CVS Pathology Material Study Guide PDF

Hello, I would like you to study and analyze the following reference and question file: My CVS pathology material And deduce from it the important and frequently asked questions and the important points mentioned in the questions In order to create 50 questions: 1. Cover all the topics in the refe...

Hello, I would like you to study and analyze the following reference and question file: My CVS pathology material And deduce from it the important and frequently asked questions and the important points mentioned in the questions In order to create 50 questions: 1. Cover all the topics in the reference 2. Serve as a comprehensive and intensive review of the material To help me prepare for the exam Question specifications: 1. To be in the form of MCQ 2. To mention the logical explanation of the answer like the model below 3. To create a brief table comparing the answers containing: Key: etiology, Pathology, clinical Manifestation, treatment To help me in the Diagnosis 1. A 55-year-old woman presents with complaints of chest pain. She states that the chest pain predictably occurs when she climbs four flights of stairs to reach her apartment or when she has been jogging for more than 10 minutes. She is particularly concerned because her mother died of a myocardial infarction at 50 years of age. Which of the following best describes this patient’s state? (A) Arrhythmia (B) Myocardial infarction (C) Prinzmetal angina (D) Stable angina pectoris (E) Unstable angina pectoris The correct answer is (D) Stable angina pectoris. Here's why: * Stable angina pectoris is characterized by chest pain that occurs predictably with exertion (like climbing stairs or jogging) and is relieved by rest. This patient's symptoms fit this description perfectly. Let's look at why the other options are incorrect: * (A) Arrhythmia: While arrhythmias can cause chest pain, it's not typically associated with exertion and relief with rest. * (B) Myocardial infarction (MI): is a more serious condition that causes prolonged and severe chest pain, often not relieved by rest. This patient's symptoms are more consistent with a less severe condition. * (C) Prinzmetal angina: This is a rare form of angina that typically occurs at rest and is often triggered by coronary artery spasms. The patient's pain is associated with exertion, making this less likely. * (E) Unstable angina pectoris: Unstable angina is characterized by chest pain that occurs more frequently, is more intense, and may happen at rest. This patient's symptoms are more consistent with stable angina. Cardiac Conditions Condition Key Cause Key Pathology Key Clinical Manifestation Arrhythmia Irregular heart Disruption of the Palpitations, rhythm due to heart's electrical dizziness, fainting, abnormal electrical system. and sometimes activity. chest pain. Myocardial Complete or near- Myocardial cell Severe, crushing Infarction (MI) complete blockage death (necrosis) in chest pain often of a coronary artery, the area supplied by radiating to the jaw, often caused by a the blocked artery, left arm, or back, blood clot forming leading to scar shortness of breath, on an tissue formation. nausea, sweating. atherosclerotic plaque. Prinzmetal Angina Spasms of the Temporary Severe chest pain coronary arteries, narrowing of the that often occurs at often triggered by coronary arteries, rest or during sleep, cold temperatures, leading to reduced sometimes  smoking, or blood flow. associated with ECG emotional stress. changes (ST elevation). Stable Angina Atherosclerosis Narrowing of Chest pain that Pectoris (narrowing of coronary arteries. occurs with coronary arteries), exertion, typically leading to reduced relieved by rest or blood flow during nitroglycerin, exertion. usually predictable in terms of intensity and onset. Unstable Angina Unstable plaque or Reduced blood flow, New, worsening Pectoris severe blockage in a possibly associated chest pain, possibly coronary artery, with ECG changes. occurring at rest, similar to stable potentially angina but more associated with ECG severe. changes (ST segment depression or T wave inversion).  Vascular Pathology 7 VASCULITIS I. BASIC PRINCIPLES A. Inflammation of the blood vessel wall 1. Arterial wall is comprised of three layers: endothelial intima, smooth muscle media, and connective tissue adventitia (Fig. 7.1). B. Etiology is usually unknown; most cases are not infectious. C. Clinical features include 1. Nonspecific symptoms of inflammation (e.g., fever, fatigue, weight loss, and myalgias) 2. Symptoms of organ ischemia - due to luminal narrowing or thrombosis of the inflamed vessels D. Divided into large-, medium-, and small-vessel vasculitides 1. Large-vessel vasculitis involves the aorta and its major branches. 2. Medium-vessel vasculitis involves muscular arteries that supply organs. 3. Small-vessel vasculitis involves arterioles, capillaries, and venules. II. LARGE-VESSEL VASCULITIS A. Temporal (Giant Cell) Arteritis 1. Granulomatous vasculitis that classically involves branches of the carotid artery 2. Most common form of vasculitis in older adults (> 50 years); usually affects females 3. Presents as headache (temporal artery involvement), visual disturbances (ophthalmic artery involvement), and jaw claudication. Flu-like symptoms with joint and muscle pain (polymyalgia rheumatica) are often present. ESR is elevated. 4. Biopsy reveals inflamed vessel wall with giant cells and intimal fibrosis (Fig. 7.2). i. Lesions are segmental; diagnosis requires biopsy of a long segment of vessel, and a negative biopsy does not exclude disease. 5. Treatment is corticosteroids; high risk of blindness without treatment B. Takayasu Arteritis 1. Granulomatous vasculitis that classically involves the aortic arch at branch points 2. Presents in adults < 50 years old (classically, young Asian females) as visual and neurologic symptoms with a weak or absent pulse in the upper extremity ('pulseless disease'). ESR is elevated. 3. Treatment is corticosteroids. III. MEDIUM-VESSEL VASCULITIS A. Polyarteritis Nodosa 1. Necrotizing vasculitis involving multiple organs; lungs are spared. 