The Management of HOCM: What are the Surgical Options

Transcription

1 The Management of HOCM: What are the Surgical Options Konstadinos A Plestis, MD System Chief of Cardiac Thoracic and Vascular Surgery Main Line Health Care System Professor Sidney Kimmel Medical College Thomas Jefferson University

2 HCM Genetic disorder Autosomal dominant Prevalence- 0.2% Myocardium Sarcomere proteins >1400 mutations, at least 8 genes Most common: β-myosin heavy chain or myosin-binding protein C gene

3 HOCM Symptoms Shortness of breath Chest pain Fainting Palpitations Heart murmur Exacerbated by exercise

4 Maron et al, Circulation, 1995

5 Maron et al, Am J Med, 2004

6 HOCM Natural History SCD due to unpredictable ventricular tachyarrhythmias, most commonly in asymptomatic patients <35 years of age Heart failure characterized by exertional dyspnea (with or without chest pain) that may be progressive AF, also associated with various degrees of heart failure and an increased risk of systemic thromboembolism and stroke

7 HOCM Pathophysiology LVOT obstruction Outflow: SAM and mitral-septal contact Midcavitary: hypertrophied papillary muscles or anomalous muscle insertion into the anterior mitral leaflet Diastolic dysfunction Mitral regurgitation Myocardial ischemia Arrhythmias

8 HOCM Screening Genetic testing ECG Echocardiography Cardiac catheterization Cardiac MRI

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10 HOCM Genetic Testing B 1. Evaluation of familial inheritance and genetic counseling is recommended as part of the assessment of patients with HCM. 2. Patients who undergo genetic testing should also undergo counseling by someone knowledgeable in the genetics of cardiovascular disease so that results and their clinical significance can be appropriately reviewed with the patient. 3. Screening (clinical, with or without genetic testing) is recommended in firstdegree relatives of patients with HCM. 4. Genetic testing for HCM and other genetic causes of unexplained cardiac hypertrophy is recommended in patients with an atypical clinical presentation of HCM or when another genetic condition is suspected to be the cause.

11 HOCM Electrocardiography B 1. A 12-lead ECG is recommended in the initial evaluation of patients with HCM. 2. Twenty-four hour ambulatory (Holter) electrocardiographic monitoring is recommended in the initial evaluation of patients with HCM to detect ventricular tachycardia (VT) and identify patients who may be candidates for ICD therapy. 3. Twenty-four hour ambulatory (Holter) electrocardiographic monitoring or event recording is recommended in patients with HCM who develop palpitations or lightheadedness.

12 HOCM Electrocardiography C 1. A repeat ECG is recommended for patients with HCM when there is worsening of symptoms. 2. A 12-lead ECG is recommended every 12 to 18 months as a component of the screening algorithm for adolescent first-degree relatives of patients with HCM who have no evidence of hypertrophy on echocardiography. 3. A 12-lead ECG is recommended as a component of the screening algorithm for first-degree relatives of patients with HCM.

13 HOCM Echocardiography B 1. A TTE is recommended in the initial evaluation of all patients with suspected HCM. 2. A TTE is recommended as a component of the screening algorithm for family members of patients with HCM unless the family member is genotype negative in a family with known definitive mutations. 3. Repeat TTE is recommended for the evaluation of patients with HCM with a change in clinical status or new cardiovascular event. 4. A transesophageal echocardiogram (TEE) is recommended for the intraoperative guidance of surgical myectomy.54-56(level of Evidence: B) 5. TTE or TEE with intracoronary contrast injection of the candidate's septal perforator(s) is recommended for the intraprocedural guidance of alcohol septal ablation.

14 HOCM Echocardiography C 1. Periodic (12 to 18 months) TTE screening is recommended for children of patients with HCM, starting by age 12 years or earlier if a growth spurt or signs of puberty are evident and/or when there are plans for engaging in intense competitive sports or there is a family history of SCD. 2. TTE should be used to evaluate the effects of surgical myectomy or alcohol septal ablation for obstructive HCM.

15 HOCM Cardiac Magnetic Resonance B 1. CMR imaging is indicated in patients with suspected HCM when echocardiography is inconclusive for diagnosis. 2. CMR imaging is indicated in patients with known HCM when additional information that may have an impact on management or decision making regarding invasive management, such as magnitude and distribution of hypertrophy or anatomy of the mitral valve apparatus or papillary muscles, is not adequately defined with echocardiography.

