Language：Japanese   Publisher：(NPO)日本小児循環器学会  

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Language：Japanese   Publisher：(NPO)日本小児循環器学会  

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Language：Japanese   Publisher：(NPO)日本小児循環器学会  

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Language：Japanese   Publisher：(NPO)日本小児循環器学会  

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Language：English   Publishing type：Research paper (scientific journal)  

Histidine-rich glycoprotein (HRG) has been reported to inhibit signaling leading to the release of high mobility group box 1 protein, a damage-associated molecular pattern. The present study aimed to determine the longitudinal change in HRG levels in extremely preterm infants and assess whether complications such as bronchopulmonary dysplasia (BPD) were associated with differences in HRG levels. In this multicenter, prospective, observational study, we measured serum HRG levels every 2 weeks from birth to 8 weeks of age. Serum HRG was measured using an enzyme-linked immunosorbent assay. We included 19 extremely preterm infants in the study and 74 samples were analyzed. The median gestational age was 26.0 weeks, and the median birth weight was 858 g. Serum HRG levels showed a significant upward trend after birth (p < 0.001); median HRG concentrations at birth and at 2, 4, 6, and 8 weeks of age were 1.07, 1.11, 2.86, 6.05, and 7.49 µg/mL, respectively. Onset of BPD was not associated with differences in serum HRG levels. Further, the serum HRG levels increased significantly after birth in extremely preterm infants.

DOI： 10.3390/biomedicines11010212

PubMed

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Language：English   Publishing type：Research paper (scientific journal)  

Pseudoachondroplasia (PSACH) is an autosomal dominant skeletal dysplasia caused by pathogenic variants of cartilage oligomeric matrix protein (COMP). Clinical symptoms of PSACH are characterized by growth disturbances after the first year of life. These disturbances lead to severe short stature with short limbs, brachydactyly, scoliosis, joint laxity, joint pain since childhood, and a normal face. Epimetaphyseal dysplasia, shortened long bones, and short metacarpals and phalanges are common findings on radiological examination. Additionally, anterior tonguing of the vertebral bodies in the lateral view is an important finding in childhood because it is specific to PSACH and normalizes with age. Here, we report five Japanese patients with PSACH, with one recurrent (p.Cys351Tyr) and four novel heterozygous pathogenic COMP variants (p.Asp437Tyr, p.Asp446Gly, p.Asp507Tyr, and p.Asp518Val). These five pathogenic variants were located in the calcium-binding type 3 (T3) repeats. In four of the novel variants, the affected amino acid was aspartic acid, which is abundant in each of the eight T3 repeats. We describe the radiological findings of these five patients. We also retrospectively analyzed the sequential changes in the vertebral body and epimetaphysis of the long bones from the neonatal to infantile periods in a patient with PSACH and congenital heart disease.

DOI： 10.1297/cpe.2023-0035

PubMed

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Authorship：Lead author,　Corresponding author   Language：English   Publishing type：Research paper (scientific journal)   Publisher：Elsevier BV  

DOI： 10.1016/j.jjcc.2022.04.005

PubMed

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Language：Japanese   Publisher：(一社)日本心臓病学会  

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Language：Japanese   Publisher：日本小児高血圧研究会  

酸化ストレスは、内部・外部刺激により生体内で活性酸素群(活性窒素も含む)が抗酸化システムで捕捉しきれないほど過剰に生じる状況である。酸化ストレス亢進は血管内皮障害と深く関連する。とりわけ、血管内皮での一酸化窒素(nitric oxide:NO)生成は生体のレドックス環境を保持するうえで必須である。酸化ストレス亢進はarginineを基質とするNO合成を抑制し、内因性NO合成酵素阻害因子であるasymmetric dimethylarginine(ADMA)の生成を刺激する。新生児の急速進行性疾患、慢性遷延性疾患ではしばしば酸化ストレスが増幅され、生体の構造や機能が酸化劣化を受けて、組織障害が不可逆的に進展する。これらの疾患の治療や管理において酸化ストレスを制御することは重要である。そのためには酸化ストレスの病態生理、それへの防御機制を把握し、特異的マーカーを用いて患者の酸化ストレス環境を非～低侵襲的に評価することが必要である。新生児医療の現場で実施される種々の治療は、酸化ストレス制御を目指すものでもある。その中で、母乳はNO、thioredoxin-1などの抗酸化因子を多量に含有し、酸化ストレス過剰病態の抑止に有効と推定される。今後は、"レドックス"に関連してより特異性の高い細胞機能修飾薬が開発され、それらが重症疾患への集学治療の中に組み込まれていくことが期待される。(著者抄録)

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Language：Japanese   Publisher：(NPO)日本小児循環器学会  

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Language：Japanese   Publisher：(NPO)日本小児循環器学会  

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Language：Japanese   Publisher：(NPO)日本小児循環器学会  

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Language：Japanese   Publisher：(NPO)日本小児循環器学会  

