Clinical Pathology Conference 62 year old woman with weakness and shortness of breath - PowerPoint PPT Presentation

Loading...

PPT – Clinical Pathology Conference 62 year old woman with weakness and shortness of breath PowerPoint presentation | free to view - id: 367af-MWZmN



Loading


The Adobe Flash plugin is needed to view this content

Get the plugin now

View by Category
About This Presentation
Title:

Clinical Pathology Conference 62 year old woman with weakness and shortness of breath

Description:

HPI: 62 year old white woman with multiple sclerosis ... Review of the literature for a correlation between multiple sclerosis and cardiomyopathy ... – PowerPoint PPT presentation

Number of Views:271
Avg rating:3.0/5.0
Slides: 66
Provided by: sw47
Category:

less

Write a Comment
User Comments (0)
Transcript and Presenter's Notes

Title: Clinical Pathology Conference 62 year old woman with weakness and shortness of breath


1
Clinical Pathology Conference 62 year old woman
with weakness and shortness of breath
  • Heather Henderson, MD
  • Internal Medicine Resident, PGY-3
  • Scott White/Texas AM HSC

2
Case Presentation
  • CC I am so weak and short of breath
  • HPI 62 year old white woman with multiple
    sclerosis
  • 2-3 days of worsening lower extremity swelling
  • Increasing shortness of breath
  • Generalized weakness
  • No prior history of heart failure symptoms

3
Case Presentation
  • Past Medical History
  • Relapsing, remitting multiple sclerosis
  • HTN
  • History of herpes zoster
  • Raynauds phenomenon
  • S/P TAH/BSO

4
Case Presentation
  • Allergies NKDA
  • Medications
  • Amantadine 200 mg po qam
  • Avonex 30 mcg IM qweekly
  • Baclofen 10 mg po qid
  • Amitriptyline 50 mg po qday
  • Oxybutynin 5 mg po bid
  • Maxzide 75/50 po daily, but has not taken for
    last week
  • Conjugated estrogen 0.625 mg po daily

5
Case Presentation
  • Social History
  • Tobacco None
  • ETOH None
  • Lives alone in Temple
  • Inactive, but performs ADLs
  • Family History
  • Prostate Ca brother
  • HTN - brother

6
Case Presentation
  • Review of Systems
  • Weak with poor appetite
  • Dyspnea on exertion with some wheezing lately
  • No fever or chills
  • Constipated for last 2-3 days
  • Nauseated at times without emesis
  • No headaches or blurred vision
  • No dysuria
  • No recent problem with Raynauds

7
Physical Examination
  • VS151/60, 120, 20, 93 RA, 36.1, Wt 91kg
  • Gen wn/wd white woman appearing chronically ill,
    weak, and tired, but no acute distress
  • HEENT normal except JVP 10 cm of water
  • Chest bilateral breath sounds decreased
    bilateral lower lobes, bibasilar crackles
  • CV PMI slightly displaced inferolaterally
    tachycardic at 120 bpm normal S1 and S2 with
    normal splitting, no S3 or S4 gallop II/VI
    diastolic decrescendo murmur at LLSB with patient
    sitting up pulses 2/2, bilateral throughout

8
Physical Examination
  • Back pitting sacral edema
  • Abd soft, nd/nt nabs, no abd bruit, no
    hepatosplenomegaly
  • Ext no clubbing/cyanosis positive 0.5cm depth
    pitting edema of the lower extremities LgtR to the
    level of the upper tibia
  • Skin chronic venous stasis changes bilateral
    lower extremities
  • Neuro bulk and tone normal in UEs, LEs with
    decrease motor strength with patient unable to
    lift left leg (known to be chronic) no new
    sensory deficits

9
Laboratory Evaluation
  • BNP1940
  • TnI0.07, CK35, CK-MB1.8
  • Na140, K3.7, Cr1.0, BUN6
  • TSH1.3
  • WBC12.7, Hbg13.9, Plat391k
  • Chol178, TG152, HDL67, LDL81

10
Electrocardiogram
  • NSR, rate 84
  • Marked t-wave inversion in the anterior leads and
    scooping of the ST segment in the inferior leads,
    suggests ischemia
  • No voltage criteria for LVH

