Language：English   Publishing type：Research paper (scientific journal)   Publisher：Heart and Vessels  

It is controversial whether children with isolated aortic valvular stenosis (vAS) initially undergo transcatheter or surgical aortic valvuloplasty (BAV or SAV). This multicenter retrospective case-control study aimed to explore outcomes after BAV or SAV for pediatric vAS. We studied children (aged < 15 years) with vAS treated at 4 tertiary congenital heart centers, and compared the rates of survival, reintervention, and valve replacement between patients with BAV and SAV. A total of 73 subjects (BAV: N = 52, SAV: N = 21) were studied. Age and aortic annulus z-score at the first presentation were 85 (26-530) days and - 0.45 (- 1.51-0.59), respectively. During the follow-up period of 121 (47-185) months, rates of 10-year survival (BAV: 88% vs. SAV: 92%, P = 0.477), reintervention (BAV: 58% vs. SAV: 31%, P = 0.626), and prosthetic/autograft valve replacement (BAV: 21% vs. SAV: 19%, P = 0.563) did not differ between the groups. Freedom from reintervention rate significantly correlated with aortic annulus z-score (hazard ratio [HR] 0.66, 95% confidence interval [CI] 0.49-0.88, P = 0.005), and freedom from prosthetic/autograft valve replacement rate significantly correlated to the degree of aortic regurgitation after the first intervention (HR: 4.58, 95% CI 1.19-17.71, P = 0.027). Propensity score-matched analysis (N = 16) did not show the differences in survival and reintervention rates between the groups. Long-term survival was acceptable, and the rates of freedom from reintervention and prosthetic/autograft valve replacement were comparable between children with vAS who underwent BAV and SAV.

DOI： 10.1007/s00380-024-02403-8

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Language：English   Publishing type：Research paper (scientific journal)   Publisher：Journal of Cardiology  

BACKGROUND: Elevated central venous pressure (CVP) and decreased arterial oxygen saturation (SaO2) are the characteristics of patients after Fontan operations and determine morbidity and mortality in the long-term. Oxygen inhalation therapy theoretically increases SaO2 and may decrease the elevated CVP in these patients. However, there is no previous study to support this hypothesis. This study aimed to determine the acute effects of oxygen inhalation on the hemodynamics of adult patients late after Fontan operations using cardiac catheterization. METHODS: This study enrolled 58 consecutive adult patients (median age, 30 years; female, n = 24) who had undergone Fontan operations. We assessed the hemodynamic changes during oxygen inhalation (2 L/min) with a nasal cannula in cardiac catheterization. We divided the studied patients into two groups according to the reduction in CVP during oxygen inhalation using the median value: responders (>2 mmHg) and non-responders (≤2 mmHg). Clinical characteristics of the responders to oxygen inhalation were investigated with uni- and multivariate analyses. RESULTS: SaO2 increased from 93.3 % (91.3-94.5 %) to 97.5 % (95.2-98.4 %) (p < 0.001) and CVP decreased from 12 mmHg (11-14 mmHg) to 10 mmHg (9-12 mmHg) (p < 0.001) after oxygen inhalation. There was a weak but significant correlation between the increase in SaO2 and the decrease in CVP (R = 0.29, p = 0.025). Pulmonary blood flow increased from 4.1 L/min (3.5-5.0 L/min) to 4.4 L/min (3.7-5.3 L/min) (p = 0.007), while systemic blood flow showed no significant changes. A multivariate analysis revealed that high baseline CVP was associated with a larger decrease in CVP (>2 mmHg) after oxygen inhalation. CONCLUSIONS: Oxygen inhalation increased SaO2 and decreased CVP, especially in patients with high baseline CVP. Further studies with home oxygen therapy are needed to investigate the long-term effects of oxygen inhalation in adult patients who underwent Fontan operations.

DOI： 10.1016/j.jjcc.2024.02.010

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Language：English   Publisher：シュプリンガー・ジャパン(株)  

Language：English   Publishing type：Research paper (scientific journal)   Publisher：American Heart Journal  

BACKGROUND: Previous reports reveal inconsistent findings of right ventricular (RV) changes following pregnancy in subjects with repaired tetralogy of Fallot (rTOF). METHODS: A two-center, retrospective cohort study which included women with rTOF who completed pregnancy that were matched to nulliparous women with rTOF by age at the time of baseline cardiac magnetic resonance (CMR), RV ejection fraction (RVEF), and indexed RV end-diastolic volume (RVEDVi). Pre-pregnancy and postpartum cardiac magnetic resonance (CMR) were analyzed and compared to sequential CMR of nulliparous subjects with rTOF. RESULTS: Thirty-six women with rTOF who completed pregnancy were matched to 72 nulliparous women with rTOF. Over a mean period of 3.1 years for the pregnancy group and 2.7 years for the comparison group, there was no significant change in the RVEDVi, RVEF, RV mass, pulmonary regurgitation severity, left ventricular (LV) volumes, LV ejection fraction (LVEF), or LV mass when comparing the baseline CMR and the follow-up CMR in either of the groups. There was a slight increase in RV indexed end-systolic volume (RVESVi) when comparing the baseline CMR and the follow-up CMR in the pregnancy group (68.93, SD 23.34 ml/m2 at baseline vs. 72.97, SD 25.24 mL/m2 at follow-up, P = .028). Using a mixed effects model for CMR parameters change over time; when adjusted for time between baseline and follow-up CMR there was no significant difference in rate of change between the pregnancy and comparison groups. CONCLUSIONS: Most ventricular remodeling parameters measured by CMR did not significantly change in subjects with rTOF who completed pregnancy or in nulliparous subjects with rTOF. In the pregnancy group, RVESVi is larger in those individuals who have undergone pregnancy without a significant change in ventricular function. These patients should be followed longitudinally to determine the long-term ventricular and clinical effects of pregnancy.

