🧵 3/17 - In a study of Maron BJ et al - JACC 2016 with 573 HCM patients, patients with rest obstruction had more progressive HF (7,4%/year), followed by patients with provocable obstruction (3,2%/year) and less in nonobstructive HCM (1,6%/year) sciencedirect.com/science/articl…… twitter.com/i/web/status/1…
🧵 4/17 - Exercise Echo in HCM is safe when performed in experienced centers: Cleveland Clinic - study with 263 HCM patients - major complications in <0,04% of patients (Drinko JK, et al. Am J Cardiol 2004)
✨Exercise Echo provides important prognostic information: in the study… twitter.com/i/web/status/1…
🧵 5/17 - There is no advantage to submit a patient with rest gradients > 50mmHg, only increased risks.
In this case there was already a LVOT gradient of 96mmHg, with mesossystolic Ao Valve closure in M-mode
🧵 5/17 - There is no advantage to submit a patient with rest gradients > 50mmHg, only increased risks.
In this case there was already a LVOT gradient of 96mmHg, with mesossystolic Ao Valve closure in M-mode
🧵 6/17 - SAM is the main mechanism of LVOT dynamic obstruction - "mitral-septal contact"
•SAM: not only result of septal asymmetric hypertrophy (⬆️Veloc / Venturi effect) 👉🏽 Crucial understanding for adequate treatment!
•Primary abnormalities of MV apparatus may have a great… twitter.com/i/web/status/1…
🧵 7/17 - Medioventricular LV obstruction is less frequent than LVOT obstruction, and has higher association with apical aneurysm, ventricular arrhythmia, embolic phenomena and death (Yan, L. R., et al. J Cardiovasc Med 2015, Liu, Q., et al. Scientific Reports 2017) @purviparwani… twitter.com/i/web/status/1…
🧵 8/17 - ✨Apical aneurysm may occur in 2% of all patients with HCM (Maron MS, Circulation 2008), In 13-15% of patients with Apical HCM (Chen CC- Clin Cardiol. 2011) and 25% of patients with medioventricular obstruction (MVO) (Efthimiadis GK - Circ J. 2013).
🧵 10/17- The definition of grade and mechanism of MR in HCM is important for choosing the best therapeutic strategy.
💫Patients with septal hypertrophy ≤ 1.8cm or medioventricular obstruction may have important contribution of additional SAM causative mechanisms - consider… twitter.com/i/web/status/1…
🧵 11/17 - In 10% primary mitral valvular disease may coexist, bringing difficulties for MV repair - degenerative (prolapse / ruptured chordae) or traumatic fibrosis (repetitive septal contact) - Holst KA, et al. Mayo Clin Proc. 2019;94(1):66‐73. @VerwerftJan@KardiologieHH… twitter.com/i/web/status/1…
🧵 12/17 - According to guidelines in symptomatic patients refractory to negative inotropic drugs (NYHA II-IV) septal reduction therapy (SRT) is indicated.
✨Evidence shows that early SRT is beneficial. Study by Cui H et al. The J Thorac and Cardiovasc Surg 2020 (n= 1351 patients… twitter.com/i/web/status/1…
✨Important improvement in symptoms and mortality when compared to nonoperated obstructive HCM patients - (Maron BJ - Ventricular Septal Myectomy for Obstructive Hypertrophic Cardiomyopathy: Analysis… twitter.com/i/web/status/1…
🧵 14/17 - Intraprocedural Echo - Myectomy: 1- Evaluation of LVOT obstruction mechanism. 2- Localize the point of contact between interventricular septum and anterior mitra leaflet 2- MR mechanism? 3- Check for results after weaning from extracorporeal circulation - MR and… twitter.com/i/web/status/1…
🧵 15/17- Good results with Alcohol Septal Ablation has been published - Euro ASA registry:
•1275 symptomatic patients (NYHA III-IV)
•Gradients LVT > 50mmHg rest or efforts
•Without significant mitral valve disease
•1% mortality in 1 month
•12% needed pacemaker implantation… twitter.com/i/web/status/1…
🧵 16/17 - Intraprocedural Echo for Alcohol septal Ablation:
✨Evaluate perfused area for each septal branch: use of intracoronary contrast (UEA) - reduces infarction area, less complete AV block, better final result.
✨TTE or TEE may be used
✨Evaluate immediate results of… twitter.com/i/web/status/1…
🧵 17/17 - Myosin Inhibitors - Mavacantem
Phase 3 trial
Pimary endpoint (Primary endpoints: improvement of peak O2 consumption (pVO2) 1,5ml/Kg/min + improvement of functional class (FC) or improvement of pVO2 > 3,0ml/Kg/min without FC deterioration) : 37% Mavacantem group x 17%… twitter.com/i/web/status/1…
🧵 5/17 - There is no advantage to submit a patient with rest gradients > 50mmHg, only increased risks.
