Comparative aspects of the care of familial hypercholesterolemia in the “Ten Countries Study”

• Published in: Journal of Clinical Lipidology
• Main Author: Pang J.; Chan D.C.; Hu M.; Muir L.A.; Kwok S.; Charng M.-J.; Florkowski C.M.; George P.M.; Lin J.; Loi D.D.; Marais A.D.; Nawawi H.M.; Gonzalez-Santos L.E.; Su T.-C.; Truong T.H.; Santos R.D.; Soran H.; Tomlinson B.; Yamashita S.; Ademi Z.; Watts G.F.
• Format: Article
• Language: English
• Published: Elsevier Ltd 2019
• Online Access: https://www.scopus.com/inward/record.uri?eid=2-s2.0-85061756946&doi=10.1016%2fj.jacl.2019.01.009&partnerID=40&md5=4a2d5c26ce0a94e653a11e176dee4ca1

Background: There is a lack of information on the health care of familial hypercholesterolemia (FH). Objective: The objective of this study was to compare the health care of FH in countries of the Asia-Pacific region and Southern Hemisphere. Methods: A series of questionnaires were completed by key opinion leaders from selected specialist centers in 12 countries concerning aspects of the care of FH, including screening, diagnosis, risk assessment, treatment, teaching/training, and research; the United Kingdom (UK)was used as the international benchmark. Results: The estimated percentage of patients diagnosed with the condition was low (overall <3%)in all countries, compared with ∼15% in the UK. Underdetection of FH was associated with government expenditure on health care (ϰ = 0.667, P <.05). Opportunistic and systematic screening methods, and the Dutch Lipid Clinic Network criteria were most commonly used to detect FH; genetic testing was infrequently used. Noninvasive imaging of coronary calcium and/or carotid plaques was underutilized in risk assessment. Patients with FH were generally not adequately treated, with <30% of patients achieving guideline recommended low-density lipoprotein cholesterol targets on conventional therapies. Treatment gaps included suboptimal availability and use of lipoprotein apheresis and proprotein convertase subtilsin-kexin type 9 inhibitors. A deficit of FH registries, training programs, and publications were identified in less economically developed countries. The demonstration of cost-effectiveness for cascade screening, genetic testing, and specialized treatments were significantly associated with the availability of subsidies from the health care system (ϰ = 0.571–0.800, P <.05). Conclusion: We identified important gaps across the continuum of care for FH, particularly in less economically developed countries. Wider implementation of primary and pediatric care, telehealth services, patient support groups, education/training programs, research activities, and health technology assessments are needed to improve the care of patients with FH in these countries. © 2019 National Lipid Association