DOI： 10.1016/j.ahj.2024.04.015

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Language：English   Publishing type：Research paper (scientific journal)   Publisher：Journal of Cardiology Cases  

DOI： 10.1016/j.jccase.2024.04.008

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

GNAO1 encodes G protein subunit alpha O1 (Gαo). Pathogenic variations in GNAO1 cause developmental delay, intractable seizures, and progressive involuntary movements from early infancy. Because the functional role of GNAO1 in the developing brain remains unclear, therapeutic strategies are still unestablished for patients presenting with GNAO1-associated encephalopathy. We herein report that siRNA-mediated depletion of Gnao1 perturbs the expression of transcripts associated with Rho GTPase signaling in Neuro2a cells. Consistently, siRNA treatment hampered neurite outgrowth and extension. Growth cone formation was markedly disrupted in monolayer neurons differentiated from iPSCs from a patient with a pathogenic variant of Gαo (p.G203R). This variant disabled neuro-spherical assembly, acquisition of the organized structure, and polarized signals of phospho-MLC2 in cortical organoids from the patient's iPSCs. We confirmed that the Rho kinase inhibitor Y27632 restored these morphological phenotypes. Thus, Gαo determines the self-organizing process of the developing brain by regulating the Rho-associated pathway. These data suggest that Rho GTPase pathway might be an alternative target of therapy for patients with GNAO1-associated encephalopathy.

DOI： 10.1038/s41598-024-68062-x

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Language：English   Publishing type：Research paper (scientific journal)   Publisher：International Journal of Hygiene and Environmental Health  

The exposure of family caregivers to anticancer drugs for pediatric patients with malignancy is a potential health risk that needs to be minimized. We monitored the amount of cyclophosphamide (CPM) that had adhered to the undershirts of patients and the personal protective equipment (PPE) of family caregivers as well as the caregivers' urine levels of CPM within the first three days after the first and second courses of high-dose CPM therapy. Liquid chromatography/mass spectrometry (LC/MS/MS) detected >0.03 ng/ml of CPM in 26% (23/88) of urine samples from 8 of 11 (72.7%) patients' family caregivers, with a peak of 0.7 ng/ml from 24 to 48 h after administration. Since urine CPM concentrations in family caregivers varied after the first and second courses, the exposure risk factors were analyzed by scoring the PPE-wearing time index (caring minutes × PPE points from wearing masks, gloves, and/or gowns) and CPM adhesion of PPE items with the caring patterns of diaper change, washing body care, oral care, eating assistance, emotional support, and co-sleeping. The closest association was observed for CPM adhesion between oral care gloves and undershirts (correlation coefficient 0.67, p = 0.001). The mixed-effect model analysis indicated only a significant correlation between the PPE-wearing time index and emotional care (playing, cuddling, and physical contact) (p = 0.016). These results suggest that prolonged emotional support results in poor PPE protection, which increases the risk of exposure in family caregivers. Strict PPE care within 48 h after high-dose CPM controls the exposure to high-risk anticancer drugs in caregivers of pediatric patients.

DOI： 10.1016/j.ijheh.2024.114402

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Language：English   Publishing type：Research paper (scientific journal)   Publisher：Clinical and Experimental Immunology  

The clinical spectrum of Down syndrome (DS) ranges from congenital malformations to premature aging and early-onset senescence. Excessive immunoreactivity and oxidative stress are thought to accelerate the pace of aging in DS patients; however, the immunological profile remains elusive. We investigated whether peripheral blood monocyte-derived dendritic cells (MoDCs) in DS patients respond to lipopolysaccharide (LPS) distinctly from non-DS control MoDCs. Eighteen DS patients (age 2-47 years, 12 males) and 22 controls (age 4-40 years, 15 males) were enrolled. CD14-positive monocytes were immunopurified and cultured for 7 days in the presence of granulocyte-macrophage colony-stimulating factor and IL-4, yielding MoDCs in vitro. After the LPS-stimulation for 48 hours from days 7 to 9, culture supernatant cytokines were measured by multiplex cytokine bead assays, and bulk-prepared RNA from the cells was used for transcriptomic analyses. MoDCs from DS patients produced cytokines/chemokines (IL-6, IL-8, TNF-α, MCP-1, and IP-10) at significantly higher levels than those from controls in response to LPS. RNA sequencing revealed that DS-derived MoDCs differentially expressed 137 genes (74 upregulated and 63 downregulated) compared with controls. A gene enrichment analysis identified 5 genes associated with Toll-like receptor signaling (KEGG: hsa04620, P = 0.00731) and oxidative phosphorylation (hsa00190, P = 0.0173) pathways. MoDCs obtained from DS patients showed higher cytokine or chemokine responses to LPS than did control MoDCs. Gene expression profiles suggest that hyperactive Toll-like receptor and mitochondrial oxidative phosphorylation pathways configure the immunoreactive signature of MoDCs in DS patients.

