title: 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.
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 (rs= 0.79) and indexed left atrial volume (rs= 0.49), demonstrated significant positive correlation, while mitral valve A (rs= -0.44), lateral e' (rs= -0.61) and lateral a' (rs= -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.
authors.affiliation: The Heart Institute, Cincinnati Children's Hospital, 3333 Burnet Ave, MLC 2003, Cincinnati, OH, 45229-2003, USA. thomas.ryan@cchmc.org.
authors.forename: Thomas D
authors.initials: TD
authors.lastname: Ryan
authors.affiliation: The Heart Institute, Cincinnati Children's Hospital, 3333 Burnet Ave, MLC 2003, Cincinnati, OH, 45229-2003, USA.
authors.forename: Peace C
authors.initials: PC
authors.lastname: Madueme
authors.affiliation: The Heart Institute, Cincinnati Children's Hospital, 3333 Burnet Ave, MLC 2003, Cincinnati, OH, 45229-2003, USA.
authors.forename: John L
authors.initials: JL
authors.lastname: Jefferies
authors.affiliation: Sibley Heart Center, Children's Healthcare of Atlanta, Atlanta, GA, USA.
authors.forename: Erik C
authors.initials: EC
authors.lastname: Michelfelder
authors.affiliation: Heart Institute, Le Bonheur Children's Hospital, Memphis, TN, USA.
authors.forename: Jeffrey A
authors.initials: JA
authors.lastname: Towbin
authors.affiliation: Division of Biostatistics and Epidemiology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA.
authors.forename: Jessica G
authors.initials: JG
authors.lastname: Woo
authors.affiliation: Division of Biostatistics and Epidemiology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA.
authors.forename: Rashmi D
authors.initials: RD
authors.lastname: Sahay
authors.affiliation: Division of Biostatistics and Epidemiology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA.
authors.forename: Eileen C
authors.initials: EC
authors.lastname: King
authors.affiliation: The Heart Institute, Cincinnati Children's Hospital, 3333 Burnet Ave, MLC 2003, Cincinnati, OH, 45229-2003, USA.
authors.forename: Roberta
authors.initials: R
authors.lastname: Brown
authors.affiliation: The Heart Institute, Cincinnati Children's Hospital, 3333 Burnet Ave, MLC 2003, Cincinnati, OH, 45229-2003, USA.
authors.forename: Ryan A
authors.initials: RA
authors.lastname: Moore
authors.affiliation: Pediatrix Cardiology Associates of New Mexico, Albuquerque, NM, USA.
authors.forename: Michelle A
authors.initials: MA
authors.lastname: Grenier
authors.affiliation: The Heart Institute, Cincinnati Children's Hospital, 3333 Burnet Ave, MLC 2003, Cincinnati, OH, 45229-2003, USA.
authors.forename: Bryan H
authors.initials: BH
authors.lastname: Goldstein
mesh_terms: D000293:Adolescent; D001783:Blood Flow Velocity; D006328:Cardiac Catheterization / Q000009:adverse effects*; D002313:Cardiomyopathy, Restrictive / Q000503:physiopathology* / Q000628:therapy*; D002648:Child; D003971:Diastole; D015150:Echocardiography, Doppler; D005260:Female; D006801:Humans; D008297:Male; D008943:Mitral Valve / Q000503:physiopathology; D011446:Prospective Studies; D012372:ROC Curve; D012680:Sensitivity and Specificity; D013318:Stroke Volume; D018487:Ventricular Dysfunction, Left / Q000000981:diagnostic imaging*; D055815:Young Adult
keywords: Catheterization; Echocardiography; Pediatric; Restrictive cardiomyopathy