title: [Management of refractory symptoms in hypertrophic cardiomyopathy with restrictive pathophysiology: novel perspectives for ranolazine].
abstract: The management of patients with hypertrophic cardiomyopathy (HCM) and refractory symptoms due to massive hypertrophy and severe diastolic dysfunction represents a real challenge for the clinical cardiologist. Such patients often require novel therapeutic approaches, both invasive and pharmacological, involving multidisciplinary teamwork; however, the implementation of potentially viable treatment options is hindered by lack of disease-specific evidence. We report the case of a young woman with severe HCM and restrictive physiology, who underwent extensive myectomy via the transaortic and transapical approach, followed by biventricular pacing for cardiac resynchronization, with significant but incomplete symptomatic improvement. The subsequent introduction of ranolazine, based on promising preclinical data, has led to an excellent final result. An ongoing randomized clinical trial is currently testing the efficacy of ranolazine in symptomatic HCM.
authors.affiliation: Centro di Riferimento per le Cardiomiopatie, Azienda Ospedaliero-Universitaria Careggi, Firenze. benedetta.tomberli@gmail.com
authors.forename: Benedetta
authors.initials: B
authors.lastname: Tomberli
authors.forename: Francesca
authors.initials: F
authors.lastname: Girolami
authors.forename: Raffaele
authors.initials: R
authors.lastname: Coppini
authors.forename: Cecilia
authors.initials: C
authors.lastname: Ferrantini
authors.forename: Alessandra
authors.initials: A
authors.lastname: Rossi
authors.forename: Franco
authors.initials: F
authors.lastname: Cecchi
authors.forename: Iacopo
authors.initials: I
authors.lastname: Olivotto
mesh_terms: D000083:Acetanilides / Q000627:therapeutic use*; D000328:Adult; D002304:Cardiac Pacing, Artificial; D006348:Cardiac Surgical Procedures; D002312:Cardiomyopathy, Hypertrophic / Q000188:drug therapy* / Q000473:pathology / Q000503:physiopathology* / Q000628:therapy; D002313:Cardiomyopathy, Restrictive / Q000188:drug therapy* / Q000473:pathology / Q000503:physiopathology* / Q000628:therapy; D002317:Cardiovascular Agents / Q000627:therapeutic use*; D017115:Catheter Ablation; D004791:Enzyme Inhibitors / Q000627:therapeutic use*; D005260:Female; D006801:Humans; D010138:Pacemaker, Artificial; D010879:Piperazines / Q000627:therapeutic use*; D000069458:Ranolazine; D012720:Severity of Illness Index; D016896:Treatment Outcome
chemical_list: D000083:Acetanilides; D002317:Cardiovascular Agents; D004791:Enzyme Inhibitors; D010879:Piperazines; D000069458:Ranolazine