T end interval was found in mice with surgical ligation of the distal left coronary artery. Morphological analyses based on both ultrasonography of the living heart, as well as histopathological findings revealed that Nedd4-2 C2 KO mice show no significant structural changes from wild-type littermates under resting conditions. These results suggested that Nedd4-2 with C2 domain might play an important role in cardio-renal syndrome through post-transcriptional modification of both ENaC and cardiac ion channels, which are critical for kidney and heart functions.

DOI： 10.3390/ijms18061268

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

The epithelial sodium channel (ENaC) plays critical roles in maintaining fluid and electrolyte homeostasis and is located in the aldosterone-sensitive distal nephron (ASDN). We previously found that Nedd4-2 C2 knockout (KO) mice showed salt-sensitive hypertension with paradoxically enhanced ENaC gene expression in ASDN under high oral salt intake. Eplerenone (EPL), a selective aldosterone blocker, is a promising therapeutic option for resistant or/and salt-sensitive hypertension. We examined the effect of EPL on Nedd4-2 C2 KO mice with respect to blood pressure, metabolic parameters, and molecular level changes in ASDN under high oral salt intake. We found that EPL failed to reduce blood pressure in KO mice with high oral salt intake and upregulated ENaC expression in ASDN. Thus, salt-sensitive hypertension in Nedd4-2 C2 KO was EPL-resistant. Gene expression analyses of laser-captured specimens in ASDN suggested the presence of non-aldosterone-dependent activation of ENaC transcription in ASDN of Nedd4-2 C2 KO mice, which was abolished by amiloride treatment. Our results from Nedd4-2 C2 KO mice suggest that enhanced ENaC gene expression is critically involved in salt-sensitive hypertension under certain conditions of specific enzyme isoforms for their ubiquitination.

DOI： 10.3390/ijms18061250

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

BACKGROUND: Recently, new non-invasive vascular indexes named arterial velocity pulse index (AVI) and arterial pressure volume index (API), which is evaluated by a multifunctional blood pressure monitoring device, were developed using cuff oscillometric technologies and suprasystolic cuff oscillometric wave measurement. However, although a few studies including a computational model have been performed, data on subjects with cardiovascular diseases in actual outpatient clinics remain scant. METHODS: We examined a total 252 consecutive outpatients and analyzed two vascular indexes with various clinical parameters to explore potential utilities of these two indexes in actual clinical settings. RESULTS: Although we found that two indexes were correlated with each other, the clinical implications of these indexes seemed to differ. Our analyses showed that AVI significantly correlated with augmentation index, but not with flow-mediated dilatation, and multivariate analyses suggested that enhanced AVI represents increased workload on the heart with elevated central blood pressure. In contrast, although the results of analyses performed to identify clinical parameters independently related to API were obscure and non-specific, after adjustment for multiple clinical variables, API was found to be significantly and independently associated with both Framingham Cardiovascular Risk Score and the Suita Score, suggesting that API is a useful predictor of future cardiovascular events. CONCLUSIONS: These two new vascular indexes might be useful in actual clinical settings to evaluate cardiovascular risks with various clinical backgrounds.

DOI： 10.1016/j.jjcc.2016.06.004

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

The relation between B2 cells and commensal microbes during atherosclerosis remains largely unexplored. Here we show that under hyperlipidemic conditions intestinal microbiota resulted in recruitment and ectopic activation of B2 cells in perivascular adipose tissue, followed by an increase in circulating IgG, promoting disease development. In contrast, disruption of the intestinal microbiota by a broad-spectrum antibiotic cocktail (AVNM) led to the attenuation of atherosclerosis by suppressing B2 cells, despite the persistence of serum lipid abnormalities. Furthermore, pharmacological depletion of B2 cells with an anti-B2-cell surface CD23 antibody also attenuated commensal microbe-induced atherosclerosis. Moreover, expression analysis of TLR-signaling-related genes in the activated B2 cell subsets, assessed using the Toll-Like Receptor Signaling Pathway RT2 Profiler PCR Array, confirmed activation of the B2-cell autoantibody-production axis, which was associated with an increased capacity of B2 cells to bind to intestinal microbiota. Together, our findings reveal the critical role of commensal microbe-specific activation of B2 cells in the development of atherogenesis through lipid metabolism-independent mechanisms.

DOI： 10.1016/j.ebiom.2016.10.030

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記述言語：英語   掲載種別：研究論文（学術雑誌）  

Epithelial sodium channels (ENaCs) play critical roles in the maintenance of fluid and electrolyte homeostasis, and their genetic abnormalities cause one type of hereditary salt-sensitive hypertension, Liddle syndrome. As we reported previously, both human and rodent Nedd4L/Nedd4-2 showed molecular diversity, with and without a C2 domain in their N-terminal. Nedd4L/Nedd4-2 isoforms with a C2 domain are hypothesized to be related closely to ubiquitination of ENaCs. We generated Nedd4-2 C2 domain knockout mice. We demonstrate here that loss of Nedd4-2 C2 isoform causes salt-sensitive hypertension under conditions of a high dietary salt intake in vivo. The knockout mice had reduced urinary sodium excretion, osmotic pressure and increased water intake and urine volume with marked dilatation of cortical tubules while receiving a high salt diet. To the contrary, there was no difference in metabolic data between wild-type and knockout mice receiving a normal control diet. In the absence of Nedd4-2 C2 domain, a high salt intake accelerated ENaC expression. Coimmunoprecipitation studies revealed suppressed ubiquitination for ENaC with a high salt intake. Taken together, our findings demonstrate that during a high oral salt intake the Nedd4-2 C2 protein plays a pivotal role in maintaining adaptive salt handling in the kidney.

