Blood Vessels and Heart Pathology PDF Spring 2025

Blood vessels and heart pathology Dr. Danny Mora Oral and Maxillofacial Pathologist Disclosure: Most photos are from Copyright © 2017, by Elsevier Inc. All rights reserved. Dr. Danny Mora Oral and Maxillofacial Pathologist  – The student will learn the diff...

Blood vessels and heart pathology Dr. Danny Mora Oral and Maxillofacial Pathologist Disclosure: Most photos are from Copyright © 2017, by Elsevier Inc. All rights reserved. Dr. Danny Mora Oral and Maxillofacial Pathologist  – The student will learn the different aspects of the blood vessels and Objectives heart disorders – Will learn to recognize and characterize pathology findings of blood vessels and the heart. Dr. Danny Mora Oral and Maxillofacial Pathologist  Blood Vessels Main component Endothelial cells Smooth muscle cells Dr. Danny Mora Oral and Maxillofacial Pathologist 4 Endothelial cells Maintenance of permeability barrier Modulation of blood flow and vascular reactivity Vasoconstrictors Elaboration of anticoagulant, Endothelin, Angiotensin Converting antithrombotic, fibrinolytic regulators Enzyme Prostacyclin Vasodilators Thrombomodulin Nitric Oxide, prostacyclin Heparine-like molecules Regulation of Inflammation and immunity Plaminogen activator IL-1, IL-6, chemokines Adhesion molecules Elaboration of prothrombotic molecules VCAM-1, ICAM, E-selectin, P-selectine Von Willebrand’s factor Histocompatibility antigens Tissue factor Regulation of cell growth Plasminogen activator inhibitor Growth stimulators PDGF, Colony Stimulating Factor, FGF Extracellular matrix production Growth inhibitors Collagen Heparin, TGF-β Proteoglycans Oxidation of LDL Dr. Danny Mora Oral and Maxillofacial Pathologist  Vascular smooth muscle cells – Normal vascular repair – Synthesize – ECM collagen – Elastin – Proteoglycans – Growth factors – Cytokines – Vasoconstriction and vasodilation Dr. Danny Mora Oral and Maxillofacial Pathologist 6 endothelium media adventitia Dr. Danny Mora Oral and Maxillofacial Pathologist 7 Hypertensive Vascular disease Dr. Danny Mora Oral and Maxillofacial Pathologist 2021  – Blood pressure is a function of cardiac output and peripheral vascular resistance. Hypertensive – Hypertension is sustained elevation of resting systolic BP (≥ 140 mm Vascular Hg), diastolic BP (≥ 90 mm Hg), or both. Disease – No symptoms develop unless hypertension is severe or long-standing. – Treatment involves lifestyle changes and drugs, including diuretics, β - blockers, ACE inhibitors, angiotensin II receptor blockers, and Ca channel blockers Dr. Danny Mora Oral and Maxillofacial Pathologist 9 (a, b) Plexiform lesions of the classical type, showing multiple capillary channels lined by plump endothelial cells; (c) “Nodule type” of plexiform (d) Angiomatoid lesion, (e, f) Fibrinoid necrosis of the medial layer ; inflammatory infiltrate of the adventitia is present in f. Dr. Danny Mora Oral and Maxillofacial Pathologist 2021  – Primary or essential hypertension Endocrine – Hypertension with no known cause Adrenocortical hyperfunction – Most common 90-95%. Exogenous hormones- – Secondary glucocorticoids, estrogen Hypertensive – Renal disorder – Acute glomerulonephritis Pheochromocytoma Hypothyroidism and Vascular – Chronic renal disease – Polycystic disease hyperthyroidism Pregnancy Disease – Renal artery stenosis – Neurologic Cardiovascular Coarctation of aorta – Psychogenic Polyarteritis nodosa – Increased intracraneal pressure Increased intravascular volume or – Sleep apnea increased cardiac output – Acute stress Dr. Danny Mora Oral and Maxillofacial Pathologist  Pathology and complications – No pathologic changes occur early in hypertension. – Severe or prolonged hypertension damages target organs – Cardiovascular system Hypertensive – Brain – Kidneys Vascular – Increasing risk of: – Coronary artery disease (CAD) Disease – Myocardial Infarction – Stroke – Renal failure. – The mechanism involves development of generalized arteriolosclerosis and acceleration of atherogenesis. Dr. Danny Mora Oral and Maxillofacial Pathologist  Vascular Pathology in Hypertension – Hypertension not only accelerates atherogenesis but also causes 12 degenerative changes in the walls of large and medium arteries that can lead to aortic dissection and cerebrovascular hemorrhage. Dr. Danny Mora Oral and Maxillofacial Pathologist  – Hypertension is associated with two forms of small blood vessel disease – Hyaline Arteriolosclerosis. – Arterioles show homogeneous, pink hyaline thickening with associated luminal narrowing. – In nephrosclerosis due to chronic hypertension, the Morphology arteriolar narrowing of hyaline arteriosclerosis causes diffuse impairment of renal blood