DOI： 10.1093/cei/uxae048

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

DOI： 10.1002/ppul.26973

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Language：Japanese   Publisher：Japanese Society for Adult Congenital Heart Disease  

Most ventricular septal defects (VSDs) are diagnosed in childhood and are often closed spontaneously or operated on by the time they reach adulthood, and surgery is rarely indicated in adulthood. VSDs in adulthood may be small VSDs with a small shunt volume or VSDs already diagnosed with pulmonary hypertension and not indicated for surgery. In particular, there are cases of VSD with severe pulmonary hypertension diagnosed as Eisenmenger’s syndrome and followed up with only conservative treatment. However, with recent advances in pulmonary hypertension medications, it has been reported that “treat and repair”, in which VSD with severe pulmonary hypertension is treated with pulmonary hypertension medications followed by surgical treatment, can be effective. In this report, we describe two cases of VSD with severe pulmonary hypertension that were successfully treated surgically by “treat and repair”.

DOI： 10.34376/jsachd.c-2023-0004

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Language：Japanese   Publisher：(一社)日本成人先天性心疾患学会  

心室中隔欠損症(VSD)の多くは幼少期に診断され,成人期到達時には自然閉鎖,または手術されていることが多く,成人期で手術適応となることは稀である.成人期のVSDではシャント量の少ない小さなVSDか,すでに肺高血圧を伴い治療適応外と診断されたVSDが見られる.特に重症肺高血圧を伴うVSDではEisenmenger症候群と診断され,保存的な治療のみで経過観察されてきた症例が存在する.しかし近年肺高血圧治療薬の進歩により,重症肺高血圧を伴うVSDであっても,肺高血圧治療薬で治療した後に外科的治療を行う"treat and repair"が有用であると報告されている.今回,重症肺高血圧を伴うVSDに対して"treat and repair"で外科的治療に到達し得た2例を報告する.(著者抄録)

Language：English   Publisher：Journal of Cardiology Cases  

UNLABELLED: We present three cases of hypoplastic left heart syndrome (HLHS) complicated by congenital esophageal atresia and trachea-esophageal fistula (EA/TEF). The standard treatment for HLHS involves a staged surgical approach, eventually reaching Fontan completion. There is no report of patients with both HLHS and EA/TEF, and no established treatment strategy exists for such cases. Given the significant risk of simultaneously operating on HLHS and EA/TEF, we elected to pursue staged repair for each condition separately. Initially, soon after birth, we performed gastrostomy to secure the nutritional pathway for EA/TEF and stabilize breathing. Subsequently, we conducted bilateral pulmonary artery banding (bil-PAB) and ductal stenting for HLHS, as the Norwood operation carried an unacceptably high risk in these patients. Two of these patients were able to transition to home care, while the other patient died during hospitalization due to complications after EA repair. A combination of bil-PAB with ductal stenting for HLHS and staged repair for EA/TEF may provide effective management for patients with both conditions. LEARNING OBJECTIVE: Hypoplastic left heart syndrome (HLHS) and congenital esophageal atresia (EA) are both life-threatening conditions that require early intervention after birth. There are few reports of patients with both conditions, and no treatment strategy is established. Although the procedure carries a high risk, we successfully performed ductal stenting with bilateral pulmonary artery banding for HLHS, as well as staged repair procedures for EA. Our approach may be a viable strategy for these conditions.

DOI： 10.1016/j.jccase.2023.10.012

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

Language：English   Publisher：Journal of Cardiology Cases  

UNLABELLED: 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

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

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

OBJECTIVES: To determine guideline adherence pertaining to pulmonary valve replacement (PVR) referral after tetralogy of Fallot (TOF) repair. METHODS: Children and adults with cardiovascular magnetic resonance imaging scans and at least moderate pulmonary regurgitation were prospectively enrolled in the Comprehensive Outcomes Registry Late After TOF Repair (CORRELATE). Individuals with previous PVR were excluded. Patients were classified according to presence (+) versus absence (-) of PVR and presence (+) versus absence (-) of contemporaneous guideline satisfaction. A validated score (specific activity scale [SAS]) classified adult symptom status. RESULTS: In total, 498 participants (57% male, mean age 32 ± 14 years) were enrolled from 14 Canadian centers (2013-2020). Mean follow-up was 3.8 ± 1.8 years. Guideline criteria for PVR were satisfied for the majority (n = 422/498, 85%), although referral for PVR occurred only in a minority (n = 167/498, 34%). At PVR referral, most were asymptomatic (75% in SAS class 1). One participant (0.6%) received PVR without meeting criteria (PVR+/indication-). The remainder (n = 75/498, 15%) did not meet criteria for and did not receive PVR (PVR-/indication-). Abnormal cardiovascular imaging was the most commonly cited indication for PVR (n = 61/123, 50%). The SAS class and ratio of right to left end-diastolic volumes were independent predictors of PVR in a multivariable analysis (hazard ratio, 3.33; 95% confidence interval, 1.92-5.8, P < .0001; hazard ratio, 2.78; 95% confidence interval, 2.18-3.55, P < .0001). CONCLUSIONS: Although a majority of patients met guideline criteria for PVR, only a minority were referred for intervention. Abnormal cardiovascular imaging was the most common indication for referral. Further research will be necessary to establish the longer-term clinical impact of varying PVR referral strategies.

