0000000000147922
AUTHOR
Olivier Huttin
Phenotype/Genotype Relationship in Left Ventricular Noncompaction: Ion Channel Gene Mutations Are Associated With Preserved Left Ventricular Systolic Function and Biventricular Noncompaction
International audience; Background: Few data exist concerning genotype-phenotype relationships in left ventricular noncompaction (LVNC).Methods and results: From a multicenter French Registry, we report the genetic and clinical spectrum of 95 patients with LVNC, and their genotype-phenotype relationship. Among the 95 LVNC, 45 had at least 1 mutation, including 14 cases of mutation in ion channel genes. In a complementary analysis including 16 additional patients with ion channel gene mutations, for a total of 30 patients with ion channel gene mutation, we found that those patients had higher median LV ejection fraction (60% vs 40%; P < .001) and more biventricular noncompaction (53.1% vs 18…
Cardiovascular outcomes after cardiac resynchronization therapy in cardiac amyloidosis
Abstract Aims Cardiac resynchronization therapy (CRT) is highly effective in dilated cardiomyopathy (DCM) patients with impaired left ventricular ejection fraction (LVEF) and left bundle block branch. In cardiac amyloidosis (CA) patients, left ventricular dysfunction and conduction defects are common, but the potential of CRT to improve cardiac remodelling and survival in this particular setting remains undefined. We investigated cardiovascular outcomes in CA patients after CRT implantation in terms of CRT echocardiographic response and major cardiovascular events (MACEs). Methods and results Our retrospective study included 47 CA patients implanted with CRT devices from January 2012 to Feb…
Prognosis of adults with left ventricular non compaction: Results from a prospective multricentric french study
Background Left ventricular non compaction (LVNC) is a rare cause of cardiomyopathy related to abnormal in utero myocardial development, leading to prominent trabeculations. Its prognosis remains unclear with conflicting data and mortality ranging from 2 to 38%. Purpose The aim of this study was to define the prognosis of newly diagnosed LVNC as compared to recently diagnosed dilated cardiomyopathy (DCM). Methods A prospective multicentric study with a 2-year follow-up was designed. The primary endpoint combined cardiovascular death, heart transplantation and hospitalisation for cardiovascular events. Three groups of patients were defined: Group A: new cases of LVNC, Group B: new cases of D…
Temporal Trends in Transcatheter Aortic Valve Replacement in France
Background - Transcatheter aortic valve replacement (TAVR) is standard therapy for patients with severe aortic stenosis who are at high surgical risk. However, national data regarding procedural characteristics and clinical outcomes over time are limited. Objectives - The aim of this study was to assess nationwide performance trends and clinical outcomes of TAVR during a 6-year period. Methods - TAVRs performed in 48 centers across France between January 2013 and December 2015 were prospectively included in the FRANCE TAVI (French Transcatheter Aortic Valve Implantation) registry. Findings were further compared with those reported from the FRANCE 2 (French Aortic National CoreValve and Edwa…