supply and causes glomerular scarring Dr. Danny Mora Oral and Maxillofacial Pathologist  – Hyperplastic arteriolosclerosis seen in severe malignant hypertension – Vessel show onion skin lesions with concentric laminated thickening of the walls and luminal narrowing – Consists of smooth muscle with reduplicated basement Hypertensive membranes Vascular – Fibrinoid deposits and necrosis of the wall Disease Dr. Danny Mora Oral and Maxillofacial Pathologist  – Once arteries are narrowed, any slight additional shortening of already hypertrophied smooth muscle reduces the lumen to a greater extent. – Increased afterload gradually hypertrophies the left ventricle, causing Hypertensive diastolic dysfunction. Vascular – The ventricle eventually dilates, causing dilated cardiomyopathy and heart failure (HF) due to systolic Disease dysfunction. – Thoracic aortic dissection is typically a consequence of hypertension; – Almost all patients with abdominal aortic aneurysms have arterial hypertension. Dr. Danny Mora Oral and Maxillofacial Pathologist  16 Normal Kidney Kidney with Nephrosclerosis Dr. Danny Mora Oral and Maxillofacial Pathologist 2021  – Hardening of the arteries – Thickening of the arterial wall Arteriosclerosis – Loss of elasticity – Three patterns – Arteriolosclerosis – Small arteries and arterioles – Mönckeberg medial necrosis 17 – Calcific necrosis in muscular arteries – Metaplasia into bone – Not clinically significant – Atherosclerosis – Intimal lesions Dr. Danny Mora Oral and Maxillofacial Pathologist  Atherosclerosis 18 – Intimal lesions called atheromas – Protrudes into the vessel – Soft yellow lipid core of cholesterol and cholesterol esters covered by a fibrious cap. – Obstructs the blood flow, may rupture and produce thrombosis, or aneurismal formation Dr. Danny Mora Oral and Maxillofacial Pathologist  19 Atherosclerosis – Risk factors have a multiplicative effect – Two risk factors have a fourfold risk – Three risk factors have a seven fold risk of myocardial infarction – Constitutional – Age 40-60 y/o, fivefold – Gender postmenopausal women – Genetics family history single most significant risk factor – Modifiable – Hyperlipidemia hypercholesterolemia major risk factor by itself – Hypertension 60% risk of IHD – Cigarette smoking 1 pack doubles risk of IHD – Diabetes twofold for IHD, 100-fold for gangrene Dr. Danny Mora Oral and Maxillofacial Pathologist Atherosclerosis Dr. Danny Mora Oral and Maxillofacial Pathologist 20 Pathogenesis 4. Platelet adhesion 1. Endothelial injury, which causes (among other 5. Factor release from activated platelets, things) increased vascular permeability, macrophages, and vascular wall cells, inducing leukocyte adhesion, and thrombosis smooth muscle cell recruitment, either from the 2. Accumulation of lipoproteins (mainly LDL and media or from circulating precursors its oxidized forms) in the vessel wall 6. Smooth muscle cell proliferation and ECM 3. Monocyte adhesion to the endothelium, production followed by migration into the intima and 7. Lipid accumulation both extracellularly and transformation into macrophages and foam within cells (macrophages and smooth muscle cells cell) Dr. Danny Mora Oral and Maxillofacial Pathologist 22 Atherosclerosis Dr. Danny Mora Oral and Maxillofacial Pathologist  – Fatty streaks – Atherosclerotic plaque – Rupture – Ulceration Atherosclerosis – Erosion – Thrombosis – Hemorrhage into plaque – Atheroembolism – Aneurism formation Dr. Danny Mora Oral and Maxillofacial Pathologist Fatty streak, a collection of foamy macrophages in the intima. A, Aorta with fatty streaks (arrows), associated largely with the ostia of branch vessels. B, Photomicrograph of fatty streak in an experimental hypercholesterolemic rabbit, demonstrating intimal, macrophage-derived foam cells (arrows). 2021 Dr. Danny Mora Oral and Maxillofacial Pathologist Vessel with partial occlusion of lumen by a calcified atheromatous plaque Calcification Lumen Dr. Danny Mora Oral and Maxillofacial Pathologist  – Atherosclerotic Stenosis. – Occlusion of vessel compromising blood flow and causing ischemic injury. – Critical stenosis – Chest pain (angina) Consequences of – Mesenteric occlusion and bowel ischemia, – Chronic Ischemic heart disease Atherosclerotic – Ischemic encephalopathy – Intermittent claudication (diminished extremity perfusion) Disease – Acute Plaque Change. – Rupture/fissuring, exposing highly thrombogenic plaque constituents – Erosion/ulceration, exposing the thrombogenic subendothelial basement membrane to blood – Hemorrhage into the atheroma Dr. Danny Mora Oral and Maxillofacial Pathologist  Consequences of Atherosclerotic Disease – Thrombosis. – Partial or total thrombosis associated with