A Stroke from the Bishop’s Cap Thomas A. Showalter, III, DO Resident, Internal Medicine CPC August...

A Stroke from A Stroke from thethe

Bishop’s Cap Bishop’s Cap

Thomas A. Showalter, III, Thomas A. Showalter, III, DODO

Resident, Internal MedicineResident, Internal MedicineCPCCPC

August 10August 10thth, 2007, 2007

The CaseThe Case

CC: ‘my right arm and leg are weak’CC: ‘my right arm and leg are weak’

HPI: HPI: 22 year old male 22 year old male 5 weeks prior5 weeks prior

Right arm and leg numbness and weaknessRight arm and leg numbness and weakness Mild dysarthriaMild dysarthria Night prior - 10-12 beers, smoked pot, taken 2 Night prior - 10-12 beers, smoked pot, taken 2

vicodin Presented to outside ERvicodin Presented to outside ER Treated with IVF for dehydration and sent Treated with IVF for dehydration and sent

home.home.

The Case continued…The Case continued…

Same day after ER dischargeSame day after ER dischargeDeveloped twitching of right side of face and armDeveloped twitching of right side of face and armReturned to ERReturned to ER

CT head negativeCT head negative All symptoms resolved with 4-5 hoursAll symptoms resolved with 4-5 hours Except for mild residual right facial numbnessExcept for mild residual right facial numbness 2 days later2 days later Reported mild dysarthriaReported mild dysarthria

Right facial droop and right facial numbnessRight facial droop and right facial numbnessReferred to Neurology at S&WReferred to Neurology at S&W


HPI:HPI: Seen 4 weeks laterSeen 4 weeks laterDenied paresthesias, paresis, involuntary Denied paresthesias, paresis, involuntary movements or dysarthria. movements or dysarthria. Complained of mild headache and Complained of mild headache and sonophobia. sonophobia.

PMHx: PMHx: Substance abuseSubstance abuseSpontaneous Pneumothorax at age 19Spontaneous Pneumothorax at age 19One episode, 1 year ago with right sided One episode, 1 year ago with right sided numbness, but no paresis.numbness, but no paresis.


PSH:PSH:Appendectomy as childAppendectomy as childChest tube placement for Spontaneous PTxChest tube placement for Spontaneous PTx

Meds:Meds:nonenone

Allergies:Allergies:nonenone


Social Hx:Social Hx:Construction workerConstruction worker+Tobacco. +THC weekly+Tobacco. +THC weeklyEtoH daily with binges on weekendEtoH daily with binges on weekendMethamphetamine once monthlyMethamphetamine once monthlyNo IV drug abuseNo IV drug abuse

Family Hx:Family Hx:Parents healthy. Sister unknown stomach Parents healthy. Sister unknown stomach disorder. No early stroke, hypercoagulable disorder. No early stroke, hypercoagulable disorders, seizures or malignancy.disorders, seizures or malignancy.


Review of Systems:Review of Systems:No fevers, weight loss, night sweats.No fevers, weight loss, night sweats.No arthralgias or myalgias. No rashes.No arthralgias or myalgias. No rashes.No abdominal pain. No chest pain.No abdominal pain. No chest pain.No dyspnea. No palpitations.No dyspnea. No palpitations.No dysphagia. No melena/hematochezia.No dysphagia. No melena/hematochezia.No hematuria/dysuria.No hematuria/dysuria.No syncope. No easy bruising or bleeding.No syncope. No easy bruising or bleeding.

Physical ExamPhysical Exam

VS: T 95.7 P 70 R 12 BP 105/62VS: T 95.7 P 70 R 12 BP 105/62Gen: thin male, NAD, healthy appearing.Gen: thin male, NAD, healthy appearing.HEENT: normalHEENT: normalNeck: normalNeck: normalHeart: normalHeart: normalChest: normalChest: normalExtr: normalExtr: normalNeuro: normalNeuro: normalSkin: normalSkin: normal

8.7

13.6

39.0273

138 100 10

4.3 23 0.8

Diff: Diff: NeutNeut 54%54% LymphLymph 27%27% MonoMono 7%7%

LFTsLFTs Normal Normal

Coags NormalCoags Normal

The DataThe Data

The Data continued…The Data continued…

CXR: CXR: normalnormal

EKG: EKG: marked sinus marked sinus bradycardia with bradycardia with sinus arrhythmia, sinus arrhythmia, early repolarizationearly repolarization

MRI Brain: MRI Brain: chronic left MCA chronic left MCA infarct and several infarct and several chronic tiny chronic tiny cerebellar infarctscerebellar infarcts

MRA Head/Neck: MRA Head/Neck: normal Circle of normal Circle of Willis and great Willis and great vessels of head vessels of head and neckand neck

The Data continued…The Data continued…Additional lab obtained…Additional lab obtained…

ESRESR 1010Total CholesterolTotal Cholesterol 138138Anticardiolipin IgG and IgM – negativeAnticardiolipin IgG and IgM – negativeLupus anticoagulant – negativeLupus anticoagulant – negative

Echocardiography:Echocardiography:normal LV function, 1.7 cm x 1.2 cm normal LV function, 1.7 cm x 1.2 cm mass on the atrial side of the anterior mass on the atrial side of the anterior leaflet of mitral valve possibly attached leaflet of mitral valve possibly attached by a stalkby a stalk

Hospital CourseHospital Course

BC for bacterial and fungal organisms done.BC for bacterial and fungal organisms done. Cardiac MRI: Cardiac MRI:

– mass on atrial surface of mitral valvemass on atrial surface of mitral valve– with no clear stalkwith no clear stalk– Impression: mass consistent with either tumor or Impression: mass consistent with either tumor or

vegetation from infective endocarditis, but more vegetation from infective endocarditis, but more likely endocarditis based on the location and that likely endocarditis based on the location and that no stalk was seen on the MRI.no stalk was seen on the MRI.

