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Management of HOCM
- 1. HOCM MANAGEMENT -DR. ROHITWALSE SENIORRESIDENT DM CARDIOLOGY SCTIMST
- 2. SCOPE OF DISCUSSION •INTRODUCTION • RISK STRATIFICATION & ROLE OF ICD • PHARMACOLOGICAL RX, INCLUDING AF AND PREGNANCY ISSUES • INVASIVETREATMENT ALCOHLIC SEPTAL ABLATION Sx MYOMECTOMY
- 3. INTRODUCTION • HISTORY: 1STPatient – Mr. Claude Brady (1959) • Age of presentation- Birth to 90 yrs • Sex predilection: M~F (M>F) • Older Annual mortality rates- 4% to 6% • Overall HCM-related mortality rates-1%/yr • Somewhat higher in children -2%/yr.
- 4. • HCM isan inherited disease of heart muscle disease with variable clinical expression and natural history. • Characterised by hypertrophy of left ventricular walls. • Sudden cardiac death –common cause of mortality. • Can be effectively treated with implantable cardioverter defibrillator.
- 5. European Heart Journal(2014):doi:10.1093/eurheartj/ehu284 HCM: Definitions Increased left ventricular wall thickness not solely explained by abnormal loading conditions ADULTS: ● LV wall thickness ≥15 mm in one or more LV myocardial segments measured by any imaging technique CHILDREN: ● LV wall thickness more than two standard deviations above the predicted mean (z-score >2)
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- 8. JACCVol. 58, No.25, 2011 Gersh et al. December 13/20, 2011:e212–60 ACCF/AHA Hypertrophic Cardiomyopathy Guideline
- 9. Dai-Yin et al,Clinical Outcomes in Patients With Non obstructive, Labile, and Obstructive Hypertrophic Cardiomyopathy, Journal of the American Heart Association March 6, 2018,Vol 7, Issue 5
- 11. RISK STRATIFICATION • Survivalfrom a cardiac arrest due toVT orVF • Spontaneous sustainedVT causing syncope or hemodynamic compromise • F/H/O SCD associated with HCM • LV wall thickness > 30 mm • Unexplained syncope within 6 months • NSVT > 3 beats • Abnormal BP response during exercise Established risk factors: Heart Rhythm,Vol 15, No 10, October 2018
- 12. RISK STRATIFICATION • <30years • Delayed hyperenhancement on cardiac MRI • LVOT obstruction • Syncope >5 y ago • LV aneurysm • LVEF <50% Potential risk modifiers: High risk subsets: Heart Rhythm,Vol 15, No 10, October 2018
- 13. CLASS I RECOMMENDATIONS •To perform risk stratification at the time of initial evaluation and periodically thereafter. • ICD is recommended for those who have survived a SCA due toVT orVF, or have spontaneous sustainedVT causing syncope or hemodynamic compromise, an if meaningful survival > 1 year is expected Heart Rhythm,Vol 15, No 10, October 2018
- 14. CLASS II aRECOMMENDATIONS • In patients with HCM who have spontaneous NSVT or an abnormal blood pressure response with exercise, who also have additional SCD risk modifiers or high-risk features, an ICD is reasonable if meaningful survival greater than 1 year is expected • Inpatients with HCM and 1 or more of the following risk factors, an ICD is reasonable if meaningful survival of greater than 1 year is expected: • Maximum LV wall thickness ‡30 mm • SCD in 1 or more first-degree relatives presumably caused by HCM • 1 or more episodes of unexplained syncope within the preceding 6 months Heart Rhythm,Vol 15, No 10, October 2018
- 15. CLASS II bRECOMMENDATIONS • In patients with HCM who have NSVT or an abnormal blood pressure response with exercise but do not have any other SCD risk modifiers, an ICD may be considered, but its benefit is uncertain. • In patients with HCM and a history of sustainedVT orVF, amiodarone may be considered when an ICD is not feasible or not preferred by the patient . Heart Rhythm,Vol 15, No 10, October 2018
- 16. SUMMARY Heart Rhythm,Vol 15,No 10, October 2018
- 17. European Heart Journal(2014):doi:10.1093/eurheartj/ehu284
- 18. European Heart Journal(2014):doi:10.1093/eurheartj/ehu284 Prevention of Sudden Cardiac Death Recommendations for ICD in each risk category take into account not only the absolute statistical risk, but also the age and general health of the patient, socio-economic factors and the psychological impact of therapy.
