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A Stroke from the Bishop’s Cap

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1 A Stroke from the Bishop’s Cap
Thomas A. Showalter, III, DO Resident, Internal Medicine CPC August 10th, 2007

2 The Case CC: ‘my right arm and leg are weak’ HPI: 22 year old male
5 weeks prior Right arm and leg numbness and weakness Mild dysarthria Night prior beers, smoked pot, taken 2 vicodin Presented to outside ER Treated with IVF for dehydration and sent home.

3 The Case continued… Same day after ER discharge
Developed twitching of right side of face and arm Returned to ER CT head negative All symptoms resolved with 4-5 hours Except for mild residual right facial numbness 2 days later Reported mild dysarthria Right facial droop and right facial numbness Referred to Neurology at S&W

4 The Case continued… HPI: Seen 4 weeks later
Denied paresthesias, paresis, involuntary movements or dysarthria. Complained of mild headache and sonophobia. PMHx: Substance abuse Spontaneous Pneumothorax at age 19 One episode, 1 year ago with right sided numbness, but no paresis.

5 The Case continued… PSH: Appendectomy as child
Chest tube placement for Spontaneous PTx Meds: none Allergies:

6 The Case continued… Social Hx: Construction worker
+Tobacco. +THC weekly EtoH daily with binges on weekend Methamphetamine once monthly No IV drug abuse Family Hx: Parents healthy. Sister unknown stomach disorder. No early stroke, hypercoagulable disorders, seizures or malignancy.

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

8 Physical Exam VS: T 95.7 P 70 R 12 BP 105/62
Gen: thin male, NAD, healthy appearing. HEENT: normal Neck: normal Heart: normal Chest: normal Extr: normal Neuro: normal Skin: normal

9 The Data Diff: Neut 54% Lymph 27% Mono 7% LFTs Normal Coags Normal
13.6 8.7 273 39.0 Diff: Neut 54% Lymph 27% Mono 7% LFTs Normal Coags Normal

10 The Data continued… CXR: normal EKG:
marked sinus bradycardia with sinus arrhythmia, early repolarization MRI Brain: chronic left MCA infarct and several chronic tiny cerebellar infarcts MRA Head/Neck: normal Circle of Willis and great vessels of head and neck

11 The Data continued… Additional lab obtained… ESR 10
Total Cholesterol 138 Anticardiolipin IgG and IgM – negative Lupus anticoagulant – negative Echocardiography: normal LV function, 1.7 cm x 1.2 cm mass on the atrial side of the anterior leaflet of mitral valve possibly attached by a stalk

12 Hospital Course BC for bacterial and fungal organisms done.
Cardiac MRI: mass on atrial surface of mitral valve with no clear stalk Impression: mass consistent with either tumor or vegetation from infective endocarditis, but more likely endocarditis based on the location and that no stalk was seen on the MRI. A diagnostic procedure was performed.

13 The Case Problem List DDx of Stroke in a Young Person DDx of a Left Atrial Mass Conclusions

14 The Problem List Multiple chronic strokes
Left MCA distribution infarct Multiple tiny cerebellar infarcts Left Atrial Mass (with or without a stalk) Substance abuse Headache with sonophobia History of… Right arm and leg numbness and weakness Dysarthria and facial numbness and weakness Muscle spasm of right face and right arm

15 Stroke in Young Adults Usually defined as age < 45 years
Worldwide incidence 9-11 per 100,000 [4] Northern Manhattan Stroke Study, Stroke 2002 Multiethnic population of 210,000 residents In a 4-yr period, 74 cases of young stroke out of 924 incident first ever strokes (8%) Higher incidence rates in Blacks and Hispanics compared to Whites

16 Stroke in Young Adults Italian epidemiological review by Gandolfo and Conti Neurological Science 2003 Western European Countries, less than 5% of all strokes occurred in patients < 45 years of age (yoa). Developing countries had 20-30% of strokes < 45 yoa United States, 8-10% of strokes in patients < 45 yoa Estimated lifetime cost of stroke $103,576 for US patients times that is young adult due to longer period of lost productivity [4]

17 DDx of Stroke in Young Adults
Subarachnoid Hemorrhage Intracerebral Hemorrhage Cerebral Ischemic Infarcts SAH - Leading cause Ruptured Saccular aneurysm Biller and associates found ruptured aneurysms in 75 of 95 (79%) patients with SAH between years of age Intracerebral Hemorrhage Etiology Ruptured arteriovenous malformation Ruptured saccular aneurysm Sympathomimetic drug use

