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1 Hypertrophic Obstructive Cardiomyopathy (Case Presentation) Ashraf Andrawis, MD Norman Bolden, MD Metrohealth medical center- CWRU Department of anesthesiology.

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Presentation on theme: "1 Hypertrophic Obstructive Cardiomyopathy (Case Presentation) Ashraf Andrawis, MD Norman Bolden, MD Metrohealth medical center- CWRU Department of anesthesiology."— Presentation transcript:

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2 1 Hypertrophic Obstructive Cardiomyopathy (Case Presentation) Ashraf Andrawis, MD Norman Bolden, MD Metrohealth medical center- CWRU Department of anesthesiology

3 2 History (1) 35 YO Hispanic female, 37 weeks pregnant Presented to OB ward on 02/ 2002 for OB f/u –H/O syncope: Pt. Collapsed at home during last pregnancy, required emergency C/S, had complicated postoperative course, required ICU admission and CVP monitoring for 1 week –HOCM (IHSS) diagnosed –Cardiology recommendation: to avoid future pregnancy and permanent sterilization

4 3 History (2) CP and chest heaviness: (like baby sitting on my chest) SOB: when doing household activities and when laying flat (use 3 big pillows or sleeping in a chair) Atypical CP: Required hospital admission X 2 during pregnancy CCU admission (11/01) to R/O (PE / MI), heparin 24 h, negative serial ECG and cardiac enz. And V/Q scan. O/B H/R admission (01/02) to R/O (MI / CHF) Heaviness: of Lt. Shoulder and Lt. Arm with exertion Nausea and palpitations when walking

5 4 History (3) PMHx –Anxiety –HOCM (IHSS) diagnosed 2000 OBHx: –37 Weeks Pregnancy, G6P3023, EDD 02/27/02 PSHx: –Ectopic pregnancy1987 –Therapeutic abortion2000 –C- section  »1985  child with spina bifida »1997  child with heart murmur »2000  child with heart murmur

6 5 Physical V.S: BP 112/67, HR 83, RR 20 Wt: 119 Kg, Ht: 6 Ft HEENT: PEERL, EOMI MP: class 2, TMD 5 cm, mouth opening 3FB, good neck mobility, and own dentition Lungs: CTA B/L Heart: ejection systolic murmur grade 3/6 at Lt. sternal border radiates to the base and the apex, no JVD, no Gallop Neurological: AAO x 3, non focal ASA: class 3

7 6 Labs and studies (1) CK-MB = 1.0 Fetal lung maturity = 77.3 CXR: slight cardiac enlargement, no infiltrate 11.6 27 8.7 190 133 4.0 97 4 22 94 0.5

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9 8 Labs and studies (2) Transthoracic echocardiogram: on 01/24/2002 –Left ventricular systolic function: EF 65%, hyperdynamic –Right ventricular systolic function: normal –Valves: AR: mild MR, PR: trivial –PAP: 34/12 –Subaortic stenosis with fibrocalcific changes, peak gradient 70 mmHg, mean gradient 41 mmHg –Compared to prior study dated 11/08/01 (peak gradient = 90, mean gradient 60)

10 9 Chronology Feb. 06, 2002: Preoperative assessments, chart, cardiology consult and echocardiography results reviewed, Anesthesia plan D/W Pt. Feb. 07, 2002 –At 09:30 AM, pt to OR, standard 5 ASA monitors applied –18 G IV Line, Right Radial A-line and Right IJ 9 Fr introducer placed, SG catheter placed, + wedge at 49 cm, no complication –Defibrillator pads applied to treat possible arrhythmia –Left uterine displacement applied –Initial VS: BP 130/70 HR 80CVP 11 PA 22/11CO 4.6SVO2 72

