Review Test 1. Ménckeberg arteriosclerosis is marked by ail of the following characteristics EXCEPT. (A) it occurs most often in elderly patients, (B) calcifications occur in the arterial medi (©) is predominantly seen in radial and ulnar arteries, (> signi int vascular obstruction occurs. 8. The orders? esion shown in the illustration below is associated with which of 2. All of the following terms and phrases con. cerning the pathogenesis of atherosclerosis are correctly matched EXCEPT (A) insudation hypothesis filtration. (B) encrustation hypothesis—repeated sur face thrombosis, (©) reaction to injury formulation: lial damage and secondary pla sition, monoclonal hypothesis—atherogenic an tibodies. {intimal lipid in. o ¢ of the following dis (Reprinted with permission from Golden A, PowsllD, and Jenrings C: Pathology: Understanding Human Disease, 2nd-ed, Balimore, Wiliams & Wiki, 1985, p 189.) (A) Congenital vascular muscle weakness (B) Medial calcification (©) Cystic medial necrosis, (D) Syphilis (B) Hyperehol terolemia 146148 / Pathology Questions 13-16 For each complication below, select the option ‘The response options for items 18-16 are the With Which tis most likely tobe associated. same. You will be required to select one answer 18, Aortic valvular insufficiency for each item in the set. (A) Congenital vascular muscle weakness 14, Aneurysm of abdominal aorta (B) Atherosclero (©) Syphilis (D) Hypertension (B) Penetrating wounds 15. High-output eardiae failure 16. Dissecting aortic aneurysm Answers and Explanations 1-D. Monckeberg arteriosclerosis is usually a nonobstructing lesion because the arterial intima is not involved. The condition is often seen in elderly persons in whom medium-sized muscular arteries, typically the radial and ulnar arteries, become stiffened by calcification within the me- dia, resulting in so-called “pipestem” arteries. 2D. The smooth muscle proliferation in atherosclerotic lesions is often monoclonal—that is, de- rived from a single precursor cell. The significance of this observation led in the past to a number of speculative concepts collectively referred to as the monoclonal hypothesis. The insudation hy~ pothesis held that the primary atherogenic event is intimal lipid infiltration, and in this regard it Is noteworthy that the fatty streak (focal accumulations of intimal lipid-laden foam cells) is con- sidered to be the earliest atherosclerotic change. The encrustation hypothesis considered repeated surface mural thrombosis to be the primary event in atherogenesis, whereas the currently held reaction to injury formulation considers arterial endothelial damage, followed by monocytic infil- tration, ipid insudation, and platelet adhesion with release of mitogenic factors, to be primary. 3-E. ‘The incidence of atherosclerosis is strongly associated with hypercholesterolemia. The fig: ure shows a large atheromatous plaque narrowing the lumen of an artery. The plaque consists of a mixture of fibrous tissue and cleft-like spaces indicating the presence of cholesterol crystals. 4-D. Atherosclerotic plaques are intimal lesions consisting of a surface fibrous cap of connective tissue with abundant collagen and smooth muscle cells overlying a necrotic core of debris, old f- brin, and pools of lipid, ipid-laden macrophages, and cholesterol. 5D. Increased serum HDL concentration is associated with a decreased risk of atherosclerosis. This cholesterol-containing lipoprotein, the so-called “good” cholesterol, is postulated to play & scavenger role, moving excess cholesterol away from peripheral cells and atherosclerotic plaques. Hypertension, diabetes mellitus, hyperuricemia, and the use of oral contraceptives are all asso- ciated with an increased risk of atherosclerosis. 6-£, Dissecting aneurysm is unrelated to atherosclerosis. It is predisposed by eystic medial necrosis and is almost always associated with severe hypertension. The presenting symptom is often severe, tearing chest pain. Most deaths result from hemorrhage into the pericardial sac. 7-A. The most serious complication of venous thrombosis (most often of the lower extremities) is pulmonary embolism with resultant pulmonary infaretion, 8D. Hereditary hemorrhagic telangiecta: is an important autosomal dominant condition of- ten presenting as bleeding of obscure ori 9-D. Polyarteritis nodosa is an immune complex disease. The antigen is most often unknown. ‘Hepatitis B surface antigen is found in approximately 30% of cases and may well be the antigen involved in the immune complexes.Chapter 9 4. Which of the following descriptions best ap: plies to atherosclerotic plaques? @) Cholesterol infltration of thickened vas (B) Cholesterol infiltration of thickened vas cular media (©) Cystic degeneration and necrosis of vas cular media (D) Intimal lesion consisting of fibrous cap overlying necrotic lipid-laden core Organized old thrombotic residues overly- ing atrophie vascular intima © 55. An increased incidence of atherosclerosis has been correlated with all ofthe following as: sociations EXCEPT “w @) © o hypertension, diabetes mellitus. hyperuricemia. increased serum high-density lipoprotein (HDL) concentration. (E) use of oral contraceptives. 