DOI： 10.1016/j.xjon.2023.11.013

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Publisher：Journal of Cardiology Cases  

The incidence of invasive group A Streptococcus (iGAS) infection has been increasing across all age groups, including pediatric patients, and is associated with high mortality rates. Although iGAS infection leads to streptococcal toxic shock syndrome and necrotizing soft tissue infections, iGAS-associated infective endocarditis (IE) is rare. Here, we report a case of iGAS-associated IE, streptococcal toxic shock syndrome, and pyomyositis that occurred after the Fontan procedure in a 7-year-old patient. Initial treatment included antibiotics and surgical intervention for pyomyositis. Despite her overall condition's improvement, persistent fever led to the discovery of IE. Furthermore, this patient developed cardiac rupture due to the progression of IE but was successfully rescued. No neurological complications occurred, and the patient was discharged without recurrence of infection. To our knowledge, this is the first case report of successful life-saving treatment for cardiac rupture due to IE caused by iGAS in a pediatric Fontan patient. This case suggests that iGAS infections in patients with complex congenital heart disease warrant a crucial search for complications of iGAS-associated IE. Learning objective: The incidence of invasive group A Streptococcus (iGAS) infection is increasing globally. While infective endocarditis (IE) caused by iGAS is rare, the risk may be elevated among patients with complex congenital heart disease. This underscores the importance of searching for iGAS-associated IE and the need for treatment with consideration for exacerbation.

DOI： 10.1016/j.jccase.2024.09.009

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Language：English   Publishing type：Research paper (scientific journal)   Publisher：Clinical and Translational Immunology  

OBJECTIVES: The objectives of this study were to investigate the pathophysiology of Kawasaki disease (KD) from immunological and oxidative stress perspectives, and to identify real-time biomarkers linked to innate immunity and oxidative stress in KD. METHODS: We prospectively enrolled 85 patients with KD and 135 patients with diverse conditions including immune, infectious and non-infectious diseases for this investigation. Flow cytometry was used to analyse the surface expression of CD14, CD38 and CD62L on monocytes, along with a quantitative assessment of CD14 down-modulation. Additionally, oxidative stress levels were evaluated using derivatives of reactive oxygen metabolites (d-ROMs) and antioxidant capacity measured by a free radical elective evaluator system. RESULTS: During the acute phase of KD, we observed a prominent CD14 down-modulation on monocytes, reflecting the indirect detection of circulating innate immune molecular patterns. Moreover, patients with KD showed a significantly higher CD14 down-modulation compared with infectious and non-infectious disease controls. Notably, the surface expression of CD14 on monocytes was restored concurrently with responses to intravenous immunoglobulin and infliximab treatment in KD. Furthermore, d-ROM levels in patients with KD were significantly elevated compared with patients with infectious and non-infectious diseases. Following intravenous immunoglobulin treatment, oxidative stress levels decreased in patients with KD. CONCLUSION: Monitoring CD14 down-modulation on monocytes in real-time is a valuable strategy for assessing treatment response, distinguishing KD relapse from concomitant infections and selecting second-line therapy after IVIG treatment in KD patients. The interplay between inflammation and oxidative stress likely plays a crucial role in the development of KD.

DOI： 10.1002/cti2.1482

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Language：English   Publishing type：Research paper (scientific journal)   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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Language：English   Publishing type：Research paper (scientific journal)   Publisher：Early Human Development  

INTRODUCTION: To establish actionable neonatal screening during the first month of life, we investigated critical diseases in seemingly healthy newborns discharged from birth hospitals. METHODS: This retrospective study enrolled previously healthy full-term infants who visited our hospital, a tertiary hospital in Japan, from home between 5 and 28 days after birth from 2009 to 2018. Infants with known perinatal or congenital diseases, positive newborn screening results, or accidental injuries were excluded. Data were collected from electronic medical records, including principal diagnosis, clinical details, and prognosis at 18 months of age. RESULTS: Ninety-seven (58 %) of 168 eligible neonates were admitted to the hospital, and 71 (42 %) were not. The median admission rate in patients with disease onset at ≤14 days after birth (80 %) was significantly higher than that in patients with disease onset at ≥15 days (42 %). Among 45 patients who received intensive medical care, 5 died and 10 developed neurodevelopmental sequelae. Four of 5 patients died by 100 days. Among 25 diseases treated in intensive care unit, 17 (68 %) diseases had a prevalence of <1 per 2000 live births. The commonly used diagnostic methods were imaging (n = 58, 35 %) and physical examination (n = 34, 20 %). CONCLUSION: Critical diseases due to rare and heterogeneous causes in ostensibly healthy newborns occurred predominantly in the first two weeks of life. Optimal newborn screening and health check-up protocols may benefit from the wide spectrum of life-threatening diseases occurring in home after birth.