a disrupted plaque is critical to the pathogenesis of the acute coronary syndromes. – Vasoconstriction. – Vasoconstriction compromises lumen size, and, by increasing the local mechanical forces can potentiate plaque disruption. Dr. Danny Mora Oral and Maxillofacial Pathologist Aneurysms A, Normal vessel. B , True aneurysm, saccular type. The wall focally bulges outward and may be attenuated but is otherwise intact. C , True aneurysm, fusiform type. There is circumferential dilation of the vessel, without rupture. D , False aneurysm. The wall is ruptured, and there is a collection of blood (hematoma) that is bounded externally by adherent extravascular tissues. E , Dissection. Blood has entered (dissected) the wall of the vessel and separated the layers. Although this is shown as occurring through a tear in the lumen, dissections can also occur by rupture of the vessels of the vaso vasorum within the media. Dr. Danny Mora Oral and Maxillofacial Pathologist  – An aortic aneurysm is a dilatation in a section of the aorta. – The dilatation might rupture and cause Abdominal severe and rapid bleeding leading to Aortic death. Aneurysms – Most common are in the abdomen, second is the thoracic area – Related to or consequence of atherosclerosis (most common). Dr. Danny Mora Oral and Maxillofacial Pathologist  30 Dr. Danny Mora Oral and Maxillofacial Pathologist  Abdominal aortic aneurysm. A. External view, gross photograph of a large aortic aneurysm that ruptured; the rupture site is indicated by the arrow. B. Opened view, with the location of the rupture tract indicated by a probe. The wall of the aneurysm is exceedingly thin, and the lumen is filled by a large quantity of layered but largely unorganized thrombus. Dr. Danny Mora Oral and Maxillofacial Pathologist  – The clinical consequences of AAA include – Rupture with massive, potentially fatal hemorrhage – Obstruction of a branch vessel – iliac (leg), renal (kidney), mesenteric (gastrointestinal tract), or vertebral (spinal cord) arteries Abdominal – Embolism from atheroma or mural thrombus Aortic – Impingement on an adjacent structure, e.g., compression of a ureter or Aneurism erosion of vertebrae – Presentation as an abdominal mass (often palpably pulsating) that simulates a tumor Dr. Danny Mora Oral and Maxillofacial Pathologist  Thoracic Aortic Aneurism – Most commonly associated with hypertension, although other causes such as Marfan – Signs and symptoms – Compression of mediastinal structures, – Respiratory difficulties – Difficulty in swallowing – Persistent cough due to irritation of or pressure on the recurrent laryngeal nerves, – Pain caused by erosion of bone (i.e., ribs and vertebral bodies), – Cardiac disease as the aortic aneurysm leads to aortic valve dilation with valvular insufficiency or narrowing of the coronary ostia causing myocardial ischemia, – Rupture. – Most patients with syphilitic aneurysms die of heart failure induced by aortic valvular incompetence. Dr. Danny Mora Oral and Maxillofacial Pathologist  – Vasculitis is an inflammation of the blood vessels – Causes changes in the walls of the blood vessels, including thickening, weakening, narrowing and scarring. – Inflammation can be short-term (acute) or long-term (chronic). – The shortage of blood can result in organ and tissue damage, even death. – Caused by immune mechanisms: Vasculitis – Immune complex deposition – Antineutrophil cytoplasmic antibodies – Antiendothelial cell antibodies Dr. Danny Mora Oral and Maxillofacial Pathologist 35 Dr. Danny Mora Oral and Maxillofacial Pathologist  VASCULITIS 36 Dr. Danny Mora Oral and Maxillofacial Pathologist  TA K AYAS U ARTERITIS – This is a granulomatous vasculitis of medium and larger arteries characterized principally by ocular disturbances and marked weakening of the pulses in the upper extremities (pulseless disease). – Transmural fibrous thickening of the aorta—particularly the aortic arch and great vessels—and severe luminal narrowing of the major branch vessels – Under 50 y/o – Traditionally associated with the Japanese population and a subset of HLA haplotypes – Takayasu aortitis has a global distribution. – The cause and pathogenesis are unknown, although immune mechanisms are suspected. Dr. Danny Mora Oral and Maxillofacial Pathologist Polyarteritis Nodosa – This form of vasculitis affects small- to medium-sized blood vessels – Seen in young adults – Affects any organ, including skin, heart, kidneys, peripheral nerves, muscles and intestines – Does no affect lungs. – Signs and symptoms include purpura, skin ulcers, muscle and joint pain, abdominal pain, and kidney problems – Fatal if not treated – 30% of patients have chronic hepatitis B with HBsAg-HbsAb complexes