Upload
simon23
View
1.338
Download
3
Tags:
Embed Size (px)
DESCRIPTION
Citation preview
Clinical Pathologic ConferenceClinical Pathologic ConferenceDivision of CardiologyDivision of Cardiology
Andrew BolinAndrew BolinMay 11May 11thth, 2007, 2007
The CaseThe Case Chief Complaint: Irregular Heart Beat, Dyspnea Chief Complaint: Irregular Heart Beat, Dyspnea
and Fatigue.and Fatigue. HPI: A 74 year old white female with a history HPI: A 74 year old white female with a history
of paroxysmal atrial fibrillation for 7 years is of paroxysmal atrial fibrillation for 7 years is referred to the cardiology clinic.referred to the cardiology clinic.
The patient had intermittent paroxysms of The patient had intermittent paroxysms of atrial fibrillation while taking flecainide for 5 atrial fibrillation while taking flecainide for 5 years. In the month prior to referral, she was years. In the month prior to referral, she was hospitalized twice for rapid atrial fibrillation. hospitalized twice for rapid atrial fibrillation.
The CaseThe Case Before the first hospitalization, the patient Before the first hospitalization, the patient
reported symptoms of: paroxysmal nocturnal reported symptoms of: paroxysmal nocturnal dyspnea, orthopnea and dyspnea on exertion.dyspnea, orthopnea and dyspnea on exertion.
During the first hospitalization, flecainide was During the first hospitalization, flecainide was discontinued and warfarin and metoprolol were discontinued and warfarin and metoprolol were started. She ruled out for an acute myocardial started. She ruled out for an acute myocardial infarction and a dipyridamole thallium study infarction and a dipyridamole thallium study did not demonstrate ischemia. An echo-did not demonstrate ischemia. An echo-cardiogram revealed normal left ventricular cardiogram revealed normal left ventricular function and a mildly enlarged left atrium.function and a mildly enlarged left atrium.
The CaseThe Case Two weeks prior to her second hospitalization, Two weeks prior to her second hospitalization,
the patient experienced the sudden onset of: the patient experienced the sudden onset of: fatigue, decreased endurance, irregular heart fatigue, decreased endurance, irregular heart beat, constant chest pressure and profound beat, constant chest pressure and profound dyspnea on exertion.dyspnea on exertion.
Prior to this illness, the patient could ambulate for Prior to this illness, the patient could ambulate for thirty minutes without fatigue or dyspnea. On the thirty minutes without fatigue or dyspnea. On the day of consultation she experienced dyspnea on day of consultation she experienced dyspnea on walking 20 yards.walking 20 yards.
Review of Symptoms: Positive for: 10 pound Review of Symptoms: Positive for: 10 pound weight gain, diffuse chronic myalgias, and anxietyweight gain, diffuse chronic myalgias, and anxiety
Past Medical HistoryPast Medical History Hypertension for 11 yearsHypertension for 11 years Breast Cancer: underwent left Breast Cancer: underwent left
mastectomy and chemotherapy 14 years mastectomy and chemotherapy 14 years before. No radiation therapy.before. No radiation therapy.
Recurrent Urinary Tract InfectionsRecurrent Urinary Tract Infections
Social History Family HistorySocial History Family History
Married to retired Married to retired Methodist ministerMethodist minister
Two adult childrenTwo adult children No alcohol, tobacco No alcohol, tobacco
or illicit drugsor illicit drugs No unusual No unusual
exposuresexposures
Mother died of Mother died of congestive heart congestive heart failure at age 79failure at age 79
Sister breast cancerSister breast cancer Sister “bone cancer”Sister “bone cancer”
MedicationsMedications Metoprolol 25 mg BIDMetoprolol 25 mg BID CoumadinCoumadin Hydrochlorothiazide Hydrochlorothiazide
25 mg Daily25 mg Daily Celexa 10 mg Daily Celexa 10 mg Daily Aspirin 81 mg DailyAspirin 81 mg Daily Bactrim DS three Bactrim DS three
days per weekdays per week
Allergies: Allergies: MacrodantinMacrodantin
Physical ExamPhysical Exam Blood Pressure 148/78 (in both arms) Blood Pressure 148/78 (in both arms)
Pulse 90 Respirations 18Pulse 90 Respirations 18 Neck: normal jugular venous pressure, Neck: normal jugular venous pressure,
symmetric brisk carotid upstrokes symmetric brisk carotid upstrokes without bruits, no lymphadenopathywithout bruits, no lymphadenopathy
Lungs: clear to auscultation, dullness to Lungs: clear to auscultation, dullness to percussion in right base percussion in right base
Physical ExamPhysical Exam Cardiac: normal point of maximal impulse, Cardiac: normal point of maximal impulse,
normal S1, physiologically split S2, no normal S1, physiologically split S2, no murmurs, rubs or gallops, strong and murmurs, rubs or gallops, strong and symmetric peripheral pulsessymmetric peripheral pulses
Benign Abdominal ExamBenign Abdominal Exam Extremities: no clubbing, cyanosis or edemaExtremities: no clubbing, cyanosis or edema Musculoskeletal: tenderness to palpation in Musculoskeletal: tenderness to palpation in
trapezius and shoulder girdle bilaterallytrapezius and shoulder girdle bilaterally
StudiesStudies Electrocardiogram: atrial fibrillation, Electrocardiogram: atrial fibrillation,
incomplete right bundle branch block incomplete right bundle branch block and nonspecific ST-T abnormalitiesand nonspecific ST-T abnormalities
Chest X-ray: possible right pleural Chest X-ray: possible right pleural effusioneffusion
Transesophageal Echocardiogram: Transesophageal Echocardiogram: unusual thickening of left atrial walls, unusual thickening of left atrial walls, small pericardial effusionsmall pericardial effusion
StudiesStudies Chest CT: pericardial effusion and Chest CT: pericardial effusion and
bilateral pleural effusions, biapical bilateral pleural effusions, biapical pleuroparenchymal scarringpleuroparenchymal scarring
Cardiac MRI: atrium unremarkableCardiac MRI: atrium unremarkable Labs: CBC, E-group, BUN, Creatinine, Labs: CBC, E-group, BUN, Creatinine,
TSH, Troponin and CK were all within TSH, Troponin and CK were all within normal limits.normal limits.
