Language：English   Publisher：Journal of Cardiology Cases  

Non-reentrant fascicular tachycardia (NRFT) developed in a 6-year-old Japanese boy. Because of drug-resistant recurrences, he received catheter mapping and ablation at age 10 years. An electrocardiogram exhibited a superior left-axis deviation, a right bundle branch block-type configuration, and relatively narrow QRS with sharp R wave. It suggested verapamil-sensitive ventricular tachycardia (VT), but showed no sensitivity to verapamil or reentrant characteristics in the electrophysiological study. Detailed VT mapping determined the earliest presystolic Purkinje potential on the left posterior fascicle at the mid-ventricular septum. Radiofrequency current applications to the lesion led to his NRFT-free life without restriction. Learning objectives: Purkinje-related idiopathic ventricular tachycardias (VTs) are commonly due to reentrant mechanisms, and non-reentrant fascicular tachycardia (NRFT) is a rare form of idiopathic VT in adults. Although it is crucial to distinguish NRFT from reentrant VTs, there is no information about the electrophysiological studies and the treatment effect in pediatric-onset NRFT.

DOI： 10.1016/j.jccase.2023.12.002

Scopus

PubMed

Language：Japanese   Publisher：Japanese Society of Pediatric Interventional Cardiology  

The Japanese Society of Congenital Interventional Cardiology (JCIC) had conducted the annual questionnaire surveillance regarding catheter-based interventional procedures and adverse events since 1993. The internet registry system named JCIC-Registry (JCIC-R) went into operation with the initial enrollment of the actual cases since January 2013. In this report, we demonstrate annual 4,554 catheter-based therapeutic sessions during 2020, the fifth year after complete transition to online registration. The JCIC-R maintains specific feature of the nationwide comprehensive real-world registry involving any types of interventions and ablations for congenital heart disease, cardiovascular sequelae following Kawasaki disease, and tachyarrhythmias. In 2020, the audit started to ensure the quality and the completion of registry data, and the JCIC-R is becoming more reliable. In addition to benchmarking and multi-institutional research, we effectively utilize the registry in various fields, such as new device development with post-marketing surveillance, application, and approval process.

DOI： 10.20599/jjcic.8.25

CiNii Research

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

<p>Vascular embolization, specifically coil embolization, is performed widely in pediatric cardiology. Indications for embolization have been increasing because of the development of devices in recent years. Provided the necessity to embolize diverse anatomic vessels in congenital heart disease, it is crtitical to carefully consider the risks and precautions associated with each vascular anatomy when developing a treatment strategy. This article describes the basic techniques and special considerations for vascular anatomy embolization that include our own approaches.</p>

DOI： 10.9794/jspccs.39.217

CiNii Research

Language：English   Publisher：Pediatric Cardiology  

Multiorgan dysfunction is a concern of Fontan patients. To clarify the pathophysiology of Fontan nephropathy, we characterize renal disease in the long-term observational study. Medical records of 128 consecutive Fontan patients [median age: 22 (range 15–37) years old] treated between 2009 and 2018 were reviewed to investigate the incidence of nephropathy and its association with other clinical variables. Thirty-seven patients (29%) showed proteinuria (n = 34) or < 90 mL/min/1.73 m2 of estimated glomerular filtration rate (eGFR) (n = 7), including 4 overlapping cases. Ninety-six patients (75%) had liver dysfunction (Forns index > 4.21). Patients with proteinuria received the Fontan procedure at an older age [78 (26–194) vs. 56 (8–292) months old, p = 0.02] and had a higher cardiac index [3.11 (1.49–6.35) vs. 2.71 (1.40–4.95) L/min/m2, p = 0.02], central venous pressure [12 (7–19) vs. 9 (5–19) mmHg, p < 0.001], and proportion with > 4.21 of Forns index (88% vs. 70%, p = 0.04) than those without proteinuria. The mean renal perfusion pressure was lower in patients with a reduced eGFR than those without it [55 (44–65) vs. 65 (45–102) mmHg, p = 0.03], but no other variables differed significantly. A multivariable analysis revealed that proteinuria was associated with an increased cardiac index (unit odds ratio 2.02, 95% confidence interval 1.12–3.65, p = 0.02). Seven patients with severe proteinuria had a lower oxygen saturation than those with no or mild proteinuria (p = 0.01, 0.03). Proteinuria or a decreased eGFR differentially occurred in approximately 30% of Fontan patients. Suboptimal Fontan circulation may contribute to the development of proteinuria and reduced eGFR.

