Cardim, Nuno

Revista Portuguesa de Cardiologia

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Cardim, Nuno

Repositório da Universidade Nova de Lisboa

RCHlM3NabRSuCsctpcw(ith0BARTICLE IN PRESS+ModelEPC-2310; No. of Pages 3Revista Portuguesa de Cardiologia xxx (xxxx) xxx--xxxwww.revportcardiol.orgRevista Portuguesa deCardiologiaPortuguese Journal of CardiologyURRENT PERSPECTIVESypertrophic  cardiomyopathy:  Paradigm  shifts in  theast 30 years  (Part  2)iocardiopatia  hipertrófica:  mudanças  de  paradigma  nos  últimos0  anos  (Parte  2)uno Cardima,bNova  Medical  School,  Lisbon,  PortugalHospital  CUF  Descobertas,  Lisbon,  Portugaleceived  16  September  2023;  accepted  1  December  2023ppodaSelsodbo(pm‘‘Half  of  what  you  are  taught  in  medical  school  will  bewrong  in  10  years’  time.’’--  Sydney  Burwell,  1944evere hypertrophic cardiomyopathy isntreatable and the prognosis is poorontemporary  advances  in  the  treatment  of  HCM  have  sub-tantially  modified  its  prognosis;  this  disease,  previouslyonsidered  untreatable  and  with  high  mortality,1 is  nowreatable,  with  a  similar  mortality  to  that  of  the  generalopulation.Risk  stratification  of  sudden  cardiac  death  (SCD)  in  theontext  of  primary  prevention  identifies  high-risk  patients,ho  should  receive  an  implantable  cardioverter-defibrillatorICD).2 However,  despite  improved  patient  selection,  theres  still  room  to  optimize  the  current  algorithms,  sincehe  incidence  of  appropriate  interventions  in  primaryE-mail address: cardimnuno@gmail.comttfittps://doi.org/10.1016/j.repc.2023.12.008870-2551/© 2024 Sociedade Portuguesa de Cardiologia. Published by EY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/)Please  cite  this  article  as:  N.  Cardim,  Hypertrophic  cardiomyopPortuguesa  de  Cardiologia,  https://doi.org/10.1016/j.repc.202revention  is  only  3--4%/year  (versus  10%/year  in  secondaryrevention1,3).For  a  long  time,  the  treatment  of  intraventricularbstruction  was  based  on  the  use  of  beta-blockers  andisopyramide,  but  surgical  septal  myectomy  is  currently highly  effective  intervention  with  very  low  mortality.1,4ince  1995,  percutaneous  alcohol  septal  ablation  hasmerged  as  a  less  invasive  alternative  to  surgery,  with  excel-ent  results,  low  mortality,  and  similar  outcomes,  despitehorter  follow-up  times,  delayed  response  and  higher  ratesf  permanent  pacemaker  implantation.4Recently,  a  new  drug  class,  myosin  inhibitors,  has  beeneveloped.5 By  reducing  the  excess  actin--myosin  cross-ridges  in  HCM,  these  drugs  decrease  the  contractilityf  the  interventricular  septum,  increasing  left  ventricularLV)  outflow  tract  dimensions,  and  thereby  interrupting  theathophysiological  cascade  of  obstruction.5In  heart  failure  with  preserved  ejection  fraction  (HFpEF),edical  treatment  involves  the  preservation  of  sinus  rhythmo  maintain  atrial  contraction.  In  addition,  heart  rate  reduc-ion  to  increase  the  duration  of  diastolic  time  and  LVlling,  and  improvement  of  the  ‘diastolic  environment’,lsevier España, S.L.U. This is an open access article under the CC.athy:  Paradigm  shifts  in  the  last  30  years  (Part  2),  Revista3.12.008 IN+ModelRCardm2wrtd(asaactcdpraamcvnuNetCcHaewBraH---bci•••••wworiwifmhftrap(titsootHmpiospCSHscoARTICLEEPC-2310; No. of Pages 3N.  ay  also  be  useful.  Recently,  sodium-glucose  cotransporter- inhibitors  have  also  shown  benefit  in  HFpEF.  Some  patientsho  are  refractory  to  medical  treatment  evolve  to  aestrictive  filling  pattern,  becoming  candidates  for  heartransplantation.6,7A  small  percentage  of  HCM  patients  progress  to  systolicysfunction  and  heart  failure  with  reduced  ejection  fractionHFrEF).  In  the  presence  of  intractability  to  medical  therapy,dvanced  HF  treatment  and  possible  heart  transplantationhould  be  considered.  