2. Classically presents in young adults as hypertension (renal artery involvement), abdominal pain with melena (mesenteric artery involvement), neurologic disturbances, and skin lesions. Associated with serum HBsAg 65 »* 9 4 pathoma.com b 'V  66 FUNDAMENTALS OF PATHOLOGY 3. Lesions of varying stages are present. Early lesion consists of transmural inflammation with fibrinoid necrosis (Fig. 7.3); eventually heals with fibrosis, producing a 'string-of-pearls' appearance on imaging 4. Treatment is corticosteroids and cyclophosphamide; fatal if not treated B. Kawasaki Disease 1. Classically affects Asian children < 4 years old 2. Presents with nonspecific signs including fever, conjunctivitis, erythematous rash of palms and soles, and enlarged cervical lymph nodes 3. Coronary artery involvement is common and leads to risk for (1) thrombosis with myocardial infarction and (2) aneurysm with rupture. 4. Treatment is aspirin and IVIG; disease is self-limited. C. Buerger Disease 1. Necrotizing vasculitis involving digits 2. Presents with ulceration, gangrene, and autoamputation of fingers and toes; Raynaud phenomenon is often present. 3. Highly associated with heavy smoking; treatment is smoking cessation. IV. SMALL-VESSEL VASCULITIS A. Wegener Granulomatosis 1. Necrotizing granulomatous vasculitis involving nasopharynx, lungs, and kidneys 2. Classic presentation is a middle-aged male with sinusitis or nasopharyngeal ulceration, hemoptysis with bilateral nodular lung infiltrates, and hematuria due to rapidly progressive glomerulonephritis. 3. Serum c-ANCA levels correlate with disease activity. 4. Biopsy reveals large necrotizing granulomas with adjacent necrotizing vasculitis (Fig. 7.4). 5. Treatment is cyclophosphamide and steroids; relapses are common. B. Microscopic Polyangiitis 1. Necrotizing vasculitis involving multiple organs, especially lung and kidney 2. Presentation is similar to Wegener granulomatosis, but nasopharyngeal involvement and granulomas are absent. 3. Serum p-ANCA levels correlate with disease activity. 4. Treatment is corticosteroids and cyclophosphamide; relapses are common. C. Churg-Strauss Syndrome 1. Necrotizing granulomatous inflammation with eosinophils involving multiple organs, especially lungs and heart 2. Asthma and peripheral eosinophilia are often present. 3. Serum p-ANCA levels correlate with disease activity. Fig. 7.1 Normal muscular artery. Fig. 7.2 Temporal (giant cell) arteritis. Fig. 7.3 Fibrinoid necrosis, polyarteritis nodosa.  Vascular Pathology 67 D. Henoch-Schönlein Purpura 1. Vasculitis due to IgA immune complex deposition; most common vasculitis in children 2. Presents with palpable purpura on buttocks and legs, GI pain and bleeding, and hematuria (IgA nephropathy); usually occurs following an upper respiratory tract infection 3. Disease is self-limited, but may recur; treated with steroids, if severe HYPERTENSION (HTN) I. BASIC PRINCIPLES A. Increased blood pressure; may involve pulmonary (see chapter 9) or systemic circulation B. Systemic HTN is defined as pressure ≥ 140/90 mm Hg (normal ≤ 120/80 mm Hg); divided into primary or secondary types based on etiology II. PRIMARY HTN A. HTN of unknown etiology (95% of cases) B. Risk factors include age, race (increased risk in African Americans, decreased risk in Asians), obesity, stress, lack of physical activity, and high-salt diet. III. SECONDARY HTN A. HTN due to an identifiable etiology (5% of cases) B. Renal artery stenosis is a common cause (renovascular hypertension). 1. Stenosis decreases blood flow to glomerulus. 2. Juxtaglomerular apparatus (JGA) responds by secreting renin, which converts angiotensinogen to angiotensin I. 3. Angiotensin I is converted to angiotensin II (ATII) by angiotensin converting enzyme (ACE). 4. ATII raises blood pressure by (1) contracting arteriolar smooth muscle, increasing total peripheral resistance and (2) promoting adrenal release of aldosterone, which increases resorption of sodium in the distal convoluted tubule (expanding plasma volume). 5. Leads to HTN with increased plasma renin and unilateral atrophy (due to low blood flow) of the affected kidney; neither feature is seen in primary hypertension. C. Important causes of stenosis include atherosclerosis (elderly males) and fibromuscular dysplasia (young females). A Fig. 7.4 Wegener granulomatosis. A, Necrotizing granuloma. B, Giant cells at edge of granuloma. Fig. 7.5 Atherosclerosis.  68 4 FUNDAMENTALS OF PATHOLOGY 1. Fibromuscular dysplasia is a developmental defect of the blood vessel wall, resulting in irregular thickening of large- and medium-sized arteries, especially the renal artery. IV. BENIGN AND MALIGNANT HTN A. HTN can also be classified as benign or malignant. B. Benign HTN is a mild or moderate elevation in blood pressure; most cases of HTN are benign. 1. Clinically silent; vessels and organs are damaged slowly over time. C. Malignant HTN is severe elevation in blood pressure ( > 180/120 mm Hg); comprises < 5% of cases 1. May arise from preexisting benign HTN or de novo 2. Presents with acute end-organ damage (e.g., acute renal failure, headache, and papilledema) and is a medical emergency ARTERIOSCLEROSIS I. BASIC PRINCIPLES A. Literally, "hard arteries;" due to thickening of the blood vessel wall B. Three pathologic patterns - atherosclerosis, arteriolosclerosis, and Mönckeberg medial calcific sclerosis II. ATHEROSCLEROSIS A. Intimal plaque that obstructs blood flow 1. Consists of a necrotic lipid core (mostly cholesterol) with a fibromuscular cap (Fig. 7.5); often undergoes dystrophic calcification B. Involves large- and medium-sized arteries; abdominal aorta, coronary artery, popliteal artery, and internal carotid artery are commonly affected. C. Risk factors for atherosclerosis are divided into modifiable and nonmodifiable. 1. Modifiable risk factors include hypertension, hypercholesterolemia (LDL increases risk; HDL decreases risk), smoking, and diabetes. 2. Nonmodifiable risk factors include age (number and severity of lesions increase with age), gender (increased risk in males and postmenopausal females; estrogen is protective), and genetics (multifactorial, but family history is highly predictive of risk). D. Pathogenesis 1. Damage to endothelium allows lipids to leak into the intima. 