Hocm mpuh

THIS IS CASE REPORT OF HOCM PATIENT,WHICH WAS FOR ORTHO.SURGERY. ANAESTHESIA CONDUCTED SUCCESSFULLY.

1. Dr. Nilesh Parikh MD,PDCC ( Card.Anaes ) Consultant Anaesthesiologist NADIAD Anaesthesia for HOCM patient

2. Hypertrophic Obstructive Cardiomyopathy Dr. Nilesh Parikh

3. Here is a patient named Kamlaben B Patel 85 yrs. Old ,having # N/F, requring DHS Surgery. pt. is a known case of HTN Since 15 yrs. and she is taking Tab Amlodepine-Atenolol combination. Patient has been investigated and diagnosed to have HOCM by 2D ECHO ., how would you proceed for this case?

4. Hypertrophic obstructive cardiomyopathy Overview Background Pathophysiology Clinical picture Diagnosis Treatment Anesthetic consideration 4

5. Hypertrophic Obstructive CardioMyopathy (HOCM) is a rare genetic disorder characterized by left ventricular outflow tract (LVOT) obstruction. Clinical presentation ranges from absence of symptoms to sudden death. These patients presents considerable challenges and requires maintenance of desired hemodynamic parameters and management of specific complications. Factors like Tachycardia, Hypovolemia , Vasodilation and increased cardiac contractility leads to exacerbation of the obstruction. Decrease in venous return and SVR or increase in myocardial contractility worsens the LVOT obstruction. These patients are highly prone to arrhythmias like AF & VT Management of anesthesia in these patients poses considerable challenges for the anesthesiologist.

6. Background Prevalence of HCM: 1:500 to 1:1000 individuals This occurrence is higher than previously thought, suggesting a large number of affected but undiagnosed people Men affected by almost 2:1 ratio over women Global disease with most cases reported from USA, Canada, Western Europe, Israel, & Asia Most common cause of SCD, age < 40 yr Most common cause of SCD, in competitive athletes

7. Normal Anatomy 7

8. Hypertophic cardiomyopathy (HOCM) 8

9. Hypertrophic cardiomyopathy (HOCM) 9

10. Pathophysiology of HOCM Involves 4 interrelated processes: Left ventricular outflow obstruction Diastolic dysfunction Myocardial ischemia Mitral regurgitation

11. Pathophysiology Hypertrophy: in any region of left ventricle SAM: systolic anterior motion of anterior MV leaflet against hypertrophic septum (Bernoulli effect) dynamic pressure gradient across LV outflow tract midsystolic intraventricular obstruction of the flow SAM - Septal Contact  dynamic obstruction increased by :  afterload  preload  contractility 11

12. Diastolic Dysfunction Due to prolongation of isovolumic relaxation time (AV closure to MV opening)  LV filling pressure  Ventricular volume Atrial contribution to ventricular filling ~ 75% Poor Compliance  LVEDP for any LVEDV  CPP gradient Subendocardial ischemia 12 Pathophysiology

13. Symptoms: Dyspnea on exertion (90%) - Reduced diastolic relaxation & LV filling Angina (70-80%) - Inadequate myocardial perfusion Syncope (20%) - Vasovagal attack/ transient arrhythmias Palpitation (10%) - AF - Loss of atrial contribution in LV filling - Rapid rate - 50 % pts of AF manifests as systemic embolisation

14. Hypertophic cardiomyopathy (HOCM) Lab studies: Blood test: non specific Genetic testing: for high risk group ECG: ST-T wave abnormalities LV hypertrophy, LA enlargement Axis deviation (left > right) Conduction abnormalities (P-R prolongation, BBB) A-fib (poor prognostic sign) 14

15. Electrocardiogram LV strain pattern LBBB/ RBBB / Lt ant hemiblock

16. ECG

17. Hypertophic cardiomyopathy (HOCM) Two - Dimensional Echocardiography and Doppler MR and Mitral prolapse Flow velocity: > 4.0 m/s LV outflow gradient: > 50 mm Hg EF : high to normal Small LV cavity Left atrial enlargement Septal thickness: 4-6 mm thicker than normal The hallmarks: SAM of Mitral valve Asymmetric septal hypertrophy 17

18. X ray Cardiomegaly LA enlargement Small aorta Pulmonary edema

19. CXR HOCM 19 Cardiac enlargement > 1/2 thoracic width

20. Hypertophic cardiomyopathy (HOCM) Goals:  V entricular contractility Myocardial depression  Ventricular volume Volume loading  Ventricular compliance and outflow tract dimensions  P ressure gradient across the LVOT Vasoconstriction 20

21. Hypertophic cardiomyopathy (HOCM) Medications Beta-Blockers: ( Metoprolol , Propranolol , Atenolol , Sotalol ) Calcium Channel blockers: ( Verapamil ) Antiarrhythmic : amiodarone and disopyramide Antitussives : avoid coughing 21