DOI： 10.1038/srep27137

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記述言語：日本語   出版者・発行元：(公財)日本心臓財団  

症例は34歳、女性。頭痛、嘔気を主訴に近医受診。くも膜下出血の診断にて当院脳神経外科入院。脳血管造影において動脈瘤は認められなかった。入院中、CRPの上昇および発熱あり、血液培養にてグラム陽性桿菌を検出。心臓超音波検査施行し僧帽弁に径2cmの疣贅を認めたため感染性心内膜炎と診断した。当科入院後、原因菌はArcanobacterium haemolyticumと同定。Ampicillin、gentamicinによる治療を開始するも疣贅は縮小せず。外科適応と考え第32病日僧帽弁置換術を施行。その後、gentamicin、penicillin、erythromycinによる治療を継続し、第60病日軽快退院した。感染性心内膜炎に合併するくも膜下出血の原因はほとんどが感染性動脈瘤によるものだが、本症例では脳血管造影上、動脈瘤は認められず、後日の頭部MRIにて静脈洞血栓症が原因であると診断した。静脈洞血栓症によるくも膜下出血を契機に診断にいたった感染性心内膜炎の報告例は現在のところ認められていない。また、A.haemolyticumによる感染性心内膜炎は極めて稀であり、現在まで3例の症例報告を認めるのみであり、うち2例は診断後1週間以内に死亡している。今回、生来健康であった女性が静脈洞血栓症によるくも膜下出血を契機にA.haemolyticumによる感染性心内膜炎の診断にいたった貴重な1例を経験したので報告する。(著者抄録)

DOI： 10.11281/shinzo.42.352

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記述言語：日本語   出版者・発行元：日本成人先天性心疾患学会  

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J-GLOBAL

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J-GLOBAL

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記述言語：日本語   出版者・発行元：(一社)日本内分泌学会  

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記述言語：日本語   出版者・発行元：(一社)日本内分泌学会  

J-GLOBAL

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記述言語：日本語   出版者・発行元：(NPO)日本高血圧学会  

J-GLOBAL

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記述言語：日本語   出版者・発行元：(一社)日本心臓病学会  

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記述言語：日本語   出版者・発行元：(一社)日本心臓病学会  

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記述言語：英語   出版者・発行元：(一社)日本循環器学会  

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記述言語：日本語   出版者・発行元：(一社)日本内分泌学会  

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記述言語：日本語   出版者・発行元：(NPO)生物試料分析科学会  

J-GLOBAL

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J-GLOBAL

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記述言語：日本語  

J-GLOBAL

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記述言語：日本語   出版者・発行元：(NPO)日本高血圧学会  

J-GLOBAL

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記述言語：日本語   出版者・発行元：(NPO)日本高血圧学会  

J-GLOBAL

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記述言語：英語   出版者・発行元：(NPO)日本高血圧学会  

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記述言語：英語   出版者・発行元：(NPO)日本高血圧学会  

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記述言語：日本語   出版者・発行元：(NPO)日本高血圧学会  

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記述言語：日本語   出版者・発行元：(NPO)日本高血圧学会  

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記述言語：日本語   出版者・発行元：(NPO)日本高血圧学会  

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記述言語：日本語   出版者・発行元：(一社)日本心臓病学会  

J-GLOBAL

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記述言語：英語  

J-GLOBAL

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記述言語：日本語   出版者・発行元：(一社)日本内分泌学会  

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記述言語：英語   出版者・発行元：(一社)日本心血管インターベンション治療学会  

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記述言語：英語   出版者・発行元：(一社)日本循環器学会  

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記述言語：日本語   出版者・発行元：(一社)日本心臓病学会  

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記述言語：英語  

J-GLOBAL

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記述言語：英語   出版者・発行元：(一社)日本循環器学会  

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記述言語：日本語   出版者・発行元：神奈川県医師会  

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記述言語：日本語   出版者・発行元：(一社)日本循環器学会  

DOI： 10.1253/circj.CJ-09-0208

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会議種別：口頭発表（一般）  

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会議種別：口頭発表（招待・特別）  

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会議種別：口頭発表（一般）  

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会議種別：口頭発表（招待・特別）  

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会議種別：ポスター発表  

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会議種別：ポスター発表  

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会議種別：口頭発表（一般）  

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会議種別：ポスター発表  

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会議種別：口頭発表（一般）  

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会議種別：ポスター発表  

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記述言語：日本語  

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記述言語：英語   会議種別：口頭発表（一般）  

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