Right ThoracentesisRight Thoracentesis Consistent with Transudative EffusionConsistent with Transudative Effusion WBC 485, Lymphocytes 89%WBC 485, Lymphocytes 89% Flow Cytometry: Consistent with a Flow Cytometry: Consistent with a
Reactive Effusion.Reactive Effusion. Cytology: No Malignant CellsCytology: No Malignant Cells
Case SummaryCase Summary Elderly White Female with A-fibElderly White Female with A-fib Progressive Symptoms of CHF Over Weeks Without Progressive Symptoms of CHF Over Weeks Without
Echocardiographic Evidence of LV DysfunctionEchocardiographic Evidence of LV Dysfunction Transudative Lymphocytic Bilateral Pleural Transudative Lymphocytic Bilateral Pleural
Effusions Effusions Biapical Pleuroparenchymal ScarringBiapical Pleuroparenchymal Scarring Pericardial Effusion Without Evidence of Pericardial Effusion Without Evidence of
TamponadeTamponade Incomplete Right Bundle Branch BlockIncomplete Right Bundle Branch Block Abnormal Left Atrial WallsAbnormal Left Atrial Walls
Case SummaryCase Summary The Patient Has a Pathologic Process The Patient Has a Pathologic Process
Involving the Pericardium and Involving the Pericardium and Myocardium that is Not Related to Myocardium that is Not Related to Valvular or Ischemic Disease. Valvular or Ischemic Disease. Additionally, Noted is an Additionally, Noted is an Echocardiogram Demonstrating Highly Echocardiogram Demonstrating Highly Abnormal Left Atrial Walls.Abnormal Left Atrial Walls.
Causes of Lymphocytic Pleural Causes of Lymphocytic Pleural EffusionsEffusions
CHF (Only Transudate)CHF (Only Transudate) NeoplasmNeoplasm Fungal InfectionFungal Infection TBTB SarcoidosisSarcoidosis Rheumatoid ArthritisRheumatoid Arthritis Hepatic HydrothoraxHepatic Hydrothorax Yellow Nail SyndromeYellow Nail Syndrome ChylothoraxChylothorax
Causes of Left Atrial Causes of Left Atrial EnlargementEnlargement
Valvular DiseaseValvular Disease Myocardial InfarctionMyocardial Infarction Obstructive CardiomyopathyObstructive Cardiomyopathy HypertensionHypertension Infiltrative CardiomyopathyInfiltrative Cardiomyopathy Inflammatory CardiomyopathyInflammatory Cardiomyopathy Primary or Metastatic TumorsPrimary or Metastatic Tumors
Differential Diagnosis for Differential Diagnosis for Pericardial EffusionPericardial Effusion
InfectiousInfectious MalignantMalignant AutoimmuneAutoimmune CardiomyopathyCardiomyopathy Drug InducedDrug Induced CardiacCardiac Constrictive Constrictive
PericarditisPericarditis
RadiationRadiation TraumaTrauma Metabolic: Metabolic:
Hypothyroidism Hypothyroidism Uremia Uremia Ovarian- Ovarian- Hyperstimulation- Hyperstimulation- SyndromeSyndrome
Pericardial EffusionPericardial Effusion All Causes of Pericardial Effusion Can All Causes of Pericardial Effusion Can
Also Cause Pleural Effusions.Also Cause Pleural Effusions.
All Causes of Pericardial Disease Can All Causes of Pericardial Disease Can Also Include the Myocardium to Varying Also Include the Myocardium to Varying Degrees. Termed Myopericarditis or Degrees. Termed Myopericarditis or Perimyocarditis.Perimyocarditis.
InfectiousInfectiousPericardial EffusionPericardial Effusion
ViralViral Pyogenic/BacterialPyogenic/Bacterial FungalFungal ParasiticParasitic TuberculousTuberculous Any Infectious Agent Can Affect the Any Infectious Agent Can Affect the
PericardiumPericardium
Viral PericarditisViral Pericarditis Most Common Infectious PericarditisMost Common Infectious Pericarditis Usually Cause Acute Pericarditis with: Fever, Rub, Typical Usually Cause Acute Pericarditis with: Fever, Rub, Typical
ECG Changes, Leukocytosis, ECG Changes, Leukocytosis, 3:1 Male to Female Ratio, Predominately in Young Adults3:1 Male to Female Ratio, Predominately in Young Adults Usually Self Limited Resolving Within 2 WeeksUsually Self Limited Resolving Within 2 Weeks Preceded by Upper Respiratory or GI Illness by 1-3 WeeksPreceded by Upper Respiratory or GI Illness by 1-3 Weeks 50% Have Recurrence Within 8 Months 50% Have Recurrence Within 8 Months Common Viruses: Enteroviruses, Coxsackie A&B, Common Viruses: Enteroviruses, Coxsackie A&B,
Adenovirus, Rhinovirus, Echovirus type 8, and InfluenzaAdenovirus, Rhinovirus, Echovirus type 8, and Influenza Diagnosis of ExclusionDiagnosis of Exclusion
Pyogenic/Bacterial PericarditisPyogenic/Bacterial Pericarditis Fulminant Course Fulminant Course Symptoms Arise Over a Few Days and Usually Lead to Symptoms Arise Over a Few Days and Usually Lead to
Tamponade and Sepsis Tamponade and Sepsis Commonly Pneumococus, Staph, StrepCommonly Pneumococus, Staph, Strep Typically Associated with High Fever (Virtually All Typically Associated with High Fever (Virtually All
Cases), Toxicity, Tachycardia, RubCases), Toxicity, Tachycardia, Rub Maybe More Insidious Course in the ElderlyMaybe More Insidious Course in the Elderly Usually Require Systemic Antibiotics and Pericardial Usually Require Systemic Antibiotics and Pericardial
Drainage With ExplorationDrainage With Exploration In the Antibiotic Era Usually Associated with Dialysis, In the Antibiotic Era Usually Associated with Dialysis,
Thoracic Surgery and Chemotherapy.Thoracic Surgery and Chemotherapy.
