Applicability of Published Guidelines for Assessment of Left Ventricular Diastolic Function in Adults to Children with Restrictive Cardiomyopathy

An Observational Study

Nao Sasaki, Mario J. Garcia, H. Helen Ko, Sangeeta Sharma, Ira A. Parness, Shubhika Srivastava

Research output: Contribution to journalArticle

7 Citations (Scopus)

Abstract

Guidelines for diagnosis and grading of diastolic dysfunction (DD) in children have not been established. The applicability of adult parameters of DD to children has been questioned by recent studies. Although normal diastolic parameters in children have been published, the data to support application of these indices for the non-invasive diagnosis of DD and quantifying its degree are still being developed. Restrictive cardiomyopathy (RCM) is the only recognized disease entity in children that presents with isolated, irreversible DD as the predominant finding. The aim of this study was to investigate the applicability of current diastolic indices used for assessment of diastolic function in adults as reliable indicators of DD in children with established RCM. Retrospective review of institutional clinical database for the period of 2002–2010 was performed to identify patients with RCM who had had a comprehensive echocardiographic assessment of diastolic function. The following parameters were obtained from apical four chamber view: mitral valve (MV) inflow Doppler early filling velocity (E), late filling velocity (A), deceleration time (DT), color M-mode flow propagation from MV to apex (Vp), Doppler tissue imaging derived early diastolic velocity E′ and late diastolic velocity A′ at the LV lateral wall at MV annulus, RV at the tricuspid valve annulus, septum, and LA area. All parameters were compared to age and gender matched controls using student t test. : LA area/BSA was significantly larger in RCM group than the control group, median 22.8 cm<sup>2</sup>/m<sup>2</sup> (range 16.9–28.6) versus 10.3 cm<sup>2</sup>/m<sup>2</sup> (range 8.3–12.3), p value <0.001. MV inflow E and A were lower, and DT was shorter in the RCM group (p = 0.04, 0.02, and 0.005, respectively). A wave was absent in 3 of 9 patients in the RCM group. Ratio of E to A (E/A) was not different between the two groups. E′ was significantly lower at all three sites in RCM group; however, there was some overlap between the two groups. E/septal E′ ratio was statistically significantly higher in RCM group. A′ was absent either at lateral wall or at septum in five patients. 7 of 9 patients in RCM group had L′ wave (at lateral wall or septum) defined as negative deflection during diastasis. Vp was higher in RCM group than in the control group 81.4 ± 44.5 versus 52.9 ± 10.9, p value <0.01. Combination of increased left atrial size, septal E/E′, and lack of A wave and presence of mid-diastolic L′-wave are the noted abnormalities in this group. Individual cut-offs for Doppler indices have very poor sensitivity in identifying restrictive physiology. These findings suggest that poor LV compliance is the hallmark of restrictive cardiomyopathy in children even in the presence of normal early relaxation and ventricular filling. These findings support the need for development of guidelines for diagnosis and physiologic grading of diastolic dysfunction in children.

Original languageEnglish (US)
Pages (from-to)386-392
Number of pages7
JournalPediatric Cardiology
Volume36
Issue number2
DOIs
StatePublished - 2015
Externally publishedYes

Fingerprint

Restrictive Cardiomyopathy
Left Ventricular Function
Observational Studies
Guidelines
Mitral Valve
Deceleration
Control Groups
Tricuspid Valve
Compliance

Keywords

  • Children
  • Diastolic dysfunction
  • Restrictive cardiomyopathy

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine
  • Pediatrics, Perinatology, and Child Health

Cite this

Applicability of Published Guidelines for Assessment of Left Ventricular Diastolic Function in Adults to Children with Restrictive Cardiomyopathy : An Observational Study. / Sasaki, Nao; Garcia, Mario J.; Ko, H. Helen; Sharma, Sangeeta; Parness, Ira A.; Srivastava, Shubhika.

In: Pediatric Cardiology, Vol. 36, No. 2, 2015, p. 386-392.

