Hypertrophic cardiomyopathy (HCM) in childhood is characterized by marked phenotypic heterogeneity and poses significant challenges in risk stratification for sudden cardiac death (SCD). We report the case of a 9.5-year-old girl in whom transthoracic echocardiography revealed progressive extreme interventricular septal hypertrophy (up to 33 mm; Z-score > +11), marked left atrial enlargement, reduced left ventricular cavity size, and significant left ventricular outflow tract obstruction. Although repeated ambulatory Holter monitoring did not reveal ventricular arrhythmias, the patient exhibited one episode of unexplained syncope at rest. These findings placed her in a high-risk category according to contemporary pediatric risk stratification approaches. Following insufficient response to medical therapy, surgical septal myectomy was performed. Given the persistent high-risk profile, an implantable cardioverter-defibrillator was implanted for primary prevention of sudden cardiac death. This case emphasizes the importance of individualized, multimodal risk stratification in guiding management decisions in pediatric HCM, even in the absence of documented malignant arrhythmias.
Abstract
Case Reports
Journal Article
eng
42093794
Smajlović, Elma, and Alma Bolic Alic. "Extreme Left Ventricular Hypertrophy in Pediatric Hypertrophic Obstructive Cardiomyopathy." Cureus, vol. 18, no. 4, 2026, pp. e106450.
Smajlović E, Bolic Alic A. Extreme Left Ventricular Hypertrophy in Pediatric Hypertrophic Obstructive Cardiomyopathy. Cureus. 2026;18(4):e106450.
Smajlović, E., & Bolic Alic, A. (2026). Extreme Left Ventricular Hypertrophy in Pediatric Hypertrophic Obstructive Cardiomyopathy. Cureus, 18(4), e106450. https://doi.org/10.7759/cureus.106450
Smajlović E, Bolic Alic A. Extreme Left Ventricular Hypertrophy in Pediatric Hypertrophic Obstructive Cardiomyopathy. Cureus. 2026;18(4):e106450. PubMed PMID: 42093794.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR
T1 - Extreme Left Ventricular Hypertrophy in Pediatric Hypertrophic Obstructive Cardiomyopathy.
AU - Smajlović,Elma,
AU - Bolic Alic,Alma,
Y1 - 2026/04/05/
PY - 2026/04/04/accepted
PY - 2026/5/7/medline
PY - 2026/5/7/pubmed
PY - 2026/5/7/entrez
KW - hypertrophic cardiomayopathy
KW - implantable cardioverter-defibrillator
KW - septal myectomy
KW - sudden cardiac death (scd)
KW - ventricular tachycardia (vt)
SP - e106450
EP - e106450
JF - Cureus
JO - Cureus
VL - 18
IS - 4
N2 - Hypertrophic cardiomyopathy (HCM) in childhood is characterized by marked phenotypic heterogeneity and poses significant challenges in risk stratification for sudden cardiac death (SCD). We report the case of a 9.5-year-old girl in whom transthoracic echocardiography revealed progressive extreme interventricular septal hypertrophy (up to 33 mm; Z-score > +11), marked left atrial enlargement, reduced left ventricular cavity size, and significant left ventricular outflow tract obstruction. Although repeated ambulatory Holter monitoring did not reveal ventricular arrhythmias, the patient exhibited one episode of unexplained syncope at rest. These findings placed her in a high-risk category according to contemporary pediatric risk stratification approaches. Following insufficient response to medical therapy, surgical septal myectomy was performed. Given the persistent high-risk profile, an implantable cardioverter-defibrillator was implanted for primary prevention of sudden cardiac death. This case emphasizes the importance of individualized, multimodal risk stratification in guiding management decisions in pediatric HCM, even in the absence of documented malignant arrhythmias.
SN - 2168-8184
UR - https://www.unboundmedicine.com/prime/citation/42093794/Extreme_Left_Ventricular_Hypertrophy_in_Pediatric_Hypertrophic_Obstructive_Cardiomyopathy.
DB - PRIME
DP - Unbound Medicine
ER -
