Residual or recurrent obstruction after septal myectomy in young children and infants with hypertrophic cardiomyopathy: cohort study

Author:

Dong Shuo1,Du Chuhao1,Song Jiangping12,Dong Jie1,Meng Hong3,Xu Haitao1,Sun Yangxue1,Zou Mengxuan1,Li Shoujun1,Yan Jun1

Affiliation:

1. Department of Pediatric Cardiac Surgery, National Center for Cardiovascular Disease and Fuwai Hospital

2. State Key Laboratory of Cardiovascular Disease, Fuwai Hospital, National Center for Cardiovascular Diseases

3. Department of ultrasound, National Center for Cardiovascular Disease and Fuwai Hospital, Chinese Academy of Medical Sciences, Peking Union Medical College, Beijing, China

Abstract

Background: The outcomes after septal myectomy in young children and infants with hypertrophic obstructive cardiomyopathy (HOCM) are not clear. The study sought to report the outcomes after septal myectomy in young children and infants and identify the mechanisms of residual or recurrent obstruction after surgery. Methods: The authors performed an observational cohort study of children and infants under the age of 14 who underwent septal myectomy for HCOM from January 2013 to December 2020. Mean follow-up among 94.3% (n=50) of hospital survivors was 42.09±24.38 months. Results: In total, 56 children and infants [mean (SD) age, 5.38 (3.78) years; 29 (58.1%) were male] underwent septal myectomy for HOCM. Cumulative survival was 100, 96.6, 93.0, and 81.4% at 1, 3, 5, and 7 years, respectively, among hospital survivors. The incidence of residual and recurrent obstruction was 14.3% (8/56) and 13.0% (6/46), respectively. The mechanisms of residual obstruction were identified as subaortic obstruction caused by inadequacy of previous septal excision in two patients, midventricular obstruction caused by inadequacy of septal excision in five patients, and untreated abnormal papillary muscles in one patient. Recurrent obstruction was caused by isolated midventricular obstruction (n=4) and newly emerged systolic anterior motion (SAM)-related subaortic obstruction combining abnormal mitral valve apparatus (n=2). Residual or recurrent obstruction was associated with age less than 2 years at surgery (OR=6.157, 95% CI: 1.487–25.487, P=0.012) and biventricular outflow obstruction (OR=6.139, 95% CI: 1.292–29.172, P=0.022). Recurrent obstruction was associated with age less than 2 years at surgery (OR=6.976, 95% CI: 1.233–39.466, P=0.028). Conclusions: Septal myectomy is still effective and safe in young children and infants. The rate of residual or recurrent obstruction with diverse causes is relatively high, which is more likely to occur in children aged less than 2 years at surgery and those with biventricular obstruction.

Publisher

Ovid Technologies (Wolters Kluwer Health)

Subject

General Medicine,Surgery

Reference24 articles.

1. Hypertrophic cardiomyopathy is predominantly a disease of left ventricular outflow tract obstruction;Maron;Circulation,2006

2. Effect of left ventricular outflow tract obstruction on clinical outcome in hypertrophic cardiomyopathy;Maron;N Engl J Med,2003

3. 2020 AHA/ACC Guideline for the Diagnosis and Treatment of Patients With Hypertrophic Cardiomyopathy: A Report of the American College of Cardiology/American Heart Association Joint Committee on Clinical Practice Guidelines;Ommen;Circulation,2020

4. 2014 ESC Guidelines on diagnosis and management of hypertrophic cardiomyopathy: the Task Force for the Diagnosis and Management of Hypertrophic Cardiomyopathy of the European Society of Cardiology (ESC);Elliott;Eur Heart J,2014

5. Clinical long-term outcome of septal myectomy for obstructive hypertrophic cardiomyopathy in infants;Schleihauf;Eur J Cardiothorac Surg,2018

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