11
Chest X-ray
  • Cardiomegaly
  • Pulmonary vascular congestion
  • Small bilateral pleural effusions

12
Echocardiogram
  • LV enlargement with EF25 (globally
    depressed)
  • Normal LV wall thickness
  • Increased echo densities in the LV apex
    suggesting possible thrombus
  • LA enlargement with mild MR
  • Moderate to severe AI with normal aortic root size

13
Transesophageal Echocardiogram
  • AI was mild to moderate
  • Prominent myocardial trabeculations
  • No LV thrombus

14
Coronary Angiogram
  • Normal coronary anatomy
  • No significant angiographic coronary artery
    disease

15
Problem List
  • Weakness
  • Shortness of breath
  • Multiple Sclerosis
  • Congestive Heart Failure
  • Dilated Cardiomyopathy EF 25
  • Tachycardia
  • Aortic Insufficiency with normal aortic root
  • Prominent myocardial trabeculations
  • No significant Coronary Artery Disease

16
Things are not as they appear
17
Objectives
  • Define cardiomyopathy
  • Discussion of causes of dilated cardiomyopathy
  • Diagnostic evaluation of a dilated
    cardiomyopathy
  • Review of the literature for a correlation
    between multiple sclerosis and cardiomyopathy
  • A discussion of a rare cause of dilated
    cardiomyopathy

18
Cardiomyopathy Defined
  • A group of disorders in which the dominant
    feature is direct involvement of the heart
    muscle.
  • (Not the result of pericardial, hypertensive,
    congenital, or valvular diseases)

19
Classification of Primary Cardiomyopathies
  • Dilated cardiomyopathy
  • Hypertrophic cardiomyopathy
  • Restrictive cardiomyopathy
  • Arrhythmogenic right ventricular cardiomyopathy
  • Unclassified cardiomyopathy

20
Specific Cardiomyopathies
  • Ischemic cardiomyopathy
  • Valvular cardiomyopathy
  • Hypertensive cardiomyopathy
  • Inflammatory cardiomyopathy
  • Metabolic cardiomyopathy
  • General-systemic disease cardiomyopathy
  • Muscular dystrophies
  • Neuromuscular disorders
  • Sensitivity and toxic reactions
  • Peripartal cardiomyopathy

21
Dilated Cardiomyopathy
  • 5-8 cases per 100,000 population/year
  • 10,000 deaths each year in the US
  • 46,000 hospitalizations each year in the United
    States
  • ¼ of the cases of congestive heart failure in the
    United States
  • 75 different diseases cause DCM

22
Objectives
  • Define cardiomyopathy
  • Discussion of causes of dilated cardiomyopathy
  • Diagnostic evaluation of a dilated cardiomyopathy
  • Review of the literature for a correlation
    between multiple sclerosis and cardiomyopathy
  • A discussion of a rare cause of dilated
    cardiomyopathy

23
Causes of Dilated Cardiomyopathy
  • Ischemia
  • Infectious diseases
  • Coxsackievirus
  • Cytomegalovirus
  • HIV
  • Varicella
  • Hepatitis
  • Epstein-Barr
  • Echovirus
  • Streptococci-rheumatic fever
  • Typhoid fever
  • Diphtheria
  • Brucellosis
  • Psittacosis
  • Rickettsial disease
  • Lyme disease
  • Histoplasmosis
  • Cryptococcosis
  • Toxoplasmosis
  • Trypanosomiasis
  • Shistosomiasis
  • Trichinosis

24
Causes of Dilated Cardiomyopathy
  • Medications
  • Chemotherapeutic agent
  • Anthracyclines
  • Cyclophosphamide
  • Trastuzumab
  • Antiretroviral drugs
  • Zidovudine
  • Didanosine
  • Zalcitabine
  • Phenothiazines
  • Chloroquine
  • Clozapine
  • Toxins
  • Ethanol
  • Cocaine
  • Amphetamines
  • Cobalt
  • Lead
  • Mercury
  • Carbon Monoxide
  • Beryllium

