Prognostic Value of Serial Echocardiography in Hypoplastic Left Heart Syndrome

Jae Sung Son, Adam James, Chun Po Steve Fan, Luc Mertens, Brian W. McCrindle, Cedric Manlhiot, Mark K. Friedberg

Research output: Contribution to journalArticle

Abstract

BACKGROUND: Hypoplastic left heart syndrome (HLHS) carries a high mortality. The prognostic value of echocardiographic variables at presentation and the importance of serial data are poorly explored. METHODS AND RESULTS: We retrospectively analyzed longitudinal clinical and echocardiographic parameters of children with hypoplastic left heart syndrome. The primary outcome was time to death or transplant after stage-1 surgery, right censored at stage 3 surgery, accounting for sequential surgical procedures. Univariable and multivariable analysis identified risk factors for transplant or mortality. Time-dependent trends in echo parameters stratified by patient survival were explored using nonparametric methods. In total 67 children with hypoplastic left heart syndrome were included. The 10-week, 20-week, and 1-year survival proportions were 77.6% (66-86), 68.7% (56-78), and 54.1% (41-65), respectively. At presentation, multivariable analysis identified a thicker interventricular septum (hazard ratio [HR], 1.64; P=0.007) to be associated with increased risk for transplant/death, whereas higher tricuspid valve annular planar systolic excursion z score (HR, 0.61; P=0.003) was associated with reduced risk of mortality/transplant. After stage-1, higher body surface area-indexed right ventricular end-systolic area (HR, 1.52; P=0.02), lower fractional area change (HR, 1.18; P=0.01), and qualitatively decreased right ventricular function (HR, 2.55; P=0.08) were associated with increased risk of transplant-free mortality, censored at stage 3. Higher body surface area-indexed right ventricular end-diastolic area (HR, 0.76; P=0.04) and better tricuspid valve annular planar systolic excursion z score (HR, 0.49; P=0.01) were associated with lower mortality/transplant. During follow-up, right ventricular dilation, dysfunction, and tricuspid regurgitation improved in transplant-free survivors and worsened in those transplanted or who died. CONCLUSIONS: Conventional echocardiographic parameters at presentation and during follow-up are potential markers for transplant-free survival in children with hypoplastic left heart syndrome.

Original languageEnglish (US)
Pages (from-to)e006983
JournalCirculation. Cardiovascular imaging
Volume11
Issue number7
DOIs
StatePublished - Jul 1 2018

Fingerprint

Hypoplastic Left Heart Syndrome
Echocardiography
Transplants
Mortality
Tricuspid Valve
Body Surface Area
Survival
Right Ventricular Dysfunction
Right Ventricular Function
Tricuspid Valve Insufficiency
Survivors
Dilatation

Keywords

  • death
  • echocardiography
  • hypoplastic left heart syndrome
  • risk factors
  • tricuspid valve

ASJC Scopus subject areas

  • Radiology Nuclear Medicine and imaging
  • Cardiology and Cardiovascular Medicine

Cite this

Son, J. S., James, A., Fan, C. P. S., Mertens, L., McCrindle, B. W., Manlhiot, C., & Friedberg, M. K. (2018). Prognostic Value of Serial Echocardiography in Hypoplastic Left Heart Syndrome. Circulation. Cardiovascular imaging, 11(7), e006983. https://doi.org/10.1161/CIRCIMAGING.117.006983

Prognostic Value of Serial Echocardiography in Hypoplastic Left Heart Syndrome. / Son, Jae Sung; James, Adam; Fan, Chun Po Steve; Mertens, Luc; McCrindle, Brian W.; Manlhiot, Cedric; Friedberg, Mark K.

In: Circulation. Cardiovascular imaging, Vol. 11, No. 7, 01.07.2018, p. e006983.

Research output: Contribution to journalArticle

Son, Jae Sung ; James, Adam ; Fan, Chun Po Steve ; Mertens, Luc ; McCrindle, Brian W. ; Manlhiot, Cedric ; Friedberg, Mark K. / Prognostic Value of Serial Echocardiography in Hypoplastic Left Heart Syndrome. In: Circulation. Cardiovascular imaging. 2018 ; Vol. 11, No. 7. pp. e006983.
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abstract = "BACKGROUND: Hypoplastic left heart syndrome (HLHS) carries a high mortality. The prognostic value of echocardiographic variables at presentation and the importance of serial data are poorly explored. METHODS AND RESULTS: We retrospectively analyzed longitudinal clinical and echocardiographic parameters of children with hypoplastic left heart syndrome. The primary outcome was time to death or transplant after stage-1 surgery, right censored at stage 3 surgery, accounting for sequential surgical procedures. Univariable and multivariable analysis identified risk factors for transplant or mortality. Time-dependent trends in echo parameters stratified by patient survival were explored using nonparametric methods. In total 67 children with hypoplastic left heart syndrome were included. The 10-week, 20-week, and 1-year survival proportions were 77.6{\%} (66-86), 68.7{\%} (56-78), and 54.1{\%} (41-65), respectively. At presentation, multivariable analysis identified a thicker interventricular septum (hazard ratio [HR], 1.64; P=0.007) to be associated with increased risk for transplant/death, whereas higher tricuspid valve annular planar systolic excursion z score (HR, 0.61; P=0.003) was associated with reduced risk of mortality/transplant. After stage-1, higher body surface area-indexed right ventricular end-systolic area (HR, 1.52; P=0.02), lower fractional area change (HR, 1.18; P=0.01), and qualitatively decreased right ventricular function (HR, 2.55; P=0.08) were associated with increased risk of transplant-free mortality, censored at stage 3. Higher body surface area-indexed right ventricular end-diastolic area (HR, 0.76; P=0.04) and better tricuspid valve annular planar systolic excursion z score (HR, 0.49; P=0.01) were associated with lower mortality/transplant. During follow-up, right ventricular dilation, dysfunction, and tricuspid regurgitation improved in transplant-free survivors and worsened in those transplanted or who died. CONCLUSIONS: Conventional echocardiographic parameters at presentation and during follow-up are potential markers for transplant-free survival in children with hypoplastic left heart syndrome.",
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AU - Manlhiot, Cedric