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Language：Japanese   Publisher：(NPO)日本小児循環器学会  

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Language：English   Publishing type：Research paper (scientific journal)   Publisher：Elsevier BV  

DOI： 10.1016/j.jtcvs.2021.08.083

PubMed

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Authorship：Corresponding author   Language：English   Publishing type：Research paper (scientific journal)   Publisher：Springer Science and Business Media LLC  

Even today, when the surgical outcome of congenital heart disease in the neonatal period has improved, the prognosis for heterotaxy syndrome and functional single ventricle complicated with total anomalous pulmonary venous connection (TAPVC), especially the infra-cardiac type, is catastrophic. We describe a strategy that combines percutaneous ductus venosus (DV) stent placement and occlusion after TAPVC repair to ensure survival from initial surgery to bidirectional cavopulmonary shunt (BCPS) procedure and facilitate subsequent treatment. Three consecutive patients with heterotaxy syndrome and functional single ventricle complicated by infra-cardiac TAPVC treated with our own strategy were retrospectively studied. In two infants, DV stent placement was performed on the day of birth. In one case at 11 days of age. The risk of pulmonary vein obstruction was reduced, and on-pump surgery, including TAPVC repair, was performed on a standby basis. Since the rapid increase in hepatic enzymes occurred on postoperative day 0 to 1 in all cases, percutaneous stent occlusion was performed until postoperative day 3. The procedure improved liver function. One patient died due to severe atrioventricular valve regurgitation, one case underwent BCPS, and one patient was waiting to undergo. DV stent placement can avoid TAPVC repair in the early neonatal period. After TAPVC repair, the portosystemic shunt remained, resulting in hepatic dysfunction, but this could be improved by stent and vertical vein occlusion. A series of stepwise treatments can be useful to help such critically ill infants survive the high-risk neonatal period and achieve good BCPS circulation.

DOI： 10.1007/s00246-021-02782-z

PubMed

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Other Link： https://link.springer.com/article/10.1007/s00246-021-02782-z/fulltext.html

Language：Japanese   Publisher：(公社)日本小児科学会  

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Language：Japanese   Publisher：日本成人先天性心疾患学会  

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Language：Japanese   Publisher：日本成人先天性心疾患学会  

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Language：Japanese   Publisher：日本成人先天性心疾患学会  

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Language：Japanese   Publisher：日本成人先天性心疾患学会  

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Language：Japanese   Publisher：日本成人先天性心疾患学会  

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Language：Japanese   Publisher：(株)東京医学社  

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Authorship：Lead author,　Corresponding author   Language：English   Publishing type：Research paper (scientific journal)   Publisher：Elsevier BV  

DOI： 10.1016/j.jjcc.2021.04.008

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Language：English   Publishing type：Research paper (scientific journal)   Publisher：Elsevier BV  

DOI： 10.1053/j.jvca.2021.02.017

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Language：Japanese   Publisher：(NPO)日本小児循環器学会  

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Language：Japanese   Publisher：(NPO)日本小児循環器学会  

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Language：English   Publishing type：Research paper (scientific journal)   Publisher：Springer Science and Business Media LLC  

DOI： 10.1007/s00540-021-02931-x

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Other Link： https://link.springer.com/article/10.1007/s00540-021-02931-x/fulltext.html

Authorship：Corresponding author   Language：English   Publishing type：Research paper (scientific journal)   Publisher：SPRINGER  

Bovine jugular vein (BJV) and expanded polytetrafluoroethylene (ePTFE) conduits have been described as alternatives to the homograft for right ventricular outflow tract (RVOT) reconstruction. This study compared RVOT reconstructions using BJV and ePTFE conduits performed in a single institution. The valve functions and outcomes of patients aged < 18 years who underwent primary RVOT reconstruction with a BJV or ePTFE conduit between 2013 and 2017 were retrospectively investigated. 44 patients (20 and 24 with BJV and ePTFE conduits, respectively) met the inclusion criteria. The mean follow-up time was 4.5 +/- 1.5 years. No significant differences in peak RVOT velocity (1.8 +/- 0.9 m/s vs 2.1 +/- 0.9 m/s,P = 0.27), branch pulmonary stenosis (P = 0.50), or pulmonary regurgitation (P = 0.44) were found between the BJV and ePTFE conduit groups, respectively. Aneurysmal dilatation of the conduit was observed in 25.0% of the patients in the BJV conduit group but not in the ePTFE conduit group (P = 0.011). All the cases with aneurysmal dilatation of the BJV conduit were complicated with branch pulmonary stenosis up to 3.0 m/s (P = 0.004). No conduit infections occurred during the follow-up period, and no significant difference in conduit replacement (20.0% vs 8.3%,P = 0.43) was found between the BJV and ePTFE conduit groups, respectively. The outcomes of the RVOT reconstructions with BJV and ePTFE conduits were clinically satisfactory. Aneurysmal dilatation was found in the BJV conduit cases, with branch pulmonary stenosis as the risk factor.

