a stroke from the bishop’s cap
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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
The Case continued…The Case continued…
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.
The Case continued…The Case continued…
PSH:PSH:Appendectomy as childAppendectomy as childChest tube placement for Spontaneous PTxChest tube placement for Spontaneous PTx
Meds:Meds:nonenone
Allergies:Allergies:nonenone
The Case continued…The Case continued…
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.
The Case continued…The Case continued…
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
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
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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.
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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
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