6. Dissecting aortic aneurysm is associated with all of the following characteristics EX- CEPT (A) death from hemoperieardium, (B)_ degenerative changes of aortic medi (©) hypertension (D) severe, tearing chest pain, (B)_ severe atherosclerosis. important complication of venous pulmonary embolism with resultant in- farction. peripheral vascular insufficiency with gangrene. arteriovenous fistula with hypervolemia, ‘thrombophlebitis, @ © my «®), 8, All of the following vascular lesions are cor- rectly matehed with the appropriate elinieal association EXCEPT (A) hemangiosarcoma—administration of Thorotrast. (B) cystic hygroma—cervieal and axillary (©) glomus tumor—painful subungual nod- ule, (D) hereditary hemorrhagic telangivetasia— X-linked inheritance. (©) Kaposi sarcoma—AIDS. Vascular System | 147 9. Hepatitis B virus is frequently associated with which of the following diseases? (A) Lymphomatoid granulomatosi (B) Temporal arteritis (C) Thromboangiitis obliterans (D) Polyarteritis nodosa (8) Takayasu arteritis 10. A child with fever, arthralgias, gastroin- testinal and renal involvement, and hemor. rhagic urticaria-like lesions of the skin most likely is exhibiting “ Be © om «eB 11. All of the following vasculitis syndromes and their associations are correctly matched EXCEPT (A) Buerger disease—high-fat diet. (B) Kawasaki disease—lymphadenitis and involvement of conjunetivae, lips, and oral ‘Takayasu arteritis. Henoch-Sehonlein purpura. polyarteritis nodosa, temporal arteritis, Wegener granulomatosis. (©) Takayasu arteritis—aortie arch, (D) Wegener granulomatosis—upper and lower respiratory tract. (B) temporal arteritis—branches of carotid artery. 12. Chronic renal disease, pheochromoey toma, Conn syndrome, coarctation of the aorta, and acromegaly are all conditions that may Tead to “w ®B © o () hypersensitivity vasculitis. Wegener granulomatosis. venous thrombosis. hypertension, thromboangiitis obliterans.Chapter 9 Vascular System / 149 10-B, The hemorrhagie urtiearia-like lesions (palpable purpura) place this disorder within the hypersensitivity vasculitis group. Associated gastrointestinal and renal abnormalities are par: ticularly characteristic of Henoch-Schénlein purpura, 11-A. Buerger disease, or thromboangiitis obliterans, is an obstructive inflammatory arterial disorder elourly related to cigarette smoking. 12-D. Chronic renal disease, pheochromocytoma, Conn syndrome, coarctation of the aorta, and acromegaly are all prominent causes of secondary hypertension. Renal disease is the most fre. ‘quent cause, Endocrine disorders, such as pheochromocytoma, Conn syndrome, and acromegaly, represent the next most common cause. Coarctation of the aorta is a frequent cause of hyper: ‘tension limited to the upper extremities. 13-C. Syphilitic aortitis characteristically involves the proximal thoracic aorta, resulting in di lation, often with aneurysm formation, Dilation of the adjacent aortic valve results in aortic in sufficiency. Although once the most common cause of these lesions, syphilis rarely causes this now because of treatment and control. 1A-B. Atherosclerosis is the most common cause of aortic aneurysm, and the abdominal aorta is, the most frequent site of involvement. 15-E, Penetrating wounds can result in arteriovenous fistula formation, which in turn is asso- ciated with hypervolemia and high-output cardiac failure, 16-D. Hypertension predispoces to dissecting aneurysm, as does eystic medial necrosis,Review Test 1. Prinametal angina is characterized by “w B) © ‘chest pain on exertion, ‘chest pain at rest. narrowing of coronary ostia by syphilitie aortitis. (D) coronary embolism. 2. A myocardial infarct that exhibits early granulation tissue has most likely occurred “@ B Jess than 1 hour previously. within 24 hours. (© within 1 week, (D) within 1 month. (&) within 3 months. 8. Following acute myocardial infarction, rup- ‘ture of the left ventricle is most likely to occur within “ B) © @) «®) 6-8 hours 16-24 hours, 4-7 days. 2-3 weeks. 