DOI： 10.1007/s00246-020-02458-0

Web of Science

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Language：English   Publishing type：Research paper (scientific journal)  

DOI： 10.1126/scitranslmed.abb3336

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Language：Japanese   Publisher：(NPO)日本小児循環器学会  

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Language：Japanese   Publisher：(NPO)日本小児循環器学会  

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Language：Japanese   Publisher：(NPO)日本小児循環器学会  

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Language：Japanese   Publisher：(NPO)日本小児循環器学会  

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Language：Japanese   Publisher：(NPO)日本小児循環器学会  

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Language：Japanese   Publisher：(公社)日本超音波医学会  

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Language：English   Publishing type：Research paper (scientific journal)   Publisher：TECH SCIENCE PRESS  

Background: There is limited information on outcomes of adult patients with pulmonary atresia with intact ventricular septum (PA-IVS) due to the low incidence of disease and the large variation of surgical histories. Methods: Among 58 patients with repaired PA-IVS, a total of 32 patients aged >= 16 years and who were followed at our institution between January 2003 and December 2018 were reviewed. Surgical history, clinical outcomes, and laboratory, echocardiographic and electrocardiographic data were obtained by chart review. Results: Follow-up was from the age of 16 years and the median age at the latest follow-up was 23.7 years. Twenty-four patients had undergone biventricular repair (BVR), 3 had undergone one-and-a half ventricular repair (1.5VR), and 5 had undergone univentricular repair. Over a median follow-up period of 7.7 years (interquartile range: 4.1-11.0 years), 1 BVR patient died suddenly and 7 patients had heart failure. Arrhythmias were present in 5 patients. Ten patients underwent surgical re-interventions, including 4 BVR take-downs with conversion to 1.5VR and 3 Fontan conversions. Overall survival, heart failure-free, arrhythmia-free, and surgical re-intervention-free rates at 5 years and 10 years from the age of 16 years were 96.2% (95% confidence interval [CI], 77.2-99.4) and 96.2% (95% CI, 77.2-99.4), 81.4% (95% CI, 62.1-92.1) and 74.6% (95%CI, 52.3-88.7), 88.7% (95% CI, 70.1-96.3) and 75.9% (95% CI, 51.7-90.2), and 80.7% (95% CI, 60.8-91.8) and 70.8% (95% CI, 49.7-85.7), respectively. Conclusion: Adults with PA-IVS have preserved long-term survival regardless of the early operative strategy, while they are at risk for heart failure, arrhythmia, and surgical re-intervention. Thus, detailed and continued follow-up is mandatory for all PA-IVS patients from childhood to adulthood.

DOI： 10.32604/CHD.2020.011579

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Language：English   Publishing type：Research paper (scientific journal)  

Purpose: To prospectively evaluate the efficacy and safety of embolization using hydrogel-coated coils for the treatment of pulmonary arteriovenous malformations (PAVMs). Materials and methods: The outcomes of 21 PAVMs in 19 patients (3 men and 16 women; mean age, 58.8 ± 15.2 [SD] years; age range 14–78 years) treated by venous sac embolization (VSE) with additional feeding artery embolization were prospectively evaluated. For VSE, using one or more 0.018-inch hydrogel-coated coils was mandatory. Recanalization and/or reperfusion were evaluated by pulmonary arteriography 1 year after embolization. Results: The mean feeding artery and venous sac sizes were 4.0 mm and 8.5 mm, respectively. Embolization was successfully completed in 20/21 PAVMs, yielding a technical success rate of 95%. The feeding artery was also embolized in 17/20 successful PAVMs (85%). A technical failure occurred in one PAVM, where embolization was abandoned because of migration of one bare coil to the left ventricle. The mean numbers of hydrogel-coated coils and bare platinum detachable coils used for VSE were 3.3 ± 2.1 (SD) (range, 1–8) and 4.4 ± 3.9 (SD) (range, 1–17), respectively. The mean percentages of hydrogel-coated coils in number, length, and estimated volume were 42.9%, 33.3%, and 72.7% respectively. One patient with one PAVM was lost to follow-up after 3 months. Neither recanalization nor reperfusion was noted in the remaining 19 PAVMs (success rate, 19/19 [100%]). One grade 4 (coil migration) adverse event occurred, and it was treated without any sequelae. Conclusion: VSE using hydrogel-coated coils with additional feeding artery embolization is a safe and effective treatment for PAVM.

DOI： 10.1016/j.diii.2019.10.008

Scopus

PubMed

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Language：English   Publisher：日本成人先天性心疾患学会  

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Authorship：Corresponding author   Language：English   Publishing type：Research paper (scientific journal)   Publisher：Cambridge University Press (CUP)  

Abstract

Background:

Pulmonary vein stenosis (PVS) is a condition with challenging treatment and leads to severe cardiac failure and pulmonary hypertension. Despite aggressive surgical or catheter-based intervention, the prognosis of PVS is unsatisfactory. This study aimed to assess the prognosis and to establish appropriate treatment strategies.

