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Pediatric heterozygous familial hypercholesterolemia patients have locally increased aortic pulse wave velocity and wall thickness at the aortic root

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Pediatric heterozygous familial hypercholesterolemia patients have locally increased aortic pulse wave velocity and wall thickness at the aortic root. / Tran, Andrew; Burkhardt, Barbara; Tandon, Animesh; Blumenschein, Sarah; van Engelen, Arna; Cecelja, Marina; Zhang, Song; Uribe, Sergio; Mura, Joaquin; Greil, Gerald; Hussain, Tarique.

In: INTERNATIONAL JOURNAL OF CARDIOVASCULAR IMAGING, Vol. 35, No. 10, 01.10.2019, p. 1903-1911.

Research output: Contribution to journalArticle

Harvard

Tran, A, Burkhardt, B, Tandon, A, Blumenschein, S, van Engelen, A, Cecelja, M, Zhang, S, Uribe, S, Mura, J, Greil, G & Hussain, T 2019, 'Pediatric heterozygous familial hypercholesterolemia patients have locally increased aortic pulse wave velocity and wall thickness at the aortic root', INTERNATIONAL JOURNAL OF CARDIOVASCULAR IMAGING, vol. 35, no. 10, pp. 1903-1911. https://doi.org/10.1007/s10554-019-01626-5

APA

Tran, A., Burkhardt, B., Tandon, A., Blumenschein, S., van Engelen, A., Cecelja, M., Zhang, S., Uribe, S., Mura, J., Greil, G., & Hussain, T. (2019). Pediatric heterozygous familial hypercholesterolemia patients have locally increased aortic pulse wave velocity and wall thickness at the aortic root. INTERNATIONAL JOURNAL OF CARDIOVASCULAR IMAGING, 35(10), 1903-1911. https://doi.org/10.1007/s10554-019-01626-5

Vancouver

Tran A, Burkhardt B, Tandon A, Blumenschein S, van Engelen A, Cecelja M et al. Pediatric heterozygous familial hypercholesterolemia patients have locally increased aortic pulse wave velocity and wall thickness at the aortic root. INTERNATIONAL JOURNAL OF CARDIOVASCULAR IMAGING. 2019 Oct 1;35(10):1903-1911. https://doi.org/10.1007/s10554-019-01626-5

Author

Tran, Andrew ; Burkhardt, Barbara ; Tandon, Animesh ; Blumenschein, Sarah ; van Engelen, Arna ; Cecelja, Marina ; Zhang, Song ; Uribe, Sergio ; Mura, Joaquin ; Greil, Gerald ; Hussain, Tarique. / Pediatric heterozygous familial hypercholesterolemia patients have locally increased aortic pulse wave velocity and wall thickness at the aortic root. In: INTERNATIONAL JOURNAL OF CARDIOVASCULAR IMAGING. 2019 ; Vol. 35, No. 10. pp. 1903-1911.

Bibtex Download

@article{c83078de96b04351bc6d1d7766813fdf,
title = "Pediatric heterozygous familial hypercholesterolemia patients have locally increased aortic pulse wave velocity and wall thickness at the aortic root",
abstract = "Familial hypercholesterolemia (FH) is an autosomal dominant disorder that affects 1 in 250 people. Aortic stiffness, measured by pulse wave velocity (PWV), is an independent predictor for cardiovascular events. Young FH patients are a unique group with early vessel wall disease that may serve to elucidate the determinants of aortic stiffness. We hypothesized that young FH patients would have early changes in aortic stiffness compared to healthy, age- and sex-matched reference values. Thirty-three FH patients (≥ 7 years age; mean age 14.6 ± 3.3 years; 26/33 on statin therapy) underwent cardiac MRI. PWV was determined using propagation of flow waveform from aortic arch phase contrast images. Distensibility and aortic wall thickness (AWT) were measured at the ascending, proximal descending, and diaphragmatic aorta. Ventricular volumes and left ventricular (LV) myocardial mass were measured from 2D cine images. These parameters were compared to age- and sex-matched reference values. FH patients had significantly higher PWV (4.5 ± 0.8 vs. 3.5 ± 0.3 m/s; p < 0.001), aortic distensibility, and ascending aortic wall thickness (1.37 ± 0.18 vs. 1.30 ± 0.02 mm; p < 0.05) compared to reference. There was no difference in aortic area or descending aortic wall thickness between groups. Young FH patients had aortic changes with increased aortic pulse wave velocity in the setting of increased aortic distensibility, accompanied by increased thickness of the ascending aortic wall. Presence of these early findings in young patients despite the majority being on statin therapy support enhanced screening and aggressive treatment of familial hypercholesterolemia to prevent potential future cardiovascular events.",
keywords = "Atherosclerosis, Familial hypercholesterolemia, Magnetic resonance imaging (MRI), Pediatrics, Prevention",
author = "Andrew Tran and Barbara Burkhardt and Animesh Tandon and Sarah Blumenschein and {van Engelen}, Arna and Marina Cecelja and Song Zhang and Sergio Uribe and Joaquin Mura and Gerald Greil and Tarique Hussain",
year = "2019",
month = oct,
day = "1",
doi = "10.1007/s10554-019-01626-5",
language = "English",
volume = "35",
pages = "1903--1911",
journal = "INTERNATIONAL JOURNAL OF CARDIOVASCULAR IMAGING",
issn = "1569-5794",
publisher = "Springer Netherlands",
number = "10",

