Ministry of Health of the Republic of Latvia
Ministry of Health of the Republic of Lithuania
Ministry of Social Affairs of Eston ja
February 28,Z}LB
call to action on improvement of management of Fanrilial
Hypercholesterolemia in the Baltic States
We are writing on behalf of professionalcardiology associations of the three
Baltic States: Latvian Society of Cardiology, Lithuanian Society of Cardiology and
Estonian Society of Cardiology. Our aim is to irnprove the treatment of patients
diagnosed with familial hypercholesterolemia in Latvia, Estonia ancl Lithuania.
Familial Hypercholesterolemia (FH) is a very severe inherited disease
manifesting with life-long exposure to extreme levels of cholesterol, which leads to
early development of atherosclerosis, myocardial infarction and other fatal and
non-fatal cardiovascular events, The risk of such events in general is increased
up to 2Z-fold. t1) In young individuals aged 20-29 years relative risk of
cardiovascular events is as high as L25 and 48 in women and men, respectlvely.
[2) Average age of the onset of myo'cardial in^farction or other cardiovascular
manifestations is 40 years in men and 50 years in women.
[3) In our practice we
have encountered with many Ft{ patients with advanced atherosclernti. dir.or.
in age group 25 to 40 years (l), The presence of FH even in mildest forms
decreases life expectancy by at least L0-20 years in men and 20-30 years in women.
(4) Thus mostly economically and socially active your-rg people are affected.
Estimated average prevalence of FH is approximately 1 in 250 indivicluals
worldwide, which makes FH as the most common inherited disease leading to
premature death in adult populations. Importantly, these deaths and no-fitat
events can be very effectively prevented with lipicl-lowering agents,
Regrettably, although there are effective interventions available to diagnose and
manage FH and prevent life-threatening cardiovascular complications, FH
remains under-diagnosed and undertreated in the Baltic States where only
minority of these patients is diagnosed and even ,less proportion managed
appropriately. Latvian Registry of the Familial Hypercholesterolemia established
in 2015 have identified 1B0 FH patients during three years, which constitures
around 2.20/o of estimated all FH patients in LaWia.
Considering all above-mentioned we believe it is crr.rcially important step in
improvement of care of FH patients in all three Baltic States to implement a new
disease code according to Internotional Classification of Diseases, namely,
878,01 "Fomilial Hypercholesterolemia". Introduction of such code will
distinguish this particularly high-risk group of patients requiring more specific
care compared [o patients with other forms of fnon-familial)
hypercholesterolemia coded as E78.0. Code E78.0 is not a specific code for FH
and it includes all patients with elevated cholesterol levels, among which va.st
majority are non-familial, The new code E78,01 would allow to senarate F'H
patients from other forms of hypercholesterolemia and would easier allow to
cost-effectively manage these very high risk individuals. It would be reasonable
that E78,01 code is confirmed at specialized centers experienced in diagnosis of
FH.
The code 78,01, has .already been successfully used in the United States of
America since october 1, 2017 in clinical Modification (cM) of ICD-10 [tcD-
10cM). [http://www.icdlodata.com/ICD10cM/codes/Eoo-EBg/E7}-EBB/E7B-
/E78.oI). Moreover, diagnosis of Familial Hypercholesterolemia is included in
the ICD-11 beta version by World Health Organization.
Considering very high cardiovascular morbidity and mortality in the Baltic States
and limited financial resources, we strongly believe that timely introduction of
E78.01 is critically needed to evaluate costs and define patient groups in need for
more specific management of FH.
Sincerely yrlurs,
Prof. Andrejs Erglis, MD, [r president ol Society of Cardiology
rnember of the l,atvian Society of
past president of the Estonian Society of
rdiology
Prof. Rimvydas Slapikas, MD, FESC, president of the thuanian Society of
Cardiology ,, , ,.., n,
t ,{
References
L. Khera AV, Won flH, Peloso GM, l,awson KS, Bartz TM, Deng X, et al. Diagnostic Yield of Sequencirrg
Fantilial Hypercholesterolemia Cenes in Patients with Severc Hypercholesterolemia, Journal of the
Anrerican College of Cardiology, 2016,
2. Croup, SSCobotSBR. Risl< of fatal coronary heart disease in familial hypercholcsterolaemia. BMJ (Clinical
research ed). 1991;303 (6807):893-6.
3. Goldstein Jl,, Schrott HG, Hazzard WR, Biernran Et,, Motulsky AG. tlyperlipidernia in coronary heart
disease. Il, Genetic analysis of lipid levels in 175 fanilies and delineation of a new inherited disorder,
conrbincd hyperlipidemia. Tbe Journal of clinical investigation. I973;52(7):7544-68.
4. Perak AM, Ning H, de Ferranti SD, Gooding HC, Wilkins JT, Lloyd-Jones DM. Long-Term Risk of
Atherosclerotic Cardiovascular Disease in US Adulls With the FamiliaL Hypercholesberolernia
PhenotypeClinical Perspective. Circulation. 2016;134(7):9.L9.
Tere,
Eesti Kardioloogide Seltsi nimel saadan Balti riikide kardioloogide seltside ühise kirja.
Selles palume võimalust perekondliku hüperkolesteroleemia kui üliraske haiguse
diagnoosi kodeerimise võimalust E 78.01 analoogiliselt Ameerika Ühendriikides rakendatule.
Praegune kodeerimise süsteem (RHK-10) ei erista seda haigust.
Lugupidamisega,
Margus Viigimaa
Eesti Kardioloogide Seltsi juhatuse liige,
preventsiooni töögrupi juht
Margus Viigimaa
Eesti Kardioloogide Selts
[email protected] Meie 17.04.2018 nr 5.2-2/958-2
Perekondliku hüperkolesteroleemia
diagnoosikoodi lisamine RHK-10 hulka
Lugupeetud Margus Viigimaa
Täname teid kirja eest, milles tegite ettepaneku täiendada rahvusvahelise haiguste ja
tervisega seotud probleemide statistilise klassifikatsiooni kümnendas väljaandes (RHK-10)
toodud diagnooside loetelu. Täpsemalt soovisite lisada RHK-10-sse perekondliku
hüperkolesteroleemia diagnoosi (E78.01) kodeerimise võimaluse nagu seda on Ameerika
Ühendriikides rakendatud. Leiame, et teie ettepanek on põhjendatud ja mõistetav.
Edastame teie ettepaneku Tervise ja Heaolu Infosüsteemide Keskusele ja Tervise Arengu
Instituudile, kes koondavad RHK-10 täiendus- ja muudatusettepanekuid ning haldavad
klassifikaatoreid tehniliselt ja sisuliselt.
Lugupidamisega
(allkirjastatud digitaalselt)
Maris Jesse
terviseala asekantsler
Lisad:
E-kiri.pdf
Letter about E78.01.pdf
Lisaadressaadid:
Tervise ja Heaolu Infosüsteemide Keskus
Tervise Arengu Instituut
Eleri Lapp 626 9137
[email protected]
Suur-Ameerika 1 / 10122 Tallinn / 626 9301 /
[email protected] / www.sm.ee / registrikood 70001952