AU - Friedberg, Mark K.

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N2 - BACKGROUND: Hypoplastic left heart syndrome (HLHS) carries a high mortality. The prognostic value of echocardiographic variables at presentation and the importance of serial data are poorly explored. METHODS AND RESULTS: We retrospectively analyzed longitudinal clinical and echocardiographic parameters of children with hypoplastic left heart syndrome. The primary outcome was time to death or transplant after stage-1 surgery, right censored at stage 3 surgery, accounting for sequential surgical procedures. Univariable and multivariable analysis identified risk factors for transplant or mortality. Time-dependent trends in echo parameters stratified by patient survival were explored using nonparametric methods. In total 67 children with hypoplastic left heart syndrome were included. The 10-week, 20-week, and 1-year survival proportions were 77.6% (66-86), 68.7% (56-78), and 54.1% (41-65), respectively. At presentation, multivariable analysis identified a thicker interventricular septum (hazard ratio [HR], 1.64; P=0.007) to be associated with increased risk for transplant/death, whereas higher tricuspid valve annular planar systolic excursion z score (HR, 0.61; P=0.003) was associated with reduced risk of mortality/transplant. After stage-1, higher body surface area-indexed right ventricular end-systolic area (HR, 1.52; P=0.02), lower fractional area change (HR, 1.18; P=0.01), and qualitatively decreased right ventricular function (HR, 2.55; P=0.08) were associated with increased risk of transplant-free mortality, censored at stage 3. Higher body surface area-indexed right ventricular end-diastolic area (HR, 0.76; P=0.04) and better tricuspid valve annular planar systolic excursion z score (HR, 0.49; P=0.01) were associated with lower mortality/transplant. During follow-up, right ventricular dilation, dysfunction, and tricuspid regurgitation improved in transplant-free survivors and worsened in those transplanted or who died. CONCLUSIONS: Conventional echocardiographic parameters at presentation and during follow-up are potential markers for transplant-free survival in children with hypoplastic left heart syndrome.

AB - BACKGROUND: Hypoplastic left heart syndrome (HLHS) carries a high mortality. The prognostic value of echocardiographic variables at presentation and the importance of serial data are poorly explored. METHODS AND RESULTS: We retrospectively analyzed longitudinal clinical and echocardiographic parameters of children with hypoplastic left heart syndrome. The primary outcome was time to death or transplant after stage-1 surgery, right censored at stage 3 surgery, accounting for sequential surgical procedures. Univariable and multivariable analysis identified risk factors for transplant or mortality. Time-dependent trends in echo parameters stratified by patient survival were explored using nonparametric methods. In total 67 children with hypoplastic left heart syndrome were included. The 10-week, 20-week, and 1-year survival proportions were 77.6% (66-86), 68.7% (56-78), and 54.1% (41-65), respectively. At presentation, multivariable analysis identified a thicker interventricular septum (hazard ratio [HR], 1.64; P=0.007) to be associated with increased risk for transplant/death, whereas higher tricuspid valve annular planar systolic excursion z score (HR, 0.61; P=0.003) was associated with reduced risk of mortality/transplant. After stage-1, higher body surface area-indexed right ventricular end-systolic area (HR, 1.52; P=0.02), lower fractional area change (HR, 1.18; P=0.01), and qualitatively decreased right ventricular function (HR, 2.55; P=0.08) were associated with increased risk of transplant-free mortality, censored at stage 3. Higher body surface area-indexed right ventricular end-diastolic area (HR, 0.76; P=0.04) and better tricuspid valve annular planar systolic excursion z score (HR, 0.49; P=0.01) were associated with lower mortality/transplant. During follow-up, right ventricular dilation, dysfunction, and tricuspid regurgitation improved in transplant-free survivors and worsened in those transplanted or who died. CONCLUSIONS: Conventional echocardiographic parameters at presentation and during follow-up are potential markers for transplant-free survival in children with hypoplastic left heart syndrome.

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