Longitudinal low density lipoprotein cholesterol goal achievement and cardiovascular outcomes among adult patients with familial hypercholesterolemia: The CASCADE FH registry

Duell, P. Barton; Gidding, Samuel S.; Andersen, Rolf; Knickelbine, Thomas; Anderson, Lars; Gianos, Eugenia; Shrader, Peter; Kindt, Iris; O’Brien, Emily C.; McCann, Dervilla; Hemphill, Linda; Ahmed, Catherine D.; Martin, Seth S.; Larry, John; Ahmad, Zahid; Kullo, Iftikhar J.; Underberg, James; Guyton, John R.; Thompson, Paul D.; Wilemon, Katherine; Roe, Matthew T.; Rader, Daniel J.; Cuchel, Marina; Linton, MacRae F.; Shapiro, Michael D.; Moriarty, Patrick M.; Knowles, Joshua

doi:10.1016/j.atherosclerosis.2019.08.007

Cited by 67 publications

(34 citation statements)

References 26 publications

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“…In most countries, less than 1% of FH patients are diagnosed (Nordestgaard et al, 2013). Furthermore, treatment for FH is still limited; for example, the CASCADE-FH Registry reported that only 48% of FH patients achieved LDL-C < 100 mg/dl and 22% achieved LDL-C < 70 mg/dl (Duell et al, 2019).…”

Section: Introductionmentioning

confidence: 99%

Genetics, Screening, and Treatment of Familial Hypercholesterolemia: Experience Gained From the Implementation of the Vietnam Familial Hypercholesterolemia Registry

Truong

Kim

et al. 2020

Front. Genet.

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Section: Introductionmentioning

confidence: 99%

Genetics, Screening, and Treatment of Familial Hypercholesterolemia: Experience Gained From the Implementation of the Vietnam Familial Hypercholesterolemia Registry

Truong

Kim

et al. 2020

Front. Genet.

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“…In the present study, more than 80% of the FH patients with established ASCVD at enrollment experienced a new event over a 10-year period, despite being treated to mean (95%CI) LDL-C 3.0 (2.9, 3.2) mmol/L, which is a 55% reduction compared to the untreated LDL-C of 6.6 (5.3, 7.9) mmol/L. As observed in other trials on treatment LDL-C is much too high in FH, Duell et al reported 2.9 mmol/L using PCSK9 inhibitors in 30% of the patients (Duell et al, 2019), Langslet et al reported 3.2 mmol/ L in 909 FH patients of whom 47% had ASCVD (Langslet et al, 2020) and in another recent trial on FH patients LDL-C was 4.0 mmol/L on conventional treatment (Raal et al, 2020). Taken together, this suggests that a mean LDL-C around 3.0 mmol/L is about as low as it is possible to achieve in real-world practice in patients with FH.…”

Section: Discussionmentioning

confidence: 70%

“…Effect of statins in FH was also investigated in a Dutch study reporting that statins decreased risk of CHD by 76% vs. those not treated (Versmissen et al, 2008). Further, several studies on real world data have shown that few FH subjects achieve their LDL cholesterol (LDL-C) treatment goals (Bogsrud et al, 2019;Duell et al, 2019;Iyen et al, 2019;Perez de Isla et al, 2019). In the most recent of these studies mean LDL-C was decreased to 2.90 mmol/L in specialized lipid clinics (Duell et al, 2019).…”

Section: Introductionmentioning

confidence: 99%

“…Further, several studies on real world data have shown that few FH subjects achieve their LDL cholesterol (LDL-C) treatment goals (Bogsrud et al, 2019;Duell et al, 2019;Iyen et al, 2019;Perez de Isla et al, 2019). In the most recent of these studies mean LDL-C was decreased to 2.90 mmol/L in specialized lipid clinics (Duell et al, 2019). This included, however, treatment with PCSK9 inhibitors in 30% of the patients.…”

Section: Introductionmentioning

confidence: 99%

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Risk of Recurrent Coronary Events in Patients With Familial Hypercholesterolemia; A 10-Years Prospective Study