2-8 months, 164 4, The most common cause of death immedi aicly after the onset of acute myocardial in- faretion is (A) archythmia. (B) left ventricular rupture. (C) congestive heart failure (D) shock. (B) pulmonary edema, 5, Manifestations of acute rheumatic fever i clude all of the following conditions EXCEPT w B © ) © mitral stenosis, pancarditis. subcutaneous nodules. erythema marginatum, chorea. 6. Characteristic lesions or complications of rheumatic heart disease include all of the fol- owing conditions EXCEPT ww ® © ) o mitral stenosis. tral insufficiency. sortie stenosis, pulmonary stenosi infective endocarditis.Chapter 10 The Heart_1 165 7. ‘The myocardial lesions shown in the illustration below ean be observed in patients with which one of the following conditions? (Reprinted wih permission from Golden A, Powel, and Jennings C: Pathology: Understanding Human Disease, 2nd'ed. Baltimore, Willams & Wikis, 1985, p 171.) “ By © wD «B) Chorea Unstable angina Systemic embolization Wasting diseases Systemic lupus erythematosus 8. Most deaths that occur during acute rheu. matie fover are caused by w B © D) pericarditis. endocarditis streptocoe: myocarditis, I sepsis 9. Nonbacterial thrombotic endocarditis. is most frequently associated with which of the following conditions? (A) Terminal neoplastic disease (B)_ Systemic lupus erythematosus (C) Old rheumatic endocarditis (D) Subdiaphragmatic abscess (B) Congenital heart disease 10, Which pair of cardiac valves listed below is least commonly associated with rheumatic heart disease? (A) Aortie and pulmonary (B) Mitral and tricuspid (©) Aortie and mitral (D) Pulmonary and tricuspid (B) Aortic and tricuspid 11. Which of the following conditions charac- teristically results in aortic valvular insu ciency’ “w @ © ) © 12. Mitral stenosis most often results from w B © @ Q Carcinoid syndrome Marantic endocarditis Bicuspid aortie valve ic heart disease ‘Sacks endocarditis rheumatic endocarditis, bacterial endocarditis. congenital heart disease Marfan syndrome, syphilitic heart disease166 / Pathology 13. Cyanosis in congenital heart disease is caused by (A) alleft-to-right shunt (B) pulmonary valvular insufficieney. (©) aortic stenosis, (D) a right-to-left shunt 14. All of the following conditions are associ- ated with a left-to-right shunt EXCEPT “~ B © ) patent ductus arteriosus, atrial septal defect. ventricular septal defect, tetralogy of Fallot, 15. ‘Tetralogy of Fallot most often includes all of the following features EXCEPT “ B) © early left-to-right shunting, overriding aorta. pulmonary infundibular or valvular steno- right ventricular hypertrophy. ventricular septal defect. o ® 16. Which one of the following conditions is as. sociated with paradoxie embolism? (A) Rheumatic heart disease (B) Pulmonary stenosis (©) Pulmonary hypertension (D) Atrial septal defects (B) Infective endocarditis 17. Notching of the undersurface of the ribs is characteristic of (A) atrial septal defect (B) ventricular septal defect. (C) tetralogy of Fallot. (D) coaretation of aorta. (B) patent ductus arteriosus. 18. A.42-year-old man is seen because of a long history of slowly developing congestive heart failure. His blood pressure is normal. Coronary artery angiography reveals no vascular dis- ‘ease. No heart murmurs are heard. The white blood cell count, differential, and erythrocyte sedimentation rate are normal. The most likely diagnosis is w @B © o) © cardiomyopathy: constrictive pericarditis. carcinoid heart disease, myocardial infarction. coarctation of the aorta. 19. Which of the following diseases is elassi- fied as a restrictive cardiomyopathy? (A) Alcoholic heart disease (B) Hypertensive heart disease (©) Ischemic heart disease (D) Amyloid heart disease (B) Asymmetric septal hypertrophy 20, Which of the fullowing is the most common primary tumor of the heart? (A) Leiomyoma (B) Rhabdomyoma (©) Myxoma D) Fibroma (©) Lipoma 21. Right ventricular failure is commonly characterized by all of the following changes EXCEPT, (A) distended neck veins (B) hepatomegaly. (©) ankle edema. (D) sodium and water retention (B) pulmonary edema. 22. Right ventricular hypertrophy is caused by all of the following conditions EXCEPT (A), chronic ling disease. (B) mitral valve disease. (©) tetralogy of Fallot. (D) hypertension (©) pulmonary arterial hypertension, 28. The most frequent cause of cor pulmonale with right-sided heart failure is “w ® © o constrictive pericarditis disease of the lungs or pulmonary vessels, left-sided heart failure. pulmonary infundibular or valvular steno- (&) systemic hypertension,Chapter 10 ‘The Heart 1167 Answers and Explanations 1-B. Prinzmetal angina is a variant of angina thought to result from coronary artery spasm and inoften seen in patients with anatomically normal coronary vessels. Discomfort is similar to that of classic angina but occurs characteristically at rest rather than upon exertion. 