Type 1 Brugada Pattern May Be Provoked by Ajmaline in Some Healthy Subjects: Results From a Clinical Trial

⚠️ Isolated DI-T1BP without clinical or genetic features of #BrS or family history of sudden death may not be diagnostic of the condition.

ahajournals.org/doi/10.1161/CI…

Safety and Efficacy of Metabolic Modulation With Ninerafaxstat in Patients With Nonobstructive Hypertrophic Cardiomyopathy - ScienceDirect sciencedirect.com/science/articl…

Many congratulations to George Joy for his PhD. Very proud as a supervisor of his hard, excellent and groundbreaking work exploring the deep phenotype of subclinical HCM UCL Institute of Cardiovascular Science

#LQTS : importance of reassessing arrhythmic risk after treatment initiation

📈M-FACT score ≥2 predicts a⬆️risk of ICD shocks, prompting therapeutic decisions in #LQTS

💡Relying solely on the M-FACT score may lead to unnecessary #ICD implants

DOI: 10.1093/eurheartj/ehae289

👉Excelentes perspectivas para los pacientes con MCH obstructiva! 👉Esperemos que pronto podamos tener comercializados mavacamten y aficamten en🇪🇸!!! Aimee Emch Sección Cardiopatías Familiares y Genética CV SEC Soc Esp Cardiología

😊Very happy to share with all of you our recent work published in JACC Journals #JACCEP

Phenotype and Clinical Outcomes in Desmin-Related Arrhythmogenic Cardiomyopathy

Has taken a lot of effort, but it's worth it. Thank you to all co-authors!

jacc.org/doi/10.1016/j.…

In summary, it is our understanding that DES-related ACM is characterised by

- Mild LV systolic dysfunction and/or dilation
- Subepicardial fibrosis (💍/ inferolateral)
- Similar adverse cardiac outcomes to historical controls with FLNC-tv and LMNA-tv ACM in males.

It's been a while since desminopathy has been on the medical radar, but it's still a condition that many of us are unaware of.
A lot of us, used to think that is was limited to RCM or DCM + AVB + some degree of skeletal involvement, but...👇👇

🏥 SAEs were reported in a small percentage of patients in both the aficamten and placebo groups, with #AF and palpitations being more common in the aficamten group.

🔍 Treatment effects of aficamten appeared similar regardless of background beta-blocker use or the presence of a pathogenic sarcomere gene variant.

📉 Incidence of LVEF <50% was low with aficamten, with no treatment interruption or exacerbation of HF.

#Aficamten for Symptomatic #OHCM in NEJM

📋Phase 3, double-blind, randomized, placebo-controlled trial

💊 Aficamten, an oral selective CMI w/ ⏱️ shorter half-life and interactions allows for rapid dose escalation and timely clinical benefit.
nejm.org/doi/full/10.10…

Disappointed to see the conservative approach to #genetic testing, recommending only the main #sarcomeric #genes . This ignores the recent evidence around FHOD3, CSRP3, TRIM63, ALPK3, etc. Also, phenocopies are not easily distinguishable and should be included from start, IMO.

They are here!!! The 2024 #HCM #Guidelines are available!
Of special interest is the inclusion of cardiac myosin inhibitors in the treatment pathway, and the recognition of valsartan as potentially beneficial to slow adverse cardiac remodeling 🫀💉🏥
jacc.org/doi/10.1016/j.…

❗️No existe un límite para grosor del miocardio apical para diagnosticar una miocardiopatia hipertrofica apical
🔎La idea : definir un grosor máximo del apex en personas sanas
📍un grosor máximo apical de 5.2 a 5.6 mm/m2 define el límite de normalidad versus hipertrofia apical

Survival analysis and gender differences in hypertrophic cardiomyopath... sciencedirect.com/science/articl…
Rebeca Lorca

International (🇪🇸, 🇵🇱,🇬🇧,🇳🇱, 🇮🇹, 🇩🇰,🇹🇩, 🇦🇺, 🇨🇿) Multicenter study on safety of pregnancy in women with DCM-associated genetic variants either with overt DCM or not. Very necessary work to inform this growing population of patients. doi.org/10.1016/j.rec.…

Penetrance of Dilated Cardiomyopathy in Genotype-Positive Relatives - ScienceDirect sciencedirect.com/science/articl…

Implantable loop recorders in patients with Brugada syndrome: the BruLoop study

European Society of Cardiology European Society of Cardiology Journals

academic.oup.com/eurheartj/arti…

New paper about Phenotypic Expression and Outcomes in Patients with the p.Arg301Gln GLA Variant in #Fabry Disease. Congratulations Rocío Blanco, MD 🇦🇷 and all collaborators from Mallorca, Santiago Alvaro Hermida Switzerland and Germany Soc Esp Cardiología Sección Cardiopatías Familiares y Genética CV SEC mdpi.com/1422-0067/25/8….

Lectura de Tesis doctoral de Javier Limeres Factores pronósticos en Miocardiopatía arritmogénica izquierda. Excelente!! Enhorabuena Dr Limeres Roberto Barriales-Villa José F. Rodríguez Palomares Soc Esp Cardiología Sección Cardiopatías Familiares y Genética CV SEC