in affected vessels – Classic idiopathic PAN – Cutaneous forms of PAN – PAN associated with chronic hepatitis Dr. Danny Mora Oral and Maxillofacial Pathologist Polyarteritis Nodosa 39 Dr. Danny Mora Oral and Maxillofacial Pathologist Dr. Danny Mora Oral and Maxillofacial Pathologist  – The leading cause of acquired heart disease in children, Kawasaki disease, is an acute febrile, usually self- limited illness – Infancy and childhood 80% are younger than 4 years – Arteritis affecting large to medium-sized, and even small, vessels, specially in the coronary arteries – Also known as mucocutaneous lymph node syndrome – It also affects lymph nodes, skin and the mucous membranes inside the mouth, nose and throat. Kawasaki – More in boys – Japanese or Korean descent Disease – The condition is not preventable, but it's treatable in most cases. – Most children recover from Kawasaki disease without serious problems. – The cause is unknown but it is thought to result from a delayed-type hypersensitivity reaction of T cells to an as yet uncharacterized antigen. – This leads to cytokine production and macrophage activation, and is accompanied by polyclonal B-cell activation. – This results in formation of autoantibodies to endothelial cells and smooth muscle cells, which precipitate the acute vasculitis. Dr. Danny Mora Oral and Maxillofacial Pathologist 42 Dr. Danny Mora Oral and Maxillofacial Pathologist 43 Dr. Danny Mora Oral and Maxillofacial Pathologist  – Necrotizing vasculitis of the blood vessels in the nose, sinuses and throat, lungs, and kidneys. – Shortness of breath, nasal stuffiness, chronic sinusitis, nosebleeds and frequent ear infections. – Pathology – Acute necrotizing granulomas – Necrotizing vasculitis Wegener – Focal necrotizing glomerulonephritis Granulomatosis Dr. Danny Mora Oral and Maxillofacial Pathologist  Raynaud phenomenon – Results from an exaggerated vasoconstriction of digital arteries and arterioles. – These vascular changes induce paroxysmal pallor or cyanosis of the digits of the hands or feet; infrequently, the nose, earlobes, or lips can also be involved. – Characteristically, the involved digits show red, white, and blue color changes from most proximal to most distal, correlating with proximal vasodilation, central vasoconstriction, and more distal cyanosis. – Raynaud phenomenon may be a primary disease entity or be secondary to a variety of conditions. Dr. Danny Mora Oral and Maxillofacial Pathologist 46 Dr. Danny Mora Oral and Maxillofacial Pathologist  Raynaud phenomenon – Primary Raynaud phenomenon reflects an – Secondary Raynaud phenomenon refers to exaggeration of central and local vasomotor vascular insufficiency of the extremities responses to cold or emotional stresses. secondary to arterial disease caused by other entities including SLE, scleroderma, – It affects 3% to 5% of the general population Buerger disease, or even atherosclerosis. and shows a predilection for young women. – Since Raynaud phenomenon may be the – Structural changes in the arterial walls are first manifestation of such conditions, any absent except late in the course, when intimal patient with new symptoms should be thickening can appear. evaluated. – The course of Raynaud phenomenon is usually – Of these individuals, some 10% will benign, but when long-standing can result in eventually manifest an underlying disease. atrophy of the skin, subcutaneous tissues, and muscles. Dr. Danny Mora Oral and Maxillofacial Pathologist  VaricoseVeins – Veins have one-way valves channel blood to the heart. – If the valves are incompetent the blood flow is affected. – The veins become enlarged because they are congested with blood. – The enlarged veins are commonly called spider veins or varicose veins. – Spider veins are small red, blue or purple veins on the surface of the skin. – Varicose veins are larger distended veins that are located deeper than spider veins. – Severe varicose veins can compromise the nutrition of the skin and lead to eczema, inflammation or even ulceration of the skin of the lower leg. Dr. Danny Mora Oral and Maxillofacial Pathologist  49 Dr. Danny Mora Oral and Maxillofacial Pathologist  – Thrombophlebitis and phlebothrombosis – Deep leg veins- 90% – Periprostatic plexus – Pelvic venous plexus – Dural sinuses Varicose – Prolonged immobilization – Congestive heart failure Veins – Pregnancy – Obesity – Systemic hypercoagulability – Superior and inferior vena cava syndrome – Neoplasms – Bronchogenic carcinoma – Mediastinal lymphoma – Thrombus from liver, renal or lower extremity Dr. Danny Mora Oral and Maxillofacial Pathologist  – Benign – Hemangioma – Lymphangiomas Tumors – Glomus tumor – Malignant – Kaposi sarcoma – Angiosarcoma