Latest data from the HICC, EAS-led HoFH Registry

Among the abstracts presented at EAS Helsinki 2021 was important news from the EAS-led Homozygous familial hypercholesterolaemia International Clinical Collaboration (HICC) registry, the largest global database of homozygous familial hypercholesterolaemia (HoFH) patients (1). HoFH is the most severe form of FH, thought to affect about one in 300,000 to one in a million people, and is characterised by extremely high levels of low-density lipoprotein cholesterol (LDL-C) from birth and severe atherosclerotic cardiovascular disease (2). Many patients may have their first heart attack in childhood (2).

The HICC was launched in 2016 aiming to provide data about the prevalence, clinical consequences and treatment of HoFH, as well as promote actions to improve care. Patients are included in the registry based on clinical or genetic criteria for HoFH.

This latest report provides data on 765 patients from 38 countries world-wide, the majority (75%) diagnosed by genetic criteria. Categorising the data by high-income countries (n=410) versus non-high-income countries (n=355) revealed disparities in FH care.

While patients from non-high-income countries were diagnosed earlier than those in high-income countries (median 10 versus 16 years), access to novel treatments which are now part of the armamentarium of managing HoFH was much more limited. Among non-high-income countries, significantly fewer patients received PCSK9 inhibitors (17% versus 25% in high-income countries, p<0.001) and lomitapide (2% versus 13%, p<0.001), and no patients (versus 4.3%) received evinacumab. Consequently on-treatment LDL-C levels were much higher for patients in lower-income countries, about double those in high-income regions (mean 9.6 versus 4.7 mmol/L, p<0.001). Overall, while only a minority of patients attained guideline-recommended LDL-C goal, almost none were in non-high-income countries (2.6% versus 21.4% in high-income countries, p<0.001). 

Importantly, limited access to novel therapies and higher on-treatment LDL-C levels significantly impacted outcome. The cardiovascular burden of HoFH was greater among non-high-income countries, with earlier onset of major coronary events (median 24 versus 35 years) and younger age of cardiovascular death (median 24 versus 40 years). Thus, inequity in treatment almost halved the age of death due to cardiovascular causes among patients in non-high-income regions.

In conclusion, patients in high-income countries had access to novel lipid-lowering therapies more frequently, attained lower LDL-C levels and had longer event-free survival. These new findings from the HICC reinforce the value of this global registry for highlighting inequities in resource allocation for FH care, in particular the limited availability of contemporary and novel therapies. Going forward, this information will be critical in discussions with all stakeholders in FH care.

According to Professor Derick Raal, co-coordinator of the HICC Registry (University of the Witwatersrand, Johannesburg, South Africa): ‘These new findings from the HICC Registry underline the need for action to ensure equity in care for this rare, severe condition, crucial to improve prognosis and freedom from early heart attack, often during childhood.’

Professor Raal discusses the HICC findings in this video.

Find more at the meeting 


  1. Tromp TR, Hartgers ML, Hovingh GK, et al. Worldwide perspective on homozygous familial hypercholesterolemia diagnosis, treatment and outcome – Results from the HICC Registry. Poster session. Presented at EAS Helsinki 2021 Virtual – 30th May -2 June 2021.
  2. Cuchel M, Bruckert E, Ginsberg HN, et al. Homozygous familial hypercholesterolaemia: new insights and guidance for clinicians to improve detection and clinical management. A position paper from the Consensus Panel on Familial Hypercholesterolaemia of the European Atherosclerosis Society. Eur Heart J 2014;35:2146-57.

For more information about the HICC: HoFH International Clinical Collaboration – HICC.

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