FungalFungal Pericarditis Pericarditis
Usually Occurs in ImmunocompromisedUsually Occurs in Immunocompromised Histoplasma: Usually Young Males in Endemic Histoplasma: Usually Young Males in Endemic
Areas, Often Benign Course Remitting Areas, Often Benign Course Remitting Spontaneously, May Have Mediastinal Spontaneously, May Have Mediastinal Lymphadnopathy and Pleural EffusionsLymphadnopathy and Pleural Effusions
Coccidiomycosis: Usually in Patients with Coccidiomycosis: Usually in Patients with Widely Disseminated InfectionWidely Disseminated Infection
Candida & Aspergillus: Opportunistic, Candida & Aspergillus: Opportunistic, Necrotizing and Leading to Thrombosis Necrotizing and Leading to Thrombosis
Parasitic PericarditisParasitic Pericarditis Clinical Course Resembles that of Clinical Course Resembles that of
Pyogenic PericarditisPyogenic Pericarditis Residents or Travelers to Endemic AreasResidents or Travelers to Endemic Areas Usually Have an Identifiable Primary Usually Have an Identifiable Primary
Source i.e. Liver or LungSource i.e. Liver or Lung EosinophilliaEosinophillia
Primary TumorsPrimary Tumors 75% Benign, 50% of Benign Tumors Are 75% Benign, 50% of Benign Tumors Are
MyxomasMyxomas
25% Malignant 25% Malignant 95% of Malignant Tumors Are Sarcomas95% of Malignant Tumors Are Sarcomas 5% of Malignant Tumors Are Lymphomas5% of Malignant Tumors Are Lymphomas Symptoms Related to Location and SizeSymptoms Related to Location and Size The Pathology of the Tumor Can Be Speculated The Pathology of the Tumor Can Be Speculated
by Appearance on Imaging and Anatomic by Appearance on Imaging and Anatomic LocationLocation
MyxomasMyxomas Occur in 3Occur in 3rdrd-6-6thth Decades Decades Female Predominance 60-70%Female Predominance 60-70% 80% Found in the Left Atrium80% Found in the Left Atrium Presentation: Obstruction of Mitral or Tricuspid Presentation: Obstruction of Mitral or Tricuspid
Valve 67% (Occasionally Positional), Embolization Valve 67% (Occasionally Positional), Embolization 29%, 15% of Cases Have Audible Tumor Plop29%, 15% of Cases Have Audible Tumor Plop
Constitutional Symptoms: Fever, Fatigue, Weight Constitutional Symptoms: Fever, Fatigue, Weight Loss, Myalgias, and ArthralgiasLoss, Myalgias, and Arthralgias
Not Commonly Associated With Pericardial Not Commonly Associated With Pericardial EffusionEffusion
MyxomasMyxomas Echo Demonstrates Endocardial Mass, Echo Demonstrates Endocardial Mass,
Rarely May Be IntramuralRarely May Be Intramural Treatment is Surgical Resection, Though Treatment is Surgical Resection, Though
Myxoma May Recur 1-5% Myxoma May Recur 1-5% 10% May Be Familial 10% May Be Familial
Benign Papillary Benign Papillary FibroelastomasFibroelastomas
22ndnd Most Common Benign Tumor Most Common Benign Tumor Predominantly Affect Valves, Account for Predominantly Affect Valves, Account for
75% of Valvular Tumors75% of Valvular Tumors Often Asymptomatic, But May Present Often Asymptomatic, But May Present
with Dyspnea, Embolization, or Chest with Dyspnea, Embolization, or Chest PainPain
Visible By Echo as Mobile Endocardial Visible By Echo as Mobile Endocardial MassMass
Other Benign Cardiac TumorsOther Benign Cardiac Tumors All Are RareAll Are Rare Rhabdomyomas: Most Present in ChildhoodRhabdomyomas: Most Present in Childhood Fibromas: Most Present in ChildhoodFibromas: Most Present in Childhood Hemangiomas: Have Characteristic Findings Hemangiomas: Have Characteristic Findings
on Imagingon Imaging Lipomas: Have Characteristic Findings on Lipomas: Have Characteristic Findings on
ImagingImaging Teratomas: Have Characteristic Findings on Teratomas: Have Characteristic Findings on
Imaging, Most Present in ChildhoodImaging, Most Present in Childhood
Malignant SarcomasMalignant Sarcomas Angiosarcomas: Most “Common” Angiosarcomas: Most “Common”
Malignant Primary Cardiac TumorMalignant Primary Cardiac Tumor Men:Women 3:1, 65-90% Have Men:Women 3:1, 65-90% Have
Metastases at PresentationMetastases at Presentation 80% Occur In the Right Atrium 80% Occur In the Right Atrium
(Other Sarcomas Are Usually in the (Other Sarcomas Are Usually in the Left Atrium)Left Atrium)
Malignant SarcomasMalignant Sarcomas Rhabdomyosarcoma: 2Rhabdomyosarcoma: 2ndnd Most Most
“Common” Malignant Primary Cardiac “Common” Malignant Primary Cardiac TumorTumor
Can Occur at Any AgeCan Occur at Any Age Most Have Aggressive Features Seen on Most Have Aggressive Features Seen on
Imaging (Invasion of Local Structures) Imaging (Invasion of Local Structures)
Primary Cardiac LymphomasPrimary Cardiac Lymphomas By Far Most Commonly Occur in the By Far Most Commonly Occur in the
Right AtriumRight Atrium More Common in AIDS and More Common in AIDS and
Immunocompromised PatientsImmunocompromised Patients Most Are Fatal Shortly After DiagnosisMost Are Fatal Shortly After Diagnosis