DOI： 10.1016/j.earlhumdev.2023.105869

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Language：English   Publisher：(一社)インターナショナルハートジャーナル刊行会  

包括的心臓MRIを施行された心血管疾患を有さない健常児23名(男児15名、女児8名、年齢0.1～15.3歳)を対象に、心室容積、局所血流量、T1緩和時間の正常値について検討した。被験者の平均身長は116±34cm、体重は23.3kg(範囲5.7～73.9kg)、体表面積は0.89±0.42m2であった。心臓MRIは1.5T装置を用いて、シネ、二次元位相差コントラスト法、非造影でのnative T1マッピング法を施行した。シネMRIは左室二腔、三腔、四腔、短軸像を撮影し、T1マッピングはTrue PISPシーケンスにて撮影した。左室および右室の拡張末期容積、収縮末期容積、心筋重量はいずれも体表面積と強く相関した。また上行および下行大動脈、肺動静脈、上下大静脈等の局所血流量も体表面積と相関した。心室容積と局所血流量に関して体表面積を変数とする正常回帰式を取得した。線形回帰分析で左室心筋T1値は年齢に応じて低下し、肝臓のT1値は年齢の二次関数として表された。級内相関係数は心筋T1値(0.868)、肝臓T1値(0.895)、右室心筋重量(0.744)以外はいずれも0.94以上であった。

Language：English   Publishing type：Research paper (scientific journal)   Publisher：Journal of the American College of Cardiology  

BACKGROUND: Comprehensive assessment of tetralogy of Fallot (TOF) outcomes extends beyond morbidity and mortality to incorporate patient-reported outcomes (PROs), including quality of life (QOL) and health status (HS). OBJECTIVES: This study explored PROs in adolescents and adults with TOF and delineated variables associated with PROs. METHODS: This was a cross-sectional observational study within a larger prospective registry of adolescents and adults with repaired TOF and moderate or greater pulmonary regurgitation from North America, Europe, and Asia. Participants completed PROs, including a QOL linear analogue scale (QOL-LAS) and an HS visual analogue scale (HS-VAS). Scores were classified according to age cohorts: <18, 18 to 25, 26 to 40, and >40 years. RESULTS: The study included 607 patients (46.3% female; median age 28.5 years). Median QOL-LAS scores (0-100) were similar across age cohorts (85, 80, 80, 80; P = 0.056). Median HS-VAS scores (0-100) were lowest for the oldest cohort (77) compared with the 3 younger cohorts (85, 80, 80) (P = 0.004). With advancing age, there were increased reports of poor mobility (P < 0.001) and pain or discomfort (P = 0.004); problems in these dimensions were reported by 19.1% and 37.2% of patients aged >40 years, respectively. Of factors associated with superior PROs on multivariable regression modeling (ie, being White, being nonsyndromic, having employment, and having better left ventricular function; P < 0.05), asymptomatic status (functional class I) was the variable associated with the greatest number of QOL and HS measures (P < 0.001). CONCLUSIONS: Strategies to improve TOF outcomes should consider PROs alongside conventional clinical variables. Factors associated with poorer PROs represent opportunities to intervene to improve the lives of patients with TOF.

DOI： 10.1016/j.jacc.2023.03.385

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Language：English   Publishing type：Research paper (scientific journal)   Publisher：European Heart Journal  

DOI： 10.1093/eurheartj/ehad112

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

DOI： 10.1111/ped.15503

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Language：English   Publishing type：Research paper (scientific journal)   Publisher：Journal of Cardiovascular Development and Disease  

Pulmonary regurgitation (PR) is a risk factor for sudden cardiac death in adult patients with repaired tetralogy of Fallot (TOF). However, transthoracic echocardiography (TTE) cannot fully visualize the pulmonary valve (PV) and PR. We investigated whether intracardiac echocardiography (ICE) could visualize the PV and PR better than TTE. Thirty adult patients with TOF (mean age 33 ± 15 years) scheduled for cardiac catheterization underwent ICE. The visualization of PV and the severity of PR were classified into three grades. ICE depicted the PV better than TTE (ICE vs. TTE: not visualized, partially visualized, and fully visualized: n = 1 [3%], n = 13 [43%], and n = 16 [53%] vs. n = 14 [47%], n = 13 [43%], and n = 3 [10%], p < 0.001). Especially in patients after pulmonary valve replacement (PVR), the PV was more fully visualized by ICE. The assessment of PR by TTE underestimated the severity of PR in comparison to cardiac magnetic resonance imaging (MRI) (severe PR: 8 [28%] vs. 22 [76%], p = 0.004), while there was no discrepancy between the results of ICE and MRI (21 [72%] vs. 22 [76%], p = 1.000). In comparison to TTE, ICE can safely provide better visualization of the PV and PR in adults with TOF, especially in patients who have undergone PVR.

