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Hypertrophic Obstructive Cardiomyopathy (Case Presentation)

Hypertrophic Obstructive Cardiomyopathy (Case Presentation). History (1). 35 YO female, 37 weeks pregnant Presented to OB ward on 02/ 2002 for OB f/u

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Hypertrophic Obstructive Cardiomyopathy (Case Presentation)

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  1. Hypertrophic Obstructive Cardiomyopathy(Case Presentation)

  2. History (1) 35 YO 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

  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

  4. History(3) • PMHx • Anxiety • HOCM (IHSS) diagnosed 2000 • OBHx: • 37 Weeks Pregnancy, G6P3023, EDD 02/27/02 • PSHx: • Ectopic pregnancy 1987 • Therapeutic abortion 2000 • C- section  • 1985  child with spina bifida • 1997  child with heart murmur • 2000  child with heart murmur

  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

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

  7. Labs and studies (2) • Transthoracic echocardiogram:on01/24/2002 • 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)

  8. 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 80 CVP 11 PA 22/11 CO 4.6 SVO2 72

  9. Intraoperative Management • 12:15 PM: Smooth IV rapid sequence induction with Sux 120 mg, STP 350 mg, ETT # 7 placed, + ETCO2 & BS B/L • IVF bolus + maintenance fluid given to keep CVP ~ 11-15 cmH2O • Surgery started at 12:22 • Maintenance of anesthesia: Enflurane 0.6-0.8, N2O • 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: 97 BP: 140/75 CVP: 15 PA: 49/25 CO: 4.5 SVO2: 70 • Postoperative course: stable, Pt. D/C home POD # 4

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

  11. 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

  12. 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

  13. Hypertophic cardiomyopathy (HOCM)Pathophysiology (2) • 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

  14. Normal Anatomy

  15. Hypertophic cardiomyopathy (HOCM)

  16. Hypertophic cardiomyopathyFACTS • 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

  17. 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

  18. Hypertrophic cardiomyopathy (HOCM)

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

  20. 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

  21. 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

  22. Hypertophic cardiomyopathy (HOCM) • Angina: • Common with no CAD • Impaired diastolic relaxation + MVO2 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

  23. Hypertophic cardiomyopathy (HOCM) • Palpitation: • Ventricular Arrhythmia 75% • SVT 25% • A- fib 5-10% • Sudden cardiac death (SCD): • 6 % in children • Related to extreme exertion • MCC of SCD is arrhythmia • 80 % ~ V-fib

  24. 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

  25. 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

  26. 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)

  27. 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

  28. 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)

  29. 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

  30. Two- Dimensional Echocardiography

  31. Two- Dimensional Echocardiography

  32. 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

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

  34. 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.)

  35. CXRHOCM Cardiac enlargement > 1/2 thoracic width

  36. Hypertophic cardiomyopathy (HOCM)Differential Diagnosis • Aortic Stenosis • Restrictive Cardiomyopathy • Glycogen Storage Disease, Type 2

  37. Hypertophic cardiomyopathy (HOCM) • Goals: •  Ventricular contractility • Myocardial depression •  Ventricular volume • Volume loading •  Ventricular compliance and outflow tract dimensions •  Pressure gradient across the LVOT • Vasoconstriction

  38. 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

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

  40. Hypertophic cardiomyopathy (HOCM)Medications • Beta-Blockers: (Metoprolol, Propranolol, Atenolol, Sotalol ) • Calcium Channel blockers: (Verapamil) • Antiarrhythmic: amiodarone and disopyramide • Antitussives: avoid coughing • Antibiotic: prophylaxis against endocarditis • Anticoagulation: Atrial fibrillation

  41. 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

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

  43. 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

  44. 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

  45. Hypertrophic cardiomyopathy (HOCM)

  46. Hypertophic cardiomyopathy (HOCM)Surgical Care (2) • Implantable Cardioverter Defibrillator (ICD) • Prevents 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

  47. 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

  48. 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

  49. 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

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