Metastatic TumorsMetastatic Tumors 20 Times More Common than Primary Tumors20 Times More Common than Primary Tumors Lung, Breast, Leukemia, Lymphoma, Melanoma, Lung, Breast, Leukemia, Lymphoma, Melanoma,
Kaposi’s SarcomaKaposi’s Sarcoma Presenting Symptoms Based on Location of Presenting Symptoms Based on Location of
Tumor, Usually Causes Pericardial EffusionTumor, Usually Causes Pericardial Effusion If the Patient Is Symptomatic the Tumor Is If the Patient Is Symptomatic the Tumor Is
Virtually Always Visible on Echo, CT or MRI Virtually Always Visible on Echo, CT or MRI Most Patients Have Mediastinal LymphadnopathyMost Patients Have Mediastinal Lymphadnopathy Most Have Aggressive Features Seen on Imaging Most Have Aggressive Features Seen on Imaging
(Invasion of Local Structures)(Invasion of Local Structures)
AutoimmuneAutoimmunePericardial EffusionPericardial Effusion
Rheumatoid ArthritisRheumatoid Arthritis SLESLE SclerodermaScleroderma Rheumatic FeverRheumatic Fever Wegener’s GranulomatosisWegener’s Granulomatosis Ankylosing SpondylitisAnkylosing Spondylitis Inflammatory Bowel DiseaseInflammatory Bowel Disease
CardiomyopathiesCardiomyopathies IschemicIschemic DilatedDilated HypertrophicHypertrophic ValvularValvular HypertensiveHypertensive Restrictive or InfiltrativeRestrictive or Infiltrative InflammatoryInflammatory
RestrictiveRestrictiveCardiomyopathyCardiomyopathy
Excessively Rigid Ventricular Walls Excessively Rigid Ventricular Walls Cause Diastolic DysfunctionCause Diastolic Dysfunction
Systolic Function Is Not ImpairedSystolic Function Is Not Impaired Commonly Present with Dyspnea, Commonly Present with Dyspnea,
Fatigue and Chest PainFatigue and Chest Pain Commonly Associated with A-FibCommonly Associated with A-Fib Difficult to Distinguish Clinically from Difficult to Distinguish Clinically from
Constrictive PericarditisConstrictive Pericarditis
RestrictiveRestrictiveCardiomyopathyCardiomyopathy
Storage Diseases Storage Diseases InfiltrativeInfiltrative EndomyocardialEndomyocardial
Restrictive CardiomyopathyRestrictive CardiomyopathyInherited Storage DisordersInherited Storage Disorders
Hemochromatosis: Deposition of Iron in Liver, Hemochromatosis: Deposition of Iron in Liver, Pancreas, Gonads and MyocardiumPancreas, Gonads and Myocardium
Fabry: Intracellular Accumulation of Fabry: Intracellular Accumulation of Glycosphingolipids in the Skin, Kidneys and Glycosphingolipids in the Skin, Kidneys and MyocardiumMyocardium
Gaucher: Accumulation of Cerebrosides in the Spleen, Gaucher: Accumulation of Cerebrosides in the Spleen, Liver, Bone Marrow, Lymph Nodes, Brain and Liver, Bone Marrow, Lymph Nodes, Brain and MyocardiumMyocardium
Glycogen Storage Diseases: Survival to Adulthood Glycogen Storage Diseases: Survival to Adulthood Uncommon Except in Type III, Where Infiltration of the Uncommon Except in Type III, Where Infiltration of the Myocardium is Usually Not Clinically RelevantMyocardium is Usually Not Clinically Relevant
Restrictive CardiomyopathyRestrictive CardiomyopathyInfiltrative DisordersInfiltrative Disorders
AmyloidAmyloid SarcoidSarcoid Carcinoid Heart Disease: Serotonin Carcinoid Heart Disease: Serotonin
Producing Tumor Causing Producing Tumor Causing Cutaneous Flushing, Diarrhea, Cutaneous Flushing, Diarrhea, Bronchoconstriction and Right Bronchoconstriction and Right Sided Endocardial Plaques Sided Endocardial Plaques Composed of Fibrous Tissue Composed of Fibrous Tissue
Restrictive Cardiomyopathy Restrictive Cardiomyopathy Endocardial Fibrotic DiseasesEndocardial Fibrotic Diseases
Endomyocardial Disease (Africa, Endomyocardial Disease (Africa, Eosinophilia)Eosinophilia)
Loffler Endocarditis (Eosinophilia)Loffler Endocarditis (Eosinophilia) Endomyocardial Fibrosis (Africa)Endomyocardial Fibrosis (Africa)
MyocarditisMyocarditis Primary Primary LymphocyticLymphocytic Acute Rheumatic FeverAcute Rheumatic Fever Eosinophilic Eosinophilic LymeLyme ChagasChagas HIVHIV Drugs Drugs RadiationRadiation Giant Cell Giant Cell
MyocarditisMyocarditis Inflammation of the Myocardium Associated with Inflammation of the Myocardium Associated with
Injury to MyocytesInjury to Myocytes Presents as an Acute Illness, Often Congestive Heart Presents as an Acute Illness, Often Congestive Heart
Failure, Chest Pain and FatigueFailure, Chest Pain and Fatigue Moderate Elevation of Cardiac Biomarkers, Moderate Elevation of Cardiac Biomarkers,
Nonspecific ST-T Changes, Arrhythmias, Nonspecific Nonspecific ST-T Changes, Arrhythmias, Nonspecific EchoEcho
Diagnosis by Endomyocardial BiopsyDiagnosis by Endomyocardial Biopsy
MyocarditisMyocarditis Primary or Acute Myocarditis Associated with Primary or Acute Myocarditis Associated with
Viral Infection (20 Viruses). Most Commonly Viral Infection (20 Viruses). Most Commonly Enteroviruses (Coxsackie). Viral Etiology Enteroviruses (Coxsackie). Viral Etiology Suggested by Antecedent Upper Respiratory Suggested by Antecedent Upper Respiratory or GI Illness. or GI Illness.