16 HOCM Treatment Medications Beta blockers Calcium channel blockers Disopyramide Alcohol septal ablation Surgical septal myectomy Mitral clip Implantable pacemaker or defibrillator Cardiac transplantation

17 HOCM Treatment Medications Beta blockers Calcium channel blockers Disopyramide

18 HOCM Beta blockers B Recommended for the treatment of symptoms (angina or dyspnea) in adult patients with obstructive or nonobstructive HCM but should be used with caution in patients with sinus bradycardia or severe conduction disease If low doses of beta-blocking drugs are ineffective for controlling symptoms (angina or dyspnea) in patients with HCM, it is useful to titrate the dose to a resting heart rate of less than 60 to 65 bpm (up to generally accepted and recommended maximum doses of these drugs)

19 HOCM Verapamil B Recommended (starting in low doses and titrating up to 480 mg/d) for the treatment of symptoms (angina or dyspnea) in patients with obstructive or nonobstructive HCM who do not respond to beta-blocking drugs or who have side effects or contraindications to beta-blocking drugs. However, verapamil should be used with caution in patients with high gradients, advanced heart failure, or sinus bradycardia

20 HOCM Septal oblation therapy C should be performed only by experienced operators in the context of a comprehensive HCM clinical program and only for the treatment of eligible patients with severe drug-refractory symptoms and LVOT obstruction C Consultation with centers experienced in performing both surgical septal myectomy and alcohol septal ablation is reasonable when discussing treatment options for eligible patients with HCM with severe drug-refractory symptoms and LVOT obstruction

21 HOCM Surgical Septal Myectomy B When performed in experienced centers, can be beneficial and is the first consideration for the majority of eligible patients with HCM with severe drug-refractory symptoms and LVOT obstruction

22 HOCM Pacemaker A In patients with HCM who have had a dualchamber device implanted for non-hcm indications, it is reasonable to consider a trial of dual-chamber atrial-ventricular pacing (from the right ventricular apex) for the relief of symptoms attributable to LVOT obstruction

23 HOCM Pacemaker B Patients with advanced heart failure (endstage ) and nonobstructive HCM not otherwise amenable to other treatment interventions, with EF less than 50% (or occasionally with preserved EF), should be considered for heart transplantation

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25 Variants of hypertrophic cardiomyopathy A B C D A- Normal B- Basal C- Midventricular D- Apical Kotkar et al Ann Cardiothorac Surg 2017

26 Transaortic Septal Myectomy

27 Ralph-Edwards et al, Annals CTS 2017

28 Ralph-Edwards et al, Annals CTS 2017

29 Ralph-Edwards et al, Annals CTS 2017

30 Final distal division made with scissors. Ralph-Edwards et al, Annals CTS 2017

31 Ralph-Edwards et al, Annals CTS 2017

32 Ralph-Edwards et al, Annals CTS 2017

33 Ralph-Edwards et al, Annals CTS 2017

34 LANKENAU HEART INSTITUTE Case Presentation 65 yo male LV outflow obstruction Systolic Anterior Motion of the Leaflet Mitral Regurgitation Aortic Stenosis

35 LANKENAU HEART INSTITUTE PMH Cerebrovascular Accident Hypertension Hypercholesterolemia

36 LANKENAU HEART INSTITUTE Operation Mini-sternotomy Septal Myomectomy MV Repair plication of the anterior leaflet

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38 Discharge Hospital Course Uncomplicated

39 LANKENAU HEART INSTITUTE Case Presentation 48 yo Female Debilitating shortness of breath Progressively worsening symptoms Intermittent chest pain

40 LANKENAU HEART INSTITUTE Echocardiogram Marked concentric LV hypertrophy Resting LVOT gradient > 60 mm Hg No systolic anterior motion of the mitral valve

41 TTE

42 TTE

43 TEE

44 TEE

45 TEE

46 LANKENAU HEART INSTITUTE Cardiac MRI Concentric left ventricular hypertrophy Maximal LV wall thickness of 2.8 cm Hypertrophy of the papillary muscles Normal RV function