Methods:

We retrospectively reviewed endovascular treatments for PVS (2001–2017) from the clinical database at the Okayama University Hospital.

Results:

A total of 24 patients underwent PVS associated with total anomalous pulmonary venous connection and 7 patients underwent isolated congenital PVS. In total, 53 stenotic pulmonary veins were subjected to endovascular treatments; 40 of them were stented by hybrid (29) and percutaneous procedures (11) (bare-metal stent, n = 34; drug-eluting stent, n = 9). Stent size of hybrid stenting was larger than percutaneous stenting. Median follow-up duration from the onset of PVS was 24 months (4–134 months). Survival rate was 71 and 49% at 1 and 5 years, respectively. There was no statistically significant difference between stent placement and survival; however, patients who underwent bare-metal stent implantation had statistically better survival than those who underwent drug-eluting stent implantation or balloon angioplasty. Early onset of stenosis, timing of stenting, and small vessel diameter of pulmonary vein before stenting were considered as risk factors for in-stent restenosis. Freedom from re-intervention was 50 and 26% at 1 and 2 years.

Conclusions:

To improve survival and stent patency, implantation of large stent is important. However, re-intervention after stenting is also significant to obtain good outcome.

DOI： 10.1017/s1047951119001495

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Language：Japanese   Publisher：(NPO)日本小児循環器学会  

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Language：Japanese   Publisher：(NPO)日本小児循環器学会  

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Language：Japanese   Publisher：(NPO)日本小児循環器学会  

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Language：Japanese   Publisher：(NPO)日本小児循環器学会  

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Language：Japanese   Publisher：(NPO)日本小児循環器学会  

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Language：Japanese   Publisher：(NPO)日本小児循環器学会  

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Language：Japanese   Publisher：(NPO)日本小児循環器学会  

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Language：Japanese   Publisher：(NPO)日本小児循環器学会  

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Language：English   Publishing type：Research paper (scientific journal)   Publisher：WILEY  

BackgroundRashkind balloon atrial septostomy (BAS) can be challenging in infants with hypoplastic left heart syndrome (HLHS) and small atrial septal defect (ASD).MethodsWe retrospectively reviewed all infants with HLHS who underwent surgery and BAS between January 2006 and December 2015. The infants were divided into three groups: no BAS; catheter BAS; and open AS. Infants who underwent catheter BAS were divided into two groups based on atrial septal anatomy: standard and complex.ResultsOf the 70 patients, 57 (81%) underwent Glenn surgery. Subsequently, a significant difference in survival was observed: 86% (44/51), 91% (10/11), and 25% (2/8) in the no BAS, catheter BAS, and open AS groups, respectively (P = 0.0002). No significant difference was seen between the no BAS and the catheter BAS groups (P = 1.0). In the 56 patients who underwent catheterization after surgery, no intergroup differences in mean pulmonary artery pressure, pulmonary vascular resistance, or pulmonary artery index were found. We classified catheter BAS into standard (n = 5) and complex (n = 5) based on ASD location, and septum thickness. All patients in the standard group underwent complete Rashkind BAS, but in the complex group, only one patient underwent complete Rashkind BAS, with the remaining requiring initial static BAS (P = 0.048). Following septostomy, ASD size, ASD flow, and percutaneous oxygen saturation (SpO(2)) were not significantly different between the two groups.ConclusionsCatheter BAS is effective in infants with HLHS and a restrictive atrial septum. Infants with standard or complex atrial septum can achieve equivalent outcomes despite more patients often requiring static BAS.

DOI： 10.1111/ped.13716

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Language：English   Publisher：(一社)日本循環器学会  

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Language：English   Publishing type：Research paper (scientific journal)  

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Language：English   Publisher：日本成人先天性心疾患学会  

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Language：Japanese   Publisher：(一社)日本心臓病学会  

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Language：English   Publishing type：Research paper (scientific journal)  

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Language：Japanese   Publisher：(NPO)日本小児循環器学会  

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Language：Japanese   Publisher：(NPO)日本小児循環器学会  

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Language：Japanese   Publisher：(NPO)日本小児循環器学会  

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Language：Japanese   Publisher：(NPO)日本小児循環器学会  

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Language：Japanese   Publishing type：Research paper (scientific journal)  

J-GLOBAL

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Language：Japanese   Publisher：日本小児高血圧研究会  