HCM  patients  have  excellent  survivalfter  transplantation  (85%  at  one  year,  75%  at  five  yearsnd  61%  at  10  years),  due  to  their  younger  age  and  feweromorbidities.6,7In  patients  with  atrial  fibrillation  (AF),7 it  is  importanto  try  to  preserve  or  restore  sinus  rhythm,  using  electri-al  or  chemical  cardioversion,  usually  with  amiodarone,isopyramide  or  sotalol,  and/or  AF  ablation.  In  cases  wherereservation  of  sinus  rhythm  is  not  predictable  or  feasible,  aate-control  strategy  should  be  adopted,  with  beta-blockersnd/or  calcium  channel  antagonists  (verapamil  or  dilti-zem).  Radiofrequency  ablation  or,  if  there  is  concomitantyectomy,  surgical  ablation  (Cox-Maze  surgery)  may  beonsidered.7The  threshold  for  oral  anticoagulation  in  HCM  should  beery  low  and  independent  of  the  CHA2DS2-VASc  score,  andovel  oral  anticoagulants  or  vitamin  K  antagonists  should  besed.7Is  severe  HCM  untreatable  and  is  the  prognosis  poor?o,  HCM  is  nowadays  a  treatable  disease.  Treatment  exists,ven  for  severe  forms,  and  its  prognosis,  when  appropriatelyreated,  is  overall  similar  to  that  of  the  normal  population.ompetitive physical exercise is alwaysontraindicated in these patientsCM  is  an  important  cause  of  SCD  in  athletes  <35  years  ofge  and  exercise  may  precipitate  SCD.  Based  on  these  data,xercise  has  historically  been  contraindicated  in  HCM,7--10ith  only  Mitchell  class  IA  exercise  being  allowed.7--10ecause  of  these  restrictions  (and  rarely  due  to  disease-elated  limitations),  HCM  patients  are  generally  sedentarynd  inactive.Over  time,  this  formal  contraindication  to  exercise  inCM  has  been  questioned  for  several  reasons7--10: Most  HCM  patients  die  of  non-cardiac  causes,  and  amongcardiac  causes  the  main  etiology  is  coronary  artery  disease(not  HCM),  for  which  regular  exercise  is  an  integral  partof  prevention. Patients  with  HCM  who  regularly  participate  in  sports  havebetter  exercise  tolerance,  higher  peak  oxygen  consump-tion  and  better  quality  of  life. In  several  small  longitudinal  studies  (including  about  500HCM  patients)  with  relatively  long  follow-up  periods  (nineyears),  physical  exercise,  even  if  vigorous,  was  not  associ-ated  with  an  increase  in  cardiovascular  events,  includingSCD.Taking  these  findings  into  account,  a  more  flexi-le  approach  is  now  recommended  and  the  indication/CTS2 PRESSimontraindication  for  exercise  in  HCM  should  be  individual-zed,  based  on  multiple  factors7--10: Individual  risk  stratification  of  SCD Patient’s  age  (higher  risk  in  younger  individuals) Sport  characteristics  (recreational  versus  competitive)and  intensity  (light,  moderate,  or  vigorous) Sports  modality  (greater  risk  in  dynamic  sports  withfrequent  stop-and-go  sequences,  such  as  soccer  and  bas-ketball) Shared  decision-making,  including  the  patient  and  familyand  never  ignoring  the  importance  of  medical  opinionsfrom  HCM  experts  and  club/school/university  physicians.However,  common  sense  and  caution  are  alwayselcome8 as  there  is  also  no  clear  scientific  evidence  withhich  to  define  the  risk  of  all  patients  with  a  high  degreef  accuracy.  A  mature  approach  without  generalizations  isecommended.  It  is  important  to  recall  that  the  risk  of  SCDn  patients  with  HCM  is  always  higher  than  in  the  populationithout  the  disease,  even  in  low-risk  groups.  Furthermore,t  should  be  borne  in  mind  that  risk  scores  were  not  designedor  athletes,  as  they  do  not  consider  hemodynamic  andetabolic  stress,  or  the  sometimes  adverse  conditions  ofigh-intensity  competitive  sports,  particularly  in  obstructiveorms,  in  which  dehydration  and  extreme  weather  condi-ions  are  important.8Accordingly,  decisions  should  be  individualized,7--10 andisk  should  be  reassessed  regularly  in  all  HCM  patient-thletes  (annually),  and  more  frequently  in  those  whosehenotype  is  evolving,  such  as  adolescents  and  young  adultsbiannually).  