2-C, By the end of the first week after a myocardial infarction, early granulation tissue is seen ‘at the periphery of the necrotie area, followed shortly by the appearance of newly formed colla: gen, Granulation tissue is gradually replaced by scar tissue within several weeks, 3-C, Rupture of the left ventricle, a catastrophic complication of acute myocardial infaretion, usually oceurs when the necrotic area has the least tensile strength, which is at about 4~7 days after an infarction when repair is just beginning, The anterior wall of the heart is the most fre- ‘quent site of rupture, usually leading to fatal cardiac tamponade. Internal rupture of the inter- ventricular septum or of a papillary muscle may also oceur. 4-A. Arrhythmias are the most important early complication of acute myocardial infarction, ac- ‘counting for almost 50% of deaths shortly after myocardial infarction. 5-A. Severe valvular disease, often manifest as mitral stenosis, s a feature of rheumatic heart disease, the chronic stage of rheumatie disease, in which damaged, fibrotic valves may become stenotic, insufficient, or both, Panearditis, subcutaneous nodules, erythema marginatum, and chorea are all manifestations of acute rheumatic fever. 6-D. The pulmonary valve is only rarely involved in rheumatic heart disease, and pulmonary stenosis is usually congenital in origin, Fibrous scarring secondary to endocardial involvement in theumatie fever leads to rheumatic heart disease (chronic valvular disease occurring as a se- quela of rheumatic fever). The valve most commonly affected is the mitral valve, The damaged fibrotic valves ean become the site of bacterial infection, as in subacute bacterial endocarditis, 7-A. The figure illustrates an Aschoff body, the characteristic lesion of rheumatic fever. This myocardial lesion is most often oval in shape and characterized by swollen fragmented collagen ‘and fibrinoid material and by characteristic large mesenchymal cells (Anitschkow myocytes) and multinucleated cells (Aschoff cells), Sydenham chorea is @ major manifestation of rheumatic fever. 8-D. The most common cause of death that occurs during acute rheumatic fever is cardiae fail: lure secondary to myocarditis, 9-A. Nonbacterial thrombotic endocarditis, or marantie endocarditis, has been associated with ‘a variety of wasting diseases and is observed most often in patients with cancer. 10-D. Valvular deformities caused by rheumatic fever tend to occur in the mitral valve or tral and aortic valves, most likely because of the hemodynamic factors associated with the left side of the heart, Tricuspid valve involvement is seen in 10%-15% of cases and is almost always associated with mitral and aortic lesions. The pulmonary valve is rarely involved. 11-D. In syphilitic aortitis, the elastica of the aorta is replaced by fibrous tissue, resulting in di lation of the ascending aorta and separation of the aortic commissures, with resultant aortic in- sullicieney. 12-A. Mitral stenosis is most often a late sequela of rheumatic fever. Bacterial endocarditis is a complication, not a cause, of mitral stenosis. Congenital heart disease is sometimes complicated by mitral stenosis, which is often of rheumatic origin. Marfan syndrome is sometimes associated with mitral valve prolapse. Syphilitic heart disease is characterized by widening of the aortic commissures, resulting in aortic insufficieney. 13-D. Cyanosis, which oceurs when the arterial concentration of reduced hemoglobin exceeds 5168 / Pathology ‘mg/ml, is seen with a right-to-left shunt in which venous blood gains direct access to the arter- il eireulation 14-D. In tetralogy of Fallot, the ineroasod right ventricular pressure caused by pulmonary stenosis results, along with the overriding aorta, in right-to-left shunting. In contrast, patent ductus arteriosus and atrial and ventricular septal defects are associated with left-to-right blood flow because of the prossure gradient from left to right. Reversal of shunting may occur as time passes, 15-A. Most cases of tetralogy of Fallot are cyanotic at birth because of right-to-left shunting. This is not invariable, however, because the direction of blood flow is dependent on the severity of pulmonary infundibular or valvular stenosis. 