【背景】腎血管性高血圧は小児高血圧の主要な原因の一つと言われているが、乳児例の報告は少なく、また診断に時間を要することが多い。今回、生後3ヵ月頃から網状チアノーゼの症状を認めたレニン依存性高血圧の男児例を経験した。【症例】周産期には異常なかったが1ヵ月健診で体重増加不良、3ヵ月健診で網状チアノーゼを指摘され、心エコーで拡張型心筋症が疑われていた。経過中に胃腸炎に罹患した際、心不全の状態となり、同時に高血圧も認められた。入院後、心機能改善に伴い高血圧が増悪したため、高血圧性心疾患として原因検索したところ、血漿レニン活性およびアルドステロン高値の状態であった。造影CTでは両側に複数腎動脈が認められ、局所的な狭窄はなかったが、いずれの腎動脈も全長にわたって狭小であり、これらの血管による腎灌流圧の低下が病態の本態と考えた。その後、降圧薬2剤で血圧は良好にコントロールできるようになり、現在外来で経過観察されている。【考察】本症例は、レニン依存性高血圧の状態であり、腎血管性が疑われた。本症例と同様に、乳児期の腎血管性高血圧の症例は、症状が非特異的であり、心不全の状態で診断されることも多いため、注意を要する。また、病態も局所的な腎血管狭窄に限らず、腎灌流圧の低下をきたしうる病変を幅広く検討する必要がある。(著者抄録)

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Authorship：Corresponding author   Language：English   Publishing type：Research paper (scientific journal)  

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Authorship：Corresponding author   Language：English   Publishing type：Research paper (scientific journal)  

BACKGROUND: Almost all reports on cardiac regeneration therapy have referred to adults, and only a few have focused on transcoronary infusion of cardiac progenitor cells using the stop-flow technique in children. METHODS: Intracoronary autologous cardiosphere-derived cell (CDC) transfer was conducted at Okayama University as a phase 1 clinical trial for seven patients with hypoplastic left heart syndrome between January 2011 and December 2012, and as a phase 2 clinical trial for 34 patients with single-ventricle physiology between July 2013 and March 2015. RESULTS: A total of 41 patients with single-ventricle physiology underwent transcoronary infusion of CDC with the stop-flow technique. The median age was 33 months (range, 5-70 months) and the median weight was 10.1 kg (range, 4.1-16.0 kg). Transient adverse events occurred during the procedure, including ST-segment elevation or depression, hypotension, bradycardia, and coronary artery vasospasm. All patients completely recovered. There were no major procedure-related adverse events. In this study, transcoronary infusion of CDC using the stop-flow technique was successfully completed in all patients. CONCLUSION: Transcoronary infusion of CDC using the stop-flow technique in children is a feasible and safe procedure.

DOI： 10.1111/ped.13485

PubMed

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Language：Japanese   Publisher：日本成人先天性心疾患学会  

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Publishing type：Research paper (scientific journal)  

DOI： 10.24509/jpccs.180206

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Language：Japanese   Publisher：(NPO)日本小児循環器学会  

背景:左心低形成症候群(HLHS)では左房容積が小さく、心房中隔の形態や心房中隔欠損(ASD)の大きさによってRashkind Balloon Atrial Septostomy(BAS)を行うことが困難な場合もある。目的:当院におけるHLHSに対するBASの有効性について検討した。対象と方法:2006年1月から2015年12月までの10年間にHLHSに対してカテーテル治療・手術を施行した患者におけるBASの有効性について検討した。またcatheter BASを施行した群についてASDの形態別に分けて有効性について両群比較検討した。結果:全HLHS70例中のGlenn手術到達は57例(81%)であり、BAS未施行群では86%(44/51)、catheter BAS群では91%(10/11)、open BAS群では25%(2/8)と統計学的有意差を認めたが(p=0.0002)、BAS未施行群とcatheter BAS群では同等の結果であった。(p=1.0)Glenn手術後にカテーテル検査を施行した56例について検討すると、BAS未施行群、catheter BAS施行群、open BAS施行群の平均肺動脈圧、肺血管抵抗、PAIいずれも3群間に差は認めなかった。次にCatheter BAS施行群をASDの位置・大きさ・atrial septumの厚さからstandard ASD(n=5)、complex ASD(n=5)に分類し、ASDの形態別にBAS施行方法を検討すると、standard ASD群では全例Rashkind BAS単独施行で効果を得たが、complex ASD群ではRashkind BAS単独施行症例は1例のみで、Static BASを先行させRashkind BASに到達した症例が4例であった(p=0.048)。BAS後のASD size、ASD flow、SpO2は2群間で統計学的有意差を認めなかった。結論:catheter BASは有効で、Glenn後のカテーテルデータではBAS未施行群と有意差は認めなかった。またcomplex ASD群の場合には、Static BASを先行し、Rashkind BASを追加することで、standard ASD群と同等の効果を得ることができた。(著者抄録)

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Other Link： https://search.jamas.or.jp/index.php?module=Default&action=Link&pub_year=2017&ichushi_jid=J02003&link_issn=&doc_id=20180418180002&doc_link_id=%2Fee7shoni%2F2017%2F003306%2F003%2F0423-0430%26dl%3D0&url=https%3A%2F%2Fwww.medicalonline.jp%2Fjamas.php%3FGoodsID%3D%2Fee7shoni%2F2017%2F003306%2F003%2F0423-0430%26dl%3D0&type=MedicalOnline&icon=https%3A%2F%2Fjk04.jamas.or.jp%2Ficon%2F00004_2.gif