18 Cerebral Ischemic Infarct
3% of all cerebral infarcts occur between years of age [3] Etiologies Atherosclerotic Nonatherosclerotic Cardioembolic Ages 15-35, cardioembolic and nonatherosclerotic causes predominate After age 35, traditional atherosclerotic stroke risk factors become prime determinants of stroke

19 Atherosclerotic causes
Traditional risk factors: Hypertension, Smoking, Hyperlipidemia, Diabetes Mellitus Age > 2% Age > 30-35% Homocystinuria – premature large vessels disease Carotid Atheroma formation due to local radiation for laryngeal tumors Cranial radiation produces a radiation vasculopathy

20 The Myriad of Nonatherosclerotic causes
Dissection Illicit drug use Infection Prothrombotic States Migraine Sickle Cell Disease Genetic Disorders Inborn Errors of Metabolism Moyamoya Hyperestrogenemic States Vasculitis Arteritis due to Neoplasms

21 The Myriad of Nonatherosclerotic causes
Dissection Illicit drug use Infection Prothrombotic States Migraine Sickle Cell Disease Genetic Disorders Inborn Errors of Metabolism Moyamoya Hyperestrogenemic States Vasculitis Arteritis due to Neoplasms

22 Cardioembolic causes 20-30% of young adult Valvular heart disease
Mitral valve prolapse Prosthetic heart valves Rheumatic heart disease Acute Myocardial Infarction Left ventricular dyskinesia Spontaneous echo contrast Left ventricular aneurysm Left atrial aneurysm Dilated Cardiomyopathy Atrial Septal defect Patent Foreman Ovale Atrial Fibrillation (Left Atrial Thrombus) Bacterial endocarditis Libmann Sachs endocarditis Marantic endocarditis Tumor


24 The Problem List Multiple chronic strokes
Left MCA distribution infarct Multiple tiny cerebellar infarcts Left Atrial Mass (with or without a stalk) Substance abuse Headache with sonophobia History of… Right arm and leg numbness and weakness Dysarthria and facial numbness and weakness Muscle spasm of right face and right arm

25 DDx of Left Atrial Mass Endocarditis Left Atrial Thrombus Tumor
Nonbacterial Thrombotic Endocarditis (Marantic) Libmann Sachs endocarditis Bacterial endocarditis Left Atrial Thrombus Tumor Metastatic Primary, Benign or Malignant

26 Marantic Endocardits Nonbacterial Thrombotic Endocarditis
“Sterile” Vegetations Microscopic to large aggregates of platelets and fibrin on heart valves (usually aortic or mitral) 27% of ischemic stroke in patients with cancer [9] Complicates many nonmalignant wasting illnesses, i.e. AIDS Continuum with Trousseau’s Syndrome Predisposed by prothrombotic states, valvular endothelial disruption and underlying valve disease

27 Libmann Sachs Endocarditis
Verrucous Endocarditis Accumulation of immune complexes, mononuclear cells, hematoxylin bodies, fibrin and platelet thrombi Occurs in minority of Systemic Lupus Erythematosis Fewer seen in Antiphospholipid Antibody Syndrome Most commonly the Aortic or Mitral valve, although the Tricuspid may be affected Typically asymptomatic, but if large enough may embolize

28 Infective Endocarditis
Microbial infection of the endocardial surface Vegetation – platelets, fibrin, microorganisms and inflammatory cells In the U.S., incidence of community-acquired native-valve endocarditis = 1.7 to 6.2 cases per 100,000 person-years Median age = years Injection drug users Higher incidence in younger persons Incidence 150 to 2000 per 100,000 person-years

29 Clinical Manifestations of Infective Endocarditis
Fever – most common sign and symptom Subacute – anorexia, weight loss, malaise and night sweats Heart murmur – new or changing, but usually preexisting Petechiae on skin, conjunctivae or oral mucosa Splenomegaly Congestive heart failure Splinter hemorrhages, Osler’s nodes, Janeway’s lesions Neurologic complications: 20-40% will have neurologic complication! 65% of embolic phenomena involve the CNS

30 The Duke Criteria Introduced by group at Duke University in 1994, modified in 2000 Specificity 99% NPV > 92% Criteria integrated Factors predisposing patients to the development of endocarditis Blood culture isolate and persistence of bacteremia Echocardiographic findings TTE – Specificity 98%, Sensitivity 60-70% TEE – Specificity 85-98%, Sensitivity 75-95%, NPV > 92% Other clinical and laboratory findings Only 5-7% of patients have sterile blood cultures*