Acute Viral Myocarditis is Associated with Acute Viral Myocarditis is Associated with Lymphocytic InfiltrateLymphocytic Infiltrate
Inflammatory MyocarditisInflammatory Myocarditis Lymphocytic Myocarditis: Most Will Improve Over 1-6 Lymphocytic Myocarditis: Most Will Improve Over 1-6
Months, a Minority Will Fail to Clear a Cardiotropic Virus Months, a Minority Will Fail to Clear a Cardiotropic Virus or Develop Persistent Inflammation That Leads to or Develop Persistent Inflammation That Leads to Chronic Cardiomyopathy, Heart Block or Ventricular Chronic Cardiomyopathy, Heart Block or Ventricular Arrhythmias. Arrhythmias.
Myocarditis Treatment Trial: Prospective Randomized, Myocarditis Treatment Trial: Prospective Randomized, Double-Blind, Placebo-Controlled Trial of Prednisone and Double-Blind, Placebo-Controlled Trial of Prednisone and Cyclosporine or Azathioprine for the Treatment of Biopsy Cyclosporine or Azathioprine for the Treatment of Biopsy Proven Lymphocytic Myocarditis in Acute CHF. There Proven Lymphocytic Myocarditis in Acute CHF. There Was No Benefit from Immunosuppression.Was No Benefit from Immunosuppression.
Immune Modulation for Acute Cardiomyopathy: Immune Modulation for Acute Cardiomyopathy: Evaluated the Role of IVIG and Found no Benefit.Evaluated the Role of IVIG and Found no Benefit.
May Lead to Dilated CardiomyopathyMay Lead to Dilated Cardiomyopathy
Inflammatory MyocarditisInflammatory Myocarditis Acute Rheumatic Fever: Pancarditis Occurring in 3% of Acute Rheumatic Fever: Pancarditis Occurring in 3% of
Untreated Streptococal A Pharyngeal InfectionsUntreated Streptococal A Pharyngeal Infections Hypersensitivity Myocarditis (Drug Induced): Tricyclics, Hypersensitivity Myocarditis (Drug Induced): Tricyclics,
Penicillins, Antipsychotics, Present with Rash & FeverPenicillins, Antipsychotics, Present with Rash & Fever Eosinophilic Myocarditis: Occurs in Conjunction with: Eosinophilic Myocarditis: Occurs in Conjunction with:
Hypereosinophilic Syndrome, Churg-Strauss Syndrome & Hypereosinophilic Syndrome, Churg-Strauss Syndrome & Loffler’s Endomyocardial Fibrosis. Peripheral EosinophiliaLoffler’s Endomyocardial Fibrosis. Peripheral Eosinophilia
Lyme Myocarditis: Borrelia burgdorferi, Often Develop Lyme Myocarditis: Borrelia burgdorferi, Often Develop Heart Block or Arrhythmias,Heart Block or Arrhythmias,
Chagas Cardiomyopathy: Trypanosoma cruzi (Protozoan), Chagas Cardiomyopathy: Trypanosoma cruzi (Protozoan), Endemic to Central & South AmericaEndemic to Central & South America
HIV MyocarditisHIV Myocarditis
Drug Induced Pericardial Drug Induced Pericardial EffusionEffusion
ProcainamideProcainamide HydralazineHydralazine Phenytoin Phenytoin IsoniazideIsoniazide MinoxidilMinoxidil Methysergide\ Methysergide\
ergot derivativeergot derivative Phenylbutazone/ Phenylbutazone/
NSAIDNSAID
AnticoagulantsAnticoagulants Cromolyn SodiumCromolyn Sodium DantroleneDantrolene ThromboticsThrombotics PenicillinPenicillin Doxorubicin/ Doxorubicin/
AdriamycinAdriamycin Cytoxan/ Cytoxan/
CyclophosphamideCyclophosphamide
CardiacCardiacPericardial EffusionsPericardial Effusions
Myocardial InfarctionMyocardial Infarction Postmyocardial Infarction “Dressler’s Postmyocardial Infarction “Dressler’s
Syndrome”Syndrome” TraumaTrauma Aortic Dissection with Hemorrhage into Aortic Dissection with Hemorrhage into
Pericardial SpacePericardial Space PostpericardiotomyPostpericardiotomy
Constrictive PericarditisConstrictive Pericarditis No Pericardial Thickening Was Noted on No Pericardial Thickening Was Noted on
ImagingImaging Few Case Reports of Constriction Few Case Reports of Constriction
Without Pericardial Thickening Without Pericardial Thickening Would Not Account for Abnormal Left Would Not Account for Abnormal Left
Atrium Seen on EchoAtrium Seen on Echo
Possible Etiologies for This Possible Etiologies for This CaseCase
Tuberculous PericarditisTuberculous Pericarditis Cardiac SarcoidosisCardiac Sarcoidosis Cardiac AmyloidosisCardiac Amyloidosis Giant Cell MyocarditisGiant Cell Myocarditis
Tuberculous PericarditisTuberculous Pericarditis 4-10% of All Acute Pericarditis is Caused 4-10% of All Acute Pericarditis is Caused
by TB (Reported up to 80% in Some 3by TB (Reported up to 80% in Some 3rdrd World Countries) World Countries)