In this case there was already a LVOT gradient of 96mmHg, with mesossystolic Ao Valve closure in M-mode
🧵 6/17 - SAM is the main mechanism of LVOT dynamic obstruction - "mitral-septal contact"
•SAM: not only result of septal asymmetric hypertrophy (⬆️Veloc / Venturi effect) 👉🏽 Crucial understanding for adequate treatment!
•Primary abnormalities of MV apparatus may have a great… twitter.com/i/web/status/1…
🧵 7/17 - Medioventricular LV obstruction is less frequent than LVOT obstruction, and has higher association with apical aneurysm, ventricular arrhythmia, embolic phenomena and death (Yan, L. R., et al. J Cardiovasc Med 2015, Liu, Q., et al. Scientific Reports 2017) @purviparwani… twitter.com/i/web/status/1…
🧵 8/17 - ✨Apical aneurysm may occur in 2% of all patients with HCM (Maron MS, Circulation 2008), In 13-15% of patients with Apical HCM (Chen CC- Clin Cardiol. 2011) and 25% of patients with medioventricular obstruction (MVO) (Efthimiadis GK - Circ J. 2013).
🧵 10/17- The definition of grade and mechanism of MR in HCM is important for choosing the best therapeutic strategy.
💫Patients with septal hypertrophy ≤ 1.8cm or medioventricular obstruction may have important contribution of additional SAM causative mechanisms - consider… twitter.com/i/web/status/1…
🧵 11/17 - In 10% primary mitral valvular disease may coexist, bringing difficulties for MV repair - degenerative (prolapse / ruptured chordae) or traumatic fibrosis (repetitive septal contact) - Holst KA, et al. Mayo Clin Proc. 2019;94(1):66‐73. @VerwerftJan@KardiologieHH… twitter.com/i/web/status/1…
🧵 12/17 - According to guidelines in symptomatic patients refractory to negative inotropic drugs (NYHA II-IV) septal reduction therapy (SRT) is indicated.
✨Evidence shows that early SRT is beneficial. Study by Cui H et al. The J Thorac and Cardiovasc Surg 2020 (n= 1351 patients… twitter.com/i/web/status/1…
✨Important improvement in symptoms and mortality when compared to nonoperated obstructive HCM patients - (Maron BJ - Ventricular Septal Myectomy for Obstructive Hypertrophic Cardiomyopathy: Analysis… twitter.com/i/web/status/1…
🧵 14/17 - Intraprocedural Echo - Myectomy: 1- Evaluation of LVOT obstruction mechanism. 2- Localize the point of contact between interventricular septum and anterior mitra leaflet 2- MR mechanism? 3- Check for results after weaning from extracorporeal circulation - MR and… twitter.com/i/web/status/1…
🧵 15/17- Good results with Alcohol Septal Ablation has been published - Euro ASA registry:
•1275 symptomatic patients (NYHA III-IV)
•Gradients LVT > 50mmHg rest or efforts
•Without significant mitral valve disease
•1% mortality in 1 month
•12% needed pacemaker implantation… twitter.com/i/web/status/1…
🧵 16/17 - Intraprocedural Echo for Alcohol septal Ablation:
✨Evaluate perfused area for each septal branch: use of intracoronary contrast (UEA) - reduces infarction area, less complete AV block, better final result.
✨TTE or TEE may be used
✨Evaluate immediate results of… twitter.com/i/web/status/1…
🧵 17/17 - Myosin Inhibitors - Mavacantem
Phase 3 trial
Pimary endpoint (Primary endpoints: improvement of peak O2 consumption (pVO2) 1,5ml/Kg/min + improvement of functional class (FC) or improvement of pVO2 > 3,0ml/Kg/min without FC deterioration) : 37% Mavacantem group x 17%… twitter.com/i/web/status/1…
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🌕 Cardiac Myxomas 1/🧵
🔘The most prevalent cardiac tumors
🔘95% are sporadic and 5% are familial (Carney’s complex)
🔘3👩🏻 : 1 👨🏽 (non-familial)
#echofirst
📝Reynen K. Cardiac myxomas. NEJM 1995
🌕 Cardiac Myxomas 2/🧵
🔘80% - Left atrium (75% of which involves atrial septum)
🔘7-20% - Right atrium
🔘Remainder 10% - biatrial, LV, RV
🔘 Carney’s - multiple and atypical sites
#echofirst
🌕 Cardiac Myxomas 3/🧵
🔘Clinical presentation depends on location, size and mobility
🔘Triad: obstruction, embolization and constitutional symptoms
🔘50% are asymptomatic
🔘Obstruction 35%
📝Bordalo et al. Rev Port Cardiol 2012