A diagnostic procedure was performed.A diagnostic procedure was performed.

I. The Case

II. Problem List

III. DDx of Stroke in a Young Person

IV. DDx of a Left Atrial Mass

V. Conclusions

The Problem ListThe Problem List

Multiple chronic strokesMultiple chronic strokes– Left MCA distribution infarctLeft MCA distribution infarct– Multiple tiny cerebellar infarctsMultiple tiny cerebellar infarcts

Left Atrial Mass (with or without a stalk)Left Atrial Mass (with or without a stalk)

Substance abuseSubstance abuse

Headache with sonophobiaHeadache with sonophobia

History of…History of…Right arm and leg numbness and weaknessRight arm and leg numbness and weaknessDysarthria and facial numbness and Dysarthria and facial numbness and

weaknessweaknessMuscle spasm of right face and right armMuscle spasm of right face and right arm

Stroke in Young AdultsStroke in Young Adults Usually defined as age < 45 yearsUsually defined as age < 45 years Worldwide incidence 9-11 per 100,000 Worldwide incidence 9-11 per 100,000 [4][4] Northern Manhattan Stroke Study, Northern Manhattan Stroke Study, Stroke Stroke

20022002– Multiethnic population of 210,000 residentsMultiethnic population of 210,000 residents– In a 4-yr period, 74 cases of young stroke out In a 4-yr period, 74 cases of young stroke out

of 924 incident first ever strokes (8%)of 924 incident first ever strokes (8%)– Higher incidence rates in Blacks and Hispanics Higher incidence rates in Blacks and Hispanics

compared to Whitescompared to Whites

Stroke in Young AdultsStroke in Young Adults

Italian epidemiological review by Gandolfo Italian epidemiological review by Gandolfo and Conti and Conti Neurological ScienceNeurological Science 2003 2003– Western European Countries, less than 5% of all Western European Countries, less than 5% of all

strokes occurred in patients < 45 years of age strokes occurred in patients < 45 years of age (yoa).(yoa).

– Developing countries had 20-30% of strokes < 45 Developing countries had 20-30% of strokes < 45 yoayoa

– United States, 8-10% of strokes in patients < 45 United States, 8-10% of strokes in patients < 45 yoayoa

Estimated lifetime cost of stroke $103,576 for Estimated lifetime cost of stroke $103,576 for US patients. 2-4 times that is young adult due US patients. 2-4 times that is young adult due to longer period of lost productivity to longer period of lost productivity [4][4]

DDx of Stroke in Young AdultsDDx of Stroke in Young Adults1.1. Subarachnoid Subarachnoid

HemorrhageHemorrhage

2.2. Intracerebral HemorrhageIntracerebral Hemorrhage

3.3. Cerebral Ischemic InfarctsCerebral Ischemic Infarcts

Cerebral Ischemic InfarctCerebral Ischemic Infarct 3% of all cerebral infarcts occur between 3% of all cerebral infarcts occur between

15-45 years of age 15-45 years of age [3][3] Etiologies Etiologies

1.1. AtheroscleroticAtherosclerotic2.2. NonatheroscleroticNonatherosclerotic3.3. CardioembolicCardioembolic

Ages 15-35, cardioembolic and Ages 15-35, cardioembolic and nonatherosclerotic causes predominatenonatherosclerotic causes predominate

After age 35, traditional atherosclerotic After age 35, traditional atherosclerotic stroke risk factors become prime stroke risk factors become prime determinants of strokedeterminants of stroke

Atherosclerotic causesAtherosclerotic causes Traditional risk factors:Traditional risk factors:

– Hypertension, Smoking, Hyperlipidemia, Diabetes Hypertension, Smoking, Hyperlipidemia, Diabetes MellitusMellitus

– Age 15-30 -> 2%Age 15-30 -> 2%– Age 30-45 -> 30-35%Age 30-45 -> 30-35%

Homocystinuria – premature large vessels Homocystinuria – premature large vessels diseasedisease

Carotid Atheroma formation due to local Carotid Atheroma formation due to local radiation for laryngeal tumorsradiation for laryngeal tumors

Cranial radiation produces a radiation Cranial radiation produces a radiation vasculopathyvasculopathy

The Myriad ofThe Myriad of Nonatherosclerotic causes Nonatherosclerotic causes

DissectionDissection

Illicit drug useIllicit drug use

InfectionInfection

Prothrombotic Prothrombotic StatesStates

MigraineMigraine

Sickle Cell Sickle Cell DiseaseDisease

Genetic DisordersGenetic Disorders

Inborn Errors of Inborn Errors of MetabolismMetabolism

MoyamoyaMoyamoya

Hyperestrogenemic Hyperestrogenemic StatesStates

VasculitisVasculitis

Arteritis due to Arteritis due to NeoplasmsNeoplasms

Cardioembolic causesCardioembolic causes 20-30% of young adult20-30% of young adult Valvular heart diseaseValvular heart disease Mitral valve prolapseMitral valve prolapse Prosthetic heart valvesProsthetic heart valves Rheumatic heart diseaseRheumatic heart disease Acute Myocardial Acute Myocardial

InfarctionInfarction Left ventricular Left ventricular

dyskinesiadyskinesia Spontaneous echo Spontaneous echo

contrastcontrast Left ventricular Left ventricular

aneurysmaneurysm

Left atrial aneurysmLeft atrial aneurysm Dilated Dilated

CardiomyopathyCardiomyopathy Atrial Septal defectAtrial Septal defect Patent Foreman OvalePatent Foreman Ovale Atrial Fibrillation Atrial Fibrillation