2. Lipids are oxidized and then consumed by macrophages via scavenger receptors, resulting in foam cells. Fig. 7.6 Atherosclerotic embolus. (Courtesy of Fig. 7.7 Hyaline arteriolosclerosis. Fig. 7.8 Arteriolonephrosclerosis. (Courtesy of Professor A. Garfia) Jerome Taxy, MD)  Vascular Pathology 69 3. Inflammation and healing leads to deposition of extracellular matrix and proliferation of smooth muscle. E. Morphologic stages 1. Begins as fatty streaks (flat yellow lesions of the intima consisting of lipid-laden macrophages); arise early in life (present in most teenagers) 2. Progresses to atherosclerotic plaque F. Complications of atherosclerosis account for> 50% of disease in Western countries. 1. Stenosis of medium-sized vessels results in impaired blood flow and ischemia leading to i. Peripheral vascular disease (lower extremity arteries, e.g., popliteal) ii. Angina (coronary arteries) iii. Ischemic bowel disease (mesenteric arteries) 2. Plaque rupture with thrombosis results in myocardial infarction (coronary arteries) and stroke (e.g., middle cerebral artery). 3. Plaque rupture with embolization results in atherosclerotic emboli, characterized by cholesterol crystals within the embolus (Fig. 7.6). 4. Weakening of vessel wall results in aneurysm (e.g., abdominal aorta). Ill. ARTERIOLOSCLEROSIS A. Narrowing of small arterioles; divided into hyaline and hyperplastic types B. Hyaline arteriolosclerosis is caused by proteins leaking into the vessel wall, producing vascular thickening; proteins are seen as pink hyaline on microscopy (Fig. 7.7). 1. Consequence of long-standing benign hypertension or diabetes 2. Results in reduced vessel caliber with end-organ ischemia; classically produces glomerular scarring (arteriolonephrosclerosis, Fig. 7.8) that slowly progresses to chronic renal failure C. Hyperplastic arteriolosclerosis involves thickening of vessel wall by hyperplasia of smooth muscle ('onion-skin' appearance, Fig. 7.9). 1. Consequence of malignant hypertension 2. Results in reduced vessel caliber with end-organ ischemia 3. May lead to fibrinoid necrosis of the vessel wall with hemorrhage; classically causes acute renal failure with a characteristic 'flea-bitten' appearance IV. MÖNCKEBERG MEDIAL CALCIFIC SCLEROSIS A. Calcification of the media of muscular (medium-sized) arteries; nonobstructive (Fig. 7.10A) B. Not clinically significant; seen as an incidental finding on x-ray or mammography (Fig. 7.10B) Fig. 7.9 Hyperplastic arteriolosclerosis. Fig. 7.10 Mönckeberg medial calcific sclerosis. A, Microscopic appearance. B, Mammogram.  70 FUNDAMENTALS OF PATHOLOGY AORTIC DISSECTION AND ANEURYSM I. AORTIC DISSECTION A. Intimal tear with dissection of blood through media of the aortic wall (Fig. 7.11) B. Occurs in the proximal 10 cm of the aorta (high stress region) with preexisting weakness of the media C. Most common cause is hypertension (older adults); also associated with inherited defects of connective tissue (younger individuals) 1. Hypertension results in hyaline arteriolosclerosis of the vasa vasorum; decreased flow causes atrophy of the media. 2. Marfan syndrome and Ehlers-Danlos syndrome classically lead to weakness of the connective tissue in the media (cystic medial necrosis). D. Presents as sharp, tearing chest pain that radiates to the back E. Complications include pericardial tamponade (most common cause of death), rupture with fatal hemorrhage, and obstruction of branching arteries (e.g., coronary or renal) with resultant end-organ ischemia. II. THORACIC ANEURYSM A. Balloon-like dilation of the thoracic aorta B. Due to weakness in the aortic wall. Classically seen in tertiary syphilis; endarteritis of the vasa vasorum results in luminal narrowing, decreased flow, and atrophy of the vessel wall. Results in a 'tree-bark' appearance of the aorta (Fig. 7.12) C. Major complication is dilation of the aortic valve root, resulting in aortic valve insufficiency. 1. Other complications include compression of mediastinal structures (e.g., airway or esophagus) and thrombosis/embolism. III. ABDOMINAL AORTIC ANEURYSM A. Balloon-like dilation of the abdominal aorta; usually arises below the renal arteries, but above the aortic bifurcation (Fig. 7.13) B. Primarily due to atherosclerosis; classically seen in male smokers > 60 years old with hypertension 1. Atherosclerosis increases the diffusion barrier to the media, resulting in atrophy and weakness of the vessel wall. C. Presents as a pulsatile abdominal mass that grows with time D. Major complication is rupture, especially when > 5 cm in diameter; presents with triad of hypotension, pulsatile abdominal mass, and flank pain 1. Other complications include compression of local structures (e.g., ureter) and thrombosis/embolism. Fig. 7.11 Aortic dissection. (Courtesy of humpath. Fig. 7.12 'Tree-bark' appearance of aorta due to Fig, 7.13 Abdominal aortic aneurysm. com) syphilis. (Courtesy of Aliya Husain, MD) (Courtesy of Aliya Husain, MD)  Vascular Pathology 71 VASCULAR TUMORS I. HEMANGIOMA A. Benign tumor comprised of blood vessels (Fig. 7.14) B. Commonly present at birth; often regresses during childhood C. Most often involves skin and liver II. ANGIOSARCOMA A. Malignant proliferation of endothelial cells; highly aggressive B. Common sites include skin, breast, and liver. 1. Liver angiosarcoma is associated with exposure to polyvinyl chloride, arsenic, and Thorotrast. III. KAPOSI SARCOMA A. Low-grade malignant proliferation of endothelial cells; associated with HHV-8 B. Presents as purple patches, plaques, and nodules on the skin (Fig. 7.15); may also involve visceral organs C. Classically seen in 1. Older Eastern European males - tumor remains localized to skin; treatment involves surgical removal. 2. AIDS - tumor spreads early; treatment is antiretroviral agents (to boost immune system). 3. Transplant recipients - tumor spreads early; treatment involves decreasing immunosuppression. Fig. 7.14 Hemangioma. Fig. 7.15 Kaposi sarcoma. MBBS Gang MBBS GANG  Cardiac Pathology 8 ISCHEMIC HEART DISEASE (IHD) I. BASIC PRINCIPLES A. Group of syndromes related to myocardial ischemia; IHD is the leading cause of death in the US. B. Usually due to atherosclerosis of coronary arteries, which decreases blood flow to the myocardium 1. Risk factors for IHD are similar to those of atherosclerosis; incidence increases with age. II. ANGINA A. Stable angina is chest pain that arises with exertion or emotional stress. 1. Due to atherosclerosis of coronary arteries with > 70% stenosis; decreased blood flow is not able to meet the metabolic demands of the myocardium during exertion. 