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No increased extracellular volume fraction or conduction time after childhood septal myectomy

Julia Schleihauf1, Julie Cleuziou2, Christian Meierhofer1

  • 1Department of Congenital Heart Disease and Pediatric Cardiology, German Heart Center Munich, Technical University of Munich, Munich, Germany.

European Journal of Cardio-Thoracic Surgery : Official Journal of the European Association for Cardio-Thoracic Surgery
|January 18, 2020
PubMed

Insights

Surgical septal myectomy in childhood for hypertrophic cardiomyopathy does not increase myocardial fibrosis or conduction problems. This procedure is safe for managing left ventricular outflow tract obstruction long-term.

Area of Science:

  • Cardiology
  • Pediatric Cardiology
  • Cardiovascular Imaging

Background:

  • Hypertrophic cardiomyopathy (HCM) is a genetic heart muscle disease.
  • Severe HCM in children can cause left ventricular outflow tract obstruction (LVOTO).
  • Surgical septal myectomy is a treatment option for drug-refractory LVOTO in pediatric HCM.

Purpose of the Study:

  • To evaluate the long-term effects of early childhood septal myectomy on myocardial extracellular volume fraction.
  • To assess the risk of atrioventricular conduction system disease after pediatric septal myectomy.
  • To investigate the impact of septal myectomy on myocardial fibrosis in pediatric HCM.

Main Methods:

  • Retrospective review of 30 pediatric HCM patients.
  • Cardiovascular magnetic resonance (CMR) with T1 mapping and late gadolinium enhancement.
  • Comparison between 12 myectomy patients and 18 non-operated patients.

Main Results:

  • Septal myocardial fibrosis (late gadolinium enhancement) was present in 53% of patients, with no group difference.
  • Extracellular volume fraction, a marker of diffuse fibrosis, was similar in myectomy and non-operated groups.
  • No significant difference in PQ-intervals or higher-degree atrioventricular conduction disease between groups.

Conclusions:

  • Pediatric septal myectomy does not increase septal extracellular volume fraction.
  • The procedure does not lead to delayed atrioventricular conduction times on long-term follow-up.
  • Septal myectomy is a safe intervention for severe obstructive HCM in early childhood.
Abstract

Keywords:
ArrhythmiaCongenital heart surgeryHypertrophic obstructive cardiomyopathyMagnetic resonance imagingMyocardial remodelling

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