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Abnormal Left Ventricular Strain Correlates with Left Ventricular Dysfunction but not Aortic Pathology in Marfan

Patrick S Connell1, Shaine A Morris1, Tam T Doan1

  • 1Section of Pediatric Cardiology, Department of Pediatrics, Baylor College of Medicine and Texas Children's Hospital, One Baylor Plaza, 6651 Main St., MC E2060.14, Houston, TX, 77030, USA.

Pediatric Cardiology
|July 28, 2023
PubMed

Insights

Abnormal ventricular strain in children with Marfan syndrome (MFS) is linked to reduced left ventricular ejection fraction (LVEF). Global circumferential strain (GCS) can predict future LVEF decline, aiding early risk assessment in pediatric MFS patients.

Area of Science:

  • Cardiology
  • Pediatric Cardiology
  • Medical Imaging

Background:

  • Cardiomyopathy is a known complication in adults with Marfan syndrome (MFS).
  • Early identification of pediatric MFS patients at high risk for cardiomyopathy is crucial for timely monitoring and intervention.
  • Ventricular strain abnormalities are understudied in children with MFS, despite their association with impaired ventricular function in adults.

Purpose of the Study:

  • To investigate the association between ventricular strain parameters and left ventricular ejection fraction (LVEF) in children with Marfan syndrome (MFS).
  • To evaluate if ventricular strain can predict the development of abnormal LVEF within a 5-year period in pediatric MFS patients.
  • To explore correlations between ventricular strain and aortic root dilation or vertebral artery tortuosity in this cohort.

Main Methods:

  • Retrospective analysis of cardiac magnetic resonance imaging (CMR) data from 31 pediatric patients (age < 19 years) with Marfan syndrome (MFS).
  • Evaluation of initial global circumferential strain (GCS) and global longitudinal strain (GLS) against LVEF, aortic root z-score, and vertebral artery tortuosity index (VTI-h).
  • Spearman correlation was used to assess relationships, and prediction of abnormal LVEF within 5 years was analyzed in patients with serial CMR.

Main Results:

  • Worse GCS and GLS were significantly associated with lower LVEF (p < 0.001 and p = 0.030, respectively).
  • A GCS cutoff of -34% predicted LVEF < 55% with 80% sensitivity and 50% specificity.
  • Neither GCS nor GLS correlated with aortic root z-score or VTI-h. Initial strain did not predict future LVEF abnormalities in those with initially normal LVEF.

Conclusions:

  • Abnormal left ventricular (LV) strain is associated with reduced LVEF in children with Marfan syndrome (MFS).
  • Global circumferential strain (GCS) shows potential as a predictor of reduced LVEF in this pediatric population.
  • Ventricular strain was not associated with aortic dilation or future LVEF decline within 5 years in this study cohort.
Keywords:
Cardiac magnetic resonance imagingCardiomyopathyMarfan syndromeStrainVentricular function

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