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23.11.2016 | Original Article | Ausgabe 2/2017

Pediatric Cardiology 2/2017

Utility of Echocardiography in the Assessment of Left Ventricular Diastolic Function and Restrictive Physiology in Children and Young Adults with Restrictive Cardiomyopathy: A Comparative Echocardiography-Catheterization Study

Zeitschrift:
Pediatric Cardiology > Ausgabe 2/2017
Autoren:
Thomas D. Ryan, Peace C. Madueme, John L. Jefferies, Erik C. Michelfelder, Jeffrey A. Towbin, Jessica G. Woo, Rashmi D. Sahay, Eileen C. King, Roberta Brown, Ryan A. Moore, Michelle A. Grenier, Bryan H. Goldstein
Wichtige Hinweise

Electronic supplementary material

The online version of this article (doi:10.​1007/​s00246-016-1526-0) contains supplementary material, which is available to authorized users.

Abstract

The aim of the study is to determine the utility of echocardiography in the assessment of diastolic function in children and young adults with restrictive cardiomyopathy (RCM). RCM is a rare disease with high mortality requiring frequent surveillance. Accurate, noninvasive echocardiographic measures of diastolic function may reduce the need for invasive catheterization. Single-center, prospective, observational study of pediatric and young adult RCM patients undergoing assessment of diastolic parameters by simultaneous transthoracic echocardiogram (TTE) and invasive catheterization. Twenty-one studies in 15 subjects [median (IQR) = 13.8 years (7.0–19.2), 60% female] were acquired with median left ventricular end-diastolic pressure (LVEDP) 21 (IQR 18–25) mmHg. TTE parameters of diastolic function, including pulmonary vein A wave duration (r s  = 0.79) and indexed left atrial volume (r s  = 0.49), demonstrated significant positive correlation, while mitral valve A (r s  = −0.44), lateral e′ (r s  = −0.61) and lateral a′ (r s  = −0.61) velocities showed significant negative correlation with LVEDP. Lateral a′ velocity (≤0.042 m/s) and pulmonary vein A wave duration (≥156 m/s) both had sensitivity and specificity ≥80% for LVEDP ≥ 20 mmHg. In pediatric and young adult patients with RCM, lateral a′ velocity and pulmonary vein A wave duration predicted elevated LVEDP with high sensitivity and specificity; however, due to technical limitations the latter was reliably measured in 12/21 patients. These noninvasive parameters may have utility in identifying patients that require further assessment with invasive testing. These findings require validation in a multicenter prospective cohort prior to widespread clinical implementation.

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Zusatzmaterial
Supplemental Table 1 Intra-class correlation coefficient (ICC) for selected echocardiogram values. Measurement of ICC was taken in 10 studies (47%). Higher values represent stronger agreement, with 0.0–0.2 indicating very little agreement, 0.2–0.4 little agreement, 0.4–0.6 moderate agreement, 0.6–0.8 strong agreement and 0.8–1.0 very strong agreement (DOCX 11 kb)
246_2016_1526_MOESM1_ESM.docx
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