Left ventricular outflow tract obstruction: indications and limitations of current therapies

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1 Left ventricular outflow tract obstruction: indications and limitations of current therapies Costas O Mahony Inherited Cardiovascular Diseases Unit, The Heart Hospital, London, UK.

2 None to declare Conflicts of interest

3 Overview Obstruction in HCM Pathophysiology Consequences Pharmacological treatment Invasive treatment Myectomy Alcohol Septal Ablation Pacing

4 LVOTO

5 Dynamic LVOTO

6 Latent LVOTO 87 symptomatic patients No LVOTO at rest/valsalva Medication stopped LVOTO with exercise 54 (62%) No LVOTO 33 (28%) Shah J et al.heart 2008

7 Mid cavity obstruction

8 Long MV leaflets

9 Intrinsic mitral valve disease

10 Assessment checklist 1 Conditions Rest Latent 2 Level LVOTO Mid cavity obstruction 3 Mitral valve Mitral valve morphology /pathology Papillary muscle morphology 4 LVH Distribution of LVH Severity of LVH

11 Consequences: symptoms Elliott P et al. EHJ 2006

12 LVOTO Rx: WHEN TO TREAT

13 Indications of therapy Symptomatic obstruction Establish mechanism(s) Correction of exacerbating factors Pharmacological treatment β-blockers Ca 2+ CB Disopyramide Septal Myectomy Invasive Treatment Alcohol Septal Ablation DDD pacing

14 LVOTO Rx: PHARMACOTHERAPY

15 Pharmacotherapy: mechanisms Negative inotropy O 2 demand β-blockers Verapamil Disopyramide Improved diastolic filling effect of catecholamine surge during exercise

16 Multicenter Study of the Efficacy and Safety of Disopyramide in Obstructive Hypertrophic Cardiomyopathy 4 centres 118 LVOTO pts FU ~4y No SCD 98% β-blocked ⅔ ⅓ Sherrid M et al. JACC 2005

17 Pharmacotherapy: pros and cons Inexpensive Readily available Good safety record Symptom improvement Side effects Bradycardia/AV disease Verapamil:LVF with PCWP Ineffective (>30% LVOTO pts)

18 LVOTO Rx: INVASIVE TREATMENTS

19 Septal myectomy Morrow A et al. Circ 1975

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21 Pre and post myectomy

22 Myectomy: pros and cons Excellent LVOTg reduction Excellent Sx improvement Rx MV/MCO Technical challenges Specialist peri-op care Limited availability VSD, CHB and AV injury Open heart surgery comp

23 Surgical variations Alfieri stitch Papillary muscle re-orientation Image courtesy of Dr D Sado, Heart Hospital Bhudia S et al. Ann Thor Surg 2004 Kwon DH. J Thor Cardio Surg 2010

24 Alcohol Septal Ablation

25 Septal anatomy Valeti J et al. JACC 2007

26 Pre and post ASA Pre-ASA: 116mmHg 2/12 post ASA: 22mmHg

27 ASA: pros and cons Good LVOTg reduction Good Sx improvement Widespread availability (potentially) Short recovery period Unfavourable septals May require repeat ASA Cannot address MV Risk of CHB/VF Vascular and TPW comp

28 Endocardial RF-SA Lawrenz T et al. JACC 2010

29 LVOTO RX: SEPTAL MYECTOMY VS ALCOHOL SEPTAL ABLATION

30 ASA Vs SM: Mortality

31 ASA Vs SM: NYHA, LVOTg, CHB

32 LVOTO Rx: DOES DDD PACING HAVE A PLACE?

33 DDD pacing with short AV delay Septal dysynchrony DDD pacing -ve inotropy Modification of MV excursion

34 DDD pacing: trials (and tribulations)

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36 DDD pacing: pros and cons LVOTg reduction Allows maximal medical Rx Widespread availability (potentially) Short recovery Opportunistic use with ICDs Inconsistent Sx relief No value in AFib Cannot address MV Complication rates

37 LVOTO: SURVIVAL

38 Consequences: prognosis Elliott P et al. EHJ 2006

39 Long-Term Effects of Surgical Septal Myectomy on Survival in Patients With Obstructive Hypertrophic Cardiomyopathy n? centres n? patients Mayo clinic 1337 HCM pts Myectomy: 289 pts LVOTO 50mmHg 3 other centres 820 pts LVOTO<30mmHg Conservative Rx: 228 pts LVOTO 30mmHg Myectomy was associated with improved HCM-related survival Non-consecutive pts Conservative arm was older Myectomy group had shorter follow-up Ommen R et al. JACC 2005

40 Long term survival in patients with resting obstructive hypertrophic cardiomyopathy 246 (38%) Conservative 649 adults LVOTO 30mmHg Asymptomatic Drug Rx Asymptomatic 46 (19%) Drug Rx Symptomatic 403 (62%) Invasive 287 (71%) SM 85 (21%) ASA 31 (8%) DDD Conservative arm was older, with co-morbid conditions and noncardiac deaths Improved NYHA and LVOTO in both arms Cons: 63 to 44mmHg Inva: 70 to 11mmHg Mean FU: 7.2y Invasive Rx: No effect on HCM related survival Ball W et al. JACC 2011

41 Conclusions Pharmacotherapy is effective in most patients Invasive treatment: ONLY if medical therapy fails Expert centres The mechanism of LVOTO determines the type of invasive therapy one-size fits all approach does not work Impact of treatment on survival is not clear

42 Ball W et al. JACC 2011

43 Latent LVOTO Shah J et al.heart 2008

44 Alternative approaches: old and new MVR Z Krajcer. Circ 1989;80:I57-I64 Alfieri stitch D Sado. Circ 2010;122; Papillary muscle re-orientation Kwon DH. J Thor Cardio Surg 2010;140: Radiofrequency septal ablation T Lawrenz. JACC 2010: 57;

45 LVOTO: MANAGEMENT OF ATYPICAL CASES

46 Pharmacotherapy: limitations β-blockers fatigue bronchoconstriction Verapamil constipation Disopyramide anti-muscarinic effects

47 LVOTO with minimal hypertrophy 70y female NYHA III LVOTO Rest 50mmHg Provocation 62mmHg LA 30cm 2

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49 LVOTO and MV pathology 56y male NYHA II MWT 15mm LVOTO Rest 64mmHg Moderate-severe MR (anterior)

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52 Small cavity/mco 37y male NYHA III Severe hypertrophy No LVOTO Mid cavity obstruction 30mmHg

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