11 10 Intraoperative Management 12:15 PM: Smooth IV rapid sequence induction with Sux 120 mg, STP 350 mg, ETT # 7 placed, + ETCO 2 & BS B/L IVF bolus + maintenance fluid given to keep CVP ~ 11-15 cmH 2 O Surgery started at 12:22 Maintenance of anesthesia: Enflurane 0.6-0.8, N 2 O Labetalol,Esmolol, Fentanyl titrated to keep BP ~ 130-140/60-70 and HR ~ 70-90 Total IVF: 3000 cc, EBL: 1200 cc, UOP: 300ml Delivery Of fetus at 12:29 Surgery end at 13:22 Pt. successfully extubated  high risk unit then to CCU Postop VS HR: 97BP: 140/75CVP: 15 PA: 49/25CO: 4.5SVO2: 70 Postoperative course: stable, Pt. D/C home POD # 4

12 11 Hypertrophic obstructive cardiomyopathy Overview Background Pathophysiology Histology Clinical picture Diagnosis and Differential Treatment Anesthetic consideration Therapeutic approach of pregnant Pt. With HOCM

13 12 Hypertophic cardiomyopathy (HOCM) Background Genetic disorder  Autosomal dominant with variable penetrance Molecular basis  Defect in sarcomeric protein genes as myosin heavy chain, actin, tropomyosin Abnormal myocardial Ca ++ kinetics  Increase Ca ++ intracellular  hypertrophy and cellular disarray Other terms –Idiopathic hypertrophic subaortic stenosis (IHSS) –Asymmetric septal hypertrophy (ASH) Leading cause of sudden death in preadolescent and adolescent

14 13 Hypertophic cardiomyopathy (HOCM) Pathophysiology (1) 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

15 14 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 Hypertophic cardiomyopathy (HOCM) Pathophysiology (2)

16 15 Normal Anatomy

17 16 Hypertophic cardiomyopathy (HOCM)

18 17 Hypertophic cardiomyopathy FACTS Sex: Male > female (younger, more symptomatic) Age: –Most common in 30’s - 40’s –Most common autopsy finding in previously healthy athletes –Recent study, elderly with severe mitral annular calcification  sub-aortic obstruction Frequency: –0.5 % of outpatient population –Prevalence 0.05-0.2% –25 % of first degree relative

19 18 Hypertrophic cardiomyopathy (HOCM) FACTS Morbidity / Mortality –Sudden death: younger Pt., aggressive genotype –Arrhythmia: A- fib, A- flutter, v-tach. or v-fib –CHF: MR and diastolic dysfunction –Angina: adults > children –Syncope and pre-syncope

20 19 Hypertrophic cardiomyopathy (HOCM)

21 20 Hypertophic cardiomyopathy (HOCM) Histologic Findings (1) Gross disorganization of the muscle bundles and myofibrillar disarray

22 21 Hypertophic cardiomyopathy (HOCM) Histologic Findings (2) Abnormal intramural coronary arteries (see arrow) Reduction in the size of the lumen Thickening of the vessel wall 80% of cases

23 22 Hypertophic cardiomyopathy (HOCM) Dizziness: –  by Exertion Hypovolemia Maneuver (rapid standing or valsalva) Medication (diuretics, NTG and Vasodilator Meds) Arrhythmia  hypotension  decrease cerebral perfusion Dyspnea: –Most common symptom, 90% –  Lt Ventricular Diastolic filling pressure   PAP Orthopnea and Paroxysmal Nocturnal Dyspnea: –Pulmonary venous congestion –Early signs of CHF

24 23 Hypertophic cardiomyopathy (HOCM) Angina: –Common with no CAD –Impaired diastolic relaxation +  MVO 2  Sub-endocardial ischemia –  Capillary density leads to  flow to hypertrophic muscle –Extramural compression of coronaries –  Systolic ejection time leads to  diastolic interval for coronaries perfusion Syncope and pre-syncope: –Very common –  CO with exertion or arrhythmia –High risk of sudden death –Urgent work-up and aggressive treatment

25 24 Hypertophic cardiomyopathy (HOCM) Palpitation: –Ventricular Arrhythmia75% –SVT25% –A- fib5-10% Sudden cardiac death (SCD): –6 % in children –Related to extreme exertion – MCC of SCD is arrhythmia 80 % ~ V-fib

