Interpretation of left ventricular diastolic dysfunction in children with cardiomyopathy by echocardiography:...

Area of Science:

• Pediatric Cardiology
• Cardiovascular Imaging
• Diastology

Background:

• Left ventricular diastolic dysfunction (DD) significantly impacts outcomes in pediatric cardiomyopathy (CM).
• Current diagnostic and classification paradigms for DD in children are largely based on adult guidelines.
• The applicability of adult DD criteria to pediatric CM populations remains uncertain.

Purpose of the Study:

• To investigate the assessment of DD in pediatric CM using adult and pediatric echocardiographic criteria.
• To evaluate the applicability of recent adult guidelines for DD to children with CM.
• To determine the diagnostic accuracy and interobserver agreement of echocardiographic parameters for DD in pediatric CM.

Main Methods:

• Independent classification of diastolic function by three investigators across four groups: controls, dilated, hypertrophic, and restrictive CM.
• Assessment of investigator agreement, failure to classify DD, and reasons for diagnostic failure.
• Evaluation of the utility of individual echocardiographic parameters for diagnosing and classifying DD in 175 children (aged 0-18 years).

Main Results:

• Discrepancies in DD diagnostic criteria were observed in the majority of pediatric CM patients.
• Delayed relaxation was diagnosed in only 14% of hypertrophic CM and never in dilated or restrictive CM.
• Key echocardiographic parameters like mitral and pulmonary venous Doppler were often uninformative, and interobserver agreement for DD grading was poor (36%).

Conclusions:

• Current echocardiographic guidelines appear inadequate for assessing DD in pediatric CM.
• Pediatric reference values for DD parameters are broad, limiting diagnosis in a small patient proportion.
• Insufficiently discriminatory echo parameters and criterion discrepancies hinder classification and lead to poor interobserver agreement in pediatric CM.