Supervised  exercise  in  recreational  areas  andhe  availability  of  an  automatic  external  defibrillator  mayncrease  safety.This  hot  topic  was  also  addressed  in  two  impor-ant  documents  published  in  2020:  the  ESC  guidelines  onports  cardiology9 and  the  American  College  of  Cardiol-gy/American  Heart  Association  guidelines  on  HCM.10 Bothf  these  guidelines,  albeit  cautiously  and  tentatively,  openhe  door  for  the  first  time  to  the  possibility  of  patients  withCM  being  able  to  exercise  beyond  Mitchell  class  IA  --  ailestone  and  a  paradigm  shift  in  the  approach  to  theseatients.Is  competitive  physical  exercise  always  contraindicatedn  these  patients?  No,  a  less  rigid  approach  is  now  rec-mmended,  with  individualized  risk  stratification,  and,  inelected  low-risk  individuals,  participation  in  specific  com-etitive  sports  may  be  considered.onclusionydney  Burwell’s  provocative  and  speculative  statement  fitsCM  like  a  glove.  Over  the  course  of  this  work,  we  havehown  how  some  30-year-old  concepts  have  substantiallyhanged,  due  to  major  scientific  advances  in  the  knowledgef  this  fascinating  disease.onflicts of interesthe  author  is  an  advisoy  board  member  of  Bristol  Myersquibb  and  Cytokinetics. IN+ModelRrdiolR1ARTICLEEPC-2310; No. of Pages 3Revista  Portuguesa  de  Caeferences1. Marón BJ, Rowin EJ, Casey SA, et al. How hypertrophic car-diomyopathy became a contemporary treatable genetic diseasewith low mortality: shaped by 50 years of clinical research andpractice. JAMA Cardiol. 2016;1:98--105 [PMID: 27437663].2. Marón BJ, Shen W-K, Link MS, et al. Efficacy of implantablecardioverter-defibrillators for the prevention of sudden deathin patients with hypertrophic cardiomyopathy. N Engl J Med.2000;342:365--73.3. Marón MS, Rowin EJ, Wessler BS, et al. Enhanced AmericanCollege of Cardiology/American Heart Association strategy forprevention of sudden cardiac death in high-risk patients withhypertrophic cardiomyopathy. JAMA Cardiol. 2019;4:644--57[PMID: 31116360].4. Vriesendorp PA, Liebregts M, Steggerda RC, et al. Long-termoutcomes after medical and invasive treatment in patientswith hypertrophic cardiomyopathy. J Am Coll Cardiol HF.2014;2:630--6.5. Olivotto I, Oreziak A, Barriales-Villa R, et al. Mavacamtenfor treatment of symptomatic obstructive hypertrophic car-diomyopathy (EXPLORER-HCM]: a randomised, double-blind,placebo-controlled, phase 3 trial. Lancet. 2020;396:759--69[Epub 29.08.20].6. McDonagh T, Metra M, Adamo M, et al. 2021 ESC Guidelinesfor the diagnosis and treatment of acute and chronic heart3 PRESSogia  xxx  (xxxx)  xxx--xxxfailure: developed by the task force for the diagnosis and treat-ment of acute and chronic heart failure of the European Societyof Cardiology (ESC) with the special contribution of the HeartFailure Association (HFA) of the ESC. Eur Heart J. 2021;42:3599--726.7. Arbelo E, Protonotarios A, Gimeno J, et al. 2023 ESC Guide-lines for the management of cardiomyopathies: developedby the task force on the management of cardiomyopathiesof the European Society of Cardiology (ESC). Eur Heart J.2023;44:3503--626.8. Drezner JA, Malhotra A, Prutkin JM, et al. Return to play withhypertrophic cardiomyopathy: are we moving too fast? A criticalreview. Br J Sports Med. 2021;55:1041--7 [Epub 20.01.21; PMID:33472848; PMCID: PMC8408577].9. Pelliccia A, Fagard R, Bjornstad HH, et al. Recommendations forcompetitive sports participation in athletes with cardiovasculardisease: a consensus document from the Study Group of SportsCardiology of the Working Group of Cardiac Rehabilitation andExercise Physiology and the Working Group of Myocardial andPericardial Diseases of the European Society of Cardiology. EurHeart J. 2005;26:1422--45.0. Ommen SR, Mital S, Burke MA, et al. 2020 AHA/ACC Guidelinefor the diagnosis and treatment of patients with hypertrophiccardiomyopathy: a report of the American College of Cardiol-ogy/American Heart Association Joint Committee on ClinicalPractice Guidelines. Circulation. 2020;142:e533--57.

Paradigm shifts in the last 30 years (Part 2)

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Paradigm shifts in the last 30 years (Part 2)

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