25
Causes of Dilated Cardiomyopathy
  • Rheumatologic diseases
  • Systemic lupus
  • Scleroderma
  • Giant cell arteritis
  • Endocrinologic disorders
  • Hypo/Hyperthyroidism
  • Growth hormone excess or deficiency
  • Pheochromocytoma
  • Diabetes Mellitus
  • Cushings disease
  • Neuromuscular diseases
  • Duchennes Muscular Dystrophy
  • Myotonic dystrophy
  • Friedreichs ataxia
  • Deposition Disease
  • Hemochromatosis
  • Amyloidosis

26
Causes of Dilated Cardiomyopathy
  • Electrolyte abnormalities
  • Hypocalcemia
  • Hypophosphatemia
  • Uremia
  • Nutritional deficiencies
  • Thiamine
  • Selenium
  • Carnitine
  • Miscellaneous
  • Peripartum cardiomyopathy
  • Tachycardia
  • Sarcoidosis
  • Familial
  • Sleep Apnea
  • Autoimmune myocarditis
  • Radiation
  • Calcium Overload
  • Oxygen free radical damage

27
Frequency of Different Causes
  • Idiopathic 50 percent
  • Myocarditis 9 percent
  • Ischemic heart disease 7 percent
  • Infiltrative disease 5 percent
  • Peripartum cardiomyopathy 4 percent
  • HIV infection 4 percent
  • Connective tissue disease 3 percent
  • Substance abuse 3 percent
  • Doxorubicin 1 percent
  • Other 10 percent

28
Differential Diagnosis
  • Dilated Cardiomyopathy
  • Idiopathic dilated cardiomyopathy
  • Valvular cardiomyopathy
  • Medications
  • Multiple Sclerosis
  • Left ventricular noncompaction

29
Objectives
  • Define cardiomyopathy
  • Discussion of causes of dilated cardiomyopathy
  • Diagnostic evaluation of a dilated cardiomyopathy
  • Review of the literature for a correlation
    between multiple sclerosis and cardiomyopathy
  • A discussion of a rare cause of dilated
    cardiomyopathy

30
Noninvasive Laboratory Evaluation
  • Ca
  • Phos
  • Creatinine, BUN
  • Thyroid function studies
  • Iron studies
  • HIV

31
Invasive Evaluation
  • Endomyocardial biopsy
  • May be of benefit in certain situations
  • Definite clinical benefit
  • Infiltrative disorders
  • Anthracycline toxicity
  • Cardiac transplant rejection
  • No definitive pattern histologically in DCM
  • Estimated that a specific diagnosis is obtained
    by biopsy in fewer than 10 percent of patients
  • Cardiac Catheterization and Angiography
  • To determine ischemic disease

32
Objectives
  • Define cardiomyopathy
  • Discussion of causes of dilated cardiomyopathy
  • Diagnostic evaluation of a dilated cardiomyopathy
  • Review of the literature for a correlation
    between multiple sclerosis and cardiomyopathy
  • A discussion of a rare cause of dilated
    cardiomyopathy

33
Multiple Sclerosis and Cardiomyopathy Is there a
link?
  • Subclinical left ventricular dysfunction in
    multiple sclerosis, per Akgul
  • 41 patients with MS and 32 healthy controls
  • LV ejection fraction was decreased in MS patients
    compared with controls (plt0.05)

34
Medications
  • Amantadine
  • lt1 CHF
  • 1-10 orthostatic hypotension, peripheral edema
  • Use in caution in patients with heart failure,
    peripheral edema, or orthostatic hypotension
  • Triamterene has been reported to increase the
    potential for toxicity with amantadine

35
More Medications
  • Interferon beta 1a Avonex
  • lt1 cardiomyopathy, CHF
  • 1-10 chest pain, vasodilatation
  • Use in caution in patients with pre-existing
    cardiovascular disease
  • Interferons increase the adverse effects of ACE
    inhibitors, specifically the development of
    granulocytopenia

36
More Medications
  • Amitriptyline
  • Rare cause of cardiomyopathy
  • 2 case reports in the literature
  • Case report Cardiomyopathy developed during
    treatment with imipramine, recovered after
    withdrawal, recurred 9 years later during
    treatment with amitriptyline
  • Case report Cardiomyopathy in a patient on
    amitriptyline and perphenazine