- 19. European Heart Journal(2014):doi:10.1093/eurheartj/ehu284 CMR and Sudden Death Risk “On balance, the extent of LGE on CMR has some utility in predicting cardiovascular mortality, but current data do not support the use of LGE in prediction of SCD risk.”
- 20. • Clear benefitto patients being treated for secondary prevention. • However, the appropriate therapy rate for primary prevention was lower than previously reported. • No single risk factor appeared to have stronger association with appropriate ICD therapy than others.
- 22. European Heart Journal(2014):doi:10.1093/eurheartj/ehu284 Atrial Fibrillation: ● Use of the CHA DS -VASc score to calculate stroke risk is NOT recommended. 2 2 ● In general, lifelong therapy with oral anticoagulants is recommended, even when sinus rhythm is restored. ● As left atrial size is a consistent predictor for AF and stroke in patients with HCM, patients in sinus rhythm with LA diameter ≥45mm should undergo 6–12 monthly 48-hour ambulatory ECG monitoring to detect AF.
- 23. European Heart Journal(2014):doi:10.1093/eurheartj/ehu284 Maternal Risk
- 24. European Heart Journal(2014):doi:10.1093/eurheartj/ehu284 Reproduction & Contraception
- 25. Asymptomatic Patients • Largeproportion are asymptomatic & most will achieve a normal life expectancy. • Educate patient • Screening of 1st -degree relatives • Avoiding strenuous activity . • Risk stratification for SCD Watchful waiting is often appropriate
- 26.
- 27. European Heart Journal(2014):doi:10.1093/eurheartj/ehu284 Treatment of Left Ventricular Outflow Tract Obstruction By convention, LVOTO is defined as a peak instantaneous Doppler LV outflow tract gradient of ≥30 mm Hg, but the threshold for invasive treatment is usually considered to be ≥50 mm Hg. There are no data to support the use of invasive procedures to reduce LV outflow obstruction in asymptomatic patients, regardless of its severity.
- 28. European Heart Journal(2014):doi:10.1093/eurheartj/ehu284 Treatment of LV Outflow Tract Obstruction
- 29. European Heart Journal(2014):doi:10.1093/eurheartj/ehu284 Drug treatment of LVOTO ß -blockers Verapamil (Diltiazem) Disopyramide Diuretics FIRST LINE SECOND LINE
- 30. MedicalTherapy • Beta-blockers • Increaseventricular diastolic filling/relaxation • Decrease myocardial oxygen consumption • Have not been shown to reduce the incidence of SCD • Verapamil • Augments ventricular diastolic filling/relaxation • Disopyramide • Used in combination with beta-blocker • Negative inotrope • Diuretics
- 32. RANOLAZINE • Late sodiumchannel current inhibitor • Reduces diastolic dysfunction, microvascular ischemia, ventricular arrhythmias, or severity of left ventricular outflow obstruction.
- 33. • Clinically significant improvementin angina and HF symptoms. • Improved quality of life.
- 34. • 80 adultpatients with non-obstructive HCM • No overall effect on exercise performance, plasma pro-BNP levels, diastolic function, or quality of life • Excellent safety profile and reduced premature ventricular complex burden
- 36. • The totalityof the data did not support continuation of the Eleclazine development program. • So, this study was terminated in March 2018 prior to the end of the double-blind phase.
- 37.
- 38. Surgical Septal Myectomy NishimuraRA et al. NEJM. 2004. 350(13):1320.
- 39. SurgicalTherapy • Transaortic septalmyectomy is currently considered most appropriate treatment for majority of patients with obstructive HCM & severe symptoms unresponsive to medical therapy . • Traditional myectomy (Morrow procedure) with about a 3-cm long resection(Tips of MV) • Extended myectomy (a resection of about 7 cm)(upto apex). • RPRrepair- (R) resection of septum, (P) plication of anterior leaflet of mitral valve, & (R) release of abnormal papillary muscle attachments.