DOI： 10.1007/s00246-023-03334-3

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PubMed

Language：English   Publisher：Pediatrics International  

DOI： 10.1111/ped.15500

Web of Science

Scopus

PubMed

Language：English   Publisher：Pediatrics International  

DOI： 10.1111/ped.15503

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Scopus

PubMed

Language：Japanese   Publisher：一般社団法人 日本移植学会  

<p>【背景】当院は2020年5月に大阪以西で唯一の小児心臓移植実施施設に認定された。それ以前は九州・山口地区で心移植を希望する小児重症心不全患者は遠方の医療施設に頼らなければならず、治療の選択が狭められることもあった。当院の小児心臓移植を中心とした重症心不全診療の現状と課題を考察する。【現状】機械的循環補助や心移植を考慮する症例は、毎年5例程度の診療依頼がある。うち2020年以降に新たに心臓移植登録したのは拡張型心筋症４例、先天性心疾患１例で、4例が登録時11歳未満であった。人口およそ1400万人の九州・山口地区で年1-2例の小児心臓移植適応患者が発生することとなる。3例が補助人工心臓を装着して待機し、2022年9月に11歳未満の小児第１例目の心臓移植を実施した。【課題】小児ドナー不足はコロナ禍で拍車がかかり、当院の移植待機患者は最長で4年目となった。待機が長期化するほど感染や血栓など合併症のリスクは増加し、患者や家族の精神的負担も大きな問題となる。また長期入院は病棟運営にも少なからず影響する。【解決に向けて】ドナーを増やす努力が解決への鍵となるが、年々移植医療に対する医療者および市民の意識は高まってきている。また九州・山口地区は地域間の繋がりも強く、情報を共有し地区全体で重症児を支える意識を持てば、より良い小児重症心不全診療に繋がる。</p>

DOI： 10.11386/jst.58.supplement_s196_2

CiNii Research

Language：English   Publisher：John Wiley & Sons Australia, Ltd  

症例は9ヵ月齢男児。Down症候群とFallot四徴症に罹患していた。右室流出路閉塞は比較的軽症であり、出生以降、チアノーゼまたは無酸素発作のエピソードはみられていなかった。9ヵ月齢になってから点頭発作を伴う強直性攣縮がみられ始めた。脳波検査でヒプスアリスミアが示されたことからWest症候群と確定診断され、副腎皮質刺激ホルモン(ACTH)治療を行うため当院へ入院となった。入院6日目にACTH治療を開始したが、同治療6日目の哺乳後に徐脈(心拍数60bpm)とチアノーゼを発症した。心エコー法では右室流出路閉塞の悪化と肺血流量の減少が示された。無酸素発作を疑い、体位をchest-knee positionとしたところ、容量輸液を行わずとも約30分後に発作は回復した。以降も徐脈がみられ無酸素発作を間欠的に4回発症したことからACTH治療は中止した。その中止から2日後の心拍数は110bpmまで回復し、無酸素発作はもはやみられなくなっていた。ACTH中止後はトピラマートを使用した。以降も無酸素発作または攣縮発作はみられず退院した。14ヵ月齢時に心内修復術が施行され、術後経過に問題はみられず、6ヵ月間の経過観察中にもてんかん性攣縮は再発しなかった。

Language：English   Publisher：John Wiley & Sons Australia, Ltd  

症例は13歳男児。夜間に強い胸痛が出現し、当院へ緊急入院となった。心電図ではST上昇が、血液検査では血清心筋トロポニンI濃度の高値が示された。血管造影中のアセチルコリン誘発試験では冠動脈攣縮の証拠が確かめられた。経皮的酸素飽和度は93%と軽症の低酸素血症が示された。造影CTでは両肺野に多発する肺動静脈奇形が描出された。履歴を聴取したところ、患児は幼い頃から鼻出血を繰り返しており、また父には皮膚動静脈奇形の家族歴があることが判明した。低酸素血症を改善させるため、全ての肺動静脈奇形に対し、Amplatzerバスキュラープラグとマイクロコイルを用いた経皮的塞栓術を施行した。塞栓術後、安静にしている状態で狭心症の症状が再発した。しかしその後はカルシウム遮断薬のベラパミル50mg/日を予防的に投与することで狭心症の症状は完全に制御された。遺伝性出血性毛細血管拡張症の4大基準のうち2つ(肺動静脈奇形と再発性鼻出血)がみられたことから分子遺伝学的検査を実施した。その結果、ENG遺伝子と他7つの遺伝子を含む、200000塩基を超えるヘテロ接合型大規模欠失が認められた。