32 Left Atrial Thrombus 45% of cardiogenic thromboemboli
13% patients with atrial fibrillation 33% patients with rheumatic mitral stenosis May complicate primary or metastatic tumors Regional or global wall motion abnormalities increase risk Associated with the left atrial appendage Generally, attached to posterior left atrial wall by a broad base, therefore immobile Can be pedunculated and mobile

33 Left Atrial Thrombus Omran in 2000
Sinus Rhythm 1% incidence of left atrial thrombus in patients with recent neurologic deficit 6/583 patients (1%) had left atrial appendage thrombus 3 mitral stenosis, 1 aortic stenosis, 1 dilated cardiomyopathy, 1 coronary artery disease Left atrial thrombi are an infrequent cause of thromboembolism in patients in sinus rhythm and are associated with valvular disease and atrial dysfunction. *Left atrial thrombus is associated with left atrial tumors…

34 Cardiac Tumors Metastatic Primary
20-40 times more common than primary tumors Pericardium > Myocardium > Endocardium 10-20% patients with disseminated cancer will have involvement of heart or pericardium Primary % incidence in unselected autopsy series 1 in 500 cardiac surgical cases, with exception of myxoma Benign -> 75% Myxomas comprise 50% of benign Myxomas comprise 80-90% of left atrial primary tumors Malignant -> 25% Sarcomas comprise 75% of malignant

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

36 Relative Incidence of Primary Malignant Tumors of the Heart
Adults (%) Children (%) Angiosarcoma 28 6 Rhabdomyosarcoma 11 41 Fibrosarcoma 8 18 Malignant fibrous histiocytoma Osteosarcoma 7 Leimyosarcoma 5 Myxosarcoma 3 Other sarcomas* 14 12 Undifferentiated sarcoma Lymphoma *Other sarcomas include liposarcomas, synovial and neurogenic sarcomas Adapted from Braunwald’s Heart Disease 7th edition

37 Malignant Cardiac Tumors
25% of all cardiac tumors are invasive or metastatic 95% of these are Sarcomas (2nd to myxoma in overall frequency) 5% are Lymphomas Sarcomas derive from mesenchyme, therefore have a wide variety of morphological types Mutations in K-ras were seen in most cardiac sarcomas Any age, but most common third and fifth decades Except for rhabdomyosarcomas and fibrosarcomas, distinctly unusual in infants and children

38 Malignant Cardiac Tumors
25-50% patients will have metastatic disease at time of diagnosis Most frequent: lungs, thoracic lymph nodes, mediastinum and vertebral column Less frequent: liver, kidneys, adrenals, pancreas, bone, spleen and bowel Transesophageal Echocardiography recommended for diagnosis CT and MRI show degree of tumor infiltration Often endomyocardial or open biopsy needed

39 Treatment of Cardiac Sarcomas
Sarcomas proliferate rapidly Death due to widespread infiltration of the myocardium, obstruction of flow within the heart or distant metastasis with a few weeks to 2 years after onset of symptoms Median survival 6-12 months Surgical excision considered to achieve local control and relieve symptoms Complete excision - median survival months Possible in less than 50% of patients Incomplete excision – median survival 3-10 months

40 Treatment of Cardiac Sarcomas
Autotransplantation Cardiac explantation, ex vivo tumor resection, cardiac reconstruction and reimplantation Chemotherapeutic benefits are unclear Data support anthracycline-based regimens is soft tissue sarcomas Adjuvant chemotherapy and/or radiation therapy usually recommended Orthotopic heart transplantation in patients with locally unresectable disease without evidence of metastasis 66% still die within 1 year either of locally recurrent or metastatic disease

41 Angiosarcoma 30% of primary cardiac sarcomas 3:1 male-to-female ratio
Predilection for right atrium, may be either intracavitary and polypoid or diffuse and infiltrative Later forms have sheet-like covering of pericardium Usually present with right-sided heart failure or tamponade Systemic signs such as fever and weight loss

42 Angiosarcoma “Cauliflower” appearance on MRI due to areas of hemorrhage and necrosis Tend to be discovered ‘late’, often already metastasized Often not amenable to complete resection Very poor prognosis Kaposi Sarcoma (HSV 8) Less than 5% of pts with AIDS or solid organ transplant

43 Rhabdomyosarcoma Most common cardiac malignancy in infants and children 10% of all primary cardiac sarcomas Diffusely infiltrate the ventricular myocardium May on occasion form polypoid extension into chamber Usually multiple foci with occasional nodular involvement of pericardium Rhabdomyoblast – histological hallmark

44 Fibromyosarcoma 5-10% of cardiac sarcomas
Fibroblastic in differentiation, composed of spindle-shaped cells containing areas of hemorrhage and necrosis Extensively infiltrate the heart Often involving more than one chamber spreading to the pericardium