(Left Atrial Thrombus)(Left Atrial Thrombus) Bacterial endocarditisBacterial endocarditis Libmann Sachs Libmann Sachs

endocarditisendocarditis Marantic endocarditisMarantic endocarditis TumorTumor

The Problem ListThe Problem List

Multiple chronic strokesMultiple chronic strokes– Left MCA distribution infarctLeft MCA distribution infarct– Multiple tiny cerebellar infarctsMultiple tiny cerebellar infarcts

Left Atrial Mass (with or without a stalk)Left Atrial Mass (with or without a stalk)

Substance abuseSubstance abuse

Headache with sonophobiaHeadache with sonophobia

History of…History of…

Right arm and leg numbness and weaknessRight arm and leg numbness and weakness

Dysarthria and facial numbness and weaknessDysarthria and facial numbness and weakness

Muscle spasm of right face and right armMuscle spasm of right face and right arm

DDx of Left Atrial MassDDx of Left Atrial Mass

EndocarditisEndocarditis– Nonbacterial Thrombotic Endocarditis Nonbacterial Thrombotic Endocarditis

(Marantic)(Marantic)– Libmann Sachs endocarditisLibmann Sachs endocarditis– Bacterial endocarditisBacterial endocarditis

Left Atrial ThrombusLeft Atrial Thrombus TumorTumor

– MetastaticMetastatic– Primary, Benign or MalignantPrimary, Benign or Malignant

Marantic EndocarditsMarantic Endocardits

Nonbacterial Thrombotic EndocarditisNonbacterial Thrombotic Endocarditis ““Sterile” VegetationsSterile” Vegetations Microscopic to large aggregates of platelets and Microscopic to large aggregates of platelets and

fibrin on heart valves (usually aortic or mitral)fibrin on heart valves (usually aortic or mitral) 27% of ischemic stroke in patients with cancer 27% of ischemic stroke in patients with cancer [9][9]

Complicates many nonmalignant wasting illnesses, Complicates many nonmalignant wasting illnesses, i.e. AIDSi.e. AIDS

Continuum with Trousseau’s SyndromeContinuum with Trousseau’s Syndrome Predisposed by prothrombotic states, valvular Predisposed by prothrombotic states, valvular

endothelial disruption and underlying valve endothelial disruption and underlying valve diseasedisease

Libmann Sachs EndocarditisLibmann Sachs Endocarditis

Verrucous EndocarditisVerrucous Endocarditis Accumulation of immune complexes, mononuclear cells, Accumulation of immune complexes, mononuclear cells,

hematoxylin bodies, fibrin and platelet thrombihematoxylin bodies, fibrin and platelet thrombi Occurs in minority of Systemic Lupus ErythematosisOccurs in minority of Systemic Lupus Erythematosis Fewer seen in Antiphospholipid Antibody SyndromeFewer seen in Antiphospholipid Antibody Syndrome Most commonly the Aortic or Mitral valve, although the Most commonly the Aortic or Mitral valve, although the

Tricuspid may be affectedTricuspid may be affected Typically asymptomatic, but if large enough may embolizeTypically asymptomatic, but if large enough may embolize

Infective EndocarditisInfective Endocarditis Microbial infection of the endocardial surfaceMicrobial infection of the endocardial surface Vegetation – platelets, fibrin, microorganisms Vegetation – platelets, fibrin, microorganisms

and inflammatory cellsand inflammatory cells In the U.S., incidence of community-acquired In the U.S., incidence of community-acquired

native-valve endocarditis = 1.7 to 6.2 cases native-valve endocarditis = 1.7 to 6.2 cases per 100,000 person-yearsper 100,000 person-years

Median age = 47-69 yearsMedian age = 47-69 years Injection drug usersInjection drug users

– Higher incidence in younger personsHigher incidence in younger persons– Incidence 150 to 2000 per 100,000 person-yearsIncidence 150 to 2000 per 100,000 person-years

Clinical Manifestations of Infective Clinical Manifestations of Infective EndocarditisEndocarditis

Fever – most common sign and symptomFever – most common sign and symptom Subacute – anorexia, weight loss, malaise and Subacute – anorexia, weight loss, malaise and

night sweatsnight sweats Heart murmur – new or changing, but usually Heart murmur – new or changing, but usually

preexistingpreexisting Petechiae on skin, conjunctivae or oral mucosaPetechiae on skin, conjunctivae or oral mucosa SplenomegalySplenomegaly Congestive heart failureCongestive heart failure Splinter hemorrhages, Osler’s nodes, Janeway’s Splinter hemorrhages, Osler’s nodes, Janeway’s

lesionslesions Neurologic complications:Neurologic complications:

– 20-40% will have neurologic complication!20-40% will have neurologic complication!– 65% of embolic phenomena involve the CNS65% of embolic phenomena involve the CNS

The Duke CriteriaThe Duke Criteria Introduced by group at Duke University in 1994, Introduced by group at Duke University in 1994,

modified in 2000modified in 2000 Specificity 99%Specificity 99% NPV > 92%NPV > 92% Criteria integratedCriteria integrated

– Factors predisposing patients to the development of Factors predisposing patients to the development of endocarditisendocarditis

– Blood culture isolate and persistence of bacteremiaBlood culture isolate and persistence of bacteremia– Echocardiographic findingsEchocardiographic findings

TTE – Specificity 98%, Sensitivity 60-70%TTE – Specificity 98%, Sensitivity 60-70% TEE – Specificity 85-98%, Sensitivity 75-95%, NPV > 92%TEE – Specificity 85-98%, Sensitivity 75-95%, NPV > 92%