Dr. Danny Mora Oral and Maxillofacial Pathologist  – Hemangiomas are a collection of extra blood vessels in the skin. – They may have different appearances depending on the depth of the increased numbers of blood vessels. – Strawberry Hemangioma is an abnormal collection of blood vessels in the skin characterized by a bright red color and well-defined border. Hemangiomas – A Deep or Cavernous Hemangioma is a large, collection of blood vessels beneath the skin surface characterized by a soft, bluish, or skin colored mass. – A Combined Hemangioma is a combination of a deep and superficial (strawberry) hemangioma. Dr. Danny Mora Oral and Maxillofacial Pathologist 53 Hemangiomas Dr. Danny Mora Oral and Maxillofacial Pathologist  Lymphangiomas – Uncommon, hamartomatous, congenital malformations of the lymphatic system that involve the skin and subcutaneous tissues. – The superficial or lymphangioma circumscriptum. – Deep-seated group: – Cavernous lymphangioma – Cystic hygroma – Rarely do cutaneous lymphangiomas interfere with the well-being of patients and they usually seek medical intervention because of cosmetic reason. Dr. Danny Mora Oral and Maxillofacial Pathologist  Glomus Tumor – Glomus tumors of the head and neck paraganglia are part of the extra-adrenal neuroendocrine system. – Histologically – chief cells or type I cells: chemoreceptive cells – sustentacular cells or type II cells: supporting cells. – The specific ratio of the 2 types of cells determines the function of that particular paraganglion. – Can increase or decrease stimulation to the brainstem respiratory centers, which affects cardiopulmonary function, including respiratory rate and cardiac output. – The vast majority of glomus tumors are benign and slow growth. – Mortality rates are 9-15%, depending on the location of the tumor. – Glomus jugulare and tympanicum tumors both can cause pulsatile tinnitus and conductive hearing loss. Dr. Danny Mora Oral and Maxillofacial Pathologist  – Types – Chronic, classic or European KS – Old men, jews, Europe – Not associated with HIV, 10% involvement of viscera Kaposi – Lymphadenopathic, African of Endemic KS – Young bantu children, aggressive, associated with HIV Sarcoma – Transplant associated KS – High doses of immunosuppressive therapy, aggressive more in viscera than skin – AIDS associated KS – Present in 1/3 of AIDS patients Dr. Danny Mora Oral and Maxillofacial Pathologist 57 Kaposi Sarcoma of the skin Dr. Danny Mora Oral and Maxillofacial Pathologist 58 Kaposi's sarcoma microscopically produces slit-like vascular spaces in the dermis of the skin with extravasation of red blood cells. This tissue resembles granulation tissue. Dr. Danny Mora Oral and Maxillofacial Pathologist Angiosarcoma – Malignant endothelial neoplasia – Older adults – Hepatic angiosarcomas are related to exposure to arsenical pesticides, thorotrast (contrast medium), and polyvinil chloride(PVC) – Also associated to radical mastectomy, radiation, and foreign material – Poor outcome at 5 years Dr. Danny Mora Oral and Maxillofacial Pathologist  60 Heart Disorders Dr. Danny Mora Oral and Maxillofacial Pathologist 61 2021 Dr. Danny Mora Oral and Maxillofacial Pathologist  – Chambers – Increased left atrial cavity size Myocardium Increased mass – Decreased left ventricle cavity size Increased subepicardical fat Effects of Aging – Valves – Aortic valve calcifications Brown atrophy Lipofuscin deposition on the Heart – Mitral valve annular calcific deposits Aorta – Fibrous thickening of leaflets Dilated ascending aorta – Epicardial coronary arteries Elongated thoracic aorta Atherosclerotic plaque – Tortuosity – Calcific deposits – Atherosclerotic plaque Dr. Danny Mora Oral and Maxillofacial Pathologist 63 Dr. Danny Mora Oral and Maxillofacial Pathologist  – Cardiac failure, CHF, Left-sided heart failure, Right-sided heart failure – The heart can't pump enough blood throughout the body. – The weakening of the heart's pumping ability causes – Blood and fluid to back up into the lungs – The buildup of fluid in the feet, ankles and legs - called edema – Tiredness and shortness of breath Heart – The leading causes: – coronary artery disease Failure – high blood pressure – diabetes. – Treatment includes treating the underlying cause of your heart failure, medicines, and heart transplantation if other treatments fail. – About 5 million people in the U.S. have heart failure. – It contributes to 300,000 deaths each year. Dr. Danny Mora Oral and Maxillofacial Pathologist 65 Dr. Danny Mora Oral and Maxillofacial Pathologist  – Causes – Ischemic heart disease – Hypertension – Aortic and mitral valvular disease – Nonischemic myocardial diseases Left sided – Organs affected secondary