Reduced Right Ventricular Fractional Area Change, Strain, and Strain Rate before Bidirectional Cavopulmonary Anastomosis is Associated with Medium-Term Mortality for Children with Hypoplastic Left Heart Syndrome

Lily Q. Lin, Jennifer Conway, Silvia Alvarez, Benjamin Goot, Jesus Serrano-Lomelin, Timothy Colen, Edythe B. Tham, Shelby Kutty, Ling Li, Nee Scze Khoo

Research output: Contribution to journalArticle

3 Citations (Scopus)

Abstract

Background: Ventricular dysfunction is associated with increased morbidity and mortality in children with hypoplastic left heart syndrome. The aim of this study was to assess the diagnostic performance of conventional and speckle-tracking echocardiographic measures of right ventricular (RV) function before bidirectional cavopulmonary anastomosis palliation in predicting death or need for heart transplantation (HTx). Methods: RV fractional area change (RVFAC) and longitudinal and circumferential strain and strain rate (SR) were measured in 64 prospectively recruited patients with hypoplastic left heart syndrome from echocardiograms obtained before bidirectional cavopulmonary anastomosis surgery. The composite end point of death or HTx was examined. Receiver operating characteristic analysis was performed, and cutoff values optimizing sensitivity and specificity were derived. Results: At a median follow-up of 5.0 years (interquartile range, 2.8–6.4 years), 13 patients meeting the composite end point had lower longitudinal strain and SR, circumferential SR, and RVFAC compared with survivors (n = 51). The conventional cutoff of RVFAC < 35% was specific for death or HTx (86%) but had poor sensitivity (46%), with an area under the curve of 0.73. Speckle-tracking echocardiographic variables showed similar areas under the curve (range, 0.69–0.79), with negative predictive values >90%. Addition of speckle-tracking echocardiographic variables to RVFAC < 35% showed no added benefit. However, in a subpopulation of patients with RVFAC ≥ 35% (n = 44), those meeting the composite end point (n = 7) had lower longitudinal SR (median, −1.0 1/sec [interquartile range, −0.8 to −1.1 1/sec] vs −1.21/sec [interquartile range, −1.0 to −1.3 1/sec], P =.03). Interobserver reproducibility was superior for longitudinal strain and SR (intraclass correlation coefficient > 0.92) compared with RVFAC (intraclass correlation coefficient = 0.75). Conclusions: Children with hypoplastic left heart syndrome with normal RVFAC and ventricular deformation before bidirectional cavopulmonary anastomosis have a low likelihood of death or HTx in the medium term. In the presence of reduced RVFAC, speckle-tracking echocardiography does not provide additional prognostic value. However, in patients with “normal” RVFAC, it may have a role in improving outcome prediction and warrants further investigation.

Original languageEnglish (US)
JournalJournal of the American Society of Echocardiography
DOIs
StateAccepted/In press - Jan 1 2018

Fingerprint

Right Heart Bypass
Hypoplastic Left Heart Syndrome
Child Mortality
Ventricular Dysfunction
Right Ventricular Function
Heart Transplantation
ROC Curve
Survivors
Echocardiography
Morbidity
Sensitivity and Specificity

Keywords

  • Hypoplastic left heart syndrome
  • Mortality
  • Speckle-tracking echocardiography
  • Strain
  • Strain rate

ASJC Scopus subject areas

  • Radiology Nuclear Medicine and imaging
  • Cardiology and Cardiovascular Medicine

Cite this

Reduced Right Ventricular Fractional Area Change, Strain, and Strain Rate before Bidirectional Cavopulmonary Anastomosis is Associated with Medium-Term Mortality for Children with Hypoplastic Left Heart Syndrome. / Lin, Lily Q.; Conway, Jennifer; Alvarez, Silvia; Goot, Benjamin; Serrano-Lomelin, Jesus; Colen, Timothy; Tham, Edythe B.; Kutty, Shelby; Li, Ling; Khoo, Nee Scze.

In: Journal of the American Society of Echocardiography, 01.01.2018.