1-4% of Patients with TB Have Pericardial 1-4% of Patients with TB Have Pericardial InvolvementInvolvement
Etiology of 20% of Constrictive Etiology of 20% of Constrictive PericarditisPericarditis
Cases 93% of all Pericardial Effusions in Cases 93% of all Pericardial Effusions in HIV PatientsHIV Patients
Four Pathologic Stages of TB Four Pathologic Stages of TB PericarditisPericarditis
I-Dry: Fibrin Deposition & Granulomatous I-Dry: Fibrin Deposition & Granulomatous Reaction. Usually Clinically Silent Reaction. Usually Clinically Silent
II-Effusive: Serous Fluid Accumulation Caused II-Effusive: Serous Fluid Accumulation Caused by Hypersensitivity to Tuberculoprotein and by Hypersensitivity to Tuberculoprotein and Impaired ResorptionImpaired Resorption
III- Absorptive: Effusion Resolves and Fibrous III- Absorptive: Effusion Resolves and Fibrous Tissue Replaces Granulomas, Pericardium Tissue Replaces Granulomas, Pericardium ThickensThickens
IV-Constrictive: Parietal Pericardial IV-Constrictive: Parietal Pericardial CalcificationCalcification
Presentation of Tuberculous Presentation of Tuberculous PericarditisPericarditis
Dyspnea 45-90%Dyspnea 45-90% Chest Pain 40-75%Chest Pain 40-75% Orthopnea 20-65%Orthopnea 20-65% Distant Heart Sounds Distant Heart Sounds
25-55%25-55%
Rub 30-85%Rub 30-85% Cough 50-95%Cough 50-95% Fever 80-100%Fever 80-100%
Presentation of Tuberculous Presentation of Tuberculous PericarditisPericarditis
50% of Patient Have Slowly Progressive 50% of Patient Have Slowly Progressive Insidious PresentationInsidious Presentation
95% Have Cardiomegally, 30% Have 95% Have Cardiomegally, 30% Have Active Pulmonary TB, 39-71% Have Active Pulmonary TB, 39-71% Have Pleural Effusions L>R. Bilateral Pleural Pleural Effusions L>R. Bilateral Pleural Effusions More Common than Rub.Effusions More Common than Rub.
Tuberculous PericarditisTuberculous Pericarditis ““Because of the variable and nonspecific Because of the variable and nonspecific
features of TB pericarditis, establishing features of TB pericarditis, establishing the diagnosis on clinical grounds alone the diagnosis on clinical grounds alone is impossible.” D.H. Spodickis impossible.” D.H. Spodick
Diagnosis by Pericardial Fluid or Biopsy: Diagnosis by Pericardial Fluid or Biopsy: Positive AFB Smear, Culture, Caseating Positive AFB Smear, Culture, Caseating Granulomas, TB DNA PCRGranulomas, TB DNA PCR
TreatmentTreatmentTuberculous PericarditisTuberculous Pericarditis
Antibiotics: Isoniazid, Rifampin, Antibiotics: Isoniazid, Rifampin, Pyrazinamide, EthambutolPyrazinamide, Ethambutol
Corticosteroids: Reduces Mortality and Corticosteroids: Reduces Mortality and Need for Subsequent PericardiocentisisNeed for Subsequent Pericardiocentisis
Cardiac SarcoidosisCardiac Sarcoidosis Causes Restrictive CardiomyopathyCauses Restrictive Cardiomyopathy Clinically Relevant in 5%, 25% Incidentally Clinically Relevant in 5%, 25% Incidentally
Noted at AutopsyNoted at Autopsy 69% of Clinically Relevant Cardiac Sarcoidosis 69% of Clinically Relevant Cardiac Sarcoidosis
Patients Have No Other Manifestations of the Patients Have No Other Manifestations of the Disease at PresentationDisease at Presentation
Presentation: Complete Heart Block (Be Presentation: Complete Heart Block (Be Suspicious in Young Patients With Complete Suspicious in Young Patients With Complete Heart Block), Ventricular Arrhythmia, Heart Block), Ventricular Arrhythmia, Congestive Heart Failure Systolic or Diastolic Congestive Heart Failure Systolic or Diastolic DysfunctionDysfunction
Cardiac SarcoidosisCardiac Sarcoidosis Echo Usually Reveals Hyperechogenic Left Echo Usually Reveals Hyperechogenic Left
Ventricular Walls, May Develop Ventricular Ventricular Walls, May Develop Ventricular Aneurysm From Myocardial Scar TissueAneurysm From Myocardial Scar Tissue
Diagnosis Based on Clinical Suspicion in a Diagnosis Based on Clinical Suspicion in a Patient with Known Sarcoid or by Patient with Known Sarcoid or by Endomyocardial Biopsy in a Patient with No Endomyocardial Biopsy in a Patient with No Evidence of Sarcoid in Other Organs. Biopsy Evidence of Sarcoid in Other Organs. Biopsy Consistent with Sarcoid 30% of Cases.Consistent with Sarcoid 30% of Cases.
