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Circ Cardiovasc Imaging. 2018 Jul;11(7):e006983. doi: 10.1161/CIRCIMAGING.117.006983.

Prognostic Value of Serial Echocardiography in Hypoplastic Left Heart Syndrome.

Author information

1
Division of Pediatric Cardiology, Konkuk University Medical Center, Konkuk University School of Medicine, Seoul, Korea (J.S.S.).
2
Division of Cardiology, Labatt Family Heart Centre, Hospital for Sick Children and University of Toronto, ON, Canada (A.J., C.-P.S.F., L.M., B.W.M., C.M., M.K.F.).
3
Division of Cardiology, Labatt Family Heart Centre, Hospital for Sick Children and University of Toronto, ON, Canada (A.J., C.-P.S.F., L.M., B.W.M., C.M., M.K.F.). mark.friedberg@sickkids.ca.

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.

KEYWORDS:

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

PMID:
30012823
DOI:
10.1161/CIRCIMAGING.117.006983
[Indexed for MEDLINE]

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