26 25 Causes of Ischemia in HOCM  Myocardial muscle mass  Myocardial oxygen demand (  wall stress)  Diastolic filling pressures  Coronary capillary density  Vasodilatory reserve Abnormal intramural coronary arteries  Systolic compression of coronary arteries

27 26 Hypertophic cardiomyopathy (HOCM) Physical (1) Double apical impulse: –Forceful left atrial contraction against non-compliant ventricle Triple apical impulse: –Late systolic bulge near isometric contraction S1: normal S2: normal or paradoxical split S3 gallop: decompensated Lt. ventricle S4: atrial systole against hypertrophic ventricle Jugular venous pulse: prominent a- wave Double carotid arterial pulse: declines in mid systole as gradient develop

28 27 Hypertophic cardiomyopathy (HOCM) Physical (2) Systolic Ejection Murmur: Crescendo - Decrescendo –Between apex and left sternal border –Radiate to suprasternal notch –  by  Preload(volume loading)  Afterload(vasopressor) –  by  Preload(nitrates, diuretic, standing)  Afterload(vasodilator)

29 28 Hypertrophic cardiomyopathy (HOCM) Physical (3) Holosystolic Murmur of MR: –Retrograde ejection of blood flow into low pressure left atrium –Best heard at apex and axilla –Pt. with SAM * and significant LV outflow gradients Diastolic Decrescendo Murmur of AR: 10% of Pt. * Systolic anterior motion

30 29 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)

31 30 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

32 31 Two- Dimensional Echocardiography

33 32 Two- Dimensional Echocardiography

34 33 Hypertophic cardiomyopathy (HOCM) Radionuclide study: –Absence of CAD –Defects of myocardial perfusion Cardiac catheterization –Degree of outflow obstruction –Diastolic characteristics of the left ventricle –LV anatomy –Coronary arteries anatomy Holter monitoring: Nonsustained atrial or ventricular arrhythmia / 24 h

35 34 HOCM Two-Dimensional Echocardiogram: apical hypertrophy and "ace-of-spades" configuration EKG : prominent T wave inversion Thallium-201 Scan: increased apical myocardial uptake

36 35 Left-Side Cardiac Pressures Aortic upstroke rates –Delayed supravalvular AS valvular AS subvalvular AS –Rapid and parallel to the LV pressure HOCM (From Criley JM, Siegel RJ: Subaortic stenosis revisited: The importance of the dynamic pressure gradient. Medicine 72:412, 1993.)

37 36 CXR HOCM Cardiac enlargement > 1/2 thoracic width

38 37 Hypertophic cardiomyopathy (HOCM) Differential Diagnosis  Aortic Stenosis  Restrictive Cardiomyopathy  Glycogen Storage Disease, Type 2

39 38 Hypertophic cardiomyopathy (HOCM) Goals: –  Ventricular contractility »Myocardial depression –  Ventricular volume »Volume loading –  Ventricular compliance and outflow tract dimensions –  Pressure gradient across the LVOT – Vasoconstriction

40 39 Hypertophic cardiomyopathy (HOCM) Medical Care Activity:  Avoid Competitive level sports when:  Significant outflow gradient  Significant arrhythmia  Marked LV hypertrophy  History of sudden death in relatives  Identified malignant genotype

41 40 Hypertophic cardiomyopathy (HOCM) Medical Care The purpose of pharmacologic therapy:   Inotropic state of left ventricle   pressure gradient   Compliance of the Lt.Ventricle   Diastolic dysfunction

42 41 Hypertophic cardiomyopathy (HOCM) Medications Beta-Blockers: (Metoprolol, Propranolol, Atenolol, Sotalol ) Calcium Channel blockers: (Verapamil) Antiarrhythmic: amiodarone and disopyramide Antitussives: avoid coughing   

43 42 Hypertophic cardiomyopathy (HOCM) Beta - blockers  Pressure gradient across LVOT –  Inotropic state of left ventricle. –  Diastolic dysfunction –  Lt. Ventricle compliance  HR –  Myocardial oxygen consumption –  Myocardial ischemia potential

