How the new ESC Guidelines on cardiomyopathies will change my practice?

Part 1: Diagnosis and management of hypertrophic cardiomyopathy

Part 2: Prevention of Complications

Speaker: Perry Elliott (UK)
Moderator: Giuseppe Limongelli (IT)

Our two experts discuss the implementation of the latest ESC Guidelines on Hypertrophic cardiomyopathies into daily practice through clinical cases: Diagnosis and management of hypertrophic cardiomyopathy and Prevention of complications

The faculty will discuss the following issues:

• Update the audience on  the diagnosis of hypertrophic cardiomyopathies (HCM).  
• How to apply the recent ESC guidelines on
  HCM in clinical practice.
• How to estimate the 5 years risk of sudden death in patients with HCM.
• Understand the principles behind the management of heart failure symptoms in patients with HCM

Learn more by reading the "Questions and Answers" with comments from our experts.

The ESC Webinars are designed to help you improve your daily practice!

The format includes a case based presentation, online assessment and live discussions with the 2 key opinion leaders who will give you some useful tips for your daily clinical practice.

Your questions answered by the experts

• At what age do you start screening the children of affected parents?
  In most cases, you do not need to start the screen before 8-10 years old. In high risk families (i.e. sudden death at very young age, malig-nant/compound mutations), you may consider to start the screening earlier
• How the asymetric form of HCM is defined. Only by comparing septum and posterior wall or any other segment? Is it ratio 1:3 or 1:5 right?
  The definition of ASH requires a septal/posterior wall thickness ratio of >1.5. However, a ratio >1.3 is considered valid when the septal thickness is increased.
• Is the sigmoid septum deformity seen in the elderly a form of hypertrophic cardiomyopathy?
  A sigmoid septum deformity can be seen in the elderly, in the absence of hypertrophic cardiomyopathy. The differential diagnosis is generally based on the severity of LVH, presence/absence of associated hypertensions, negative family history (and family screening).
• Is the hypertrophic segment in the same region in relatives with the same mutation or can one fam memb have hyperprophia in the apex asnd another in th septum with the same genotype?
  There is an extreme heterogeneity in HCM, also among members of the same family (“Intrafamilial”). Patients with the same mutation may also present different phenotypes (e.g. HCM and LVNC; HCM and RCM). Enviromental and genetic modifiers, as well as epigenetic modifiers are likely to be implicated.
• Why does cardiac amyloid's ECG have the characteristic pathognomic feature of pseudo-anterior infarct and reduced limb lead voltage?
  The precise cause of the ECG abnormalities in amyloidosis are not always clear. The absence of hypertrophy is explained by the fact that cardiomyocyte volume is not increased. Low voltages may relate to the loss of cardiomyocytes with progressive disease and pericardial effusions. The pseudoinfact pattern may relate to regional deposition of amyloid fibrils.
• Should we perform cardiac MRI in every patient with confirmed HCM?
  The ESC guidelines recommend CMR in patients when resources are available. The test is complementary to ECG and echo.
• Is interatrial septum affection + LVH characteristic for amyloidosis?
  Thickening of the interatrial septum and valves is common in amyloidosis but is non-specific. If present, other signs and symptoms should be taken into account.
• Should we follow ESC or US guidelines about ICD in patients with septums > 30 mm?
  The ESC recommends use of the ESC SD risk score as it is based on a robust statistical model derived and validated in the biggest cohort describe in literature. However, caution should be used in patients with extreme hypertrophy (≥35 mm) as this group are unrepresented in most series of patients
• What about abdominal fat biopsy?
  It is indicated in suspected AL amyloidosis but not TTR.
• Which is the role of cardiac MRI in prognostic stratification of patients with HCM?
  The ESC taskforce evaluated the current literature on the association between sudden death and gadolinium enhancement and felt that there was insufficient evidence to show that it is an independent predictor for sudden cardiac death.
• In a patient without symptoms having slight hypertrophy 12-15mm on echo without HTA and no family history should one do CMR?
  In non athletic, non hypertensive individuals having slight hypertrophy (12-15mm), there are a number of tools/parameters which may be used, including family history/screening, personal history (i.e. exclude steroids use, doping, etc.), ECG, BNP/proBNP, cardiopulmonary stress test. Imaging may help, since a combination of normal diastolic function, no LVOT gradient, a normal mitral valve apparatus and a normal left atrium, is rare in HCM. CMRI may be of help to detect hypertrophy, aneurysms/crypts, and scar.
• Would you allow a football player who has apical CMO to continue playing?
  In general, competitive sports activity should be discouraged in HCM. This is independent from the pattern/distribution of LVH. However, in patients at low risk for cardiac complications (low SD risk score, no symptoms, no ventricular arrhythmias, small LA, no compound muta-tions, low degree LGE at cardiac MRI, normal BP at stress test), aerobic sports (i.e. running, swimming, etc) may be considered case by case, taking into account a national law/rules, psychological and long term physical implications, and with 6-12 months follow-up visits.
• Can you use Novel Oral Anticoagulants in patients with HCM + Atrial Fibrilation?
  There are no data on NOAC in HCM but the ESC guideline allows their use when patients are unwilling or unable to take warfarin.
• What antiarrhythmic drug is most appropriate to prevent recurence of AF and what is contrandicated?
  In general, sotalol or amiodarone are recommended for prevention/rate control in patients with HCM. Routine QT length evaluation should be advised. Class I agents should be used with caution in HCM.
