National & International Guidelines on Lipoprotein Apheresis for refractory FH and HyperLp(a)aemia

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1 National & International Guidelines on Lipoprotein Apheresis for refractory FH and HyperLp(a)aemia Europe Nordestgaard et al. Lipoprotein (a) as a cardiovascular risk factor. Eur Heart J 2010; 31: Germany Working Party on Medical Treatment of the Federal Committee of Physicians and Health Insurance Companies. Code of Social Law 2003; vol V (SGB V). Italy Stefanutti C. 2nd Italian Consensus Conference on LDL-apheresis. Nutr Metab Cardiovasc Dis 2010; 20: Japan Harada-Shiba et al. Guidelines for the management of familial hypercholesterolemia. J Atheroscler Thromb 2012; 19: Spain Civiera F for International Panel on Management of Familial Hypercholesterolemia. Atherosclerosis 2004;173: UK Thompson GR, HEART-UK LDL Apheresis Working Group. Atherosclerosis 2008; 198: NICE clinical guideline 71. Identification and management of familial hypercholesterolaemia. August USA Sczepiorkowski et al. Guidelines on the use of therapeutic apheresis in clinical practice - American Society for Apheresis. J Clin Aph 2007; 22: Ito et al. National Lipid Association Expert Panel on Familial Hypercholesterolemia. J Clin Lipidol 2011; 5: S Courtesy of Prof. Gilbert R Thompson

2 Food and Drug Administration Criteria Required for Commencement of LDL Apheresis (1996) Patient characteristic LDL cholesterol (mg/dl) Homozygous FH 500 Heterozygous FH and failure of medical therapy Heterozygous FH with documented coronary disease and failure of medical therapy *FH=familial hypercholesterolemia LDL=low-density lipoprotein

3 Criteri di ammissione al trattamento di LDL-aferesi previsti dal Ministero della Sanità Tedesco Caratteristiche del paziente Ipercolesterolemia familiare omozigote da difetto recettoriale con LDL > 500 mg/dl Ipercolesterolemia grave con CHD documentata con target LDL< 130 mg/dl non raggiunto, nonostante massima terapia farmacologica e dietetica di 3 mesi Ipercolesterolemia grave con progressiva CHD documentata con Lp(a) > 60 mg/dl, anche se LDL < 130 mg/dl Linee guida approvate dalla Società Tedesca di Cardiologia

4 Extention of for RI of lipid apheresis in Germany valid from June 19th 2008 Isolated elevated Lp(a) level to > 60 mg/dl and normal LDL-cholesterol and simultaneously progressive cardiovascular disease documented by clinical and imaging techiques

5 Normal level of LDL-c not defined (probably according to target level of risk group acc. to EAS guidelines) First step is always first to get LdL-c on normal (targeted) level Cardiovascul. Disease: coronary heart disease, PAOD, cerebro-vascular disease

6 LDL-apheresis: I Italian Consensus Conference Guidelines (Ostuni 1990 Roma 1992) Homozygous Familial Hypercholesterolaemia (or double heterozygous) Heterozygous Familial Hypercholesterolaemia and other primary hypercholesterolaemia showing at least two of the following criteria: - lack of response to multiple dietary and pharmacological treatment - presence of severe atheromasia (symptomatic or asymptomatic, including preexisting Acute Myocardial Infarction) - coronary angioplasty, A-C by-pass (whenever pharmacological therapy does not guarantee the occurrence of restenosis) - heart transplant Claudia Stefanutti

7 GALLARATE MILANO TRIESTE FORLÌ REGGIO EMILIA PISTOIA PRATO L AQUILA VITERBO BARI SASSARI OZIERI ROMA Coordinating centre NUORO CAGLIARI PALERMO Italian Multicenter Study on LDL-apheresis Working Group (# 23 Centers)

8 IMSLDLa-WG centers not participating at 2009 survey, and Non- IMSLDLa-WP centres: sites, type of Unit (Specialty), patients per center, and devices used plus patients treated per device IMSLDLa-WG Center * Specialty # patients Device (# patients treated) Cagliari IM 24 HELP (13); Kaneka (6); DALI (5) Sassari 1 AR 22 Kaneka (14); HELP (4); DALI (4) Bari N 7 HELP (7) Total 53 Non- IMSLDLa-WG Center Matera IMSLDLa-WG: ITM 1 # 157 HELP (1) Catanzaro IM 3 HELP (3) Siena IM 2 HELP (2) Pisa CP (CNR) 28 Kaneka (18) HELP (5) Kaneka or Not belonging: # 54 HELP (5) Bologna (S.Orsola) ITM 7 DALI (4) HELP (3) Bologna (Malpighi) N 1 HELP (1) Orbassano ITM 3 Kaneka (2) HELP (1) Legnago N 2 HELP (2) Firenze Total patients under ITM treatment 2 in Italy: Kaneka # 211 (2) Genova ITM 2 DALI (2) Pietra Ligure ITM 1 DALI (1) Torino ITM 1 DALI (1) Novara ITM 1 DALI (1) Vicenza ITM 3 DALI (3) Total 57 *: Not participating at 2009 survey; : Source: Medical DeviceCompanies IM: Internal Medicine AR: Anesthesiology and Resuscitation N: Nephrology ITM: Immunohematology and Transfusion Medicine CP: Clinical Physiology (CNR: Consiglio Nazionale delle Ricerche; National Research Council)

9 Pediatric LDL-apheresis Refractoriness to diet and combined cholesterol-lowering drugs (at reasonably high doses) must be proven; diagnosis and complete cardiovascular examination (including catetherization) must be done; Begin LDLa between 6 and 7 years of age (or before); The delay in beginning LDLa (8-9 years) leads to the onset and progression of the aortic valvular disease; Treatment with LDLa imposes the reaching of therapeutic target (LDLC < 70mg/dL - ATP III) in children of high cardiovascular risk; Collateral effects of pediatric LDLa are rare if the procedure is carried out by a team of experts. The nd Italian Consensus Conference on LDL-apheresis Guidelines and recommendations