– Other clinical and laboratory findingsOther clinical and laboratory findings Only 5-7% of patients have sterile blood cultures*Only 5-7% of patients have sterile blood cultures*

Left Atrial ThrombusLeft Atrial Thrombus 45% of cardiogenic thromboemboli45% of cardiogenic thromboemboli 13% patients with atrial fibrillation13% patients with atrial fibrillation 33% patients with rheumatic mitral stenosis33% patients with rheumatic mitral stenosis May complicate primary or metastatic May complicate primary or metastatic

tumorstumors Regional or global wall motion abnormalities Regional or global wall motion abnormalities

increase riskincrease risk Associated with the left atrial appendageAssociated with the left atrial appendage Generally, attached to posterior left atrial Generally, attached to posterior left atrial

wall by a broad base, therefore immobilewall by a broad base, therefore immobile Can be pedunculated and mobileCan be pedunculated and mobile

Left Atrial ThrombusLeft Atrial Thrombus Omran in 2000 Omran in 2000

– Sinus RhythmSinus Rhythm– 1% incidence of left atrial thrombus in patients with 1% incidence of left atrial thrombus in patients with

recent neurologic deficitrecent neurologic deficit– 6/583 patients (1%) had left atrial appendage 6/583 patients (1%) had left atrial appendage

thrombusthrombus– 3 mitral stenosis, 1 aortic stenosis, 1 dilated 3 mitral stenosis, 1 aortic stenosis, 1 dilated

cardiomyopathy, 1 coronary artery diseasecardiomyopathy, 1 coronary artery disease

Left atrial thrombi are an infrequent cause of Left atrial thrombi are an infrequent cause of thromboembolism in patients in sinus rhythm and thromboembolism in patients in sinus rhythm and are associated with valvular disease and atrial are associated with valvular disease and atrial dysfunction.dysfunction.

*Left atrial thrombus is associated with left atrial *Left atrial thrombus is associated with left atrial tumors…tumors…

Cardiac TumorsCardiac Tumors MetastaticMetastatic

– 20-40 times more common than primary tumors20-40 times more common than primary tumors– Pericardium > Myocardium > EndocardiumPericardium > Myocardium > Endocardium– 10-20% patients with disseminated cancer will have 10-20% patients with disseminated cancer will have

involvement of heart or pericardiuminvolvement of heart or pericardium PrimaryPrimary

– 0.17-0.19% incidence in unselected autopsy series0.17-0.19% incidence in unselected autopsy series– 1 in 500 cardiac surgical cases, with exception of 1 in 500 cardiac surgical cases, with exception of

myxomamyxoma– Benign -> 75%Benign -> 75%

Myxomas comprise 50% of benignMyxomas comprise 50% of benign Myxomas comprise 80-90% of left atrial primary tumorsMyxomas comprise 80-90% of left atrial primary tumors

– Malignant -> 25%Malignant -> 25% Sarcomas comprise 75% of malignantSarcomas comprise 75% of malignant

Frequency of Cardiac TumorsFrequency of Cardiac Tumors

Atlas of Heart Disease: Cardiopulmonary Diseases and Cardiac Tumors. Vol III. 1995. Philadelphia: Mosby.

Relative Incidence of Primary Malignant Tumors of the Relative Incidence of Primary Malignant Tumors of the HeartHeart

Malignant TumorsMalignant Tumors Adults Adults (%)(%)

Children Children (%)(%)

AngiosarcomaAngiosarcoma 2828 66

RhabdomyosarcomaRhabdomyosarcoma 1111 4141

FibrosarcomaFibrosarcoma 88 1818

Malignant fibrous Malignant fibrous histiocytomahistiocytoma

66 66

OsteosarcomaOsteosarcoma 77 00

LeimyosarcomaLeimyosarcoma 55 00

MyxosarcomaMyxosarcoma 33 66

Other sarcomas*Other sarcomas* 1414 1212

Undifferentiated sarcomaUndifferentiated sarcoma 1212 1212

LymphomaLymphoma 66 00Adapted from Braunwald’s Heart Disease 7th edition

*Other sarcomas include liposarcomas, synovial and neurogenic sarcomas

Malignant Cardiac TumorsMalignant Cardiac Tumors 25% of all cardiac tumors are invasive or metastatic25% of all cardiac tumors are invasive or metastatic 95% of these are Sarcomas95% of these are Sarcomas

(2(2ndnd to myxoma in overall frequency) to myxoma in overall frequency) 5% are Lymphomas5% are Lymphomas Sarcomas derive from mesenchyme, therefore have Sarcomas derive from mesenchyme, therefore have

a wide variety of morphological typesa wide variety of morphological types Mutations in K-Mutations in K-rasras were seen in most cardiac were seen in most cardiac

sarcomassarcomas Any age, but most common third and fifth decadesAny age, but most common third and fifth decades Except for rhabdomyosarcomas and fibrosarcomas, Except for rhabdomyosarcomas and fibrosarcomas,

distinctly unusual in infants and childrendistinctly unusual in infants and children

Malignant Cardiac TumorsMalignant Cardiac Tumors

25-50% patients will have metastatic disease at 25-50% patients will have metastatic disease at time of diagnosistime of diagnosis

Most frequent: lungs, thoracic lymph nodes, Most frequent: lungs, thoracic lymph nodes, mediastinum and vertebral columnmediastinum and vertebral column

Less frequent: liver, kidneys, adrenals, pancreas, Less frequent: liver, kidneys, adrenals, pancreas, bone, spleen and bowelbone, spleen and bowel