}

RIS (suitable for import to EndNote) Download

TY - JOUR

T1 - Pediatric heterozygous familial hypercholesterolemia patients have locally increased aortic pulse wave velocity and wall thickness at the aortic root

AU - Tran, Andrew

AU - Burkhardt, Barbara

AU - Tandon, Animesh

AU - Blumenschein, Sarah

AU - van Engelen, Arna

AU - Cecelja, Marina

AU - Zhang, Song

AU - Uribe, Sergio

AU - Mura, Joaquin

AU - Greil, Gerald

AU - Hussain, Tarique

PY - 2019/10/1

Y1 - 2019/10/1

N2 - Familial hypercholesterolemia (FH) is an autosomal dominant disorder that affects 1 in 250 people. Aortic stiffness, measured by pulse wave velocity (PWV), is an independent predictor for cardiovascular events. Young FH patients are a unique group with early vessel wall disease that may serve to elucidate the determinants of aortic stiffness. We hypothesized that young FH patients would have early changes in aortic stiffness compared to healthy, age- and sex-matched reference values. Thirty-three FH patients (≥ 7 years age; mean age 14.6 ± 3.3 years; 26/33 on statin therapy) underwent cardiac MRI. PWV was determined using propagation of flow waveform from aortic arch phase contrast images. Distensibility and aortic wall thickness (AWT) were measured at the ascending, proximal descending, and diaphragmatic aorta. Ventricular volumes and left ventricular (LV) myocardial mass were measured from 2D cine images. These parameters were compared to age- and sex-matched reference values. FH patients had significantly higher PWV (4.5 ± 0.8 vs. 3.5 ± 0.3 m/s; p < 0.001), aortic distensibility, and ascending aortic wall thickness (1.37 ± 0.18 vs. 1.30 ± 0.02 mm; p < 0.05) compared to reference. There was no difference in aortic area or descending aortic wall thickness between groups. Young FH patients had aortic changes with increased aortic pulse wave velocity in the setting of increased aortic distensibility, accompanied by increased thickness of the ascending aortic wall. Presence of these early findings in young patients despite the majority being on statin therapy support enhanced screening and aggressive treatment of familial hypercholesterolemia to prevent potential future cardiovascular events.

AB - Familial hypercholesterolemia (FH) is an autosomal dominant disorder that affects 1 in 250 people. Aortic stiffness, measured by pulse wave velocity (PWV), is an independent predictor for cardiovascular events. Young FH patients are a unique group with early vessel wall disease that may serve to elucidate the determinants of aortic stiffness. We hypothesized that young FH patients would have early changes in aortic stiffness compared to healthy, age- and sex-matched reference values. Thirty-three FH patients (≥ 7 years age; mean age 14.6 ± 3.3 years; 26/33 on statin therapy) underwent cardiac MRI. PWV was determined using propagation of flow waveform from aortic arch phase contrast images. Distensibility and aortic wall thickness (AWT) were measured at the ascending, proximal descending, and diaphragmatic aorta. Ventricular volumes and left ventricular (LV) myocardial mass were measured from 2D cine images. These parameters were compared to age- and sex-matched reference values. FH patients had significantly higher PWV (4.5 ± 0.8 vs. 3.5 ± 0.3 m/s; p < 0.001), aortic distensibility, and ascending aortic wall thickness (1.37 ± 0.18 vs. 1.30 ± 0.02 mm; p < 0.05) compared to reference. There was no difference in aortic area or descending aortic wall thickness between groups. Young FH patients had aortic changes with increased aortic pulse wave velocity in the setting of increased aortic distensibility, accompanied by increased thickness of the ascending aortic wall. Presence of these early findings in young patients despite the majority being on statin therapy support enhanced screening and aggressive treatment of familial hypercholesterolemia to prevent potential future cardiovascular events.

KW - Atherosclerosis

KW - Familial hypercholesterolemia

KW - Magnetic resonance imaging (MRI)

KW - Pediatrics

KW - Prevention

UR - http://www.scopus.com/inward/record.url?scp=85068004604&partnerID=8YFLogxK

U2 - 10.1007/s10554-019-01626-5

DO - 10.1007/s10554-019-01626-5

M3 - Article

AN - SCOPUS:85068004604

VL - 35

SP - 1903

EP - 1911

JO - INTERNATIONAL JOURNAL OF CARDIOVASCULAR IMAGING

JF - INTERNATIONAL JOURNAL OF CARDIOVASCULAR IMAGING

SN - 1569-5794

IS - 10

ER -

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