DOI： 10.3390/jcdd10010024

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

DOI： 10.1111/ped.15500

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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   Publishing type：Research paper (scientific journal)   Publisher：International Heart Journal  

Age-related mean and reference ranges for ventricular volumes and mass, regional blood flow measurements, and T1 values using cardiovascular magnetic resonance (CMR) imaging are yet to be established for the pediatric population. Especially in infants and toddlers, no consistent flow volume sets or T1 values have been reported. The purpose of this study was to determine the relevant normal values.Twenty-three children (aged 0.1-15.3 years) without cardiovascular diseases were included. Comprehensive CMR imaging including cine, 2-dimensional phase-contrast, and native T1 mapping, were performed. Ventricular volumes and masses, 11 sets of regional blood flow volumes, and myocardial and liver T1 values were measured. All intraclass correlation coefficient values were > 0.94, except for the right ventricular mass (0.744), myocardial (0.868) and liver T1 values (0.895), reflecting good to excellent agreement between rates.Regression analysis showed an exponential relationship between body surface area (BSA) and ventricular volumes, mass, and regional blood flow volumes (normal value = a*BSAb). Left ventricular myocardial T1 values were regressed on linear regression with age (normal value = -7.39*age + 1091), and hepatic T1 values were regressed on a quadratic function of age (normal value = 0.923*age2 -18.012*age + 613).Comparison of the 2 different methods for the same physical quantities by Bland-Altman plot showed no difference except that the right ventricular stroke volume was 1.5 mL larger than the main pulmonary trunk flow volume.This study provides the normal values for comprehensive CMR imaging in Japanese children.

DOI： 10.1536/ihj.23-022

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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   Publishing type：Research paper (scientific journal)   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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Language：English   Publishing type：Research paper (scientific journal)   Publisher：International Journal of Cardiovascular Imaging  

To investigate the usefulness of high-resolution systolic T1 mapping using compressed sensing for right ventricular (RV) evaluation. Phantoms and normal volunteers were scanned at 3 T by using a high-resolution (HR) modified look-locker inversion recovery (MOLLI) pulse sequence and a conventional MOLLI pulse sequence. The T1 values of the left ventricular (LV) and RV myocardium and blood pool were measured for each sequence. T1 values of HR-MOLLI and MOLLI sequences were compared in the LV myocardium, blood pool, and RV myocardium. The T1 values of HR-MOLLI and MOLLI showed good agreement in both phantoms and the LV myocardium and blood pool of volunteers. However, there was a significant difference between HR-MOLLI and MOLLI in the RV myocardium (1258 ± 52 ms vs. 1327 ± 73 ms; P = 0.0005). No significant difference was observed between the T1 value of RV and that of LV (1217 ± 32 ms) in HR-MOLLI, whereas the T1 value of RV was significantly higher than that of LV in MOLLI (P < 0.0001). The interclass correlation coefficients of intraobserver variabilities from HR-MOLLI and MOLLI were 0.919 and 0.804, respectively, and the interobserver variabilities from HR-MOLLI and MOLLI were 0.838 and 0.848, respectively. Assessment of RV myocardium by using HR systolic T1 mapping was superior to the conventional MOLLI sequence in terms of accuracy and reproducibility.

DOI： 10.1007/s10554-022-02622-y

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Language：English   Publishing type：Research paper (scientific journal)   Publisher：International Heart Journal Association  

<p>Sleep-disordered breathing is one of the complications commonly seen in patients with adult congenital heart disease (ACHD) due to multiple causes including complex underlying cardiac defects, cardiomegaly, previous thoracotomies, obesity, scoliosis, and paralysis of the diaphragm. It is often hard to determine its main cause and predict the efficacy of each treatment in its management. We herein report a 30-year-old woman after biventricular repair of pulmonary atresia with intact ventricular septum diagnosed as sleep-related hypoventilation disorder. Simultaneous treatment targeting obesity, paralysis of the diaphragm, and cardiomegaly followed by respiratory muscle reinforcement through non-invasive ventilation resolved her sleep-related hypoventilation disorder. Such management for each factor responsible for the hypoventilation is expected to provide synergetic therapeutic efficacy and increase daily activity in a patient with ACHD.</p>

DOI： 10.1536/ihj.22-073

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Language：English   Publisher：(一社)インターナショナルハートジャーナル刊行会  

症例は30歳女性。心室中隔欠損を伴わない肺動脈閉鎖症の既往があり、生後2ヵ月でBlalock-Taussigシャント手術を受けた。1歳時にパッチ拡大術による二心室修復術が施行され、4歳時には左肺動脈形成術が施行された。術後に横隔膜麻痺が出現した。労作時呼吸困難と日中の過度の眠気を自覚し、近医を受診したところ、日本語版Epworth Sleepiness Scale(JESS)で20/24点の高値のため、紹介となった。受診時血圧は103/74mmHg、心拍数は64bpm、BMIは35.1kg/m2であった。拡張期雑音を胸骨右縁に聴取した。胸部X線検査で心拡大、横隔膜麻痺、脊椎側彎症を認めた。右室拡張末期容量係数(RVEDVi)は235.6mL/m2、左室駆出率は29%であった。重度の肺動脈弁逆流症を認めた。終夜睡眠ポリグラフ検査で睡眠関連低換気障害と診断された。非侵襲的陽圧換気を開始した。肺動脈弁逆流症に対して肺動脈弁置換術、三尖弁輪形成術、横隔膜縫縮術を施行した。肥満に対して食事制限と運動療法を開始した。6ヵ月後にRVEDViは131.2mL/m2、左室駆出率は63%に改善した。労作時呼吸困難は消失し、日中の眠気は改善した。