Transesophageal Echocardiography Transesophageal Echocardiography recommended for diagnosisrecommended for diagnosis

CT and MRI show degree of tumor infiltrationCT and MRI show degree of tumor infiltration Often endomyocardial or open biopsy neededOften endomyocardial or open biopsy needed

Treatment of Cardiac Treatment of Cardiac SarcomasSarcomas

Sarcomas proliferate rapidlySarcomas proliferate rapidly Death due to widespread infiltration of the Death due to widespread infiltration of the

myocardium, obstruction of flow within the heart myocardium, obstruction of flow within the heart or distant metastasis with a few weeks to 2 years or distant metastasis with a few weeks to 2 years after onset of symptomsafter onset of symptoms

Median survival 6-12 monthsMedian survival 6-12 months Surgical excision considered to achieve local Surgical excision considered to achieve local

control and relieve symptomscontrol and relieve symptoms Complete excision - median survival 12-24 monthsComplete excision - median survival 12-24 months

– Possible in less than 50% of patientsPossible in less than 50% of patients Incomplete excision – median survival 3-10 monthsIncomplete excision – median survival 3-10 months

Treatment of Cardiac Treatment of Cardiac SarcomasSarcomas

AutotransplantationAutotransplantation– Cardiac explantation, ex vivo tumor resection, cardiac Cardiac explantation, ex vivo tumor resection, cardiac

reconstruction and reimplantationreconstruction and reimplantation Chemotherapeutic benefits are unclearChemotherapeutic benefits are unclear

– Data support anthracycline-based regimens is soft tissue Data support anthracycline-based regimens is soft tissue sarcomassarcomas

– Adjuvant chemotherapy and/or radiation therapy usually Adjuvant chemotherapy and/or radiation therapy usually recommendedrecommended

Orthotopic heart transplantation in patients with Orthotopic heart transplantation in patients with locally unresectable disease without evidence of locally unresectable disease without evidence of metastasismetastasis– 66% still die within 1 year either of locally recurrent or 66% still die within 1 year either of locally recurrent or

metastatic diseasemetastatic disease

AngiosarcomaAngiosarcoma

30% of primary cardiac sarcomas30% of primary cardiac sarcomas 3:1 male-to-female ratio3:1 male-to-female ratio Predilection for right atrium, may be either Predilection for right atrium, may be either

intracavitary and polypoid or diffuse and intracavitary and polypoid or diffuse and infiltrativeinfiltrative

Later forms have sheet-like covering of Later forms have sheet-like covering of pericardiumpericardium

Usually present with right-sided heart failure Usually present with right-sided heart failure or tamponadeor tamponade

Systemic signs such as fever and weight lossSystemic signs such as fever and weight loss

AngiosarcomaAngiosarcoma

““Cauliflower” appearance on MRI due to Cauliflower” appearance on MRI due to areas of hemorrhage and necrosisareas of hemorrhage and necrosis

Tend to be discovered ‘late’, often already Tend to be discovered ‘late’, often already metastasizedmetastasized

Often not amenable to complete resectionOften not amenable to complete resection Very poor prognosisVery poor prognosis Kaposi Sarcoma (HSV 8) Kaposi Sarcoma (HSV 8)

Less than 5% of pts with AIDS or solid Less than 5% of pts with AIDS or solid organ transplantorgan transplant

RhabdomyosarcomaRhabdomyosarcoma

Most common cardiac malignancy in infants Most common cardiac malignancy in infants and childrenand children

10% of all primary cardiac sarcomas10% of all primary cardiac sarcomas Diffusely infiltrate the ventricular myocardiumDiffusely infiltrate the ventricular myocardium May on occasion form polypoid extension into May on occasion form polypoid extension into

chamberchamber Usually multiple foci with occasional nodular Usually multiple foci with occasional nodular

involvement of pericardiuminvolvement of pericardium Rhabdomyoblast – histological hallmarkRhabdomyoblast – histological hallmark

FibromyosarcomaFibromyosarcoma

5-10% of cardiac sarcomas5-10% of cardiac sarcomas Fibroblastic in differentiation, composed of Fibroblastic in differentiation, composed of

spindle-shaped cells containing areas of spindle-shaped cells containing areas of hemorrhage and necrosishemorrhage and necrosis

Extensively infiltrate the heartExtensively infiltrate the heart Often involving more than one chamber Often involving more than one chamber

spreading to the pericardiumspreading to the pericardium

Relative Incidence of Benign Tumors of the Relative Incidence of Benign Tumors of the HeartHeart

Benign TumorsBenign Tumors Adults (%)Adults (%)

MyxomaMyxoma 5252

Papillary Papillary fibroelastomafibroelastoma

1616

LipomaLipoma 1616

RhabdomyomaRhabdomyoma 11

FibromaFibroma 33

TeratomaTeratoma 11

HemangiomaHemangioma 66

Other tumors*Other tumors* 55

*other tumors include cystic tumors of the atrioventricular node, endocrine tumors and histiocytoid tumors

Adapted from Braunwald’s Heart Disease 7th edition

Relative Incidence of Benign Tumors of the Relative Incidence of Benign Tumors of the HeartHeart

Benign TumorsBenign Tumors Adult (%)Adult (%) Children Children (%)(%)

MyxomaMyxoma 5252 1717

Papillary Papillary fibroelastomafibroelastoma

1616 00

LipomaLipoma 1616 00

RhabdomyomaRhabdomyoma 11 4242

FibromaFibroma 33 1818

TeratomaTeratoma 11 1212

HemangiomaHemangioma 66 55

Other tumors*Other tumors* 55 44*other tumors include cystic tumors of the atrioventricular node, endocrine tumors and histiocytoid tumors