Language：Japanese   Publisher：(一社)日本心臓病学会  

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DOI： 10.1155/2017/2594231

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Language：Japanese   Publisher：(公社)日本小児科学会  

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Language：Japanese   Publisher：(NPO)日本小児循環器学会  

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Language：Japanese   Publisher：(NPO)日本小児循環器学会  

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Language：Japanese   Publisher：(NPO)日本小児循環器学会  

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Language：Japanese   Publishing type：Research paper (scientific journal)   Publisher：Japanese Society of Pediatric Cardiology and Cardiac Surgery  

<p>Background: Protein-losing enteropathy (PLE) following the Fontan operation has been an extremely difficult complication to treat successfully. This study aimed to assess the prognosis and to establish appropriate treatment strategies for PLE.</p><p>Methods: From 1991 to 2014, 23 patients with PLE following the Fontan operation were identified from clinical database at Okayama University Hospital. Data were collected retrospectively.</p><p>Results: A total of 401 patients underwent Fontan operation at our institution during the study period. In this cohort, the 5.7％ incidence of PLE was relative to the total number of Fontan operations. Following PLE diagnosis, patients had a 68％ and 54％ survival rate at 5 and 10 years, respectively. The causes of death included sepsis, multiple organ failure, heart failure, and intracranial bleeding. In the treatment of PLE, 23 (100％) patients were treated with medication, 10 (43％) patients underwent catheter intervention and 15 (65％) patients underwent surgery. Patients who died were older at the time of PLE diagnosis and were associated with a longer interval between the Fontan operation and PLE diagnosis. Decreased survival was observed in patients with the following conditions post-PLE treatment: a cardiac index (CI)＜2.8 L/min/mm, decreased ventricular ejection fraction (vEF)＜50％, and high central venous pressure (meanCVP)≧15 mmHg.</p><p>Conclusion: Our current treatment for PLE was only partially successful. Strategies for reducing CVP and improving both CI and vEF may be effective. Further studies are necessary to determine better treatment strategies for the future.</p>

DOI： 10.9794/jspccs.33.202

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Language：Japanese   Publisher：(一社)日本小児救急医学会  

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Language：Japanese   Publisher：(一社)日本小児外科学会  

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Language：English   Publishing type：Research paper (scientific journal)   Publisher：LIPPINCOTT WILLIAMS & WILKINS  

Rationale: Patients with single ventricle physiology are at high risk of mortality resulting from ventricular dysfunction. The preliminary results of the phase 1 trial showed that cardiosphere-derived cells (CDCs) may be effective against congenital heart failure.Objective: To determine whether intracoronary delivery of autologous CDCs improves cardiac function in patients with single ventricle physiology.Methods and Results: We conducted a phase 2 randomized controlled study to assign in a 1: 1 ratio 41 patients who had single ventricle physiology undergoing stage 2 or 3 palliation to receive intracoronary infusion of CDCs 4 to 9 weeks after surgery or staged reconstruction alone (study A). The primary outcome measure was to assess improvement in cardiac function at 3-month follow-up. Four months after palliation, controls had an alternative option to receive late CDC infusion on request (study B). Secondary outcomes included ventricular function, heart failure status, somatic growth, and health-related quality of life after a 12-month observation. At 3 months, the absolute changes in ventricular function were significantly greater in the CDC-treated group than in the controls (+ 6.4% [SD, 5.5] versus + 1.3% [SD, 3.7]; P= 0.003). In study B, a late CDC infusion in 17 controls increased the ventricular function at 3 months compared with that at baseline (38.8% [SD, 7.7] versus 34.8% [SD, 7.4]; P< 0.0001). At 1 year, overall CDC infusion was associated with improved ventricular function (41.4% [SD, 6.6] versus 35.0% [SD, 8.2]; P< 0.0001) and volumes (P< 0.001), somatic growth (P< 0.0001) with increased trophic factors production, such as insulin-like growth factor-1 and hepatocyte growth factor, and quality of life, along with a reduced heart failure status (P< 0.0001) and cardiac fibrosis (P= 0.014) relative to baseline.Conclusions: Intracoronary infusion of CDCs after staged palliation favorably affected cardiac function by reverse remodeling in patients with single ventricle physiology. This impact may improve heart failure status, somatic growth, and quality of life in patients and reduce parenting stress for their families.