• Why is this patient on AAS?
  The patient was started on aspirin prior to referral on the assumption that he has coronary disease.
• Did you include the Left Atrium diameter (and not the left atrium volume index) in the calculator for SCD estimation?
  The data on which the HCM-SCD risk score is based were collected over a 20 year period. Left atrial diameter was the most complete data-set available although we recognize that atrial area or volume is the modern standard.
• Can we use fibrosis (in CMR) for risk stratification? What are the cutoff values for increased risk?
  The ESC taskforce evaluated the current literature on the association between sudden death and gadolinium enhancement and felt that there was insufficient evidence to show that it is an independent predictor for sudden cardiac death.
• The competitive sports activities in patients with hypertrophic cardiomyopathy is always contraindicated or there are exceptions in special cases? And if yes in which cases and how to behave?
  In clinical practice cardiologists must always make a judgement based on the patients symptoms and clinical risk profile, but the new ESC guideline recommends avoidance of competitive exercise in patients with HCM.
• What level of INR is recommended in patients with hypertrophic cardiomyopathy after restoring sinus rhytm receiving warfarin life-long?
  We recommend and INR of 2-3
• How to classify angina functional class in patients with hypertrophic cardiomyopathy?
  In the guideline, we used the Canadian Cardiovascular Society (CCS) Chest Pain Score.
• LA chamber size normal ranges are different for males and females. Why isn't sex taken into account?
  This is an important point but most data suggest that sex correction does not alter the association between LA chamber and risk.
• Did patient have BB a the time of echo?
  yes
• It is clear that the sudden death risk es very high but do we need to do something with the LVOTO
  The ESC guideline recommends invasive treatment of left ventricular outflow tract gradients only when they are associated with drug refractory symptoms of chest pain and breathlessness.
• Is there a place for exercise testing for predicting suden cardiac death?
  The new HCM RISK-SCD score does not include exercise blood pressure as a risk factor because it has never been shown to be an independent predictor. However, the guideline suggests that blood pressure response should be included in the overall assessment of risk, particularly in young adults.
• Do Late Potentials play a role in risk stratification?
  At present, there are insufficient data on late potentials to recommend their use in HCM.
• What is the role of myocardial fibrosis on MRI in HCM patients
  The ESC taskforce evaluated the current literature on the association between sudden death and gadolinium enhancement and felt that there was insufficient evidence to show that it is an independent predictor for sudden cardiac death.
• When you do stress echo for HCM do you do it on the beta blocker (or CCB)- usually at high dose and how does this affect interpretation?
  Ideally, exercise stress echo should be performed off ß-blockers.
• What about Therapy if You could not implanted ICD?
  Amiodarone has been shown to reduce the incidence of death in pa-tients with non-sustained ventricular tachycardia in a single study, but the literature shows that sudden deaths still occur in patients treated with the drug. For this reason, ICDs are recommended for patients deemed to be at high risk of sudden cardiac death.
• And what about abnormal BP response?
  The new HCM RISK-SCD score does not include exercise blood pressure as a risk factor because it has never been shown to be an independent predictor. However, the guideline suggests that blood pressure response should be included in the overall assessment of risk, particularly in young adults.
• What about the BP drop in the exercise test? Thank you!
  The new HCM RISK-SCD score does not include exercise blood pressure as a risk factor because it has never been shown to be an independent predictor. However, the guideline suggests that blood pressure response should be included in the overall assessment of risk, particularly in young adults.
• Do you think there is a prognostic difference between pts who have only short NSVT (3 beat) and a more prolonged NSVT?
  There is no evidence to show that the frequency, duration or rate of NSVT influences its association with SCD.
• Is the use of verapmail/diltiazem in patients who continue have LVOT obstruction but also have developed systolic HF
  LVOT obstruction tends to reduce as systolic performance declines. There may be rare occasions when this is not the case but ß-blockers are the preferred option should this occur.
• The role of mechanlical dispersion ( time to peak strain) as a predictor) for SD in HCM ?
  There are insufficient data to support the use of this parameter in risk assessment.
• If we can not detect a NSVT in holter device but still our patient suffers from syncopal episodes how do we proceed?
  It important to exclude provocable left ventricular outflow tract ob-struction in such cases. As unexplained non-vasovagal syncope is a risk factor for sudden cardiac death, treatment with a prophylactic implantable cardioverter defibrillator (ICD) may be appropriate in individuals with other features indicative of high sudden death risk. When there are no such features, the ESC guideline recommends consideration of an implantable loop recorder.
• Which is the role of dysopiramide nowawadays?
  Disopyramide is recommended as a second-line drug in the treatment of left ventricular outflow tract obstruction. Ideally it should be combined with ß-blocker or verapamil

This programme is supported by Bayer HealthCare Pharmaceuticals, Bristol-Myers Squibb and Pfizer Alliance, Daiichi Sankyo Europe GmbH, Novartis Pharma AG and Servier in the form of unrestricted educational grants.  The scientific programme has not been influenced in any way by its sponsors.