2. Represents reversible injury to myocytes (no necrosis) 3. Presents as chest pain (lasting < 20 minutes) that radiates to the left arm or jaw, diaphoresis, and shortness of breath 4. EKG shows ST-segment depression due to subendocardial ischemia. 5. Relieved by rest or nitroglycerin B. Unstable angina is chest pain that occurs at rest. 1. Usually due to rupture of an atherosclerotic plaque with thrombosis and incomplete occlusion of a coronary artery (Fig. 8.1A). 2. Represents reversible injury to myocytes (no necrosis) 3. EKG shows ST-segment depression due to subendocardial ischemia. 4. Relieved by nitroglycerin 5. High risk of progression to myocardial infarction C. Prinzmetal angina is episodic chest pain unrelated to exertion. 1. Due to coronary artery vasospasm 2. Represents reversible injury to myocytes (no necrosis) 3. EKG shows ST-segment elevation due to transmural ischemia. 4. Relieved by nitroglycerin or calcium channel blockers III. MYOCARDIAL INFARCTION A. Necrosis of cardiac myocytes B. Usually due to rupture of an atherosclerotic plaque with thrombosis and complete occlusion of a coronary artery (Fig. 8.1B) 1. Other causes include coronary artery vasospasm (due to Prinzmetal angina or cocaine use), emboli, and vasculitis (e.g., Kawasaki disease). C. Clinical features include severe, crushing chest pain (lasting > 20 minutes) that radiates to the left arm or jaw, diaphoresis, and dyspnea; symptoms are not relieved by nitroglycerin. D. Infarction usually involves the left ventricle (LV); right ventricle (RV) and both atria are generally spared. pathoma.com 73  74 FUNDAMENTALS OF PATHOLOGY 1. Occlusion of left anterior descending artery (LAD) leads to infarction of the anterior wall and anterior septum of the LV; LAD is the most commonly involved artery in MI (45% of cases). 2. Occlusion of right coronary artery (RCA) leads to infarction of the posterior wall, posterior septum, and papillary muscles of the LV; RCA is the 2nd most commonly involved artery in MI. 3. Occlusion of left circumflex artery leads to infarction of lateral wall of the LV. E. Initial phase of infarction leads to subendocardial necrosis involving < 50% of the myocardial thickness (subendocardial infarction, Fig. 8.2); EKG shows ST-segment depression. 1. Continued or severe ischemia leads to transmural necrosis involving most of the myocardial wall (transmural infarction); EKG shows ST-segment elevation. F. Laboratory tests detect elevated cardiac enzymes. 1. Troponin I is the most sensitive and specific marker (gold standard) for Ml. Levels rise 2-4 hours after infarction, peak at 24 hours, and return to normal by 7-10 days. 2. CK-MB is useful for detecting reinfarction that occurs days after an initial MI; creatine kinase MB (CK-MB) levels rise 4-6 hours after infarction, peak at 24 hours, and return to normal by 72 hours. G. Treatment includes 1. Aspirin and/or heparin - limits thrombosis 2. Supplemental O2 - minimizes ischemia 3. Nitrates - Vasodilate veins and coronary arteries 4. β- blocker - slows heart rate, decreasing O2 demand and risk for arrhythmia 5. ACE inhibitor - decreases LV dilation 6. Fibrinolysis or angioplasty - opens blocked vessel i. Reperfusion of irreversibly - damaged cells results in calcium influx, leading to hypercontraction of myofibrils (contraction band necrosis, Fig. 8.3). ii. Return of oxygen and inflammatory cells may lead to free radical generation, further damaging myocytes (reperfusion injury). H. Complications of myocardial infarction are closely related to gross and microscopic changes (Table 8.1). Table 8.1: Morphologic Changes in Myocardial Infarction TIME FROM GROSS CHANGES MICROSCOPIC CHANGES COMPLICATIONS INFARCTION Cardiogenic shock (massive infarction), V < s L * 1 ’ W * Fig. 8.17C Splinter hemorrhages. Fig. 8.17D Roth spots. Fig. 8.18 Dilated cardiomyopathy. (Courtesy of Jamie Steinmetz, MD)  Cardiac Pathology 83 II. DILATED CARDIOMYOPATHY A. Dilation of all four chambers of the heart (Fig. 8.18); most common form of cardiomyopathy B. Results in systolic dysfunction (ventricles cannot pump), leading to biventricular CHF; complications include mitral and tricuspid valve regurgitation and arrhythmia. C. Most commonly idiopathic; other causes include 1. Genetic mutation (usually autosomal dominant) 2. Myocarditis (usually due to coxsackie A or B) - characterized by a lymphocytic infiltrate in the myocardium (Fig. 8.19); results in chest pain, arrhythmia with sudden death, or heart failure. Dilated cardiomyopathy is a late complication. 3. Alcohol abuse 4. Drugs (e.g., doxorubicin) 5. Pregnancy - seen during late pregnancy or soon (weeks to months) after childbirth 6. Hemochromatosis D. Treatment is heart transplant. III. HYPERTROPHIC CARDIOMYOPATHY A. Massive hypertrophy ofthe left ventricle B. Usually due to genetic mutations in sarcomere proteins; most common form is autosomal dominant. C. Clinical features include 1. Decreased cardiac output - Left ventricular hypertrophy leads to diastolic dysfunction (ventricle cannot fill). 2. Sudden death due to ventricular arrhythmias; hypertrophic cardiomyopathy is a common cause of sudden death in young athletes. 3. Syncope with exercise - Subaortic hypertrophy ofthe ventricular septum results in functional aortic stenosis. D. Biopsy shows myofiber hypertrophy with disarray (Fig. 8.20). IV. RESTRICTIVE CARDIOMYOPATHY A. Decreased compliance of the ventricular endomyocardium that restricts filling during diastole B. Causes include amyloidosis, sarcoidosis, endocardial fibroelastosis (children, Fig. 8.21), and Loeffler syndrome (endomyocardial fibrosis with an eosinophilic infiltrate and eosinophilia). C. Presents as congestive heart failure; classic finding is low-voltage EKG with diminished QRS amplitude. Fig. 8.19 Myocarditis. Fig. 8.20 Myofiber disarray, hypertrophic Fig. 8.21 Endocardial fibroelastosis. {Courtesy cardiomyopathy. of humpath.com) 84 FUNDAMENTALS OF PATHOLOGY CARDIAC TUMORS I. MYXOMA A. Benign mesenchymal tumor with a gelatinous appearance and abundant ground substance on histology 1. Most common primary cardiac tumor in adults B. Usually forms a pedunculated mass in the left atrium that causes syncope due to obstruction of the mitral valve II. RHABDOMYOMA A. Benign hamartoma of cardiac muscle 1. Most common primary cardiac tumor in children; associated with tuberous sclerosis B. Usually arises in the ventricle III. METASTASIS A. Metastatic tumors are more common in the heart than primary tumors. 