to heart failure – Lungs Heart Failure – Pulmonary edema – Kidneys – Activation od renin-angiotensin-aldosterone system – Pre-renal azotemia – Brain – Hypoxic encephalopathy Dr. Danny Mora Oral and Maxillofacial Pathologist  Anterior surface of the heart demonstrating the left anterior descending 67 coronary artery with recent coronary thrombosis. To the lower right of the thrombus is an area consistent with underlying myocardial infarction. Dr. Danny Mora Oral and Maxillofacial Pathologist 2021  – Causes – Left sided heart failure – Pulmonary hypertension – Organs affected Right Sided – Liver and portal system Heart Failure – – Spleen Kidneys – Brain – Pleura – Subcutaneous tissue Dr. Danny Mora Oral and Maxillofacial Pathologist  – Ventricular septal defect 42% – Atrial septal defect 10% – Pulmonary stenosis 8% – Patent ductus arteriosus 7% – Tetralogy of Fallot 5% Congenital – Coarctation of the aorta 5% Heart Disease – – Atrioventricular septal defect Aortic stenosis 4% 4% – Transposition of great arteries 4% – Truncus arteriosus 1% – Total anomalous pulmonary venous connection 1% – Tricuspid atresia 1% Dr. Danny Mora Oral and Maxillofacial Pathologist  Ventricular Septal Defect (VSD) – There is an orifice within the membranous or muscular portions of the intraventricular septum that produces a left-to-right shunt, more severe with larger defects Congenital Atrial Septal Defect (ASD) – An orifice from a septum secundum or septum primum defect in the Heart Disease interatrial septum produces a modest left-to-right shunt Patent Ductus Arteriosus (PDA) – The ductus arteriosus, which normally closes soon after birth, remains open, and a left-to-right shunt develops Tetralogy of Fallot – Pulmonic stenosis results in right ventricular hypertrophy and a right- to-left shunt across a VSD, which also has an overriding aorta Dr. Danny Mora Oral and Maxillofacial Pathologist Fallot Tetralogy Transposition of great vessels Dr. Danny Mora Oral and Maxillofacial Pathologist  – Transposition of Great Vessels – The aorta arises from the right ventricle and the pulmonic trunk from the left ventricle. A VSD, or ASD with PDA, is needed for extrauterine survival. There is right-to-left shunting. – Truncus Arteriosus – There is incomplete separation of the aortic and pulmonary outflows, along with VSD, which allows mixing of oxygenated and deoxygenated blood and right-to-left shunting Congenital – Hypoplastic Left Heart Syndrome – There are varying degrees of hypoplasia or atresia of the aortic and mitral valves, along Heart Disease with a small to absent left ventricular chamber – Coarctation of Aorta – Either just proximal (infantile form) or just distal (adult form) to the ductus is a narrowing of the aortic lumen, leading to outflow obstruction – Total Anomalous Pulmonary Venous Return (TAPVR) – The pulmonary veins do not directly connect to the left atrium, but drain into left innominate vein, coronary sinus, or some other site, leading to possible mixing of blood and right-sided overload Dr. Danny Mora Oral and Maxillofacial Pathologist  90% is caused by reduction of coronary blood flow due to atherosclerosis – Clinical manifestation Ischemic – Myocardial infarction Heart Disease – Angina pectoris – Chronic ischemic heart disease with heart failure – Sudden cardiac death Dr. Danny Mora Oral and Maxillofacial Pathologist  Myocardial Infarction – It is a Localized coagulative necrosis of the myocardium caused by vascular occlusion, or decreased flow – Atherosclerosis of coronary arteries (99%) – Decreased supply (fixed atherosclerotic 74 stenosis, thrombosis, and/or vasospasm, e.g.) – Increased demand (LVH due to hypertension or aortic stenosis) – Decreased pO2 – Male to female ratio is 2-6:1 Dr. Danny Mora Oral and Maxillofacial Pathologist  – Four stages, (gross in parentheses) – Early acute: eosinophilia, wavy fibers, few PMNs (barely visible) Myocardial – Acute: coagulative necrosis, lots of PMNs (yellow) Infarction – Organizing: granulation tissue border (red-brown edge around yellow center) – Old: collagen (white scar, wall thinning) Dr. Danny Mora Oral and Maxillofacial Pathologist  – May occur at virtually any age – 10% of myocardial infarcts occur in people under age 40 Myocardial – 45% occur in people under age 65. Infarction – – Blacks and whites are equally affected. The decrease of estrogen following menopause is associated with rapid development of CAD, and IHD is the most common cause of death in elderly women. – Postmenopausal hormonal replacement therapy is not currently felt to protect against atherosclerosis and IHD Dr. Danny Mora Oral and Maxillofacial Pathologist Myocardial Infarction – Is diagnosed by: – Clinical symptoms – Laboratory