Research output: Contribution to journalArticle

@article{574c710a97d24bf09bf4aaea38e83869,
title = "Reduced Right Ventricular Fractional Area Change, Strain, and Strain Rate before Bidirectional Cavopulmonary Anastomosis is Associated with Medium-Term Mortality for Children with Hypoplastic Left Heart Syndrome",
abstract = "Background: Ventricular dysfunction is associated with increased morbidity and mortality in children with hypoplastic left heart syndrome. The aim of this study was to assess the diagnostic performance of conventional and speckle-tracking echocardiographic measures of right ventricular (RV) function before bidirectional cavopulmonary anastomosis palliation in predicting death or need for heart transplantation (HTx). Methods: RV fractional area change (RVFAC) and longitudinal and circumferential strain and strain rate (SR) were measured in 64 prospectively recruited patients with hypoplastic left heart syndrome from echocardiograms obtained before bidirectional cavopulmonary anastomosis surgery. The composite end point of death or HTx was examined. Receiver operating characteristic analysis was performed, and cutoff values optimizing sensitivity and specificity were derived. Results: At a median follow-up of 5.0 years (interquartile range, 2.8–6.4 years), 13 patients meeting the composite end point had lower longitudinal strain and SR, circumferential SR, and RVFAC compared with survivors (n = 51). The conventional cutoff of RVFAC < 35{\%} was specific for death or HTx (86{\%}) but had poor sensitivity (46{\%}), with an area under the curve of 0.73. Speckle-tracking echocardiographic variables showed similar areas under the curve (range, 0.69–0.79), with negative predictive values >90{\%}. Addition of speckle-tracking echocardiographic variables to RVFAC < 35{\%} showed no added benefit. However, in a subpopulation of patients with RVFAC ≥ 35{\%} (n = 44), those meeting the composite end point (n = 7) had lower longitudinal SR (median, −1.0 1/sec [interquartile range, −0.8 to −1.1 1/sec] vs −1.21/sec [interquartile range, −1.0 to −1.3 1/sec], P =.03). Interobserver reproducibility was superior for longitudinal strain and SR (intraclass correlation coefficient > 0.92) compared with RVFAC (intraclass correlation coefficient = 0.75). Conclusions: Children with hypoplastic left heart syndrome with normal RVFAC and ventricular deformation before bidirectional cavopulmonary anastomosis have a low likelihood of death or HTx in the medium term. In the presence of reduced RVFAC, speckle-tracking echocardiography does not provide additional prognostic value. However, in patients with “normal” RVFAC, it may have a role in improving outcome prediction and warrants further investigation.",
keywords = "Hypoplastic left heart syndrome, Mortality, Speckle-tracking echocardiography, Strain, Strain rate",
author = "Lin, {Lily Q.} and Jennifer Conway and Silvia Alvarez and Benjamin Goot and Jesus Serrano-Lomelin and Timothy Colen and Tham, {Edythe B.} and Shelby Kutty and Ling Li and Khoo, {Nee Scze}",
year = "2018",
month = "1",
day = "1",
doi = "10.1016/j.echo.2018.02.001",
language = "English (US)",
journal = "Journal of the American Society of Echocardiography",
issn = "0894-7317",
publisher = "Mosby Inc.",

}

TY - JOUR

T1 - Reduced Right Ventricular Fractional Area Change, Strain, and Strain Rate before Bidirectional Cavopulmonary Anastomosis is Associated with Medium-Term Mortality for Children with Hypoplastic Left Heart Syndrome

AU - Lin, Lily Q.

AU - Conway, Jennifer

AU - Alvarez, Silvia

AU - Goot, Benjamin

AU - Serrano-Lomelin, Jesus

AU - Colen, Timothy

AU - Tham, Edythe B.

