Left ventricular non-compaction (LVNC) is a rare cardiomyopathy characterized by prominent trabeculations and deep recesses. Neonatal cases, particularly with severe metabolic disturbances, are uncommon. We report a 2-day-old neonate with LVNC and dilated cardiomyopathy, presenting recurrent heart failure associated with hyperkalemia, metabolic acidosis, hyperlactatemia, and hypoglycemia. Management included mechanical ventilation, metabolic correction, and heart failure therapy, leading to improved cardiac function. Genetic analysis revealed a de novo heterozygous pathogenic ACTN2 deletion spanning exons 2–6. This case broadens the phenotypic spectrum of ACTN2-related LVNC and suggests a potential link between metabolic disturbances and cardiac deterioration.
{"title":"De Novo ACTN2 Variant in a Chinese Neonate With Left Ventricular Non-Compaction and Metabolic Disturbances: A Rare Case Report","authors":"Jinqiu Huang, Ziyue Zhang, Juxian Yang","doi":"10.1111/anec.70117","DOIUrl":"10.1111/anec.70117","url":null,"abstract":"<p>Left ventricular non-compaction (LVNC) is a rare cardiomyopathy characterized by prominent trabeculations and deep recesses. Neonatal cases, particularly with severe metabolic disturbances, are uncommon. We report a 2-day-old neonate with LVNC and dilated cardiomyopathy, presenting recurrent heart failure associated with hyperkalemia, metabolic acidosis, hyperlactatemia, and hypoglycemia. Management included mechanical ventilation, metabolic correction, and heart failure therapy, leading to improved cardiac function. Genetic analysis revealed a de novo heterozygous pathogenic <i>ACTN2</i> deletion spanning exons 2–6. This case broadens the phenotypic spectrum of <i>ACTN2</i>-related LVNC and suggests a potential link between metabolic disturbances and cardiac deterioration.</p>","PeriodicalId":8074,"journal":{"name":"Annals of Noninvasive Electrocardiology","volume":"31 1","pages":""},"PeriodicalIF":1.1,"publicationDate":"2026-01-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12818458/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146008540","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
His bundle typically passes through the central fibrous body of the atrioventricular node and then enters the membranous portion of the interventricular septum, where it branches into the left and right bundle branches. The feasibility and safety of left bundle branch area pacing (LBBAP) in patients with perimembranous ventricular septal defect (pmVSD) have not been reported.
{"title":"Is Left Bundle Branch Pacing Feasible in Patients With Ventricular Septal Defect?","authors":"Binbin Luo, Longfu Jiang, Lu Zhang, Jiabo Shen","doi":"10.1111/anec.70150","DOIUrl":"10.1111/anec.70150","url":null,"abstract":"<p>His bundle typically passes through the central fibrous body of the atrioventricular node and then enters the membranous portion of the interventricular septum, where it branches into the left and right bundle branches. The feasibility and safety of left bundle branch area pacing (LBBAP) in patients with perimembranous ventricular septal defect (pmVSD) have not been reported.</p>","PeriodicalId":8074,"journal":{"name":"Annals of Noninvasive Electrocardiology","volume":"31 1","pages":""},"PeriodicalIF":1.1,"publicationDate":"2026-01-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12820410/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146008563","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}