44 43 Hypertophic cardiomyopathy (HOCM) Antiarrhythmics Amiodarone (Cordarone) To date, Only one pharmacological agent, has been shown to reduce the incidence of arrhythmogenic sudden cardiac death

45 44 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

46 45 Hypertrophic cardiomyopathy (HOCM) Surgical Care (1) Mitral Valve Replacement Catheter septal ablation –96% ethanol infusion of LAD to destroy myocardial tissue Left ventricular myomectomy or septal myotomy: –Indications: severe symptoms refractory to medical therapy outflow gradient ~ >50 mm Hg –Verapamil (improve diastolic) and myomectomy (improve systolic) –2% mortality overall –Retrospective study: survival rate higher with surgical treatment

47 46 Hypertrophic cardiomyopathy (HOCM)

48 47 Hypertophic cardiomyopathy (HOCM) Surgical Care (2) Implantable Cardioverter Defibrillator (ICD) –P revents sudden death –Automatically detects, recognizes, and treats arrhythmia –Many prospective studies In adults with CAD and  EF, the ICD has been demonstrated to be superior to antiarrhythmic drug therapy

49 48 Hypertophic cardiomyopathy (HOCM) Anesthetic considerations (1) 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

50 49 Hypertophic cardiomyopathy (HOCM) Anesthetic considerations (2) Intra-operative Monitoring Contractility and HR: avoid direct or reflex increase Arterial BP: – Avoid hypotension – Bifid shape waveform "spike-and-dome" CVP: high normal - elevated / vasoactive meds. PAC –PCWP: high normal - elevated Overestimates pt. true volume status –PAC with pacing capability CPP: use vasoconstrictor / avoid inotropes

51 50 Hypertophic cardiomyopathy (HOCM) Anesthetic considerations (3) 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

52 51 Hypertophic cardiomyopathy (HOCM) Anesthetic considerations (4) Regional anesthesia Relatively contraindicated Continuos spinal/epidural Avoid bolus administration Avoid hypotension Replace intravascular volume Vasopressor

53 52 Hypertophic cardiomyopathy (HOCM) Anesthetic considerations (5) IV Vasopressors Phenylepherine –Low risk / high yield choice for hypotension –Augment perfusion and CPP –Decrease pressure gradient –Increase vagal reflex

54 53 Hypertrophic cardiomyopathy (HOCM) Anesthetic considerations (6) MR with HOCM ¼Inotropes and Vasodilators worsen ventricular ejection ¼Vasoconstrictors improve ventricular ejection

55 54 Hypertophic cardiomyopathy (HOCM) Pregnancy Reported 100 pregnancies in patients with HOCM –Favorable outcome in most cases –New onset or worsening CHF reported in 20 % of cases –SVT and A - fib. with fetal distress reported, leading to hemodynamic deterioration and direct-current cardioversion –Sudden death in one Pt. at 28 weeks –Fetal outcome in most cases is not affected by maternal HOCM

56 55 Hypertophic cardiomyopathy (HOCM) Therapeutic Approach of Pregnancy Indications for drug therapy during gestation include –Arrhythmias –Elevated left ventricular filling pressure » beta - blocker and calcium antagonists Dual-chamber pacing –before pregnancy in symptomatic patients. ICD – syncope or life-threatening arrhythmias

57 56 Induction of labor: –Prostaglandin  risky because of vasodilator effect –Oxytocin  well tolerated Tocolytic agents –  -agonist  LVOT obstruction –MgSO 4 is preferred Avoid –Blood loss –Vasodilators –Sympathetic stimulation Hypertophic cardiomyopathy (HOCM) Therapeutic Approach of Pregnancy

58 57 Hypertophic cardiomyopathy (HOCM) Therapeutic Approach of Pregnancy Continous spinal and epidural anesthetics –Avoid vasodilator effect by bolus administration Antibiotic prophylaxis –higher risk for infective endocarditis

59 58 Thank You, The End


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