Bruno Lefort1, Samir Saheb2, Eric Bruckert2, Christine Giraud3, Olivier Hequet4, Régis Hankard5. 1. INSERM, UMR 1069, "Nutrition, Croissance et Cancer", Tours, France; Université François Rabelais, Tours, France; Centre Hospitalier Universitaire de Tours, Tours, France. Electronic address: bruno.lefort@univ-tours.fr. 2. Centre Hospitalier Universitaire Pitié-Salpêtrière, Paris, France. 3. Centre Hospitalier Universitaire de Poitiers, Poitiers, France. 4. Centre Hospitalier Universitaire Lyon, Lyon, France. 5. INSERM, UMR 1069, "Nutrition, Croissance et Cancer", Tours, France; Université François Rabelais, Tours, France; Centre Hospitalier Universitaire de Tours, Tours, France.
Abstract
BACKGROUND: Homozygous familial hypercholesterolemia (HFH) is a rare genetic disease leading to early onset atherosclerosis, due to high concentrations of LDL-C in the blood. Aortic root atheromas may be complicated by obstruction to left ventricle outflow or coronary stenosis. The aim of this study was to describe the progression of aortic root atheroma in patients requiring lipoprotein apheresis before 16 years of age and to examine the requirement of these patients for aortic surgery. METHOD: Clinical reports, lipid profiles and echocardiogram results were obtained retrospectively for patients with HFH from three French hemapheresis centers. Data are presented as group medians. RESULTS: Twenty patients were included, of which 53% had aortic root atheroma (as assessed by echocardiogram) before starting lipoprotein apheresis. These patients began lipoprotein apheresis later than children without aortic root atheroma (10.3 years old [range 5.6-15.9 years] vs. 5.0 years old [range 4.5-11.6 years], respectively, p < 0.05). After 16.4 years (range 2.2-22.8 years) of lipoprotein apheresis treatment, aortic root atheroma had progressed in 64% of patients. Five patients needed surgery for aortic stenosis, which was associated with a coronary artery by-pass for two of them. There were significantly more operations among patients with an aortic root atheroma at the beginning of lipoprotein apheresis than among patients without preexisting lesions (p < 0.01). One patient died after aorta replacement surgery during this period. CONCLUSION: Our results suggest that the initiation of lipoprotein apheresis before the onset of aortic root atheroma should reduce the requirement for aortic surgery.
BACKGROUND: Homozygous familial hypercholesterolemia (HFH) is a rare genetic disease leading to early onset atherosclerosis, due to high concentrations of LDL-C in the blood. Aortic root atheromas may be complicated by obstruction to left ventricle outflow or coronary stenosis. The aim of this study was to describe the progression of aortic root atheroma in patients requiring lipoprotein apheresis before 16 years of age and to examine the requirement of these patients for aortic surgery. METHOD: Clinical reports, lipid profiles and echocardiogram results were obtained retrospectively for patients with HFH from three French hemapheresis centers. Data are presented as group medians. RESULTS: Twenty patients were included, of which 53% had aortic root atheroma (as assessed by echocardiogram) before starting lipoprotein apheresis. These patients began lipoprotein apheresis later than children without aortic root atheroma (10.3 years old [range 5.6-15.9 years] vs. 5.0 years old [range 4.5-11.6 years], respectively, p < 0.05). After 16.4 years (range 2.2-22.8 years) of lipoprotein apheresis treatment, aortic root atheroma had progressed in 64% of patients. Five patients needed surgery for aortic stenosis, which was associated with a coronary artery by-pass for two of them. There were significantly more operations among patients with an aortic root atheroma at the beginning of lipoprotein apheresis than among patients without preexisting lesions (p < 0.01). One patient died after aorta replacement surgery during this period. CONCLUSION: Our results suggest that the initiation of lipoprotein apheresis before the onset of aortic root atheroma should reduce the requirement for aortic surgery.
Authors: Jérôme Caron; Véronique Pène; Laia Tolosa; Maxime Villaret; Eléanor Luce; Angélique Fourrier; Jean-Marie Heslan; Samir Saheb; Eric Bruckert; María José Gómez-Lechón; Tuan Huy Nguyen; Arielle R Rosenberg; Anne Weber; Anne Dubart-Kupperschmitt Journal: Stem Cell Res Ther Date: 2019-07-29 Impact factor: 6.832