et al. 2021

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Background and Aim: Real world evidence on long term treatment of patients with familial hypercholesterolemia (FH) is important. We studied the effects of intensive lipid lowering medication (LLM) and optimized lifestyle in the study TTTFH–Treat To Target FH.Materials and Methods: Adults with a first known total cholesterol of mean (95% CI) 9.8 mmol/L (9.5, 10.1) were included consecutively in their routine consultation during 2006. Of the patients 86.4% had a pathogenic FH-mutation and the remaining were clinically diagnosed. We included 357 patients and 279 met for follow-up after median 10.0 (min 8.1, max 12.8) years.Results: Mean (95% CI) low density lipoprotein (LDL-C) was reduced from 3.9 (3.8, 4.1) to 3.0 (2.9, 3.2). More men than women used high intensity statin treatment, 85.2 and 60.8%, respectively. Women (n = 129) had higher LDL-C; 3.3 mmol/L (3.0, 3.5), than men; (n = 144) 2.8 mmol/L (2.6, 3.0), p = 0.004. Add-on PCSK9 inhibitors (n = 25) reduced mean LDL-C to 2.0 (1.4, 2.6) mmol/L. At enrollment 57 patients (20.4%) had established atherosclerotic cardiovascular disease (ASCVD), and 46 (80.4%) of them experienced a new event during the study period. Similarly, 222 (79.6%) patients had no detectable ASCVD at enrollment, and 29 of them (13.1%) experienced a first-time event during the study period.Conclusion: A mean LDL-C of 3.0 mmol/L was achievable in FH, treated intensively at a specialized clinic with few users of PCSK9 inhibitors. LDL-C was higher (0.5 mmol/L) in women than in men. In patients with ASCVD at enrollment, most (80.7%) experienced a new ASCVD event in the study period. The FH patients in primary prevention had more moderate CV risk, 13% in ten years.

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“…In the future, adoption of quality measures that includes either appropriate percent lowering of LDL‐cholesterol, such as 50% lowering, and/or specific targets, such as LDL‐c < 70 mg/dl, should be required for those with ASCVD or for higher risk primary prevention. Those at high‐risk need to engage in a risk discussion with their clinician regarding the potential for benefit versus risk for additional cholesterol lowering therapy, such as ezetimibe or PCSK9 inhibitors 24‐28 . Moreover, risk discussions should remind physicians to increase statin intensity as necessary for patients with a history of SH.…”

Section: Discussionmentioning

confidence: 99%

Cardiology clinic visit increases likelihood of evidence‐based cholesterol prescribing in severe hypercholesterolemia

Groth

Stone

Benziger

2020

Clinical Cardiology

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Background Patients with phenotypic severe hypercholesterolemia (SH), low‐density lipoprotein‐cholesterol (LDL‐c) ≥ 190 mg/dl, atherosclerotic cardiovascular disease (ASCVD) or adults 40–75 years with diabetes with risk factors or 10‐year ASCVD risk ≥20% benefit from maximally tolerated statin therapy. Rural patients have decreased access to specialty care, potentially limiting appropriate treatment. Hypothesis Prior visit with cardiology will improve treatment of severe hypercholesterolemia. Methods We used an electronic medical record‐based SH registry defined as ever having an LDL‐c ≥ 190 mg/dl since January 1, 2000 (n = 18 072). We excluded 3205 (17.7%) patients not alive or age 20–75 years. Patients defined as not seen by cardiology if they had no visit within the past 3 years (2017–2019). Results We included 14 867 patients (82.3%; mean age 59.7 ± 10.3 years; 58.7% female). Most patients were not seen by cardiology (n = 13 072; 72.3%). After adjusting for age, sex, CVD, hypertension, diabetes and obesity, patients seen by cardiology were more likely to have any lipid‐lowering medication (OR = 1.46, 95% CI: 1.29–1.65), high‐intensity statin (OR = 1.81, 95% CI: 1.61–2.03), or proprotein convertase subtilisin‐kexin type 9 (PCSK9) inhibitor (OR = 5.96, 95% CI: 3.34–10.65) compared to those not seen by cardiology. Mean recent LDL‐c was lower in patients seen by cardiology (126.8 ± 51.6 mg/dl vs. 152.4 ± 50.2 mg/dl, respectively; p < .001). Conclusion In our predominantly rural population, a visit with cardiology improved the likelihood to be prescribed any statin, a high‐intensity statin, or PCSK9 inhibitor. This more appropriately addressed their high life‐time risk of ASCVD. Access to specialty care could improve SH patient's outcomes.

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Longitudinal low density lipoprotein cholesterol goal achievement and cardiovascular outcomes among adult patients with familial hypercholesterolemia: The CASCADE FH registry

Cited by 67 publications

References 26 publications

Genetics, Screening, and Treatment of Familial Hypercholesterolemia: Experience Gained From the Implementation of the Vietnam Familial Hypercholesterolemia Registry

Genetics, Screening, and Treatment of Familial Hypercholesterolemia: Experience Gained From the Implementation of the Vietnam Familial Hypercholesterolemia Registry

Risk of Recurrent Coronary Events in Patients With Familial Hypercholesterolemia; A 10-Years Prospective Study

Cardiology clinic visit increases likelihood of evidence‐based cholesterol prescribing in severe hypercholesterolemia

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