Vol.:(0123456789) 1 3 Internal and Emergency Medicine https://doi.org/10.1007/s11739-018-1925-8 IM - ORIGINAL Role of lipoprotein (a) and LPA KIV2 repeat polymorphism in bicuspid aortic valve stenosis and calcifcation: a proof of concept study Elena Sticchi 1,2  · Betti Giusti 1,2,3  · Antonella Cordisco 4  · Anna Maria Gori 1,3  · Alice Sereni 1  · Francesco Sof 1,5,6  · Fabio Mori 7  · Stefania Colonna 8  · Maria Pia Fugazzaro 9  · Guglielmina Pepe 1,2,3  · Stefano Nistri 9  · Rossella Marcucci 1,3 Received: 6 June 2018 / Accepted: 5 August 2018 © Società Italiana di Medicina Interna 2018 Abstract Hemodynamic valvular impairment is a frequent determinant of the natural history of bicuspid aortic valve (BAV). The role of elevated Lp(a) levels and LPA Kringle IV type 2 (KIV-2) size polymorphism in infuencing aortic valve calcifcation and stenosis development in patients with tricuspid aortic valve was recognized. In this study, we investigate the association between Lp(a) and LPA KIV-2 repeat number, and the presence of calcifcation and stenosis in BAV patients. Sixty-nine patients [79.7% males; median age 45(30–53) yrs], consecutively referred to Center for Cardiovascular Diagnosis or Refer- ral Center for Marfan syndrome or related disorders, AOU Careggi, from June to November 2014, were investigated. For each patient, clinical (ECG and echocardiography) and laboratory [Lp(a) (Immunoturbidimetric assay) and LPA KIV-2 repeat number (real-time PCR)] evaluation were performed. Patients were compared with 69 control subjects. No signifcant association between Lp(a) circulating levels and LPA KIV-2 repeat number and BAV was evidenced. Among BAV patients, signifcantly higher Lp(a) levels according to calcifcation degree were found [no calcifcations:78(42–159) mg/L, mild/ moderate: 134(69–189) mg/L; severe: 560(286–1511) mg/L, p = 0.008]. Conversely, lower LPA KIV-2 repeat numbers in subjects with more severe calcifcation degree were observed. Furthermore, higher Lp(a) levels in patients with aortic stenosis [214(67–501) mg/L vs 104(56–169) mg/L, p = 0.043] were also found. In conclusion, present data suggest the potential role for Lp(a) as a possible risk marker useful to stratify, among BAV patients, those with a higher chance to develop valvular calcifcations and aortic stenosis. Keywords Lipoprotein (a) · Kringle IV type 2 · Bicuspid aortic valve · Calcifcation · Stenosis * Betti Giusti betti.giusti@unif.it 1 Department of Experimental and Clinical Medicine, Section of Critical Medical Care and Medical Specialities, University of Florence, Largo Brambilla 3, 50134 Florence, Italy 2 Marfan Syndrome and Related Disorders Regional Referral Center, Careggi Hospital, Florence, Italy 3 Atherothrombotic Diseases Regional Referral Center, Careggi Hospital, Florence, Italy 4 Cardiovascular and Perioperative Internal Medicine Unit, Careggi Hospital, Florence, Italy 5 Clinical Nutrition Unit, Careggi Hospital, Florence, Italy 6 Don Carlo Gnocchi Foundation Italy, Onlus Istituto di Ricerca e Cura a Carattere Scientifco, Florence, Italy 7 Non-Invasive Vascular Diagnosis Regional Referral Center, Cardiovascular Diagnostics Unit, Careggi Hospital, Florence, Italy 8 Outpatient Cardiology Unit, Health District 1 ULSS 6, Vigonza and Carmignano di Brenta, Padua, Italy 9 Cardiology Service, CMSR Veneto Medica, Altavilla Vicentina, Italy