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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

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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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Abbreviations

IQR:

Interquartile range

LVEDP:

Left ventricular end-diastolic pressure

RCM:

Restrictive cardiomyopathy

ROC:

Receiver operating characteristic

RVEDP:

Right ventricular end-diastolic pressure

TTE:

Transthoracic echocardiogram

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Acknowledgements

The authors wish to acknowledge support from the Heart Institute Research Core, as well as the dedicated sonographers and cardiac catheterization laboratory personnel who made performance of this study possible.

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Authors and Affiliations

  1. The Heart Institute, Cincinnati Children’s Hospital, 3333 Burnet Ave, MLC 2003, Cincinnati, OH, 45229-2003, USA

    Thomas D. Ryan, Peace C. Madueme, John L. Jefferies, Roberta Brown, Ryan A. Moore & Bryan H. Goldstein

  2. Sibley Heart Center, Children’s Healthcare of Atlanta, Atlanta, GA, USA

    Erik C. Michelfelder

  3. Heart Institute, Le Bonheur Children’s Hospital, Memphis, TN, USA

    Jeffrey A. Towbin

  4. Division of Biostatistics and Epidemiology, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH, USA

    Jessica G. Woo, Rashmi D. Sahay & Eileen C. King

  5. Pediatrix Cardiology Associates of New Mexico, Albuquerque, NM, USA

    Michelle A. Grenier

Authors
  1. Thomas D. Ryan
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  2. Peace C. Madueme
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  4. Erik C. Michelfelder
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  8. Eileen C. King
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  9. Roberta Brown
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  10. Ryan A. Moore
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  12. Bryan H. Goldstein
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Corresponding author

Correspondence to Thomas D. Ryan.

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Conflict of interest

TD Ryan, PC Madueme, JL Jefferies, EC Michelfelder, JA Towbin, JG Woo, RD Sahay, EC King, R Brown, RA Moore, MA Grenier, BH Goldstein declares that they have no conflict of interest.

Ethical Approval

All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki Declaration and its later amendments or comparable ethical standards.

Informed Consent

Informed consent was obtained from all individual participants included in the study.

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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)

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Ryan, T.D., Madueme, P.C., Jefferies, J.L. et al. 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. Pediatr Cardiol 38, 381–389 (2017). https://doi.org/10.1007/s00246-016-1526-0

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