Adapted from Braunwald’s Heart Disease 7th edition

RhabdomyomaRhabdomyoma Most common cardiac tumors Most common cardiac tumors

in infants and childrenin infants and children ¾ occur in patients younger ¾ occur in patients younger

than 1 yearthan 1 year Left and right ventricular and Left and right ventricular and

septal myocardiumseptal myocardium 1/3 involve either or one atria1/3 involve either or one atria Nearly all are multipleNearly all are multiple Small, lobulatedSmall, lobulated Diameter range 2 mm to 2 cmDiameter range 2 mm to 2 cm

RhabdomyomaRhabdomyoma Most common presentation Most common presentation

is heart block or other is heart block or other arrhythmiasarrhythmias

Echo: multiple small, Echo: multiple small, lobulated, homogenous, lobulated, homogenous, hyperechoic intramural hyperechoic intramural tumorstumors

Association with Tuberous Association with Tuberous Sclerosis (80%)Sclerosis (80%)– HamartomasHamartomas– EpilepsyEpilepsy– Mental deficiencyMental deficiency– Adenoma sebaceumAdenoma sebaceum

LipomaLipoma RareRare Occur at any age with equal Occur at any age with equal

male/female ratiomale/female ratio Diameter 1-15 cmDiameter 1-15 cm Sessile or polypoidSessile or polypoid Occur in the Occur in the

subendocardium or subendocardium or subpericardium,subpericardium,

¼ are completely ¼ are completely intramuscularintramuscular

Most common chambers Most common chambers affected are the left affected are the left ventricle, right atrium and ventricle, right atrium and interatrial septuminteratrial septum

Primary Cardiac Valve Primary Cardiac Valve TumorsTumors

Retrospective study by Edwards et al 1991Retrospective study by Edwards et al 1991

from 1932 to 1990from 1932 to 1990

Walter Reed and Brooke Army Medical Walter Reed and Brooke Army Medical CentersCenters

53 patients had 56 primary cardiac valve 53 patients had 56 primary cardiac valve tumorstumors

Age range 2 to 88 yearsAge range 2 to 88 years Average 52 years of age – 79% maleAverage 52 years of age – 79% male Aortic valve most commonly affectedAortic valve most commonly affected Tumor size ranged 3 mm to 7 cmTumor size ranged 3 mm to 7 cm Average size = 1.15 cmAverage size = 1.15 cm

Primary Cardiac Valve Primary Cardiac Valve TumorsTumors

52/56 (93%) tumors were benign52/56 (93%) tumors were benign– 41 papillary fibroelastomas41 papillary fibroelastomas– 5 myxomas5 myxomas– 4 fibromas4 fibromas– 1 hamartoma1 hamartoma– 1 hemangioma1 hemangioma

Mitral valve most commonly symptomaticMitral valve most commonly symptomatic 8/53 (15%) patients had neurologic 8/53 (15%) patients had neurologic

symptomssymptoms 6 had mitral valve tumors6 had mitral valve tumors

– 3 myxomas and 3 malignancies3 myxomas and 3 malignancies

Comparison with NonvalvularComparison with NonvalvularCardiac TumorsCardiac Tumors

Most common nonvalvular -> MyxomaMost common nonvalvular -> Myxoma Most common valvular -> Papillary fibroelastomaMost common valvular -> Papillary fibroelastoma

Edwards et al “Primary Cardiac Valve Tumors” Ann Thorac Surg 1991; 32:1131

Papillary FibroelastomaPapillary Fibroelastoma

Most common tumor of the cardiac valvesMost common tumor of the cardiac valves Average age 60 at detection (range Average age 60 at detection (range

neonates to 92 yoa)neonates to 92 yoa) Men = WomenMen = Women Most have concomitant valvular disease Most have concomitant valvular disease

suggesting this may predispose to suggesting this may predispose to papillary fibroelastomapapillary fibroelastoma

90% solitary90% solitary

Papillary FibroelastomaPapillary Fibroelastoma

Median diameter = 8 Median diameter = 8 mm, largest reported 4 mm, largest reported 4 cmcm

Any valve, aortic and Any valve, aortic and mitral most commonlymitral most commonly– arterial side of semilunar arterial side of semilunar

valvesvalves– atrial side of AV valvesatrial side of AV valves

Short pedicle 50% of Short pedicle 50% of timetime

Papillary Fibroelastoma PathPapillary Fibroelastoma Path Grossly, a frond-like Grossly, a frond-like

appearance resembling appearance resembling a sea anemonea sea anemone

Histological, numerous Histological, numerous papillary fronds papillary fronds consisting of a collagen consisting of a collagen core surrounded by core surrounded by elastic fibers and loose elastic fibers and loose connective tissue, all connective tissue, all covered by endocardial covered by endocardial endotheliumendothelium

MyxomasMyxomas

33rdrd and 6 and 6thth decades of life decades of life Mean age at presentation = 50 yearsMean age at presentation = 50 years Age range = newborn to 95 yearsAge range = newborn to 95 years 2/3’s females2/3’s females

MyxomasMyxomas

LocationLocation 75% Left Atria75% Left Atria 15-20% Right 15-20% Right

AtriaAtria 3-4% Left 3-4% Left

VentricleVentricle 3-4% Right 3-4% Right

VentricleVentricle

AttachmentAttachment

1.1. Interatrial SeptumInteratrial Septum– Limbus of Fossa Limbus of Fossa

OvalisOvalis

2.2. Posterior Atrial wallPosterior Atrial wall

3.3. Anterior Atrial wallAnterior Atrial wall

4.4. Atrial appendageAtrial appendage

5.5. Rarely endocardialRarely endocardial

MyxomasMyxomas Average size 5-6 cm in Average size 5-6 cm in

diameterdiameter Size Range < 1 to > 15 Size Range < 1 to > 15

cmcm Echo shows a mobile,Echo shows a mobile,

distensible tumor distensible tumor connectedconnected

to the interatrial septum to the interatrial septum by a narrow stalkby a narrow stalk