Treat with SteroidsTreat with Steroids
Cardiac AmyloidosisCardiac Amyloidosis Causes Restrictive CardiomyopathyCauses Restrictive Cardiomyopathy Myocyte Destruction and Replacement Myocyte Destruction and Replacement
with Amyloid Fibrils (Beta Pleated with Amyloid Fibrils (Beta Pleated Sheets) Leading to Progressive Sheets) Leading to Progressive Thickening of the Ventricular Walls.Thickening of the Ventricular Walls.
More Common in Primary (AL) More Common in Primary (AL) Amyloidosis, a Plasma Cell Dyscrasia Amyloidosis, a Plasma Cell Dyscrasia Where Immunoglobulins Form Amyloid Where Immunoglobulins Form Amyloid ProteinProtein
Cardiac AmyloidosisCardiac Amyloidosis EKG: Often Have Bundle Branch Block, Low EKG: Often Have Bundle Branch Block, Low
Voltage Despite Thick Ventricles, or A-Fib Voltage Despite Thick Ventricles, or A-Fib Most Patients with Amyloidosis, Even Those Most Patients with Amyloidosis, Even Those
with no Cardiac Symptoms, Have Abnormal with no Cardiac Symptoms, Have Abnormal Echocardiograms with: Ventricular Wall Echocardiograms with: Ventricular Wall Thickening (70%), Isolated Septal Wall Thickening (70%), Isolated Septal Wall Thickening (30%), Diastolic Dysfunction (57%), Thickening (30%), Diastolic Dysfunction (57%), Systolic Dysfunction (27%), Pericardial Systolic Dysfunction (27%), Pericardial Effusion (40%), Myocardial Sparkling Pattern in Effusion (40%), Myocardial Sparkling Pattern in 2D (27%)2D (27%)
Cardiac AmyloidosisCardiac Amyloidosis Diagnosis with Tissue Biopsy Revealing Diagnosis with Tissue Biopsy Revealing
Amyloidosis, Often Abdominal Fat PadAmyloidosis, Often Abdominal Fat Pad Prognosis Less Than 1 Year, Survival at Prognosis Less Than 1 Year, Survival at
5 Years <5% 5 Years <5% Treatment: Primary Amyliodosis Limited Treatment: Primary Amyliodosis Limited
Benefit of Alkylating AgentsBenefit of Alkylating Agents Secondary Amyloidosis: Treat Secondary Amyloidosis: Treat
Underlying DiseaseUnderlying Disease
Giant Cell MyocarditisGiant Cell Myocarditis Causes Inflammatory CardiomyopathyCauses Inflammatory Cardiomyopathy Unknown Etiology Unknown Etiology Causes Progressive Left Ventricular Failure Causes Progressive Left Ventricular Failure
and Arrhythmias, Over Weeks to Monthsand Arrhythmias, Over Weeks to Months Rare, 80 Cases Reported by 1997 Rare, 80 Cases Reported by 1997 Average Age 43 (Infants to Elderly) Usually Average Age 43 (Infants to Elderly) Usually
Affects Otherwise Healthy PatientsAffects Otherwise Healthy Patients Equal Predominance of Men and WomenEqual Predominance of Men and Women 90% Are Caucasian90% Are Caucasian
Giant Cell MyocarditisGiant Cell Myocarditis Diagnosed by Endomyocardial Biopsy Diagnosed by Endomyocardial Biopsy
Demonstrating: Diffuse Myocardial Demonstrating: Diffuse Myocardial Necrosis with Multinucleated Giant Cells Necrosis with Multinucleated Giant Cells in the Absence of Sarcoid Like in the Absence of Sarcoid Like Granuloma. Inflammatory Infiltrate in Granuloma. Inflammatory Infiltrate in Close Apposition to Myocyte Necrosis. Close Apposition to Myocyte Necrosis. Negative Culture and Stains for Infection, Negative Culture and Stains for Infection, No Viral Particles on Electron No Viral Particles on Electron Microscopy.Microscopy.
Giant Cell MyocarditisGiant Cell Myocarditis 75% Present with CHF, 25% with Ventricular 75% Present with CHF, 25% with Ventricular
Arrhythmias, Sudden Cardiac Death (50%), Heart Arrhythmias, Sudden Cardiac Death (50%), Heart Block, Diffuse ST-T Abnormalities on EKG.Block, Diffuse ST-T Abnormalities on EKG.
May be Localized Rather Than Diffuse in Earlier May be Localized Rather Than Diffuse in Earlier Stages. There Are Case Reports of Giant Cell Stages. There Are Case Reports of Giant Cell Myocarditis Affecting Only the Atria. Myocarditis Affecting Only the Atria.