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

<p><b>Background</b>: The purpose of this study was to look at the current state of the adult patients with tetralogy of Fallot, the most common cyanotic congenital heart disease, to encourage social independence and healthy lifestyles.</p><p><b>Methods</b>: On 186 patients, a questionnaire survey (understanding and anxiety about their heart diseases, treatments, social independence, and lifestyles) was administered. These data were also compared between patients with and without physical disability certification (a certified group and a noncertified group). Clinical data were extracted from the medical records.</p><p><b>Results</b>: After excluding the cases without meeting the inclusion criteria, 112 patients (41 males, mean age 28 years) were studied. Eighty-three percent of 93 patients after excluding 19 students, were employed (66％ full-time employee), half of them lived with their parents, and 71％ were concerned about their heart diseases. In terms of lifestyle, 28％ were dissatisfied with the quality of their sleep. The noncertified group (n=59) was assigned more professional tasks, whereas the certified group (n=53) was assigned more office duties. The certified group had more regular outpatient clinic visits and dental consultations, but also had a greater experience to drink alcohol and take a sleeping pill.</p><p><b>Conclusion</b>: The study patients had a relatively good job rate and a high level of social independence, despite having a variety of anxiety disorders. It was suggested that some supports for anxiety and sleep disorder issues be implemented especially in the certified group.</p>

DOI： 10.9794/jspccs.38.128

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

DOI： 10.9794/jspccs.38.103

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

<p><b>Background</b>: The number of adult patients with congenital heart disease (CHD) who get married, become pregnant, and give birth is increasing with the improvement in their prognosis. We examined the hope and cognition of the marriages, pregnancies, and childbirth of women with CHD.</p><p><b>Methods</b>: A questionnaire-based survey was conducted on 307 adult women with CHD (aged 20–49 years) regarding their heart diseases, marriages, pregnancies, and childbirth. We divided the subjects into two groups according to whether they had or had not acquired the first grade of the physical disability certificate.</p><p><b>Results</b>: Responses were obtained from 89 participants (valid response rate: 29.0％), including 53 acquirers and 36 nonacquirers. The number of married people was significantly more frequent in the acquirer than in the nonacquirer group. In both groups, 80％ were aware of the “Burden on the heart due to pregnancy” and “Necessity of prepregnancy examinations.” The acquirers were highly aware of “Influences of heart disease medications on the fetus” and “Inheritance of congenital heart disease by the fetus.”</p><p><b>Conclusions</b>: Both groups reported high aspirations for marriage, pregnancy, and childbirth. They need to be supported and educated continuously for a safe pregnancy and childbirth by a multidisciplinary team.</p>

DOI： 10.9794/jspccs.38.105

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Language：English   Publisher：Pediatric Pulmonology  

Pulmonary veno-occlusive disease (PVOD) and idiopathic/heritable pulmonary arterial hypertension (I/HPAH) cause progressive PH on the distinct genetic impact. A 29-month-old boy presented with a loss of consciousness. He had severe PH refractory to pulmonary vasodilators. Hypoxemia and ground-glass opacity on the chest computed tomography were present, and significant pulmonary edema developed after the introduction of continuous intravenous prostaglandin I2 . Based on the clinical diagnosis of PVOD, he underwent a single living-donor lobar lung transplantation with the right lower lobe of his mother. The pathological findings of his explanted lung showed intimal thickening and luminal narrowing of the pulmonary vein. A genetic test revealed a novel heterozygous splice acceptor variant (c.77-2A>C) in BMPR2, which is typically associated with I/HPAH. This is the first pediatric case of PVOD with BMPR2 variant, supporting the concept that I/HPAH and PVOD are part of a spectrum of pulmonary vascular disease.

DOI： 10.1002/ppul.25877

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

背景・目的:近年,先天性心疾患(CHD)患者の多くが成人を迎えることが可能となり,増加する成人CHD(ACHD)女性の結婚,妊娠・出産に関する認識を検討した.方法:ACHD女性307名(20～49歳)へ心疾患と結婚・妊娠・出産に関する質問紙調査を行い,対象者を身体障害者手帳1級取得者と非取得者に分けて比較した.結果:回答した取得者53名,非取得者36名の89名(有効回答率29.0%)を対象とした.既婚者は取得者に多く,結婚の希望は非取得者に多い傾向があり,両群共に80%が「妊娠・出産による心臓の負担」「妊娠前検査の必要性」を認識していた.取得者は,「治療薬の胎児への影響」「心疾患の胎児への遺伝」の認識が高かった.結論:ACHD女性は,妊娠・出産による心臓への負担を認識していても,その願望は高く,看護職者や医師が連携して心疾患重症度に応じて妊娠可能性やリスクについて早期からの啓発や継続支援が必要である.(著者抄録)