Research output: Contribution to journalArticle

@article{ad56d1bef9d547ce98094f960c887ca1,
title = "Applicability of Published Guidelines for Assessment of Left Ventricular Diastolic Function in Adults to Children with Restrictive Cardiomyopathy: An Observational Study",
abstract = "Guidelines for diagnosis and grading of diastolic dysfunction (DD) in children have not been established. The applicability of adult parameters of DD to children has been questioned by recent studies. Although normal diastolic parameters in children have been published, the data to support application of these indices for the non-invasive diagnosis of DD and quantifying its degree are still being developed. Restrictive cardiomyopathy (RCM) is the only recognized disease entity in children that presents with isolated, irreversible DD as the predominant finding. The aim of this study was to investigate the applicability of current diastolic indices used for assessment of diastolic function in adults as reliable indicators of DD in children with established RCM. Retrospective review of institutional clinical database for the period of 2002–2010 was performed to identify patients with RCM who had had a comprehensive echocardiographic assessment of diastolic function. The following parameters were obtained from apical four chamber view: mitral valve (MV) inflow Doppler early filling velocity (E), late filling velocity (A), deceleration time (DT), color M-mode flow propagation from MV to apex (Vp), Doppler tissue imaging derived early diastolic velocity E′ and late diastolic velocity A′ at the LV lateral wall at MV annulus, RV at the tricuspid valve annulus, septum, and LA area. All parameters were compared to age and gender matched controls using student t test. : LA area/BSA was significantly larger in RCM group than the control group, median 22.8 cm2/m2 (range 16.9–28.6) versus 10.3 cm2/m2 (range 8.3–12.3), p value <0.001. MV inflow E and A were lower, and DT was shorter in the RCM group (p = 0.04, 0.02, and 0.005, respectively). A wave was absent in 3 of 9 patients in the RCM group. Ratio of E to A (E/A) was not different between the two groups. E′ was significantly lower at all three sites in RCM group; however, there was some overlap between the two groups. E/septal E′ ratio was statistically significantly higher in RCM group. A′ was absent either at lateral wall or at septum in five patients. 7 of 9 patients in RCM group had L′ wave (at lateral wall or septum) defined as negative deflection during diastasis. Vp was higher in RCM group than in the control group 81.4 ± 44.5 versus 52.9 ± 10.9, p value <0.01. Combination of increased left atrial size, septal E/E′, and lack of A wave and presence of mid-diastolic L′-wave are the noted abnormalities in this group. Individual cut-offs for Doppler indices have very poor sensitivity in identifying restrictive physiology. These findings suggest that poor LV compliance is the hallmark of restrictive cardiomyopathy in children even in the presence of normal early relaxation and ventricular filling. These findings support the need for development of guidelines for diagnosis and physiologic grading of diastolic dysfunction in children.",
keywords = "Children, Diastolic dysfunction, Restrictive cardiomyopathy",
author = "Nao Sasaki and Garcia, {Mario J.} and Ko, {H. Helen} and Sangeeta Sharma and Parness, {Ira A.} and Shubhika Srivastava",
year = "2015",
doi = "10.1007/s00246-014-1018-z",
language = "English (US)",
volume = "36",
pages = "386--392",
journal = "Pediatric Cardiology",
issn = "0172-0643",
publisher = "Springer New York",
number = "2",

}

TY - JOUR

T1 - Applicability of Published Guidelines for Assessment of Left Ventricular Diastolic Function in Adults to Children with Restrictive Cardiomyopathy

T2 - An Observational Study

AU - Sasaki, Nao

AU - Garcia, Mario J.

AU - Ko, H. Helen

AU - Sharma, Sangeeta

AU - Parness, Ira A.