16-D. The term paradoxic embolism denotes the passage of an embolus of venous origin into the arterial circulation through a right-to-left shunt (e.g., patent foramen ovale, atrial septal defect) ‘The likelihood of right-to-left passage of the embolus is often enhanced by pulmonary hyperten- sion secondary to pulmonary thromboembolism, 17-D. Coaretation of the aorta is characterized by aortic narrowing, most often near the ductus arteriosus. This condition results in hypertension proximal to the obstruction and hypotension distal to it. Eventually collateral circulation develops, involving the subclavian, internal mam- ‘ars, and intercostal arteries us well a others. Notching ofthe undersurface ofthe ribs is the result of atrophy eaused by pressure on the adjacent ribs from the dilated, pulsating intercostal arteries 18-A. Cardiomyopathies are noninflammatory myocardial disorders not associated with eoro: nary artery obstruction, hypertension, valvular disease, congenital heart disease, ot infectious disease. They are most often characterized by otherwise unexplained ventricular dysfunction, such as cardiac failure, ventricular enlargement, or ventricular arrhythmias. 19-D. Restrictive, or obliterative-restrictive, cardiomyopathy is characterized by restricted ven- tricular filling, most often associated with amyloidosis. Congestive, or dilated, cardiomyopathy is characterized by biventricular dilation associated with alcoholism, thiamine deficiency (beriberi), and cobalt toxicity. Hypertrophic cardiomyopathy is characterized by marked increase in ventricular wall thickness with small ventricular eavities, often with outflow tract obstrue- tion, as in asymmetric septal hypertrophy. 20-C. Myxoma of the heart, although rare, is the most common primary cardiac tumor. Because of the jelly-like appearance and myxoid histology similar to that of some organized thrombi, the neoplastic nature of this lesion was debated for many years. However, authorities now generally believe that myxoma is a true neoplasm. 21-E. Pulmonary edema characteristically results from severe left ventricular failure. Right ventricular failure results in increased right atrial pressure, leading to distention of neck veins and passive congestion and enlargement of the liver. Renin- mediated release of aldosterone re- sults in sodium and water retention, hypervolemia, and peripheral edema. 22-D. Systemic arterial hypertension results in hypertrophy of the left ventricle, Right ventrie- ular hypertrophy most often results from pulmonary hypertension, as in chronic lung disease: from increased left atrial pressure, as in mitral valve disease; or from congenital eardiae defects, asin the tetralogy of Fallot with its overriding aorta and pulmonary infundibular stenosis, 23-B. The term cor pulmonale refers to right ventricular hypertrophy caused by pulmonary hy: pertension secondary to disarders of the lungs or pulmonary vessels, Other causes of right ven- tricular hypertrophy and failure, such as valvular disease, congenital defects, and left-sided heart failure, are precluded by this definition. Constrictive pericarditis ean clinically mimie right-sided heart failure but is entirely unrelated to cor pulmonale,11 Anemia I. General Concepts—Anemia A. Definitions —anemia is a decrease in whole body red cell mass, a definition that pre- cludes relative decreases in red blood cell count, hemoglobin, or hemat- ocrit, which oceur when the plasma volume is increased, O-w —anemia of pregnancy is not anemia but is rather a manifestation of in: creased plasma volume. —a practical working definition of anemia is a decrease in red blood cell count, hemoglobin, or hematocrit, commonly measured red cell. par: meters, B, Causes of anemia (Table 11-1) —anemia may be caused by two major mechanisms: 1, Decreased red cell production resulting from: a, Hematopoietic cell damage from infection, drugs, radiation, and other similar agents b, Deficiency of factors necessary for heme synthesis (iron) or DNA synthesis (vitamin B,, or folate) 2, Increased red cell loss due to: a, External blood loss b, Red cell destruction (hemolytic anemia) Il. Acute Post-hemorrhagic Anemia —within the first few hours of acute blood loss, prior to hemodilution (compen satory increase in plasma volume), there may be no decrease in the hemoglobin, hematoerit, and red blood cell count because of a parallel loss of both red cells and plasma. There is often a marked reactive increase in platelet count. —significant clinical findings are related to hypovolemia. 169