PolypoidPolypoid Usually pedunculatedUsually pedunculated Round or oval with smooth Round or oval with smooth

or lobulated surfaceor lobulated surface

Myxoma PathMyxoma Path Embryonic Embryonic

mesenchymal cells mesenchymal cells with multipotent with multipotent capabilitycapability

Myxoid matrix of acid- Myxoid matrix of acid- mucopolysaccharide- mucopolysaccharide- rich stromarich stroma

Immunohistochemical Immunohistochemical studiesstudies– VimentinVimentin– neuroendocrine neuroendocrine

markers (S-100)markers (S-100)– gene product 9.5 and gene product 9.5 and

calretinincalretinin

MyxomasMyxomas Surface of tumor is Surface of tumor is

often covered with often covered with thrombithrombi– Embolism occurs in Embolism occurs in

30-40% of patients30-40% of patients– Usually systemic, Usually systemic,

majority are cerebralmajority are cerebral

Infected myxomas Infected myxomas have been describedhave been described– Greater danger of Greater danger of

systemic embolismsystemic embolism Excised Villous Left Atrial Myxoma

MyxomasMyxomas Intracardiac obstructionIntracardiac obstruction

– 70% have heart failure or syncope70% have heart failure or syncope– Dyspnea, pulmonary edema, sudden Dyspnea, pulmonary edema, sudden

deathdeath Systemic embolizationSystemic embolization

– 30% of patients with myxoma with 30% of patients with myxoma with 2/3 cerebral2/3 cerebral

– 25% with emboli have evidence of 25% with emboli have evidence of multiple embolic eventsmultiple embolic events

Constitutional symptomsConstitutional symptoms– Unique to myxoma, 30-40% of Unique to myxoma, 30-40% of

patientspatients– Synthesis of interleukin – 6Synthesis of interleukin – 6– Fatigue, fever, erythematous rash, Fatigue, fever, erythematous rash,

arthralgia, myalgia and weight lossarthralgia, myalgia and weight loss

MyxomasMyxomas The Physical ExamThe Physical Exam

– Murmur heard > 50%Murmur heard > 50%– Diastolic due to obstructed filling of the ventricleDiastolic due to obstructed filling of the ventricle– Systolic due to interference with closure of the AV valveSystolic due to interference with closure of the AV valve

– SS11 often loud and widely split often loud and widely split– Delay in closure of the AV valveDelay in closure of the AV valve

– ““Tumor Plop” in 33% of patientsTumor Plop” in 33% of patients– A diastolic murmur heard 80 to 150 msec after the A diastolic murmur heard 80 to 150 msec after the

second heart soundsecond heart sound

– Pericardial friction rubPericardial friction rub– Right atrial tumorsRight atrial tumors

The “Wrecking Ball Effect”The “Wrecking Ball Effect”– Recurrent collision with the pedunculated Recurrent collision with the pedunculated

myxoma and the mitral valve may cause myxoma and the mitral valve may cause permanent damagepermanent damage

MyxomasMyxomas

Familial MyxomasFamilial Myxomas– 10% or less of all myxomas10% or less of all myxomas– Autosomal dominantAutosomal dominant– Median age 20 yearsMedian age 20 years– Atypical locations, often multiple and recurrent Atypical locations, often multiple and recurrent

tumorstumors

Carney ComplexCarney Complex– Myxomas, Spotty skin pigmentation and Myxomas, Spotty skin pigmentation and

endocrine overactivityendocrine overactivity

Treatment of Benign Cardiac Treatment of Benign Cardiac TumorsTumors

Operative excision under direct vision using Operative excision under direct vision using cardiopulmonary bypasscardiopulmonary bypass– Schaff and Mullany, 2000Schaff and Mullany, 2000

Orthotopic heart transplantOrthotopic heart transplant

AutotransplantationAutotransplantation

DiscussionDiscussion Myxomas are source of most tumor emboli Myxomas are source of most tumor emboli

because of their friable consistency and because of their friable consistency and intracavitary location, but other types may intracavitary location, but other types may embolize.embolize.

““An embolic stroke in a young person without An embolic stroke in a young person without evidence of cerebrovascular disease, evidence of cerebrovascular disease, particularly in the presence of sinus rhythm, particularly in the presence of sinus rhythm, should raise the suspicion of intracardiac should raise the suspicion of intracardiac myxoma, as well as infective endocarditis.” myxoma, as well as infective endocarditis.” – Braunwald’s – Braunwald’s Heart DiseaseHeart Disease, 7, 7thth Ed. Ed.

DiscussionDiscussion

Multiple StrokesMultiple Strokes

Left Atrial MassLeft Atrial Mass

Tumor MyxomaPapillary Fibroelastoma

Tumor with or without thrombus?Tumor with or without infection?