DOI： 10.1161/CIRCRESAHA.116.310253

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Language：Japanese   Publisher：日本成人先天性心疾患学会  

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Language：English   Publishing type：Research paper (scientific journal)   Publisher：OXFORD UNIV PRESS INC  

To achieve the growth of right-sided heart structures, our choice of the first palliation for patients with pulmonary atresia and intact ventricular septum (PA-IVS) includes a modified Blalock-Taussig shunt (BTS) with pulmonary valvotomy. We sought to analyse the impact of the first palliation on the growth of right-sided heart structures and factors associated with a choice of definitive surgical procedure.Fifty patients with PA-IVS who underwent a staged surgical approach from 1991 to 2012 were retrospectively reviewed.Right ventricular (RV)-coronary artery fistulas were seen in 42% of patients at the time of birth. All 50 patients had a modified BTS with pulmonary valvotomy. Six patients died after the first palliation or inter-stage. Thirty patients achieved a biventricular repair (BVR group), 6 patients had a 1 + 1/2 ventricular repair (1 + 1/2V group) and 5 patients had a Fontan completion (Fontan group). After modified BTS with pulmonary valvotomy, tricuspid valve z-score did not increase in any of the group (BVR: pre -2.79 vs post -2.24, 1 + 1/2V: pre -5.25 vs post -6.69, Fontan: pre -6.82 vs post -7.94). Normalized RV end-diastolic volume increased only in BVR group after modified BTS with pulmonary valvotomy (BVR: pre 32% vs post 64%, 1 + 1/2V: pre 43% vs post 42%, Fontan: pre 29% vs post 32%). Major RV-coronary artery fistula was a strong factor with proceeding single-ventricle palliation [BVR: 4/30 (13%) patients, 1 + 1/2V: 1/6 (17%) and Fontan: 4/5 (80%)].Tricuspid valve growth was not obtained by modified BTS with pulmonary valvotomy; therefore, tricuspid valve size at birth appeared to be a predictor for achieving BVR. Proportionate RV growth was seen only in patients who achieved BVR. However, RV growth was not seen in patients having 1 + 1/2 ventricular repair. Major RV-coronary artery fistula was a strong predictor for proceeding single-ventricle palliation.

DOI： 10.1093/ejcts/ezw124

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Language：Japanese   Publisher：(一社)日本先天性心疾患インターベンション学会  

背景:心筋再生医療は,成人を対象としたものがほとんどで小児を対象とした臨床研究は極めて少ない.また当院で実施した小児の機能的単心室症に対する自己心臓内幹細胞移植の際に我々が行っている冠動脈バルーン閉鎖下心筋幹細胞注入の小児におけるまとまった報告は少ない.方法:岡山大学病院において冠動脈バルーン閉鎖下心筋幹細胞注入による自己心臓内幹細胞移植が第1相試験として2011年1月から2012年12月までに左心低形成症候群患者を対象に7例,また第2相試験として2013年7月から2015年3月までに機能的単心室症患者を対象に34例,合計41例に行われた.小児に対する冠動脈バルーン閉鎖下心筋幹細胞注入の手技,有害事象に関して検討を行った.結果:対象41例の自己心臓内幹細胞移植時の年齢と体重はそれぞれ中央値33ヵ月,10.1kgであった.冠動脈バルーン閉鎖下心筋幹細胞注入時に心電図上ST変化,血圧低下,脈拍低下,冠動脈攣縮を認めたが,いずれも一過性であり,重篤な合併症を認めず,全例で安全に手技を完遂できた.結語:冠動脈バルーン閉鎖下心筋幹細胞注入は機能的単心室症を有する小児においても安全に施行可能である.(著者抄録)

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Language：Japanese   Publisher：(NPO)日本小児循環器学会  

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Language：Japanese   Publisher：(NPO)日本小児循環器学会  

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Language：Japanese   Publisher：(NPO)日本小児循環器学会  

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Language：Japanese   Publisher：(NPO)日本小児循環器学会  

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Language：Japanese   Publisher：(一社)日本小児救急医学会  

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Language：Japanese   Publisher：(公社)日本超音波医学会  

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Language：Japanese   Publisher：(公社)日本小児科学会  

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Language：Japanese   Publisher：(公社)日本小児科学会  

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Language：English   Publisher：(NPO)日本小児循環器学会  

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Language：Japanese   Publisher：(NPO)日本小児循環器学会  

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Language：English   Publisher：(NPO)日本小児循環器学会  

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Language：Japanese   Publisher：(NPO)日本小児循環器学会  

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Language：Japanese   Publisher：(NPO)日本小児循環器学会  

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Language：Japanese   Publisher：(NPO)日本小児循環器学会  

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Language：English   Publishing type：Research paper (scientific journal)   Publisher：Lippincott Williams & Wilkins  

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Language：Japanese   Publisher：(NPO)日本小児循環器学会  

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Language：Japanese   Publisher：(NPO)日本小児循環器学会  

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Authorship：Lead author   Language：English   Publishing type：Research paper (scientific journal)  

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Language：Japanese   Publisher：(NPO)日本小児循環器学会  

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Language：Japanese   Publisher：(NPO)日本小児循環器学会  

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Language：Japanese   Publisher：(NPO)日本小児循環器学会  