AU - Srivastava, Shubhika

PY - 2015

Y1 - 2015

N2 - Guidelines for diagnosis and grading of diastolic dysfunction (DD) in children have not been established. The applicability of adult parameters of DD to children has been questioned by recent studies. Although normal diastolic parameters in children have been published, the data to support application of these indices for the non-invasive diagnosis of DD and quantifying its degree are still being developed. Restrictive cardiomyopathy (RCM) is the only recognized disease entity in children that presents with isolated, irreversible DD as the predominant finding. The aim of this study was to investigate the applicability of current diastolic indices used for assessment of diastolic function in adults as reliable indicators of DD in children with established RCM. Retrospective review of institutional clinical database for the period of 2002–2010 was performed to identify patients with RCM who had had a comprehensive echocardiographic assessment of diastolic function. The following parameters were obtained from apical four chamber view: mitral valve (MV) inflow Doppler early filling velocity (E), late filling velocity (A), deceleration time (DT), color M-mode flow propagation from MV to apex (Vp), Doppler tissue imaging derived early diastolic velocity E′ and late diastolic velocity A′ at the LV lateral wall at MV annulus, RV at the tricuspid valve annulus, septum, and LA area. All parameters were compared to age and gender matched controls using student t test. : LA area/BSA was significantly larger in RCM group than the control group, median 22.8 cm2/m2 (range 16.9–28.6) versus 10.3 cm2/m2 (range 8.3–12.3), p value <0.001. MV inflow E and A were lower, and DT was shorter in the RCM group (p = 0.04, 0.02, and 0.005, respectively). A wave was absent in 3 of 9 patients in the RCM group. Ratio of E to A (E/A) was not different between the two groups. E′ was significantly lower at all three sites in RCM group; however, there was some overlap between the two groups. E/septal E′ ratio was statistically significantly higher in RCM group. A′ was absent either at lateral wall or at septum in five patients. 7 of 9 patients in RCM group had L′ wave (at lateral wall or septum) defined as negative deflection during diastasis. Vp was higher in RCM group than in the control group 81.4 ± 44.5 versus 52.9 ± 10.9, p value <0.01. Combination of increased left atrial size, septal E/E′, and lack of A wave and presence of mid-diastolic L′-wave are the noted abnormalities in this group. Individual cut-offs for Doppler indices have very poor sensitivity in identifying restrictive physiology. These findings suggest that poor LV compliance is the hallmark of restrictive cardiomyopathy in children even in the presence of normal early relaxation and ventricular filling. These findings support the need for development of guidelines for diagnosis and physiologic grading of diastolic dysfunction in children.

AB - Guidelines for diagnosis and grading of diastolic dysfunction (DD) in children have not been established. The applicability of adult parameters of DD to children has been questioned by recent studies. Although normal diastolic parameters in children have been published, the data to support application of these indices for the non-invasive diagnosis of DD and quantifying its degree are still being developed. Restrictive cardiomyopathy (RCM) is the only recognized disease entity in children that presents with isolated, irreversible DD as the predominant finding. The aim of this study was to investigate the applicability of current diastolic indices used for assessment of diastolic function in adults as reliable indicators of DD in children with established RCM. Retrospective review of institutional clinical database for the period of 2002–2010 was performed to identify patients with RCM who had had a comprehensive echocardiographic assessment of diastolic function. The following parameters were obtained from apical four chamber view: mitral valve (MV) inflow Doppler early filling velocity (E), late filling velocity (A), deceleration time (DT), color M-mode flow propagation from MV to apex (Vp), Doppler tissue imaging derived early diastolic velocity E′ and late diastolic velocity A′ at the LV lateral wall at MV annulus, RV at the tricuspid valve annulus, septum, and LA area. All parameters were compared to age and gender matched controls using student t test. : LA area/BSA was significantly larger in RCM group than the control group, median 22.8 cm2/m2 (range 16.9–28.6) versus 10.3 cm2/m2 (range 8.3–12.3), p value <0.001. MV inflow E and A were lower, and DT was shorter in the RCM group (p = 0.04, 0.02, and 0.005, respectively). A wave was absent in 3 of 9 patients in the RCM group. Ratio of E to A (E/A) was not different between the two groups. E′ was significantly lower at all three sites in RCM group; however, there was some overlap between the two groups. E/septal E′ ratio was statistically significantly higher in RCM group. A′ was absent either at lateral wall or at septum in five patients. 7 of 9 patients in RCM group had L′ wave (at lateral wall or septum) defined as negative deflection during diastasis. Vp was higher in RCM group than in the control group 81.4 ± 44.5 versus 52.9 ± 10.9, p value <0.01. Combination of increased left atrial size, septal E/E′, and lack of A wave and presence of mid-diastolic L′-wave are the noted abnormalities in this group. Individual cut-offs for Doppler indices have very poor sensitivity in identifying restrictive physiology. These findings suggest that poor LV compliance is the hallmark of restrictive cardiomyopathy in children even in the presence of normal early relaxation and ventricular filling. These findings support the need for development of guidelines for diagnosis and physiologic grading of diastolic dysfunction in children.

KW - Children

KW - Diastolic dysfunction

KW - Restrictive cardiomyopathy

UR - http://www.scopus.com/inward/record.url?scp=84921726473&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=84921726473&partnerID=8YFLogxK

U2 - 10.1007/s00246-014-1018-z

DO - 10.1007/s00246-014-1018-z

M3 - Article

VL - 36

SP - 386

EP - 392

JO - Pediatric Cardiology

JF - Pediatric Cardiology

SN - 0172-0643

IS - 2

ER -