Reversible left ventricular noncompaction caused by hypertensive hydrocephalus: a pediatric case report

BMC Pediatr. 2021 Apr 28;21(1):205. doi: 10.1186/s12887-021-02680-6.

Abstract

Background: Left ventricular noncompaction cardiomyopathy (LVNC) is characterized by prominent ventricular trabeculations on cardiovascular imaging. Acquired reversible LVNC has not been reported in pediatrics without a genetic background.

Case presentation: A 9-year-old girl with a ventriculoperitoneal (VP) shunt for neonatal posthemorrhagic hydrocephalus was referred due to exacerbation of hydrocephalus caused by VP shunt dysfunction. Transthoracic echocardiography (TTE) revealed depressed left ventricular (LV) systolic function and thick prominent trabeculae in the LV, predominantly in the apex, suggesting LVNC. Following treatment with extraventricular drainage for hydrocephalus, prominent trabeculation of the LV was diminished on TTE within 3 months. Genetic testing using next-generation sequencing was performed, and no significant variants were identified.

Conclusions: We revealed for the first time a pediatric case of reversible LVNC without genetic predisposition. This case report provides valuable information on the pathogenesis of acquired LVNC and suggests that detailed evaluation is required to elucidate the diagnosis of this wide spectrum of etiologic-pathogenetic disorders.

Keywords: Hypertensive hydrocephalus; Left ventricular noncompaction.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Child
  • Echocardiography
  • Female
  • Heart Defects, Congenital*
  • Humans
  • Hydrocephalus* / diagnostic imaging
  • Hydrocephalus* / etiology
  • Infant, Newborn
  • Isolated Noncompaction of the Ventricular Myocardium* / diagnosis
  • Isolated Noncompaction of the Ventricular Myocardium* / diagnostic imaging
  • Pediatrics*