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Language：Japanese   Publisher：(NPO)日本小児循環器学会  

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Language：Japanese   Publisher：(NPO)日本小児循環器学会  

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Authorship：Lead author   Language：English   Publishing type：Research paper (scientific journal)  

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Language：Japanese   Publisher：(公社)日本小児科学会  

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Language：Japanese   Publisher：(公社)日本小児科学会  

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Authorship：Lead author   Language：English   Publishing type：Research paper (scientific journal)  

BACKGROUND: Hybrid and Norwood strategies differ substantially in terms of stage II palliative procedures. We sought to compare these strategies with an emphasis on survival and reintervention after stage II and subsequent Fontan completion. METHODS AND RESULTS: Of 110 neonates with functionally single-ventricle physiology who underwent stage I palliation between 2004 and 2010, 75 (69%) infants (Norwood, n=43; hybrid, n=32) who subsequently underwent stage II palliation were studied. Survival and reintervention rates after stage II palliation, anatomic and physiologic variables at pre-Fontan assessment, and Fontan outcomes were compared between the groups. Predictors for reintervention were analyzed. Freedom from death/transplant after stage II palliation was equivalent between the groups (Norwood, 80.4% versus hybrid, 85.6% at 3 years, P=0.66). Hybrid patients had a higher pulmonary artery (PA) reintervention rate (P=0.003) and lower Nakata index at pre-Fontan evaluation (P=0.015). Aortic arch and atrioventricular valve reinterventions were not different between the groups. Ventricular end-diastolic pressure, mean PA pressure, and ventricular function were equivalent at pre-Fontan assessment. There were no deaths after Fontan completion in either group (Norwood, n=25, hybrid, n=14). CONCLUSIONS: Survival after stage II palliation and subsequent Fontan completion is equivalent between the groups. The hybrid group had a higher PA reintervention rate and smaller PA size. Both strategies achieved adequate physiology for Fontan completion. Evolution of the hybrid strategy requires refinement to provide optimal PA growth.

PubMed

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Language：Japanese   Publisher：(NPO)日本小児循環器学会  

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Language：Japanese   Publisher：(NPO)日本小児循環器学会  

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Language：Japanese   Publisher：(NPO)日本小児循環器学会  

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Language：Japanese   Publisher：(NPO)日本小児循環器学会  

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Language：Japanese   Publisher：(NPO)日本小児循環器学会  

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Language：Japanese   Publisher：(NPO)日本小児循環器学会  

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Language：Japanese   Publisher：(公社)日本小児科学会  

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Language：English   Publishing type：Research paper (scientific journal)   Publisher：Wiley  

DOI： 10.1111/j.1442-200x.2008.02731.x

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Language：Japanese   Publisher：(NPO)日本小児循環器学会  

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Authorship：Lead author,　Corresponding author   Language：Japanese   Publishing type：Article, review, commentary, editorial, etc. (trade magazine, newspaper, online media)  

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Language：Japanese   Publisher：(NPO)日本小児循環器学会  

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J-GLOBAL

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J-GLOBAL

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Authorship：Lead author,　Corresponding author   Language：Japanese  

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Language：Japanese   Publisher：(公財)日本心臓財団  

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Language：Japanese   Publisher：(NPO)日本小児循環器学会  

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Language：English   Publisher：日本成人先天性心疾患学会  

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Authorship：Lead author,　Corresponding author   Language：Japanese   Publishing type：Article, review, commentary, editorial, etc. (scientific journal)  

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Language：Japanese   Publisher：(NPO)日本小児循環器学会  

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Language：English   Publishing type：Research paper, summary (international conference)   Publisher：ELSEVIER SCIENCE INC  

DOI： 10.1016/S0735-1097(16)30948-2

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Language：Japanese   Publisher：Japanese Society of Pediatric Cardiology and Cardiac Surgery  

DOI： 10.9794/jspccs.32.509

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J-GLOBAL

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Language：English   Publishing type：Research paper, summary (international conference)   Publisher：LIPPINCOTT WILLIAMS & WILKINS  

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Authorship：Lead author,　Corresponding author   Language：Japanese  

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J-GLOBAL

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Authorship：Lead author,　Corresponding author  

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Language：Japanese   Publisher：(公社)日本小児科学会  

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Event date： 2017.1.26 - 2017.1.28

Language：Japanese   Presentation type：Oral presentation (general)  

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Event date： 2017.1.26 - 2017.1.28

Language：Japanese   Presentation type：Oral presentation (general)  

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Event date： 2017.1.14 - 2017.1.15

Language：Japanese   Presentation type：Oral presentation (general)  

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Event date： 2015.7

Language：Japanese   Presentation type：Symposium, workshop panel (public)  

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Event date： 2015.1

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Event date： 2014.7

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Event date： 2014.6

Language：English   Presentation type：Poster presentation  

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Event date： 2011.11.12 - 2011.11.16

Language：English   Presentation type：Oral presentation (general)  

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