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

背景:チアノーゼ性成人先天性心疾患の中で頻度の高いファロー四徴症において社会的自立や生活習慣を調査し保健指導の基盤を得ることを目的とした.方法:186名に質問紙調査(病気や治療に関する理解や不安,社会的自立,生活習慣等)を行い,身体障害者手帳認定の有無で比較した.臨床情報は診療録より抽出した.結果:有効回答者112名(男41名,平均28歳)の半数が親と同居し,学生を除いた93名の83%が就労していた.71%が病気に対する不安をもち,28%は睡眠満足度が低かった.職種では非認定群は専門職が多いが,認定群では事務職が多かった.認定群の医療へのアクセスは高かったが,就労内容や周囲の理解などに不安を持っており習慣飲酒,睡眠導入剤の使用も多い傾向がみられた.結論:本対象者は比較的高い就労率で社会的自立度も高かったが,様々な不安を抱えており特に心疾患重症度の高い認定群は不安や睡眠障害に対する支援の必要性が示唆された.(著者抄録)

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

The number of women with congenital heart disease (CHD) reaching reproductive age has been increasing. Many women with CHDs are desirous of pregnancy, but they face issues regarding preconception, antepartum, and postpartum management. On the other hand, the fetal diagnosis of CHD has improved with advances in the technique and equipment for fetal echocardiography. Recently, experiences with fetal intervention have been reported in patients with severe CHD, such as critical aortic stenosis. Nevertheless, some types of CHD are challenge to diagnose prenatally, resulting in adverse outcomes. Medical care is part of the transitional care for women and fetuses with CHD during the perinatal period. Pre-conceptional and prenatal counseling play an important role in transitional care. Sex and reproductive education need to be performed as early as possible. We herein review the current status, important issues to be resolved, and the future of maternal and fetal CHD to relevant caregivers.

DOI： 10.1111/ped.15098

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

With advances in medical care, the majority of infants and children with chronic diseases are now able to reach adulthood. However, many of them still need special health care because of their original diseases, sequelae, and complications. The transition from the child health care system to the adult health care system is a crucial step for these patients. The goal of transitional care is to maximize the lifelong function and potential of these patients by uninterruptedly providing appropriate health-care services. To achieve this goal, we should (i) coordinate the transfer to adequate medical institutions and departments for adults, (ii) educate patients to improve self-management, and (iii) support the transition to social and welfare services for adults. Transitional care in pediatric cardiology has been a step ahead of such care in other diseases because of the relatively high incidence and the long history of adult congenital heart disease. Education of the patients to establish autonomy reduces dropping out and unexpected hospitalizations and it is the most important part of transitional care. To achieve this goal, we should provide explanations to pediatric patients according to their age and level of understanding from their first visit, rather than waiting until they reach a certain age. Tools for education and readiness checks are also being developed. To achieve a situation in which pediatric patients with chronic disease can take care of their own health and fully utilize their abilities at the growing step, transitional care plays a crucial role not only in pediatric cardiology but also in other subspecialties.

DOI： 10.1111/ped.15096

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Language：Japanese   Book type：Scholarly book

Event date： 2013.5

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Venue：Kuching   Country：Malaysia  

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Venue：Taipei   Country：Taiwan, Province of China  

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Venue：Chiba   Country：Japan  

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Venue：Taipei   Country：Taiwan, Province of China  

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

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

Language：Japanese   Publisher：(株)ライフメディコム  

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

Language：Japanese   Publisher：(株)日本臨床社  

Language：Japanese   Publisher：(株)へるす出版  

Language：Japanese   Publisher：大道学館出版部  

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

<Key Poins>(1)チアノーゼ残存先天性心疾患の多血症では,まずはバランスのとれた代償性多血症か,過粘稠度症候群を伴う非代償性多血症かを見極める。(2)相対的鉄欠乏があれば補充し,漫然と行われている瀉血は中止を検討する。ただ,中止後の出血性合併症には注意が必要である。(3)多血症だけを理由に抗血小板薬や抗凝固薬が投与されている場合,とくに喀血などの出血性合併症がみられている場合は,中止を検討する。(4)全身の臓器障害に注意し,検尿(蛋白尿),腹部超音波(胆石)を定期的に行う。脳膿瘍,感染性心内膜炎,褐色細胞腫といった,比較的まれだが重要な合併症の可能性も常に念頭において診療する。(著者抄録)

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

Language：Japanese   Publisher：日本産婦人科・新生児血液学会  

診断や外科治療などの進歩により、先天性心疾患を持つ小児の大多数が成人期に到達する時代となった。成人した患者の既婚率は女性に限ると一般と同等であるとの報告もあり、若年女性に対する教育と指導はきわめて重要である。欧米に遅れてスタートした我が国の移行期医療であるが、徐々に取り組みがなされ、患者教育のツールなども整いつつある。思春期以降の女性に対する抗凝固療法には特有の注意を要し、特にビタミンK拮抗薬と抗血小板薬の併用は、過多月経などの出血性合併症をしばしば経験するため注意が必要である。妊娠中の至適抗凝固療法については未だ不明点も多く、今後の課題である。(著者抄録)

Language：Japanese   Publisher：(有)科学評論社  

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

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

Type：Competition, symposium, etc. 

Type：Competition, symposium, etc. 

Type：Competition, symposium, etc. 

Number of participants：400

Type：Competition, symposium, etc. 

Type：Competition, symposium, etc.