Language：English   Publisher：Pediatric Pulmonology  

Introduction: Pulmonary agenesis is a complete absence of the pulmonary parenchyma, airways, and vasculature unilaterally or bilaterally. Although bilateral cases are lethal, the outcome of unilateral cases remains not well described. We performed a comprehensive literature review to assess the clinical features of pulmonary agenesis. Methods: Four database sources were searched on October 10, 2021 and two cases were included from our institution. Studies related to the clinical impact of comorbidity and intervention on the survival outcome in pulmonary agenesis were included for full-text review. Results: We identified 259 patients—with right-sided (59%), left-sided (34%), and bilateral agenesis (7%)—among 195 articles and our two cases. Additional anomalies included cardiovascular (40%), skeletal (30%), gastrointestinal (20%), tracheal (20%: all stenoses), and genitourinary (14%) anomalies. Fifty-seven (24%) individuals in unilateral pulmonary agenesis had isolated disease. Outcomes related to survival were not uniformly reported, but the 2-year overall survival rate of unilateral agenesis was 62% and no subsequent death was reported until 13 years of age. The right-sided agenesis was more frequently associated with tracheal stenosis (27% vs. 11%, p = 0.003) than the left-sided disease. A multivariable analysis indicated that tracheal stenosis (hazard ratio [HR]: 2.2, 95% confidence interval [CI]: 1.3–4.1, p = 0.003) and gastrointestinal anomalies (HR: 2.0, 95% CI: 1.1–3.3, p = 0.010) were prognostic factors for mortality. Conclusions: The poor prognostic factors were tracheal stenosis, right agenesis, and gastrointestinal anomalies. Treatment for these comorbidities is a key point for improving the survival of unilateral pulmonary agenesis.

DOI： 10.1002/ppul.26135

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PubMed

Language：English   Publisher：BMC Pediatrics  

Background: 22q11.2 deletion syndrome (22qDS) is the most common chromosomal microdeletion syndrome and is associated with a high rate of congenital heart disease (CHD) and neurodevelopmental abnormalities. Congenital portosystemic venous shunts (CPSS) are rare developmental abnormalities of the portal venous system. The clinical manifestations of CPSS are varied, and some patients have CHD or genetic chromosomal abnormalities, but their relationship remains unknown. We report the first case of CPSS associated with 22qDS. Case presentation: A newborn boy referred to our institution was diagnosed with 22qDS due to characteristic facial features and complications of tetralogy of Fallot. A subsequent newborn screening test indicated hypergalactosemia and high blood levels of ammonia and bile acids. Upon closer examination, these abnormalities were found to be caused by the CPSS. Abdominal contrast-enhanced computed tomography and angiography confirmed that abnormal blood vessels ascended from the splenic vein and short-circuited to the left renal vein. Intracardiac repair for CHD was performed at 1 year of age, followed by transcatheter occlusion of the CPSS using a multilayer device (vascular plug) and detachable coil at 2 years of age. After treatment, the abnormal blood parameters promptly normalized. Conclusions: As the blood flow of CPSS bypasses the liver, the levels of galactose, bile acids, and ammonia in the systemic veins can increase. Some patients with CPSS have CHD, and these toxic substances may cause liver and lung lesions as well as portosystemic encephalopathy (PSE). Several genetic chromosomal abnormalities, including 22qDS, and CPSS have similar symptoms, and neurodevelopmental abnormalities, particularly those caused by PSE, may be difficult to diagnose. Blood tests, such as newborn screening, and abdominal imaging are useful in the early diagnosis of CPSS.

DOI： 10.1186/s12887-022-03447-3

Web of Science

Scopus

PubMed

Language：Japanese   Publisher：Japanese Society of Pediatric Interventional Cardiology  

The Japanese Society of Congenital Interventional Cardiology (JCIC) had conducted the annual questionnaire surveillance regarding catheter-based interventional procedures and adverse events since 1993. The internet registry system named JCIC-Registry (JCIC-R) went into operation with the initial enrollment of the actual cases since January 2013. In this report, we demonstrate annual 4,805 catheter-based therapeutic sessions during 2019, the fourth year after complete transition to online registration. The JCIC-R maintains specific feature of the nationwide comprehensive real-world registry involving any types of interventions and ablations for congenital heart disease, cardiovascular sequelae following Kawasaki disease, and tachyarrhythmias. In addition to benchmarking and multi-institutional research, we effectively utilize the registry in various fields, such as new device development with post-marketing surveillance, application and approval process.

DOI： 10.20599/jjcic.6.17

CiNii Research

Language：English   Publisher：European Heart Journal - Case Reports  

Background: Multiple coronary-to-pulmonary artery fistulas (CPAFs) with giant coronary aneurysms (CAs) are extremely rare. The appropriate therapeutic indication and strategy for CPAFs have not been established. Case summary: Herein, we report the case of an asymptomatic 74-year-old woman with multiple CPAFs associated with giant CAs that had gradually developed over a 4-year period. After heart team discussion, we were successfully treated by minimally invasive intervention using transcatheter coil embolization and coronary stent implantation to prevent ruptures. Discussion: Coronary-to-pulmonary artery fistulas required evaluation of the appropriate timing of therapy initiation with reference to the presence of symptoms and fistula and aneurysm sizes, and determination of the optimal therapeutic approach with reference to the anatomy of the fistula with aneurysm and patient background characteristics.

DOI： 10.1093/ehjcr/ytac104

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Scopus

PubMed

Language：Japanese   Publisher：一般社団法人 日本超音波検査学会  

DOI： 10.11272/jssabst.47.0_s186_1

CiNii Research

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

DOI： 10.1016/j.ijcard.2018.04.062

Event date： 2021.8

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Country：Japan  

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