37
More Medications
  • Mitoxantrone
  • Cause of cardiomyopathy
  • Dose related, approved cumulative dose is 140
    mg/m2
  • Prospective study in Germany in 73 patients
    showed no significant change in end-diastolic
    diameter, end-systolic diameter, fractional
    shortening, or EF in 23 month follow up with
    mean dose of 114 mg/m2

38
Objectives
  • Define cardiomyopathy
  • Discussion of causes of dilated cardiomyopathy
  • Diagnostic evaluation of a dilated cardiomyopathy
  • Review of the literature for a correlation
    between multiple sclerosis and cardiomyopathy
  • A discussion of a rare cause of dilated
    cardiomyopathy

39
This is not a zebra!
40
Isolated Left Ventricular Noncompaction
  • Characteristics of Isolated Left Ventricular
    Concompaction
  • Prevalence
  • Genetics
  • Noncompaction associated with other diseases
  • Clinical Manifestations
  • Imaging
  • Managment

41
Isolated Left Ventricular Noncompaction
  • Characterized by the following feautures
  • Altered myocardial wall
  • Prominent trabeculae and deep intertrabecular
    recesses
  • Thickened myocardium with two layers consisting
    of compacted and noncompacted myocardium

42
Isolated Left Ventricular Noncompaction
43
Isolated Left Ventricular Noncompaction
  • Also Characterized by the following feautures
  • Continuity between the left ventricular cavity
    and the deep intratrabecular recesses, which are
    filled with blood
  • No communication to epicardial coronaries
  • Decreased coronary flow reserve

44
Prevalence of Isolated Left Ventricular
Noncompaction
  • A rare form of cardiomyopathy
  • All adult echocardiograms with global LV
    dysfunction and an EF of lt45 were reviewed for
    signs of LV compaction
  • 3.7 prevalence for LVEF lt45
  • 0.26 for all patients
  • A review from Switzerland identified 34 cases in
    15 years

45
Genetics of Isolated Left Ventricular
Noncompaction
  • LVNC can be familial
  • Mutations have been found in the following genes
  • G4.5
  • P121L
  • Cypher/ZASP
  • Chromosome 11p15
  • Family Screening

46
Left Ventricular Noncompaction
  • Congenital right or left ventricular outflow
    tract abnormalities
  • Pulmonary atresia with intact ventricular septum
  • Rarely seen with other congenital cardiac
    disorders
  • Ebsteins anomaly
  • Bicuspid aortic valve
  • Aorta-to-left ventricular tunnel
  • Congenitally corrected transposition
  • Isomerism of the left atrial appendage
  • VSD

47
Left Ventricular Noncompaction
  • LVNC is associated with Neuromuscular diseases
  • 86 patients with LVNC underwent neurological
    evaluation
  • Metabolic myopathy(14), Lebers hereditary optic
    neuropathy(3), myotonic(2), Becker(1),
    Duchenne(1), NMD of unknown etiology in 32,
    normal in 13, 20 patients refused

48
LV Noncompaction and NMD
  • Noncompaction and neuromuscular disease in a
    nonagerian
  • 94 year old male presented with a surprising find
    of left ventricle hypertrabeculation
  • Upon neurologic investigation, patient had a
    polyneuropathy and possible myopathy

49
Clinical Manifestations
  • Report from Switzerland on 34 patients
  • At the time of diagnosis, clinical manifestations
    included
  • Dyspnea 27 (79)
  • NYHA Class III or IV heart failure 12 (35)
  • Chest Pain 9 (26)
  • Chronic Atrial Fibrillation 9 (26)

50
ECG in Noncompaction
  • No characteristic changes
  • Usually abnormal

51
Diagnosis of Noncompaction
  • Echocardiography
  • Cardiac MRI
  • Cardiac CT Scan
  • Left Ventriculography

52
Echocardiographic Criteria for Diagnosis
  • Absence of coexisting cardiac abnormalities
  • Segmental thickening of the left ventricular
    myocardial wall consisting of two layers a ratio
    of noncompacted to compacted myocardium of gt21
    and end-systole with thickening of the myocardial
    wall
  • Predominant localization of the pathology in the
    apical mid-lateral, and mid-inferior regions of
    the left ventricle
  • Color doppler evidence of flow within the deep
    perfused intertrabecular recesses