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49 Long segment septal hypertrophy involving both the subaortic and midventricular regions Hang et al. Annals of Cardiothoracic Surgery 2017

50 Transapical Septal Myectomy

51 The location of the apical myectomy incision, lateral to the LAD Kotkar et al, Annals of CTS, 2017

52 The left ventricular apex is obliterated by hypertrophied muscle, and care should be taken not to injure the papillary muscles and mitral valve apparatus. Kotkar et al, Annals of CTS, 2017

53 The main focus of the procedure is on the ventricular septum Kotkar et al, Annals of CTS, 2017

54 The papillary muscles and the chordae are visualized after the myectomy has been completed The papillary muscles can be shaved to further increase left ventricular volume and reduce risk of midventricular obstruction Kotkar et al, Annals of CTS, 2017

55 Management Surgical Myectomy Transaortic Transapical

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60 Postoperative Course Uncomplicated Extubated on POD # 1 Discharged on POD # 7

61 Surgical Myectomy Misfeld 2017 (n=115) Vanderlaan 2017 (n=150) Sedehi 2014 (n=171) Michels 2014 (n=253) Yao 2016 (n=139) Age Male 52% 62% 49% 54% 37% SAM 74% 50% 85% N/A N/A MV Regurgitation (mod+sev) 38% 53% N/A N/A N/A

62 Surgical Myectomy Misfeld 2017 (n=115) Vanderlaan 2017 (n=150) Sedehi 2014 (n=171) Yao 2016 (n=139) CPB CC N/A

63 Surgical Myectomy Misfeld 2017 (n=115) Vanderlaan 2017 (n=150) Yao 2016 (n=139) MV Repair MV Replacement CABG AV Replacement Isolated Myectomy 30% 0 12% 23% 0 5% 6% 0 8% 3% 0 3% 18% 100% N/A

64 Surgical Myectomy Misfeld 2017 (n=115) Vanderlaan 2017 (n=150) Sedehi 2014 (n=171) Michels 2014 (n=253) Yao 2016 (n=139) Stroke (%) N/A 2% 0% N/A N/A Mortality (%) 6.9% 0.7% 2.9% 1.2% 0%

65 Long Term Survival Surgical Myectomy Misfeld 2017 (n=115) Sedehi 2014 (n=171) Michels 2014 (n=253) Yao 2016 (n=139) 1 year 5 years 90% 100% 89% 93% 92% 98% 10 years 80% 20 years 81% 85% 47%

66 Survival free from all-cause mortality after surgical myectomy Ommen et al, 2005 JACC

67 Survival free from hypertrophic cardiomyopathy-related death Ommen et al, 2005 JACC

68 LANKENAU HEART INSTITUTE Conclusion Surgical myectomy produces long term excellent results Experience is necessary to determine the depth and extend of resection Appropriate selection of the procedure

69 Literature Review

70 LANKENAU HEART INSTITUTE Thank You

71 Variants of hypertrophic cardiomyopathy A B C D A- Normal B- Basal C- Midventricular D- Apical Kotkar et al Ann Cardiothorac Surg 2017

72 The transaortic and transapical approach Hang et al. Annals of Cardiothoracic Surgery 2017

73 Operative steps of the current Mayo Clinic technique. An initial excision of the hypertrophied septum at the point of mitral-septal contact (i.e., white fibrous scar) in Panels A and B, followed by extension of the excision towards the apex of the left ventricle after adequate exposure in Panel C Hang et al. Annals of Cardiothoracic Surgery 2017

74 traditional Morrow technique utilizing parallel incisions into the hypertrophied septum followed by excision of the resulting bar of septal muscle

75 Long segment septal hypertrophy involving both the subaortic and midventricular regions Hang et al. Annals of Cardiothoracic Surgery 2017

76 Misfeld 2017 (n=115) El Khoury 2012 (n=475) Schafers 2010 (n=640) Holubec 2014 (n=100) Svensson 2014 (720) Preop Interventricular Wall Thickness Preop Interventricular Wall Thickness Preop LVOT Gradient Postop LVOT Gradient 20.9 mm 15.1 mm 70.1 mmhg 9.7 mmhg

77 Misfeld 2017 (n=115) Vanderla an 2017 (n=150) Partial Sternotomy 18%