tests for the presence of myocardial proteins in the plasma – Characteristic electrocardiographic changes – Patients with MI classically present: – Prolonged (more than 30 minutes) chest pain described as crushing, stabbing, or squeezing – Associated with a rapid, weak pulse – Profuse sweating (diaphoresis) – Nausea and vomiting are common – Dyspnea due to impaired contractility of the ischemic myocardium – Pulmonary congestion and edema – 25% of patients the onset is entirely asymptomatic (e.g., in the setting of diabetic neuropathy) and the disease is discovered only by electrocardiographic changes or laboratory tests that show evidence of myocardial damage – The laboratory evaluation of MI is based on measuring the blood levels of proteins that leak out of irreversibly damaged myocytes – Cardiac-specific troponins T and I (cTnT and cTnI) are elevated in 3 to 12 hours – MB fraction of creatine kinase (CK-MB) peak in 24 hours – CK-MB returns to normal in 48-72 hrs, cTnI in 5-10 days, and cTnT in 5 to 14 days Dr. Danny Mora Oral and Maxillofacial Pathologist  – Complications – Rupture (free wall, septum, or papillary muscle) – Mural thrombus (cause of systemic embolism) Myocardial – Congestive heart failure (CHF) Infarction – Left Ventricle Aneurysm – Dysrhythmia, arrhythmia – Pericarditis – Pulmonary thromboembolism Dr. Danny Mora Oral and Maxillofacial Pathologist  79 Myocardial infarction evolution in a) 24-48 hour; b) 3 to 4 days; c) 1-2 weeks; d) months a b c d 2021 Dr. Danny Mora Oral and Maxillofacial Pathologist 80 Dr. Danny Mora Oral and Maxillofacial Pathologist 81 Dr. Danny Mora Oral and Maxillofacial Pathologist Arrhythmias – Abnormalities in myocardial conduction can be sustained or sporadic (paroxysmal). – Aberrant rhythms can be originated in any part of the heart – Tachycardia (fast heart rate) – Bradycardia (slow heart rate) – Irregular rhythm with normal ventricular contraction – Chaotic depolarization without functional ventricular contraction (ventricular fibrillation) – No electrical activity at all (asystole). – Loss of adequate cardiac output due to sustained arrhythmia can produce light-headedness (near syncope), loss of consciousness (syncope), or sudden cardiac death – Ischemic injury is the most common cause of rhythm disorders due to: – Direct damage – Dilation of heart chambers that alters conduction system firing – Abnormalities in the structure or spatial distribution of gap junctions, can cause arrhythmias. – Ischemia, myocyte hypertrophy, and inflammation (e.g., myocarditis or sarcoidosis) also promote increased “irritability” that leads to spontaneous aberrant myocyte depolarization – Deposition of nonconducting material (e.g., amyloid), and even small areas of fibrosis, can disrupt myocyte-to- myocyte signaling, again sowing the seeds for development of reentry circuits that can give rise to potentially fatal arrythmias Dr. Danny Mora Oral and Maxillofacial Pathologist  – Late complication of hypertension – High blood pressure increases the heart's workload. Hypertensive – Causes left ventricle hypertrophy Heart Disease – High blood pressure is the most common risk factor for heart disease and stroke. – It can cause ischemic heart disease from the increased need of oxygen supply. Dr. Danny Mora Oral and Maxillofacial Pathologist  – High blood pressure also contributes to thickening of the blood vessel walls, which in turn may aggravate atherosclerosis Hypertensive – Hypertensive heart disease is the leading cause of illness and death from hypertension. Heart Disease – It affects approximately 7 out of 1,000 people. – High blood pressure is known as a silent killer – Congestive heart failure is result of hypertensive heart disease. Dr. Danny Mora Oral and Maxillofacial Pathologist  – Mitral valve disease – Mitral stenosis – Rheumatic heart disease – Mitral Regurgitation Valvular – Infective endocarditis Heart Disease – Mitral valve prolapse – Fen-phen-induced valvular fibrosis – Rupture of papillary muscle or chordae tendineae – Myocarditis – Calcification of mitral ring Dr. Danny Mora Oral and Maxillofacial Pathologist  – Aortic valve disease – Aortic stenosis – Rheumatic fever – Senile calcific aortic stenosis Valvular – Calcification of congenitally deformed valve Heart Disease – Aortic regurgitation – Infective endocarditis – Syphilitic aortitis – Rheumatoid arthritis – Marfan syndrome Dr. Danny Mora Oral and Maxillofacial Pathologist 87 Aortic acute endocarditis Mitral vlave stenosis secondary to rheumatic fever Dr. Danny Mora Oral and Maxillofacial Pathologist  Mitral Valve Prolapse (Myxomatous Degeneration of the Mitral Valve) – In mitral valve prolapse (MVP), one or both mitral valve leaflets are “floppy” and prolapse, or balloon back, into the left atrium during systole. – MVP affects approximately 2-3% of adults in the United States with an approximate 7 : 1 female-to-male ratio – It is most often an incidental finding on physical examination, but in a small minority of affected individuals may lead to serious complications. 