記述言語：日本語   出版者・発行元：Journal of the American Heart Association  

BACKGROUND: Left ventricular noncompaction (LVNC) is a hereditary type of cardiomyopathy characterized by prominent tra-beculations. Detailed characteristics of biventricular noncompaction (BiVNC) remain unknown. This study aimed to elucidate the clinical characteristics and genetic landscape of BiVNC.METHODS AND RESULTS: We recruited children with left ventricular noncompaction from Japanese multi-institutional centers from 2013 to 2021. Left ventricular noncompaction was classified as BiVNC, congenital heart disease, arrhythmia, dilated car-diomyopathy, or normal function. In these patients, cardiomyopathy-associated genes were screened. A total of 234 patients (127 male; mean age, 4 months [range, 0–6.6 years]) were enrolled in this study, of whom 25 had BiVNC; 55, normal function; 84, dilated cardiomyopathy; 38, congenital heart disease; and 32, arrhythmia. BiVNC was diagnosed during the perinatal period in 10 patients, in whom the prevalence was higher than that in other patients. A total of 14 patients in the group with BiVNC had congenital heart disease, but not necessarily right heart lesions. Left ventricular dyskinesis was frequently observed in the lateral wall (24%) and apex (28%). Eleven pathogenic variants were found in 11 patients with BiVNC (44.0%). The group with BiVNC had a higher ratio of mitochondrial and developmental gene variants than the other groups. Among all groups, the group with BiVNC had the worst survival rate (P=0.0009). CONCLUSIONS: Pediatric patients with BiVNC had a high rate of ventricular dyskinesis and poor outcome. A comprehensive and careful screening for disease-causing genes and phenotype may help identify specific patients with left ventricular noncom-paction and mortality-related cardiac phenotypes.

DOI： 10.1161/JAHA.124.035614

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PubMed

記述言語：日本語   出版者・発行元：Pediatric Infectious Disease Journal  

Background: Surgical site infection (SSI) is a major complication of pediatric cardiac surgery. If the risk of developing SSI can be predicted based on the patient's preoperative background, appropriate preoperative management to prevent the development of SSI can be achieved. Methods: We retrospectively studied cases for patients younger than 7 years of age among surgeries performed through a median sternotomy at Kagoshima University Hospital from April 2011 to March 2021. SSI was diagnosed according to the Centers for Disease Control and Prevention guidelines and classified into 3 types: superficial incisional SSI (SiSSI), deep incisional SSI (DiSSI) and mediastinitis. Results: Of the 765 consecutive pediatric cardiac surgeries, 597 were included in the analysis based on the exclusion criteria. Of these, 17 (2.8%) developed SSI (3 SiSSI cases, 2 DiSSI cases and 12 mediastinitis cases), with Staphylococcus aureus as the major pathogen. Univariate analysis revealed that low preoperative serum protein (P = 0.049) and low serum albumin levels (P = 0.023) were risk factors for the development of SSI. No findings suggested impaired hepatic synthesis, inflammatory disease or protein loss from the kidney or intestinal tract. We concluded that malnutrition caused low serum protein and albumin levels. Conclusions: Low preoperative serum protein and albumin levels are risk factors for SSI development of SSI. Nutritional status should be regularly assessed in children scheduled for cardiac surgery, and interventions, such as nutritional guidance, should be considered if malnutrition is suspected.

DOI： 10.1097/INF.0000000000004039

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PubMed

記述言語：日本語   出版者・発行元：Pediatric Nephrology  

Background: We validated the prevalence of albuminuria and its association with kidney function and hemodynamics in pre-school children who underwent surgery for congenital heart disease (CHD). Methods: From 403 patients who had undergone surgery for CHD at least 6 months before pre-school and were admitted to our hospital between 2011 and 2015, 75 who underwent blood and urine tests and cardiac catheterization were included in this study. The urinary albumin-to-creatinine ratio (ACR) was quantified, and the relationship of ACR with physical and laboratory findings and hemodynamics assessed using cardiac catheterization was analyzed. Results: The study cohort was divided into three groups: Fontan group (n = 25), tetralogy of Fallot (TOF) group (n = 18), and control group (other biventricular CHDs; n = 32). The median age of patients was 5.9 years. ACR was higher in the Fontan group than in the TOF and control groups (median: 15.0 vs. 5.0 and 0.0 mg/g, p < 0.001). Moreover, albuminuria (ACR > 30 mg/g) was observed in 20.0% of Fontan patients, while ACR was associated with potential complicating factors of Fontan circulation: high central venous pressure, high mean pulmonary artery pressure, and worse than moderate atrioventricular regurgitation. ACR showed a moderate correlation with the cystatin C-based estimated glomerular filtration rate (r = − 0.725, p < 0.001). Conclusions: Measurement of albuminuria in Fontan patients before they join elementary school is useful because it reflects kidney function and hemodynamic factors that can worsen their condition. Identification and management of patients with albuminuria may facilitate early therapeutic intervention for worsening Fontan factors, eventually delaying the deterioration of kidney function. Graphical abstract: [Figure not available: see fulltext.].