Diagnosis:

Requested Procedure:

Myxoma

Surgical Resection

ReferencesReferences1.1. Gandolfo, C. and M. Conti. “Stroke in young adults: epidemiology” Gandolfo, C. and M. Conti. “Stroke in young adults: epidemiology” Neurological ScienceNeurological Science 2003; 24: S1-S3. 2003; 24: S1-S3. 2.2. Jacobs, B.S., et al. “Stoke in the young in the Northern Manhattan Stroke Study” Jacobs, B.S., et al. “Stoke in the young in the Northern Manhattan Stroke Study” StrokeStroke 2002; 33: 2789- 2002; 33: 2789-

96.96.3.3. Hart, R.G. et al., “Diagnosis and management of ischemic stroke. II. Selected controversies” Hart, R.G. et al., “Diagnosis and management of ischemic stroke. II. Selected controversies” Current Current

Problems in Cardiology Problems in Cardiology 1983; 8(7): 43-53.1983; 8(7): 43-53.4.4. Birgitte, H. et al. “Stroke in Young Adults and Children” Birgitte, H. et al. “Stroke in Young Adults and Children” Current Neurology and Neuroscience ReportsCurrent Neurology and Neuroscience Reports

2001; 1: 2001; 1: 54-66.54-66.5.5. Martin, P.J., et al. “Causes of ischaemic stroke in the young” Martin, P.J., et al. “Causes of ischaemic stroke in the young” Postgraduate Medical JournalPostgraduate Medical Journal 1997; 73: 8- 1997; 73: 8-

16.16.6.6. Guillon, B. et al., “Internal carotid artery dissection: an update” Guillon, B. et al., “Internal carotid artery dissection: an update” Journal of Neurologic ScienceJournal of Neurologic Science 1998; 153: 1998; 153:

146-146- 158.158.7.7. Sloan, M.A., “Illicit drug-associated ischemic stroke in the Baltimore-Washinton Young Stroke Study” Sloan, M.A., “Illicit drug-associated ischemic stroke in the Baltimore-Washinton Young Stroke Study”

NeurologyNeurology 1998; 19: 1688-93. 1998; 19: 1688-93.8.8. Biller, J. et al., “Spontaneous subarachnoid hemorrhage in young adults” Biller, J. et al., “Spontaneous subarachnoid hemorrhage in young adults” NeurolosurgeryNeurolosurgery 1987; 21(5): 1987; 21(5):

664-7.664-7.9.9. Mylonakis, E. and Calderwood, S.B., “Infective Endocarditis in Adults” Mylonakis, E. and Calderwood, S.B., “Infective Endocarditis in Adults” NEJM NEJM 2001; 345(18): 1318-1330.2001; 345(18): 1318-1330.10.10. Cerebral Embolism Task Force. “Cardiogenic Brain Embolism. The Second Report of the Cerebral Cerebral Embolism Task Force. “Cardiogenic Brain Embolism. The Second Report of the Cerebral

Embolism Embolism Task Force” Task Force” Archives of NeurologyArchives of Neurology 1989; 46: 727-43 1989; 46: 727-4311.11. Klein, A.L. et al., “Use of transesophageal echocardiography to guide cardioversion in patients with atrial Klein, A.L. et al., “Use of transesophageal echocardiography to guide cardioversion in patients with atrial

fibrillation” fibrillation” NEJMNEJM 2001 May 10; 344(19): 1411-20. 2001 May 10; 344(19): 1411-20.12.12. Srimannaraya, J. et al., “Prevalence of left atrial thrombus is rheumatic mitral stenosis with atrial Srimannaraya, J. et al., “Prevalence of left atrial thrombus is rheumatic mitral stenosis with atrial

fibrillation fibrillation and its response to anticoagulation: a transesophageal echocardiographic study” and its response to anticoagulation: a transesophageal echocardiographic study” Indian Indian Heart Journal Heart Journal 2003 Jul-Aug; 55(4): 358-61.2003 Jul-Aug; 55(4): 358-61.

13.13. Omran, H. et al., “Incidence of left atrial thrombi in patients in sinus rhythm and with a recent neurologic Omran, H. et al., “Incidence of left atrial thrombi in patients in sinus rhythm and with a recent neurologic deficit” deficit” American Heart Journal American Heart Journal 2000; 140(4): 685-62.2000; 140(4): 685-62.

14.14. Reardon, M.J. and Smythe, W.R. Reardon, M.J. and Smythe, W.R. Cardiac Surgery in the AdultCardiac Surgery in the Adult. 2. 2ndnd Ed. 2003. Chapter 58 Cardiac Ed. 2003. Chapter 58 Cardiac Neoplasms. Neoplasms. New York: McGraw-Hill.New York: McGraw-Hill.

15.15. Sabatine, M.S. et al. Sabatine, M.S. et al. Braunwald’s Heart Disese: A Textbook of Cardiovascular MedicineBraunwald’s Heart Disese: A Textbook of Cardiovascular Medicine 7 7thth Ed. 2005. Ed. 2005. Chapter Chapter 63 Primary Tumors of the Heart. Saunders.63 Primary Tumors of the Heart. Saunders.

16.16. Edwards, F.H., et al. “Primary Cardiac Valve Tumors” Edwards, F.H., et al. “Primary Cardiac Valve Tumors” Annals of Thoracic SurgeryAnnals of Thoracic Surgery 1991; 52: 1127-31. 1991; 52: 1127-31.17.17. Reynen, M.D. “Cardiac Myxomas” Reynen, M.D. “Cardiac Myxomas” NEJMNEJM 1995; 333(24): 1610-1617. 1995; 333(24): 1610-1617.18.18. www.uptodate.comwww.uptodate.com19.19. The Grand TetonsThe Grand Tetons20.20. The Showalter FamilyThe Showalter Family

Special ThanksSpecial Thanks

Dr. ChilesDr. Chiles

Dr. EliesonDr. Elieson

Dr. SibbittDr. Sibbitt

Dr. MockDr. Mock

Dr. FillmoreDr. Fillmore

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A Stroke from the Bishop’s Cap Thomas A. Showalter, III, DO Resident, Internal Medicine CPC August...

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