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A Stroke from the Bishop’s Cap

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

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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 - 10-12 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: none

  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. Diff: Neut 54% Lymph 27% Mono 7% The Data 13.6 138 100 10 8.7 273 39.0 4.3 23 0.8 LFTs Normal Coags Normal

  10. 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 The Data continued…

  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. 2-4 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

  18. Cerebral Ischemic Infarct • 3% of all cerebral infarcts occur between 15-45 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 15-30 -> 2% • Age 30-45 -> 30-35% • Homocystinuria – premature large vessels disease • Carotid Atheroma formation due to local radiation for laryngeal tumors • Cranial radiation produces a radiation vasculopathy

  20. 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 The Myriad of Nonatherosclerotic causes

  21. 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 The Myriad of Nonatherosclerotic causes

  22. 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 Cardioembolic causes

  23. 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

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

  25. 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

  26. 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

  27. 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 = 47-69 years • Injection drug users • Higher incidence in younger persons • Incidence 150 to 2000 per 100,000 person-years

  28. 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

  29. 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*

  30. 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

  31. 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…

  32. Cardiac Tumors • Metastatic • 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 • 0.17-0.19% 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

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

  34. Relative Incidence of Primary Malignant Tumors of the Heart *Other sarcomas include liposarcomas, synovial and neurogenic sarcomas Adapted from Braunwald’s Heart Disease 7th edition

  35. 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

  36. 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

  37. 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 12-24 months • Possible in less than 50% of patients • Incomplete excision – median survival 3-10 months

  38. 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

  39. 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

  40. 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

  41. 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

  42. 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

  43. Relative Incidence of Benign Tumors of the Heart *other tumors include cystic tumors of the atrioventricular node, endocrine tumors and histiocytoid tumors Adapted from Braunwald’s Heart Disease 7th edition

  44. Relative Incidence of Benign Tumors of the Heart *other tumors include cystic tumors of the atrioventricular node, endocrine tumors and histiocytoid tumors Adapted from Braunwald’s Heart Disease 7th edition

  45. 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

  46. 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

  47. 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

  48. 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

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