Universität zu Köln

Taylan, Christina ORCID: 0000-0001-9585-054X, Driemeyer, Joenna, Schmitt, Claus P., Pape, Lars, Buescher, Rainer, Galiano, Matthias, Koenig, Jens, Schuerfeld, Carsten, Spitthoever, Ralf, Versen, Axel, Koziolek, Michael, Marsen, Tobias A., Stein, Holger, Schaefer, Juergen R., Heibges, Andreas, Klingel, Reinhard, Oh, Jun, Weber, Lutz T. and Klaus, Gunter (2020). Cardiovascular Outcome of Pediatric Patients With Bi- Allelic (Homozygous) Familial Hypercholesterolemia Before and After Initiation of Multimodal Lipid Lowering Therapy Including Lipoprotein Apheresis. Am. J. Cardiol., 136. S. 38 - 49. BRIDGEWATER: EXCERPTA MEDICA INC-ELSEVIER SCIENCE INC. ISSN 1879-1913

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Abstract

Twenty-four patients with bi-allelic familial hypercholesterolemia commencing chronic lipoprotein apheresis (LA) at a mean age of 8.5 +/- 3.1 years were analysed retrospectively and in part prospectively with a mean follow-up of 17.2 +/- 5.6 years. Mean age at diagnosis was 6.3 +/- 3.4 years. Untreated mean LDL-C concentrations were 752 mg/di +/- 193 mg/di (19.5 mmol/l +/- 5.0 mmol/l). Multimodal lipid lowering therapy including LA resulted in a mean LDL-C concentration of 184 mg/di (4.8 mmol/l), which represents a 75.5% mean reduction. Proprotein convertase subtilisin/kexin type 9-antibodies contributed in 3 patients to LDL-C lowering with 5 patients remaining to be tested. After commencing chronic LA, 16 patients (67%) remained clinically stable with only subclinical findings of atherosclerotic cardiovascular disease (ASCVD), and neither cardiovascular events, nor need for vascular interventions or surgery. In 19 patients (79%), pathologic findings were detected at the aortic valve (AV), which in the majority were mild. AV replacement was required in 2 patients. Mean Lipoprotein(a) concentration was 42.4 mg/dl, 38% had >50 mg/dl. There was no overt correlation of AV pathologies with other ASCVD complications, or Lipoprotein(a) concentration. Physicochemical elimination of LDL particles by LA appears indispensable for patients with bi-allelic familial hypercholesterolemia and severe hypercholesterolemia to maximize the reduction of LDL-C. In conclusion, in this rare patient group regular assessment of both the AV, as well as all arteries accessible by ultrasound should be performed to adjust the intensity of multimodal lipid lowering therapy with the goal to prevent ASCVD events and aortic surgery. (C) 2020 Elsevier Inc. All rights reserved.

Item Type:	Journal Article
Creators:	Creators Email ORCID ORCID Put Code Taylan, Christina UNSPECIFIED orcid.org/0000-0001-9585-054X UNSPECIFIED Driemeyer, Joenna UNSPECIFIED UNSPECIFIED UNSPECIFIED Schmitt, Claus P. UNSPECIFIED UNSPECIFIED UNSPECIFIED Pape, Lars UNSPECIFIED UNSPECIFIED UNSPECIFIED Buescher, Rainer UNSPECIFIED UNSPECIFIED UNSPECIFIED Galiano, Matthias UNSPECIFIED UNSPECIFIED UNSPECIFIED Koenig, Jens UNSPECIFIED UNSPECIFIED UNSPECIFIED Schuerfeld, Carsten UNSPECIFIED UNSPECIFIED UNSPECIFIED Spitthoever, Ralf UNSPECIFIED UNSPECIFIED UNSPECIFIED Versen, Axel UNSPECIFIED UNSPECIFIED UNSPECIFIED Koziolek, Michael UNSPECIFIED UNSPECIFIED UNSPECIFIED Marsen, Tobias A. UNSPECIFIED UNSPECIFIED UNSPECIFIED Stein, Holger UNSPECIFIED UNSPECIFIED UNSPECIFIED Schaefer, Juergen R. UNSPECIFIED UNSPECIFIED UNSPECIFIED Heibges, Andreas UNSPECIFIED UNSPECIFIED UNSPECIFIED Klingel, Reinhard UNSPECIFIED UNSPECIFIED UNSPECIFIED Oh, Jun UNSPECIFIED UNSPECIFIED UNSPECIFIED Weber, Lutz T. UNSPECIFIED UNSPECIFIED UNSPECIFIED Klaus, Gunter UNSPECIFIED UNSPECIFIED UNSPECIFIED
URN:	urn:nbn:de:hbz:38-309313
DOI:	10.1016/j.amjcard.2020.09.015
Journal or Publication Title:	Am. J. Cardiol.
Volume:	136
Page Range:	S. 38 - 49
Date:	2020
Publisher:	EXCERPTA MEDICA INC-ELSEVIER SCIENCE INC
Place of Publication:	BRIDGEWATER
ISSN:	1879-1913
Language:	English
Faculty:	Unspecified
Divisions:	Unspecified
Subjects:	no entry
Uncontrolled Keywords:	Keywords Language LDL-APHERESIS; EFFICACY; SAFETY; TOLERABILITY; MANAGEMENT; STATEMENT; CHILDREN; SOCIETY; DISEASE Multiple languages Cardiac & Cardiovascular Systems Multiple languages
URI:	http://kups.ub.uni-koeln.de/id/eprint/30931