45 Relative Incidence of Benign Tumors of the Heart
Adults (%) Myxoma 52 Papillary fibroelastoma 16 Lipoma Rhabdomyoma 1 Fibroma 3 Teratoma Hemangioma 6 Other tumors* 5 *other tumors include cystic tumors of the atrioventricular node, endocrine tumors and histiocytoid tumors Adapted from Braunwald’s Heart Disease 7th edition

46 Relative Incidence of Benign Tumors of the Heart
Adult (%) Children (%) Myxoma 52 17 Papillary fibroelastoma 16 Lipoma Rhabdomyoma 1 42 Fibroma 3 18 Teratoma 12 Hemangioma 6 5 Other tumors* 4 *other tumors include cystic tumors of the atrioventricular node, endocrine tumors and histiocytoid tumors Adapted from Braunwald’s Heart Disease 7th edition

47 Rhabdomyoma Most common cardiac tumors in infants and children
¾ occur in patients younger than 1 year Left and right ventricular and septal myocardium 1/3 involve either or one atria Nearly all are multiple Small, lobulated Diameter range 2 mm to 2 cm

48 Rhabdomyoma Most common presentation is heart block or other arrhythmias Echo: multiple small, lobulated, homogenous, hyperechoic intramural tumors Association with Tuberous Sclerosis (80%) Hamartomas Epilepsy Mental deficiency Adenoma sebaceum

49 Lipoma Rare Occur at any age with equal male/female ratio
Diameter 1-15 cm Sessile or polypoid Occur in the subendocardium or subpericardium, ¼ are completely intramuscular Most common chambers affected are the left ventricle, right atrium and interatrial septum

50 Primary Cardiac Valve Tumors
Retrospective study by Edwards et al 1991 from 1932 to 1990 Walter Reed and Brooke Army Medical Centers 53 patients had 56 primary cardiac valve tumors Age range 2 to 88 years Average 52 years of age – 79% male Aortic valve most commonly affected Tumor size ranged 3 mm to 7 cm Average size = 1.15 cm

51 Primary Cardiac Valve Tumors
52/56 (93%) tumors were benign 41 papillary fibroelastomas 5 myxomas 4 fibromas 1 hamartoma 1 hemangioma Mitral valve most commonly symptomatic 8/53 (15%) patients had neurologic symptoms 6 had mitral valve tumors 3 myxomas and 3 malignancies

52 Comparison with Nonvalvular Cardiac Tumors
Most common nonvalvular -> Myxoma Most common valvular -> Papillary fibroelastoma Edwards et al “Primary Cardiac Valve Tumors” Ann Thorac Surg 1991; 32:1131

53 Papillary Fibroelastoma
Most common tumor of the cardiac valves Average age 60 at detection (range neonates to 92 yoa) Men = Women Most have concomitant valvular disease suggesting this may predispose to papillary fibroelastoma 90% solitary

54 Papillary Fibroelastoma
Median diameter = 8 mm, largest reported 4 cm Any valve, aortic and mitral most commonly arterial side of semilunar valves atrial side of AV valves Short pedicle 50% of time

55 Papillary Fibroelastoma Path
Grossly, a frond-like appearance resembling a sea anemone Histological, numerous papillary fronds consisting of a collagen core surrounded by elastic fibers and loose connective tissue, all covered by endocardial endothelium

56 Myxomas 3rd and 6th decades of life
Mean age at presentation = 50 years Age range = newborn to 95 years 2/3’s females

57 Myxomas Location 75% Left Atria 15-20% Right Atria 3-4% Left Ventricle
3-4% Right Ventricle Attachment Interatrial Septum Limbus of Fossa Ovalis Posterior Atrial wall Anterior Atrial wall Atrial appendage Rarely endocardial

58 Myxomas Average size 5-6 cm in diameter
Size Range < 1 to > 15 cm Echo shows a mobile, distensible tumor connected to the interatrial septum by a narrow stalk Polypoid Usually pedunculated Round or oval with smooth or lobulated surface

59 Myxoma Path Embryonic mesenchymal cells with multipotent capability
Myxoid matrix of acid- mucopolysaccharide- rich stroma Immunohistochemical studies Vimentin neuroendocrine markers (S-100) gene product 9.5 and calretinin

60 Myxomas Surface of tumor is often covered with thrombi
Embolism occurs in 30-40% of patients Usually systemic, majority are cerebral Infected myxomas have been described Greater danger of systemic embolism Excised Villous Left Atrial Myxoma

