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This meta-analysis aimed to assess surgical outcomes and long-term survival in this population. A systematic review was conducted using PubMed, Embase, Scopus, and Cochrane databases to identify studies evaluating septal myectomy in pediatric patients with obstructive HCM. Eight clinical trials comprising a total of 490 pediatric patients met the inclusion criteria. Among children undergoing septal myectomy for HOCM, the pooled early and late mortality rates were 3.3% and 8.5%, respectively. Concomitant mitral valve repair was performed in 7.3% of patients. Rates of complete atrioventricular block and permanent pacemaker implantation were 9.9% and 5.1%, respectively. The mean durations of intensive care and hospital stay were 34.6\u00a0h and 12.9 days. Postoperative wound infection and reoperation occurred in 3.4% and 6.4% of cases. Echocardiographic assessments demonstrated a marked reduction in LVOT gradient (from 86.9 to 16.2 mmHg) with preserved left ventricular ejection fraction. Mitral regurgitation decreased from 49.8 to 6.4%, and systolic anterior motion from 84.9 to 19.9%. Septal myectomy in pediatric patients with HOCM is a safe and effective procedure, associated with favorable survival and substantial symptomatic and hemodynamic improvement. However, results must be interpreted cautiously due to moderate-quality retrospective data.<\/jats:p>","DOI":"10.1007\/s00246-025-04088-w","type":"journal-article","created":{"date-parts":[[2025,11,17]],"date-time":"2025-11-17T12:57:02Z","timestamp":1763384222000},"page":"2820-2827","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Septal Myectomy in Pediatric Obstructive Hypertrophic Cardiomyopathy: A Systematic Review and Meta-analysis"],"prefix":"10.1007","volume":"47","author":[{"given":"Anelise Poluboiarinov","family":"Cappellaro","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Luiz Felipe Costa","family":"de Almeida","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ronaldo Altenburg Odebrecht Curi","family":"Gismondi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rafael","family":"Ayala","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Myat Soe","family":"Thet","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Joseph A.","family":"Dearani","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2025,11,17]]},"reference":[{"issue":"37","key":"4088_CR1","doi-asserted-by":"publisher","first-page":"3503","DOI":"10.1093\/eurheartj\/ehad194","volume":"44","author":"E Arbelo","year":"2023","unstructured":"Arbelo E, Protonotarios A, Gimeno JR, Arbustini E, Barriales-Villa R, Basso C et al (2023) 2023 ESC guidelines for the management of cardiomyopathies. 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