Edgardo Alania
Edgardo Alania

@Ed_Alania

14 Tweets 123 reads Aug 28, 2023
💗NEW 2023 @escardio guidelines on Cardiomyopathies #ESCCongress2023 #cardioEd #Cardiology #Cardiotwitter
‼️New guideline, not an update of existing ones
Exception: HCM 2014 ESC Guidelines. As such, most of the recommendations in this guidelines are NEW
🧵Some points⬇️⬇️⬇️
🔵A new phenotypic description of cardiomyopathies
✅HCM
✅DCM
✅NDLVC 🆕
✅ARVC
✅RCM
🚫ACM: lacks morphological or functional definition consistent with the existing classification scheme
🚫LVNC
🚫Tako-Tsubo
🚫LVNC: does not consider to be a cardiomyopahty. “Hypertrabeculation” is recommended, particularly transient or clearly adult onset
🚫Tako-Tsubo: given the transient nature, the Task Force does not recommend its classification as a cardiomyopathy
🔵Cardiomyopathies 2nd external factors
🍺Cardiomyopathy (13.5%) TTNtv
☠️Cancer therapy-induced cardiomyopahty (7.5%) TTNtv
🤰PPCM (15%) truncating variants in 8 genes & 2/3 TTNtv(10%) others DSP, FLNC and BAG3
🦠Acute miocarditis (8-22%) genes in DCM, NDLVC & ARVC
🔵Multidisciplinary Cardiomyopathy Teams
📍Creation of local/regional/national/international networks
🌏ERN GUARD-Heart: allows clinicians and health professionals to share information, for the benefit of cardiomyopathy patients
Link guardheart.ern-net.eu
🔴Recommendations for management of atrial fibrillation & atrial flutter in patients with cardiomyopathy
🟩🟦Oral anticoagulation all patients with HCM or Cardiac Amyloidosis (unless contraindicated)
🔴Recommendations for 💊treatment of LVOTO
🟩🟦Non-vasodilating BB 1st line therapy improve symptoms in LVOTO
🟩🟦Verapamil/Diltiazem intolerant or contraindications BB
🟩🟦Disopyramide in addition if LVOTO persist
🟨Mavacamten considerer i addition if LVOTO persist
🔴Recommendation for prevention SCD in HCM
‼️Task Force does not recommend the use of sarcomeric variant(s) to guide decisions around ICD implantation for PP with LOW or INTERMEDIATE risk score
🔴Recommendations for an ICD in DCM & NDLVC
🧬PLN, DSP, LMNA, FLNC, TMEM43 and RBM20⬆️⬆️rate of major arrhythmic events⚡️
🔴Spectrum of restrictives heart diseases
🔴Syndromic and Metabolic cardiomyopathies: Anderson-Fabry and Amyloidosis
🔴Exercise recommendations for cardiomyopathies
🧬DCM and NDLVC: not recommended symptomatic, LVEF<40%, exercise-induced arrhythmias or LMNA or TMEM43
🔴Key messages I
✅Cardiomyopathies are more common than previously thought
✅Aetiology fundamental to the management. Morpho/Functional PHENOTYPE crucial 1st step
✅Multimodality Imaging
✅GENETIC testing: Dx/Prognosis/risk stratification/Tx/Counselling
🔴Key messages II
✅Cardiac myosin Inh should be considered HCM and LVOTO who remain symptomatic despite OMT
✅SCD risk DCM & NDLVC varies depending underlying cause & 🧬subtype (even if LVEF >35%)
✅DEFINE aetiology for Tx: ERT/chaperone (Fabry), tafamidis, silencers (ATTR)
🔴Key messages III
✅All cardiomyopathy patients should have an individualized risk assessment for exercise prescription
✅A multidisciplinary approach to cardiomyopathies that has the patient and their family at its heart
🧵(End)🫀

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