20% Have Other Autoimmune Disease: Hashimoto 20% Have Other Autoimmune Disease: Hashimoto Thyroiditis, RA, MG, Takayasu Arteritis, Alopecia, Thyroiditis, RA, MG, Takayasu Arteritis, Alopecia, Vitiligo, Pernicious Anemia, Crohn’s Disease, Vitiligo, Pernicious Anemia, Crohn’s Disease, Ulcerative Colitis, ITP, Celiac DiseaseUlcerative Colitis, ITP, Celiac Disease
Giant Cell MyocarditisGiant Cell Myocarditis Survival 5.5 Months After Onset of Symptoms, Survival 5.5 Months After Onset of Symptoms,
Normally Succumb to CHF or Arrhythmia Normally Succumb to CHF or Arrhythmia Immunosuppresive Therapy with Corticosteroids, Immunosuppresive Therapy with Corticosteroids,
Cyclosporin, Azathioprine Increases Survival to 12 Cyclosporin, Azathioprine Increases Survival to 12 Months, Case Reports of Dramatic Improvement with Months, Case Reports of Dramatic Improvement with ImmunotherapyImmunotherapy
Cardiac Transplantation (with 25% recurrence), 71% Cardiac Transplantation (with 25% recurrence), 71% Survival at 5 Years Survival at 5 Years
The Giant Cell Myocarditis Treatment Trial and The Giant Cell Myocarditis Treatment Trial and Registry, Randomized Controlled Trial of T-Cell Registry, Randomized Controlled Trial of T-Cell Targeted Treatment with Muromonab-CD3 and Targeted Treatment with Muromonab-CD3 and CyclosporineCyclosporine
Possible Etiologies for This CasePossible Etiologies for This Case Tuberculous PericarditisTuberculous Pericarditis No Fever, No Exposure, Not Immunocompromized, No Fever, No Exposure, Not Immunocompromized,
Would Not Explain Abnormal AtriumWould Not Explain Abnormal Atrium Cardiac SarcoidosisCardiac Sarcoidosis No Evidence of Systemic Sarcoid, Normal Left No Evidence of Systemic Sarcoid, Normal Left
Ventricular WallsVentricular Walls Cardiac AmyloidosisCardiac Amyloidosis No Low Voltage EKG, No Ventricular Wall ThickeningNo Low Voltage EKG, No Ventricular Wall Thickening Giant Cell MyocarditisGiant Cell Myocarditis Time Course Is Consistant with Our Patient, Could Time Course Is Consistant with Our Patient, Could
Account for Symptoms of CHF, Effusions, and Account for Symptoms of CHF, Effusions, and Abnormal Left AtriumAbnormal Left Atrium
DiagnosisDiagnosis
Giant Cell MyocarditisGiant Cell Myocarditis
Diagnostic Test of Choice: Diagnostic Test of Choice: Endomyocardial Biopsy Endomyocardial Biopsy
ReferencesReferences Spodick DH. The Pericardium. New York: Marcel Dekker; 1997Spodick DH. The Pericardium. New York: Marcel Dekker; 1997 Braunwald E, Fauci AS. Harrison’s Textbook of Internal Medicine. McGraw-Hill; 2001Braunwald E, Fauci AS. Harrison’s Textbook of Internal Medicine. McGraw-Hill; 2001 Up To Date, 2007Up To Date, 2007 Garay S, Rom WN. Tuberculosis. Boston: Libble, Brown & CO; 1996Garay S, Rom WN. Tuberculosis. Boston: Libble, Brown & CO; 1996 Hall HD, Hammar SP. Pulmonary Pathology. New York: Springer-Verlag; 1994Hall HD, Hammar SP. Pulmonary Pathology. New York: Springer-Verlag; 1994 Roth JA, Ruckdeschel JC. Weisenburger, T. H. Thoracic Oncology. Philadelphia: W.B. Roth JA, Ruckdeschel JC. Weisenburger, T. H. Thoracic Oncology. Philadelphia: W.B.
Saunders Company; 1995Saunders Company; 1995 Murphy JG, Lloyd MA. Mayo Clinic Cardiology. Rochester: Mayo Clinic Scientific Press; 2007Murphy JG, Lloyd MA. Mayo Clinic Cardiology. Rochester: Mayo Clinic Scientific Press; 2007 Zipes DP, Libby P, Bonow RO, Braunwald E. Braunwald’s Heart Disease. Philadelphia: Zipes DP, Libby P, Bonow RO, Braunwald E. Braunwald’s Heart Disease. Philadelphia:
Elsevier Saunders; 2005Elsevier Saunders; 2005 Silver MD, Gotlieb AI, Schoen FJ. Cardiovascular Pathology. Philadelphia: Churchill Silver MD, Gotlieb AI, Schoen FJ. Cardiovascular Pathology. Philadelphia: Churchill
Livingstone; 2001Livingstone; 2001 Cooper LT, Berry GJ, et al: Idiopathic Giant-Cell Myocarditis. NE J Medicine, June, 1997Cooper LT, Berry GJ, et al: Idiopathic Giant-Cell Myocarditis. NE J Medicine, June, 1997 Frustaci A, Chimenti C, et al: Giant Cell Myocarditis, Responding to Immunosuppressive Frustaci A, Chimenti C, et al: Giant Cell Myocarditis, Responding to Immunosuppressive
Therapy. Chest, March, 2000Therapy. Chest, March, 2000 Cooper LT: Giant Cell Myocarditis: Diagnosis and Treatment. Herz, May, 2000Cooper LT: Giant Cell Myocarditis: Diagnosis and Treatment. Herz, May, 2000 Rosenstein ED, Zucker MJ, et al: Giant Cell Myocarditis: Most Fatal of Autoimmune Diseases. Rosenstein ED, Zucker MJ, et al: Giant Cell Myocarditis: Most Fatal of Autoimmune Diseases.
Seminars in Arthrithritis and Rheumatism, August, 2000Seminars in Arthrithritis and Rheumatism, August, 2000
Special Thanks To:Special Thanks To:
Dr. ErwinDr. Erwin Dr. StarrDr. Starr Dr. HurleyDr. Hurley