- 41. Mechanism… • LVOT gradientreduction with myectomy results from basal septal thinning with resultant enlargement of LVOT area (and redirection of forward flow with loss of the drag & Venturi effects on mitral valve)& consequently abolition of SAM & mitral-septal contact. • MR is also usually eliminated without need for additional MV surgery. • With myectomy, LA size, risk forAF is reduced, & LV pressures & wall stress are normalized.
- 44. Alcohol Septal Ablation(ASA) • A 68-year-old lady, unresponsive to DDD pacemaker & optimal medical therapy for HOCM, agreed to become first patient for ASA for Dr Sigwart 1994 who initially noticed that significant reduction in LVOT gradient when angioplasty balloon was inflated in 1st septal artery, • This was further supported by disappearance of typical auscultatory findings, & echo manifestations of obstruction of HOCM following MI • However it took a decade for ethical clearance for this revolutionary idea of instilling alcohol & producing a controlled infarction.
- 45. Patient selection …. •Clinical: NYHA III or IV-despite optimal medical therapy • Hemodynamic: Dynamic LVOT gradient at rest or with physiologic provocation 50 mm Hg associated with septal hypertrophy & SAM of mitral valve • Anatomic:Targeted sufficient anterior septal thickness Avoided if septal thickness <18 mm
- 46. Alcohol Septal Ablation(ASA)…Technique • Contrast angiography of septal perforator through balloon central lumen with simultaneous echo Guidance confirms delivery to only target myocardium. • A short (∼10mm) OTW balloon is advanced into septal artery, balloon material should not disintegrate on exposure, should be at least equal or slightly bigger than septal artery (2- 2.5 mm) • About 1 -3 mL of alcohol is infused in controlled fashion. • It is important that balloon be inflated & that a contrast injection also show that there is no extravasation of dye into distal LAD. • Contrast enhancement of other regions (papillary muscles, free wall) indicates collateral circulation from septal perforator artery, & alcohol should not be infused.
- 47. Alcohol Septal Ablation Braunwald.Atlas of Heart Diseases: Cardiomyopathies, Myocarditis, and Pericardial Disease. 1998.
- 48.
- 49. LVOTO gradient showsa triphasic response followingASA. • Stage 1: Stunning phase:There is immediate decrease in gradient following ASA. Stunning of septum. • Stage 2: Edema phase: There might be some increase in LVOTO due to peri-infarction edema.This is reason for recurrence of gradient during discharge time. Lasts for 5–10 days after procedure. • Stage 3: Scar phase: LVOTO gradually decreases, as scar forms over wks -months & septum becomes thinner gradually. This stage lasts for 3–12 months after ASA.Thus, accurate success of ASA can only be determined after 3 months.
- 50. Alcohol septal ablation: Advantages Greater patient satisfaction : • Absence of a surgical incision & GA • Less overall discomfort • Much shorter recovery time. Selective advantage in older patients.
- 51. Complications • Temporary CHB50% occurs during procedure. • Persistent CHB prompting permanent pacemaker occurs in 10% - 20% • 5% of patients have sustainedVT during hospitalization. • In-hospital mortality rate is up to 2%.(0-4%) • Because of potential for creating aVSD, septal ablation should not be performed if target septal thickness is < 18 mm. • LAD dissection, remote infarction, ventricular fibrillation, stroke, pericardial effusion, are relatively uncommon complications.
- 52. JOURNAL OFTHE AMERICANCOLLEGE OF CARDIOLOGYVOL. 72, NO. 24, 2018
- 53. JOURNAL OFTHE AMERICANCOLLEGE OF CARDIOLOGYVOL. 72, NO. 24, 2018
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- 55. • Long-term mortalityand (aborted) SCD rates after ASA and myectomy are similarly low. • Patients who undergoASA have more than twice the risk of permanent pacemaker implantation and a 5 times higher risk of the need for additional septal reduction therapy compared with those who undergo myectomy.
- 58. • There wereno differences in survival of patients undergoing myectomy or alcohol septal ablation, but freedom from reintervention and early and late reduction of left ventricular outflow tract gradient are superior in patients undergoing septal myectomy.
- 61.