22. Hypertophic cardiomyopathy (HOCM) Beta - blockers  Pressure gradient across LVOT  I notropic state of left ventricle.  D iastolic dysfunction  Lt. Ventricle compliance  HR  Myocardial oxygen consumption  Myocardial ischemia potential 22

23. Calcium Channel Blockers : Verapamil Improves symptoms and exercise capacity (patients without marked obstruction to LV outflow) Beneficial effect on ventricular relaxation and filling Better angina control than BB Hemodynamic deterioration with CCB agents - lowering of the afterload in the presence of severe outflow tract gradients and high diastolic filling pressures

24. Hypertophic cardiomyopathy (HOCM) Antiarrhythmics Amiodarone (Cordarone) To date, Only one pharmacological agent, has been shown to reduce the incidence of arrhythmogenic sudden cardiac death 24

25. Hypertophic cardiomyopathy (HOCM) Contraindication Inotropic Sympathomimetic Nitrates Except in patients with CAD Digitalis Except with uncontrolled A-fib. Diuretics  Preload and ventricular volume  Outflow gradient 25

26. Hypertophic cardiomyopathy (HOCM) Anesthetic considerations Pre-operative period Pre-medication Avoid anxiety producing tachycardia  -blocker and/or Ca ++ channel blocker Continue untill the day of surgery and postoperative Avoid arrhythmia Aggressive treatment of arrhythmia Antiarrhythmic Meds Cardioversion Maintain adequate intravascular volume and preload 26

27. Hypertophic cardiomyopathy (HOCM) Anesthetic considerations Inhalation Anesthetics Negative inotropy Decrease SAM- Septal contact Ideal for dose dependant myocardial depression ( Halothane > Enflurane > Isoflurane > Desflurane , Sevoflurane ) Avoid hypotension due to underlying hypovolemia 27

28. Hypertophic cardiomyopathy (HOCM) Anesthetic considerations Regional anesthesia Relatively contraindicated Spinal should be avoided Epidural can be given Avoid bolus administration Avoid hypotension Replace intravascular volume Use Vasopressors like noradrenaline and phenylephrine 28

29. Hypertophic cardiomyopathy (HOCM) Anesthetic considerations IV Vasopressors Phenylepherine / Noradrenaline Low risk / high yield choice for hypotension Augment perfusion and CPP Decrease pressure gradient Increase vagal reflex 29

30. Hypertrophic cardiomyopathy (HOCM) Anesthetic considerations MR with HOCM Inotropes and Vasodilators worsen ventricular ejection Vasoconstrictors improve ventricular ejection 30

31. Here is a patient named Kamlaben B Patel 85 yrs. Old ,having # N/F, requring DHS Surgery. pt. is a known case of HTN Since 15 yrs. and she is taking Tab Amlodepine-Atenolol combination. Patient has been investigated and diagnosed to have HOCM by 2D ECHO ., how would you proceed for this case?

32. What would we the choice of Anaesthesia ? ? Spinal ? Epidural ? General anaesthesia

33. I planned Epidural for this patient. Pt.taken in OT. Venous access with two peripheral lines. Oxygen started and connected with monitor. First dose inj.xylocaine 2% 15 cc given. Sooner after this dose, inj. Noradrenaline infusion started @ 1ml/hr.(4mg/50cc)It was incresed to 2ml/ hr.as per need. (dose of Norad . is 0.04-0.4 mcg/kg/min) IV fluids inj.Hydrxyethyl starch 500 ml and one unit of Blood. Oxygen @3-4 lit/min given continuously. inj.Antibiotic , patoprazole , ondansetron given. Second dose of Epidural inj.Bupivacaine 3 cc given. Patient remained stable intra and post op. Shifed to ICU ,Where oxygen continued with monitoring. Patient’s vitals were as follow:

34. TIME HEART RATE SYSTOLIC BP DIASTOLIC BP SPO2% 8:28 am 56 153 58 93 8:33 54 154 58 99 9:10 58 116 42 98 9:23 57 107 38 98 9:33 53 111 39 98 9:43 52 108 39 99 9:53 54 129 40 99 10:03 54 148 44 100 10:13 55 158 45 100 10:23 54 156 45 99 11:30 55 158 50 99 Vital parameters of patient : HOCM-DHS 10/1/2013

35. Comments: Any patient with HTN and ECG changes always get cardiac evaluation done. Patients with HOCM ,presenting for surgery where regional anaes . is preferred,Always maintain Hemodynamics with vasopressors and fluids. Don’t give any Inotropic or chronotropic agents. Keep Defibrillator available ,as pts. are highly prone for Arrythmias . Pt’s preload should be maintained adequetely .

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