61 Myxomas Intracardiac obstruction Systemic embolization
70% have heart failure or syncope Dyspnea, pulmonary edema, sudden death Systemic embolization 30% of patients with myxoma with 2/3 cerebral 25% with emboli have evidence of multiple embolic events Constitutional symptoms Unique to myxoma, 30-40% of patients Synthesis of interleukin – 6 Fatigue, fever, erythematous rash, arthralgia, myalgia and weight loss

62 Myxomas The Physical Exam The “Wrecking Ball Effect”
Murmur heard > 50% Diastolic due to obstructed filling of the ventricle Systolic due to interference with closure of the AV valve S1 often loud and widely split Delay in closure of the AV valve “Tumor Plop” in 33% of patients A diastolic murmur heard 80 to 150 msec after the second heart sound Pericardial friction rub Right atrial tumors The “Wrecking Ball Effect” Recurrent collision with the pedunculated myxoma and the mitral valve may cause permanent damage

63 Myxomas Familial Myxomas Carney Complex 10% or less of all myxomas
Autosomal dominant Median age 20 years Atypical locations, often multiple and recurrent tumors Carney Complex Myxomas, Spotty skin pigmentation and endocrine overactivity

64 Treatment of Benign Cardiac Tumors
Operative excision under direct vision using cardiopulmonary bypass Schaff and Mullany, 2000 Orthotopic heart transplant Autotransplantation

65 Discussion Myxomas are source of most tumor emboli because of their friable consistency and intracavitary location, but other types may embolize. “An embolic stroke in a young person without evidence of cerebrovascular disease, particularly in the presence of sinus rhythm, should raise the suspicion of intracardiac myxoma, as well as infective endocarditis.” – Braunwald’s Heart Disease, 7th Ed.

66 Discussion Multiple Strokes Left Atrial Mass Tumor Myxoma
Papillary Fibroelastoma Tumor with or without thrombus? Tumor with or without infection?

67 Diagnosis: Myxoma Requested Procedure: Surgical Resection

68 References Gandolfo, C. and M. Conti. “Stroke in young adults: epidemiology” Neurological Science 2003; 24: S1-S3. Jacobs, B.S., et al. “Stoke in the young in the Northern Manhattan Stroke Study” Stroke 2002; 33: Hart, R.G. et al., “Diagnosis and management of ischemic stroke. II. Selected controversies” Current Problems in Cardiology 1983; 8(7): Birgitte, H. et al. “Stroke in Young Adults and Children” Current Neurology and Neuroscience Reports 2001; 1: Martin, P.J., et al. “Causes of ischaemic stroke in the young” Postgraduate Medical Journal 1997; 73: 8-16. Guillon, B. et al., “Internal carotid artery dissection: an update” Journal of Neurologic Science 1998; 153: Sloan, M.A., “Illicit drug-associated ischemic stroke in the Baltimore-Washinton Young Stroke Study” Neurology 1998; 19: Biller, J. et al., “Spontaneous subarachnoid hemorrhage in young adults” Neurolosurgery 1987; 21(5): Mylonakis, E. and Calderwood, S.B., “Infective Endocarditis in Adults” NEJM 2001; 345(18): Cerebral Embolism Task Force. “Cardiogenic Brain Embolism. The Second Report of the Cerebral Embolism Task Force” Archives of Neurology 1989; 46: Klein, A.L. et al., “Use of transesophageal echocardiography to guide cardioversion in patients with atrial fibrillation” NEJM 2001 May 10; 344(19): Srimannaraya, J. et al., “Prevalence of left atrial thrombus is rheumatic mitral stenosis with atrial fibrillation and its response to anticoagulation: a transesophageal echocardiographic study” Indian Heart Journal Jul-Aug; 55(4): Omran, H. et al., “Incidence of left atrial thrombi in patients in sinus rhythm and with a recent neurologic deficit” American Heart Journal 2000; 140(4): Reardon, M.J. and Smythe, W.R. Cardiac Surgery in the Adult. 2nd Ed Chapter 58 Cardiac Neoplasms. New York: McGraw-Hill. Sabatine, M.S. et al. Braunwald’s Heart Disese: A Textbook of Cardiovascular Medicine 7th Ed Chapter 63 Primary Tumors of the Heart. Saunders. Edwards, F.H., et al. “Primary Cardiac Valve Tumors” Annals of Thoracic Surgery 1991; 52: Reynen, M.D. “Cardiac Myxomas” NEJM 1995; 333(24): The Grand Tetons The Showalter Family

69 Special Thanks Dr. Chiles Dr. Elieson Dr. Sibbitt Dr. Mock
Dr. Fillmore

70 Thank You

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