DOI： 10.1007/s00467-023-06130-6

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PubMed

記述言語：英語   出版者・発行元：(一社)日本心臓病学会  

症例は2歳男児で、在胎39週で経腟分娩にて出生し、出生時体重は2890gであった。Apgarスコアは1分後8点、5分後9点であった。血管炎、結合組織異常症、遺伝性疾患の家族歴はなかった。三尖弁閉鎖症と診断され、BTシャント術を受けた。1歳3ヵ月時に両方向性グレン手術を施行した。術前の心臓カテーテル検査で平均肺動脈圧は10mmHg、肺動脈楔入圧は7mmHg、肺血管抵抗は0.61 Wood unit/m2であった。2歳9ヵ月時に心外導管を用いた両大静脈肺動脈吻合手術(TCPC)を施行した。術後に血圧は80～100mmHgで安定していたが、中心静脈圧は15mmHgの高値を呈した。一酸化窒素吸入療法を施行したが、中心静脈圧は21mmHgに上昇した。静脈血栓を疑い、ヘパリン静注を開始した。術後7日のCTで導管狭窄および導管内に血栓形成を認め、血栓溶解療法を施行した。術後9日に導管内血栓は消失し、中心静脈圧は10mmHg未満に低下した。術後36日のCTで左内胸動脈に径6.8×8.1mmの仮性動脈瘤を認めた。内胸動脈瘤に対してコイル塞栓術を施行した。術後経過は良好で、40日後に退院となった。

記述言語：日本語   出版者・発行元：Pediatric Cardiology  

Although the number of pediatric patients with long-term survival following cardiac surgery is increasing, concerns regarding chronic kidney disease (CKD) after surgery are growing. We examined the frequency of and risk factors for pediatric CKD development in patients with congenital heart disease (CHD) at least 2 years after cardiac surgery. This was a cross-sectional study of 147 patients who underwent open-heart surgery for CHD at Kagoshima University Hospital from April 2010 to March 2017. Data on demographics, acute kidney injury after cardiac surgery, cyanotic heart disease, Fontan circulation, medications in the perioperative period, and Risk Adjustment for Congenital Heart Surgery-1 (RACHS-1) category were recorded. CKD was defined using the current classification system described in the National Kidney Foundation’s Kidney Disease Outcomes Quality Initiative and assessed during early childhood within 2–3 years of cardiac surgery. Statistical analyses were performed using SPSS Statistics for Windows version 25.0. We consecutively enrolled 147 patients, of whom 22 (15.0%) had CKD, all with stage-2 severity. Among patients with CKD, a higher proportion underwent Fontan surgery (P < 0.001), a higher proportion had cyanotic heart disease (P = 0.009), and the RACHS-1 category was high (P = 0.003). Patients with CKD appeared more frequently than patients without CKD in RACHS-1 categories 3, 5, and 6. It is essential to evaluate renal function longitudinally and monitor for CKD, given that patients who underwent Fontan surgery or complicated surgery in infancy have a high rate of developing postoperative CKD in early childhood.

DOI： 10.1007/s00246-022-02817-z

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PubMed

記述言語：日本語   出版者・発行元：Journal of Cardiology Cases  

We herein describe the first pediatric case of an internal mammary artery (IMA) aneurysm caused by a median sternotomy. He was a 2-year-old with tricuspid atresia who underwent an extracardiac conduit Fontan procedure. On the 36th postoperative day, an asymptomatic left IMA aneurysm was detected via contrast computed tomography, which was successfully treated with coil embolization. The patient had no underlying disease such as vasculitis, connective tissue disease, or other hereditary diseases, and there were no episodes of infection or hypertension before or after the onset of the IMA aneurysm. Because the left IMA ran medially to the periphery and was in a vulnerable position during median sternotomy, we considered the IMA aneurysm was caused by the median sternotomy. We pediatric cardiologists should be aware that IMA aneurysms can occur in pediatric cardiac surgery, and we should be proactive in performing postoperative imaging studies in cases where the preoperative internal thoracic artery runs medially toward the periphery. Learning objective: Internal mammary artery (IMA) aneurysm is a rare vascular disease, especially in children. One-third of adult IMA aneurysms have been reported to be caused by sternotomy, but not in children. We report the first pediatric case of an IMA aneurysm caused by sternotomy. We should recognize that there is a potential risk of IMA aneurysms in pediatric cardiac surgery as well.

DOI： 10.1016/j.jccase.2022.05.016

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PubMed