AU - Kutty, Shelby

AU - Li, Ling

AU - Khoo, Nee Scze

PY - 2018/1/1

Y1 - 2018/1/1

N2 - Background: Ventricular dysfunction is associated with increased morbidity and mortality in children with hypoplastic left heart syndrome. The aim of this study was to assess the diagnostic performance of conventional and speckle-tracking echocardiographic measures of right ventricular (RV) function before bidirectional cavopulmonary anastomosis palliation in predicting death or need for heart transplantation (HTx). Methods: RV fractional area change (RVFAC) and longitudinal and circumferential strain and strain rate (SR) were measured in 64 prospectively recruited patients with hypoplastic left heart syndrome from echocardiograms obtained before bidirectional cavopulmonary anastomosis surgery. The composite end point of death or HTx was examined. Receiver operating characteristic analysis was performed, and cutoff values optimizing sensitivity and specificity were derived. Results: At a median follow-up of 5.0 years (interquartile range, 2.8–6.4 years), 13 patients meeting the composite end point had lower longitudinal strain and SR, circumferential SR, and RVFAC compared with survivors (n = 51). The conventional cutoff of RVFAC < 35% was specific for death or HTx (86%) but had poor sensitivity (46%), with an area under the curve of 0.73. Speckle-tracking echocardiographic variables showed similar areas under the curve (range, 0.69–0.79), with negative predictive values >90%. Addition of speckle-tracking echocardiographic variables to RVFAC < 35% showed no added benefit. However, in a subpopulation of patients with RVFAC ≥ 35% (n = 44), those meeting the composite end point (n = 7) had lower longitudinal SR (median, −1.0 1/sec [interquartile range, −0.8 to −1.1 1/sec] vs −1.21/sec [interquartile range, −1.0 to −1.3 1/sec], P =.03). Interobserver reproducibility was superior for longitudinal strain and SR (intraclass correlation coefficient > 0.92) compared with RVFAC (intraclass correlation coefficient = 0.75). Conclusions: Children with hypoplastic left heart syndrome with normal RVFAC and ventricular deformation before bidirectional cavopulmonary anastomosis have a low likelihood of death or HTx in the medium term. In the presence of reduced RVFAC, speckle-tracking echocardiography does not provide additional prognostic value. However, in patients with “normal” RVFAC, it may have a role in improving outcome prediction and warrants further investigation.

AB - Background: Ventricular dysfunction is associated with increased morbidity and mortality in children with hypoplastic left heart syndrome. The aim of this study was to assess the diagnostic performance of conventional and speckle-tracking echocardiographic measures of right ventricular (RV) function before bidirectional cavopulmonary anastomosis palliation in predicting death or need for heart transplantation (HTx). Methods: RV fractional area change (RVFAC) and longitudinal and circumferential strain and strain rate (SR) were measured in 64 prospectively recruited patients with hypoplastic left heart syndrome from echocardiograms obtained before bidirectional cavopulmonary anastomosis surgery. The composite end point of death or HTx was examined. Receiver operating characteristic analysis was performed, and cutoff values optimizing sensitivity and specificity were derived. Results: At a median follow-up of 5.0 years (interquartile range, 2.8–6.4 years), 13 patients meeting the composite end point had lower longitudinal strain and SR, circumferential SR, and RVFAC compared with survivors (n = 51). The conventional cutoff of RVFAC < 35% was specific for death or HTx (86%) but had poor sensitivity (46%), with an area under the curve of 0.73. Speckle-tracking echocardiographic variables showed similar areas under the curve (range, 0.69–0.79), with negative predictive values >90%. Addition of speckle-tracking echocardiographic variables to RVFAC < 35% showed no added benefit. However, in a subpopulation of patients with RVFAC ≥ 35% (n = 44), those meeting the composite end point (n = 7) had lower longitudinal SR (median, −1.0 1/sec [interquartile range, −0.8 to −1.1 1/sec] vs −1.21/sec [interquartile range, −1.0 to −1.3 1/sec], P =.03). Interobserver reproducibility was superior for longitudinal strain and SR (intraclass correlation coefficient > 0.92) compared with RVFAC (intraclass correlation coefficient = 0.75). Conclusions: Children with hypoplastic left heart syndrome with normal RVFAC and ventricular deformation before bidirectional cavopulmonary anastomosis have a low likelihood of death or HTx in the medium term. In the presence of reduced RVFAC, speckle-tracking echocardiography does not provide additional prognostic value. However, in patients with “normal” RVFAC, it may have a role in improving outcome prediction and warrants further investigation.

KW - Hypoplastic left heart syndrome

KW - Mortality

KW - Speckle-tracking echocardiography

KW - Strain

KW - Strain rate

UR - http://www.scopus.com/inward/record.url?scp=85045249850&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=85045249850&partnerID=8YFLogxK

U2 - 10.1016/j.echo.2018.02.001

DO - 10.1016/j.echo.2018.02.001

M3 - Article

JO - Journal of the American Society of Echocardiography

JF - Journal of the American Society of Echocardiography

SN - 0894-7317

ER -