88 – The etiology is mostly unknown, seen in: – Marfan syndrome, caused by fibrillin-1 (FBN-1) mutations – Genetic linkage analyses have also mapped inherited forms of MVP to loci involved in the remodeling of valvular extracellular matrix and cell : cell adhesion. – 3% develop one of four serious complications: – Infective endocarditis – Mitral insufficiency, sometimes with chordal rupture – Stroke or other systemic infarct, resulting from embolism of leaflet thrombi – Arrhythmias, both ventricular and atrial. – Rarely, MVP is the only finding in sudden cardiac death. Dr. Danny Mora Oral and Maxillofacial Pathologist  – Acute, immunologically mediated, multisystem inflammatory disease that occurs a few weeks after an Rheumatic episode of group A streptococcal pharyngitis. – Acute rheumatic carditis is a frequent manifestation during Heart Disease the active phase of RF and may progress over time to chronic rheumatic heart disease (RHD), of which valvular abnormalities are the key manifestations. Dr. Danny Mora Oral and Maxillofacial Pathologist Rheumatic Heart Disease – Characterized by deforming fibrotic valvular disease, particularly mitral stenosis – The incidence and mortality rate has declined due to improved socioeconomic conditions and rapid diagnosis and treatment of streptococcal pharyngitis. – Rheumatic fever only rarely follows infections by streptococci at other sites, such as the skin. Dr. Danny Mora Oral and Maxillofacial Pathologist  – Migratory polyarthritis of the large joints, – Pancarditis, – Subcutaneous nodules, – Erythema marginatum of the skin, – Sydenham chorea, a neurologic disorder with involuntary rapid, Rheumatic purposeless movements. Fever – The diagnosis is established by the so-called Jones criteria: – Evidence of a preceding group A streptococcal infection, with the presence of two of the major manifestations listed above or one major and two minor manifestations – Fever, arthralgia, or elevated blood levels of acute-phase reactants Dr. Danny Mora Oral and Maxillofacial Pathologist  – In Cardiomyopathy the heart becomes abnormally enlarged, thickened and/or stiffened. – This condition is generally progressive and may lead to heart failure. – Causes – Chronic diseases – Alcoholism – Viral diseases – Heart attacks Cardiomyopathies – Abnormalities: – Thickened and/or dilated ventricles, especially the left ventricle. – Scar tissue, after a heart attack or inflammation of the heart muscle. – Overall enlargement of the heart. – Tendency to form blood clots within the heart due to stagnation of blood in the heart chambers – Atrial fibrillation. – Embolism to brain, extremities, and GI tract. Dr. Danny Mora Oral and Maxillofacial Pathologist Cardiomyopathies – Dilated cardiomyopathy – Caused by peripartum, alcohol, genetic, hemochromatosis, chronic anemia, viruses, abuse of illicit drugs doxorubicin, and sarcoidosis. – Involves dilation or enlargement of the heart’s ventricles and an increase in the size of the heart overall – A major cause for heart transplantation. – Systolic dysfunction Dr. Danny Mora Oral and Maxillofacial Pathologist 94 Dr. Danny Mora Oral and Maxillofacial Pathologist Cardiomyopathies – Hypertrophic cardiomyopathy – Involves an abnormal growth of muscle fibers in the heart muscle, usually in the left ventricle. – It is a diastolic dysfunction. – Cause is usually a genetic disorders, but can also be caused by high blood pressure and disease of the aortic valve. – Severe thickening of the heart muscle may cause obstruction of blood flow from the left ventricle to the aorta, resulting in low heart output, fatigue, fainting and arrhythmias. Dr. Danny Mora Oral and Maxillofacial Pathologist Dr. Danny Mora Oral and Maxillofacial Pathologist Cardiomyopathies – Restrictive cardiomyopathy – The heart muscle cannot adequately fill with blood after contraction – Diastolic disorder. – This disease is more common in the tropics, where it may be caused by a condition known as endomyocardial fibrosis, seen in children. – Causes are idiopathic, amyloidosis, radiation- induced fibrosis, Loeffler endomyocarditis, Endocardial fibroelastosis (related to mumps during pregnancy) Dr. Danny Mora Oral and Maxillofacial Pathologist 98 Dr. Danny Mora Oral and Maxillofacial Pathologist 99 Dr. Danny Mora Oral and Maxillofacial Pathologist Questions? Dr. Danny Mora Oral and Maxillofacial Pathologist