53
Echocardiogram
54
Echocardiogram
55
MRI in Noncompaction
  • Noninvasive way to evaluate the presence and
    extent of myocardial fibrosis
  • Cardiac MRI shows trabecular delayed
    hyperenhancement in left ventricle noncompaction

56
Cardiac MRI
57
Management
  • No specific therapy
  • Treat heart failure, arrhythmias, etc
  • Holter monitoring once a year
  • Heart transplantation

58
What is?
59
What is the answer?
  • Final Diagnosis

60
What is the answer?
  • Final Diagnosis
  • Left Ventricular Noncompaction associated with
    Multiple Sclerosis
  • vs.
  • Idiopathic Dilated Cardiomyopathy

61
What is the answer?
  • Final Diagnosis
  • Left Ventricular Noncompaction associated with
    Multiple Sclerosis
  • vs.
  • Idiopathic Dilated Cardiomyopathy
  • Diagnostic Study/Procedure
  • Cardiac MRI

62
References
  • Uptodate
  • Kasper, Braunwald, Fauci, Hauser, Longo, Jameson.
    Harrisons Principles of Internal Medicine. 16th
    edition. 2005
  • Zipes, Libby, Bonow, Braunwald. Braunwalds
    Heart Disease Textbook of Cardiovascular
    Medicine. 7th edition. 2005
  • Kuhn H, Lawrenz T, Beer G. Indication for
    Myocardial Biopsy in myocarditis and dilated
    cardiomyopathy. Med Klin. 2005 Sep15100(9)553-61
    .
  • Alsaileek AA, Syed I, Seward JB, Julsrud P.
    Myocardial fibrosis of left ventricle Magnetic
    resonance imaging in noncompaction. J Magn Reson
    Imaging. 2008 Jan 24
  • Bruder O, et al. Detection and characterization
    of left ventricular thrombi by MRI compared to
    transthoracic echocardiography. Rofo. 2005
    Mar177(3)344-9.
  • Sandhu R, et al. Prevalence and characteristics
    of left ventricular noncompaction in a community
    hospital cohort of patients with systolic
    dysfunction. Echocardiography. 2008
    Jan25(1)8-12.
  • Zaragoza MV, et al. Noncompaction of the left
    ventricle primary cardiomyopathy with an elusive
    genetic etiology. Curr Opin Pediatr. 2007
    Dec19(6)619-27.

63
References
  • Finsterer J, et al. Noncompaction and
    neuromuscular disease with positive troponin-T in
    a nonagenerian. Clin Cardiol. 2007
    Oct(10)527-8.
  • Dodd JD, et al. Quantification of left
    ventricular noncompaction and trabecular delayed
    hyperenhancement with cardiac MRI correlation
    with clinical severity. AJR AM J Roentgenol. 2007
    Oct189(4)974-80.
  • Briec F, et al. Recurrence of dilated
    cardiomyopathy after re-introduction of a
    tricyclic antidepressant. Arch Mal Coeur Vaiss.
    2006 Oct99(10)933-5.
  • Ansari A, et al. Drug induced toxic myocarditis.
    Tex Heart Inst J. 200330(1)76-9.
  • Akgul F, et al. Subclinical left ventricular
    dysfunction in multiple sclerosis. Acta Neurol
    Scand. 2006 Aug114(2)114-8.
  • Cohen BA, Mikol DD. Mitoxantrone treatment of
    multiple sclerosis safety considerations.
    Neurology. 2004 Dec 2863(12 Suppl 6)s28-32.
  • Zingler VC, et al. Assessment of potential
    cardiotoxic side effects of mitoxantrone in
    patients with multiple sclerosis. Eur Neurol.
    200554(1)28-33
  • Stollberger C, Winkler-Dworak M, et al.
    Cardiology. 2007107(4)374-9.

64
Thank you
  • Dr. Scott
  • Dr. Pruett
  • Dr. Hager
  • Dr. Mock
  • Dr. Brust

65
Questions?
About PowerShow.com