1. Common metastases to the heart include breast and lung carcinoma, melanoma, and lymphoma. B. Most commonly involve the pericardium, resulting in a pericardial effusion This page intentionally left blank  11 Blood Vessels PBD7 and PBD8 Chapter 11: Blood Vessels BP7 and BP8 Chapter 10: Blood Vessels 1 The development of atheromatous plaque formation with subsequent complications is observed in an experiment. Atherosclerotic plaques are shown to change slowly but con- stantly in ways that can promote clinical events, including acute coronary syndromes. In some cases, changes occurred that were not significantly associated with acute coronary syn- dromes. Which of the following plaque alterations is most likely to have such an association?  (A) Thinning of the media  (B) Ulceration of the plaque surface  (C) Thrombosis  (D) Hemorrhage into the plaque substance  (E) Intermittent platelet aggregation 2 A 60-year-old woman has reported increasing fatigue over the past year. Laboratory studies show a serum creatinine level of 4.7 mg/dL and urea nitrogen level of 44 mg/dL. An abdominal ultrasound scan shows that her kidneys are sym- metrically smaller than normal. The high-magnification microscopic appearance of the kidneys is shown in the figure. These findings are most likely to indicate which of the follow- ing underlying conditions?  (A) Escherichia coli septicemia  (B) Systemic hypertension  (C) Adenocarcinoma of the colon  (D) Tertiary syphilis  (E) Polyarteritis nodosa 133 134 UNIT II Systemic Pathology: Diseases of Organ Systems 3 A 55-year-old woman visits her physician for a routine 6 A 61-year-old man had a myocardial infarction 1 year health maintenance examination. On physical examination, ago, which was the first major illness in his life. He now wants her temperature is 36.8°C, pulse is 70/min, respirations are to prevent another myocardial infarction and is advised to 14/min, and blood pressure is 160/105 mm Hg. Her lungs are begin a program of exercise and to change his diet. A reduc- clear on auscultation, and her heart rate is regular. She feels tion in the level of which of the following serum laboratory fine and has had no major medical illnesses or surgical proce- findings 1 year later would best indicate the success of this diet dures during her lifetime. An abdominal ultrasound scan and exercise regimen? shows that the left kidney is smaller than the right kidney. A  (A) Cholesterol renal angiogram shows a focal stenosis of the left renal artery.  (B) Glucose Which of the following laboratory findings is most likely to  (C) Potassium be present in this patient?  (D) Renin  (A) Anti–double-stranded DNA titer 1 : 512  (E) Calcium  (B) C-ANCA titer 1 : 256  (C) Cryoglobulinemia  (D) Plasma glucose level 200 mg/dL  (E) HIV test positive  (F) Plasma renin 15 mg/mL/hr  (G) Serologic test for syphilis positive 4 A 7-year-old child has had abdominal pain and dark urine for 10 days. Physical examination shows purpuric skin lesions on the trunk and extremities. Urinalysis shows hema- turia and proteinuria. Serologic test results are negative for P-ANCAs and C-ANCAs. A skin biopsy specimen shows nec- * rotizing vasculitis of small dermal vessels. A renal biopsy specimen shows immune complex deposition in glomeruli, * with some IgA-rich immune complexes. Which of the follow- ing is the most likely diagnosis?  (A) Giant cell arteritis  (B) Henoch-Schönlein purpura  (C) Polyarteritis nodosa  (D) Takayasu arteritis  (E) Telangiectasias  (F) Wegener granulomatosis 7 A 23-year-old man experiences sudden onset of severe, 5 A 30-year-old woman has had coldness and numbness sharp chest pain. On physical examination, his temperature is in her arms and decreased vision in the right eye for the past 36.9°C, and his lungs are clear on auscultation. A chest radio- 5 months. On physical examination, she is afebrile. Her blood graph shows a widened mediastinum. Transesophageal echo- pressure is 100/70 mm Hg. Radial pulses are not palpable, but cardiography shows a dilated aortic root and arch, with a tear femoral pulses are strong. She has decreased sensation and in the aortic intima 2 cm distal to the great vessels. The rep- cyanosis in her arms, but no warmth or swelling. A chest resentative microscopic appearance of the aorta with elastic radiograph shows a prominent border on the right side of the stain is shown in the figure. Which of the following is the most heart and prominence of the pulmonary arteries. Laboratory likely cause of these findings? studies show serum glucose, 74 mg/dL; creatinine, 1 mg/dL;  (A) Scleroderma total serum cholesterol, 165 mg/dL; and negative ANA test  (B) Diabetes mellitus result. Her condition remains stable for the next year. Which  (C) Systemic hypertension of the following is the most likely diagnosis?  (D) Marfan syndrome  (A) Aortic dissection  (E) Wegener granulomatosis  (B) Kawasaki disease  (F) Takayasu arteritis  (C) Microscopic polyangiitis  (D) Takayasu arteritis  (E) Tertiary syphilis  (F) Thromboangiitis obliterans  CHAPTER 11 Blood Vessels 135 outer left arm. The parents state that this lesion has been present since infancy. The lesion is excised, and its micro- scopic appearance is shown in the figure. Which of the fol- lowing is the most likely diagnosis?  (A) Kaposi sarcoma  (B) Angiosarcoma  (C) Lymphangioma  (D) Telangiectasia  (E) Hemangioma 10 A pharmaceutical company is developing an antiathero- sclerosis agent. An experiment investigates mechanisms of action of several potential drugs to determine their efficacy in reducing atheroma formation. Which of the following mecha- nisms of action is likely to have the most effective antiathero- sclerotic effect?  (A) Inhibits PDGF/Inhibits macrophage-mediated lipoprotein oxidation  (B) Inhibits PDGF/Promotes macrophage-mediated 8 A 40-year-old man with a history of diabetes mellitus lipoprotein oxidation has had worsening abdominal pain for the past week. On  (C) Promotes PDGF/Promotes macrophage-mediated physical examination, his vital signs are temperature, 36.9°C; lipoprotein oxidation pulse, 77/min; respirations, 16/min; and blood pressure,  (D) Decreases HDL/Inhibits macrophage-mediated 140/90 mm Hg. An abdominal CT scan shows the findings in lipoprotein oxidation the figure. Laboratory studies show his hemoglobin A1C is  (E) Increases HDL/Promotes macrophage-mediated 10.5%. Which of the following is the most likely underlying lipoprotein oxidation disease process in this patient?  (F) Decreases ICAM-1/Promotes macrophage-  (A) Polyarteritis nodosa mediated lipoprotein oxidation  (B) Obesity  (G) Increases ICAM-1/Inhibits macrophage-mediated  (C) Diabetes mellitus lipoprotein oxidation  (D) Systemic lupus erythematosus  (E) Syphilis 11 A 73-year-old man who has had progressive dementia for the past 6 years dies of bronchopneumonia. Autopsy shows that the thoracic aorta has a dilated root and arch, giving the intimal surface a “tree-bark” appearance. Microscopic exami- nation of the aorta shows an obliterative endarteritis of the vasa vasorum. Which of the following laboratory findings is most likely to be recorded in this patient’s medical history?  (A) High double-stranded DNA titer  (B) P-ANCA positive 1 : 1024  (C) Sedimentation rate 105 mm/hr  (D) Ketonuria 4+  (E) Antibodies against Treponema pallidum 12 For the past 3 weeks, a 70-year-old woman has been bedridden while recuperating from a bout of viral pneumonia complicated by bacterial pneumonia. Physical examination now shows some swelling and tenderness of the right leg, which worsens when she raises or moves the leg. Which of the following terms best describes the condition involving the Courtesy of Tom Rogers, MD, Department of Pathology, patient’s right leg? University of Texas Southwestern Medical School, Dallas, TX.  (A) Lymphedema  (B) Disseminated intravascular coagulopathy 9 A 10-year-old boy is brought to the physician for a  (C) Thrombophlebitis routine health maintenance examination. The physician notes  (D) Thromboangiitis obliterans a 2-cm spongy, dull red, circumscribed lesion on the upper  (E) Varicose veins 136 UNIT II Systemic Pathology: Diseases of Organ Systems 13 A 49-year-old man is feeling well when he visits his physician for a routine health maintenance examination for the first time in 20 years. On physical examination, his vital signs are temperature, 37°C; pulse, 73/min; respirations, 14/min; and blood pressure, 155/95 mm Hg. He has had no serious medical problems and takes no medications. Which of the following is most likely to be the primary factor in this patient’s hypertension?  (A) Increased catecholamine secretion  (B) Renal retention of excess sodium  (C) Gene defects in aldosterone metabolism  (D) Renal artery stenosis  (E) Increased production of atrial natriuretic factor 14 A 50-year-old man has a 2-year history of angina pec- toris that occurs during exercise. On physical examination, his 17 A 35-year-old man is known to have been HIV-positive blood pressure is 135/75 mm Hg, and his heart rate is 79/min for the past 10 years. Physical examination shows several skin and slightly irregular. Coronary angiography shows a fixed lesions with the appearance shown in the figure. These lesions 75% narrowing of the anterior descending branch of the left have been slowly increasing for the past year. Which of the coronary artery. Which of the following types of cells is the following infectious agents is most likely to play a role in the initial target in the pathogenesis of this arterial lesion? development of these skin lesions?  (A) Monocytes  (A) Human herpesvirus-8  (B) Smooth muscle cells  (B) Epstein-Barr virus  (C) Platelets  (C) Cytomegalovirus  (D) Neutrophils  (D) Hepatitis B virus  (E) Endothelial cells  (E) Adenovirus 18 A 50-year-old man complains of a chronic cough that has persisted for the past 18 months. Physical examination 15 A study of atheroma formation leading to atheroscle- shows nasopharyngeal ulcers, and the lungs have diffuse rotic complications evaluates potential risk factors for rele- crackles bilaterally on auscultation. Laboratory studies include vance in a population. Three factors are found to play a a serum urea nitrogen level of 75 mg/dL and a creatinine significant role in the causation of atherosclerosis: smoking, concentration of 6.7 mg/dL. Urinalysis shows 50 RBCs per hypertension, and hypercholesterolemia. These factors are high-power field and RBC casts. His serologic titer for C- analyzed for their relationship to experimental models for ANCA is elevated. A chest radiograph shows multiple, small, atherogenesis. Which of the following events is the most bilateral pulmonary nodules. A nasal biopsy specimen shows important direct biologic consequence of these factors? mucosal and submucosal necrosis and necrotizing granulo-  (A) Endothelial injury and its sequelae matous inflammation. A transbronchial lung biopsy specimen  (B) Conversion of smooth muscle cells to foam cells shows a vasculitis involving the small peripheral pulmonary  (C) Alterations of hepatic lipoprotein receptors arteries and arterioles. Granulomatous inflammation is seen  (D) Inhibition of LDL oxidation within and adjacent to small arterioles. Which of the following  (E) Alterations of endogenous factors regulating is the most likely diagnosis? vasomotor tone  (A) Fibromuscular dysplasia  (B) Glomus tumors  (C) Granuloma pyogenicum 16 A 55-year-old woman has noted the increasing promi-  (D) Hemangiomas nence of unsightly dilated superficial veins over both lower  (E) Kaposi sarcoma legs for the past 5 years. Physical examination shows tempera-  (F) Polyarteritis nodosa ture of 37°C, pulse of 70/min, respirations of 14/min, and  (G) Takayasu arteritis blood pressure of 125/85 mm Hg. There is no pain, swelling,  (H) Wegener granulomatosis or tenderness in either lower leg. Which of the following 19 While cleaning debris out of the gate in an irrigation complications is most likely to occur as a consequence of this canal, a 50-year-old man cuts his right index finger on a sharp condition? metal shard. The cut stops bleeding within 3 minutes, but 6  (A) Stasis dermatitis hours later he notes increasing pain in the right arm and goes  (B) Gangrenous necrosis of the lower legs to his physician. On physical examination, his temperature is  (C) Pulmonary thromboembolism 38°C. Red streaks extend from the right hand to the upper  (D) Disseminated intravascular coagulation arm, and the arm is swollen and tender when palpated. Mul-  (E) Atrophy of the lower leg muscles tiple tender lumps are noted in the right axilla. A blood culture  CHAPTER 11 Blood Vessels 137 grows group A hemolytic streptococci. Which of the following  (A) Cap of smooth muscle cells overlying a core of terms best describes the process that is occurring in this lipid debris patient’s right arm?  (B) Collection of foam cells with necrosis and  (A) Capillaritis calcification  (B) Lymphangitis  (C) Granulation tissue with a lipid core and areas of  (C) Lymphedema hemorrhage  (D) Phlebothrombosis  (D) Lipid-filled foam cells and small numbers of T  (E) Polyarteritis nodosa lymphocytes  (F) Thrombophlebitis  (E) Cholesterol clefts surrounded by proliferating  (G) Varices smooth muscle cells and foam cells 20 An experiment studies early atheromas. Lipid streaks on arterial walls are examined microscopically and biochemically to determine their cellular and chemical constituents and the factors promoting their formation. Early lesions show increased attachment of monocytes to endothelium. The monocytes migrate subendothelially and become macro- phages; these macrophages transform themselves into foam cells. Which of the following is most likely to produce these effects?  (A) C-reactive protein  (B) Homocysteine  (C) Lp(a)  (D) Oxidized LDL  (E) Platelet-derived growth factor Courtesy of Tom Rogers, MD, Department of Pathology,  (F) VLDL University of Texas Southwestern Medical School, Dallas, TX. 22 A 59-year-old man has experienced chest pain at rest for the past year. On physical examination, his pulse is 80/min and irregular. The figure shows the microscopic appearance repre- sentative of the patient’s left anterior descending artery. Which of the following laboratory findings is most likely to have a causal relationship to the process illustrated?  (A) Low Lp(a)  (B) Positive VDRL  (C) Low HDL cholesterol  (D) Elevated platelet count  (E) Low plasma homocysteine 23 After falling down a flight of stairs, a 59-year-old woman experiences mild intermittent right hip pain. Physical exami- nation shows a 3-cm contusion over the right hip. The area is tender to palpation, but she has full range of motion of the right leg. A radiograph of the pelvis and right upper leg shows no fractures, but does show calcified, medium-sized arterial branches in the pelvis. This radiographic finding is most likely to represent which of the following?  (A) Long-standing diabetes mellitus From the teaching collection of the Department of Pathology,  (B) Benign essential hypertension University of Texas Southwestern Medical School, Dallas, TX.  (C) An incidental observation  (D) Increased risk for gangrenous necrosis  (E) Unsuspected hyperparathyroidism 24 For more than a decade, a 45-year-old man has had 21 A 12-year-old boy died of complications of acute lym- poorly controlled hypertension ranging from 150/90 mm Hg phocytic leukemia. The gross appearance of the aorta at to 160/95 mm Hg. Over the past 3 months, his blood pressure autopsy is shown in the figure. Histologic examination of the has increased to 250/125 mm Hg. On physical examination, linear pale marking is most likely to show which of the fol- his temperature is 36.9°C. His lungs are clear on auscultation, lowing features? and his heart rate is regular. There is no abdominal pain on 138 UNIT II Systemic Pathology: Diseases of Organ Systems palpation. A chest radiograph shows a prominent border on 28 A 61-year-old man has smoked two packs of cigarettes the left side of the heart. Laboratory studies show that his per day for the past 40 years. He has experienced increasing serum creatinine level has increased during this time from dyspnea for the past 6 years. On physical examination, his vital 1.7 mg/dL to 3.8 mg/dL. Which of the following vascular signs are temperature, 37.1°C; pulse, 60/min; respirations, 18/ lesions is most likely to be found in this patient’s kidneys? min and labored; and blood pressure, 130/80 mm Hg. On aus-  (A) Hyperplastic arteriolosclerosis cultation, expiratory wheezes are heard over the chest bilater-  (B) Granulomatous arteritis ally. His heart rate is regular. A chest radiograph shows  (C) Fibromuscular dysplasia increased lung volume, with flattening of the diaphragms,  (D) Polyarteritis nodosa greater lucency to all lung fields, prominence of pulmonary  (E) Hyaline arteriolosclerosis arteries, and a prominent border on the right side of the heart. Laboratory studies include blood gas measurements of Po2 of 25 After a mastectomy with axillary node dissection for 80 mm Hg, Pco2 of 50 mm Hg, and pH of 7.35. He dies of breast cancer 1 year ago, a 47-year-old woman has developed pneumonia. At autopsy, the pulmonary arteries have athero- persistent swelling and puffiness in the left arm. Physical matous plaques. Which of the following is most likely to have examination shows firm skin over the left arm and “doughy” caused these findings? underlying soft tissue. The arm is not painful or discolored.  (A) Chronic renal failure She developed cellulitis in the left arm 3 months ago. Which  (B) Coronary atherosclerosis of the following terms best describes these findings?  (C) Cystic fibrosis  (A) Thrombophlebitis  (D) Diabetes mellitus  (B) Subclavian arterial thrombosis  (E) Familial hypercholesterolemia  (C) Tumor embolization  (F) Obesity  (D) Lymphedema  (G) Phlebothrombosis  (E) Vasculitis  (H) Pulmonary emphysema 29 A 75-year-old man has experienced headaches for the 26 A study is conducted to investigate the pathogenesis of past 2 months. On physical examination, his vital signs are atherosclerosis. The investigators have developed genetically temperature, 36.8°C; pulse, 68/min; respirations, 15/min; and modified mice that have hypercholesterolemia and spontane- blood pressure, 130/85 mm Hg. His right temporal artery is ously develop atherosclerosis. Next, the investigators selec- prominent, palpable, and painful to the touch. His heart rate tively delete individual genes to determine the factors that are is regular, and there are no murmurs. A temporal artery biopsy crucial to the development of atherosclerosis. Deletion of the is performed, and the segment of temporal artery excised is gene encoding for which of the following is most likely to grossly thickened and shows focal microscopic granulomatous reduce the experimentally observed atherosclerosis in these inflammation. He responds well to corticosteroid therapy. modified mice? Which of the following complications of this disease is most  (A) Von Willebrand factor likely to occur in untreated patients?  (B) Homocysteine  (A) Renal failure  (C) T-cell receptor  (B) Hemoptysis  (D) Endothelin  (C) Malignant hypertension  (E) Fibrillin  (D) Blindness  (F) LDL receptor  (E) Gangrene of the toes  (G) Factor VIII  (H) Apolipoprotein 30 A 30-year-old woman has smoked one pack of cigarettes per day since she was a teenager. She has had painful throm- 27 An 80-year-old man with a lengthy history of smoking boses of the superficial veins of the lower legs for 1 month and survived a small myocardial infarction several years ago. He episodes during which her fingers become blue and cold. Over now reports chest and leg pain during exercise. On physical the next year, she develops chronic, poorly healing ulcerations examination, his vital signs are temperature, 36.9°C; pulse, of her feet. One toe becomes gangrenous and is amputated. 81/min; respirations, 15/min; and blood pressure, Histologically, at the resection margin, there is an acute and 165/100 mm Hg. Peripheral pulses are poor in the lower chronic vasculitis involving medium-sized arteries, with seg- extremities. There is a 7-cm pulsating mass in the midline of mental involvement. Which of the following is the most the lower abdomen. Laboratory studies include two fasting appropriate next step in treating this patient? serum glucose measurements of 170 mg/dL and 200 mg/dL.  (A) Hemodialysis Which of the following vascular lesions is most likely to be  (B) Smoking cessation present in this patient?  (C) Corticosteroid therapy  (A) Aortic dissection  (D) Antibiotic therapy for syphilis  (B) Arteriovenous fistula  (E) Insulin therapy  (C) Atherosclerotic aneurysm  (D) Glomus tumor 31 A 40-year-old man has experienced malaise, fever, and  (E) Polyarteritis nodosa a 4-kg weight loss over the past month. On physical examina-  (F) Takayasu arteritis tion, his blood pressure is 145/90 mm Hg, and he has mild  (G) Thromboangiitis obliterans diffuse abdominal pain, but no masses or hepatospleno­megaly.  CHAPTER 11 Blood Vessels 139 Laboratory studies include a serum urea nitrogen concentra- They are found to have increased cardiac output and increased tion of 58 mg/dL and a serum creatinine level of 6.7 mg/dL. peripheral vascular resistance. Renal angiograms show no Renal angiography shows right renal arterial thrombosis, and abnormal findings, and CT scans of the abdomen show no the left renal artery and branches show segmental luminal masses. Laboratory studies show normal levels of serum cre- narrowing with focal aneurysmal dilation. During hemodialy- atinine and urea nitrogen. The subjects take no medications. sis 1 week later, the patient experiences abdominal pain and Which of the following laboratory findings is most likely to diarrhea and is found to have melena. Which of the following be present in this group of subjects? serologic laboratory findings is most likely to be positive in  (A) Lack of angiotensin-converting enzyme this patient?  (B) Decreased urinary sodium  (A) C-ANCA  (C) Elevated plasma renin  (B) ANA  (D) Hypokalemia  (C) HIV  (E) Increased urinary catecholamines  (D) HBsAg  (E) Scl-70 36 A 3-year-old child from Osaka, Japan, developed a fever  (F) RPR and a rash and swelling of her hands and feet over 2 days. On physical examination, her temperature is 37.8°C. There is a 32 A 30-year-old schoolteacher is known to be a strict dis- desquamative skin rash, oral erythema, erythema of the palms ciplinarian in the classroom. She has angina pectoris of 6 and soles, edema of the hands and feet, and cervical lymph- months’ duration. On physical examination, her blood pres- adenopathy. The child improves after a course of intravenous sure is 135/85 mm Hg. She is 168 cm (5 ft 5 in) tall and weighs immunoglobulin therapy. Which of the following is most 82 kg (BMI 29). Coronary angiography shows 75% narrowing likely to be a complication of this child’s disease if it is of the anterior descending branch of the left coronary artery. untreated? Angioplasty with stent placement is performed. Which of the following is the major risk factor associated with these  (A) Asthma findings?  (B) Glomerulonephritis  (C) Intracranial hemorrhage  (A) Obesity  (D) Myocardial infarction  (B) Type A personality  (E) Pulmonary hypertension  (C) Diabetes mellitus  (D) Sedentary lifestyle  (E) Age 37 An epidemiologic study seeking to determine possible risk factors for neoplasia is reviewing patient cases of neo- 33 A 46-year-old man visits his physician because he has plasms reported to tumor registries. Analysis of the data shows noted increasing abdominal enlargement over the past 15 that one type of neoplasm is seen in two widely disparate situ- months. Physical examination shows several skin lesions on ations: (1) the liver of individuals exposed to polyvinyl chlo- the upper chest that have central pulsatile cores. Pressing on ride and (2) the soft tissue of the arm ipsilateral to a prior a core causes a radially arranged array of subcutaneous arte- radical mastectomy. The pathology reports about the neo- rioles to blanch. The size of the lesions, from core to periph- plasms in these two groups of patients show a similar gross ery, is 0.5 to 1.5 cm. Laboratory studies show serum glucose appearance—an irregular, infiltrative, soft reddish mass—and of 119 mg/dL, creatinine of 1.1 mg/dL, total protein of 5.8 g/ a similar microscopic appearance—pleomorphic spindle cells dL, and albumin of 3.4 g/dL. Which of the following underly- positive for CD31. Which of the following neoplasms is most ing diseases is most likely to be present in this patient? likely to be described by these findings?  (A) Wegener granulomatosis  (A) Angiosarcoma  (B) Micronodular cirrhosis  (B) Hemangioendothelioma  (C) Marfan syndrome  (C) Hemangioma  (D) AIDS  (D) Hemangiopericytoma  (E) Diabetes mellitus  (E) Kaposi sarcoma  (F) Lymphangioma 34 A 22-year-old woman complains of itching with burning pain in the perianal region for the past 4 months. Physical examination shows dilated and thrombosed external hemor- 38 A cohort study is performed involving healthy adult rhoids. Which of the following underlying processes is most men and women born 20 years ago. They are followed to likely to be present in this patient? assess development of atherosclerotic cardiovascular diseases. Multiple laboratory tests are performed yearly during this  (A) Rectal adenocarcinoma study. An elevation in which of the following test results is  (B) Pregnancy most likely to indicate the greatest relative risk for develop-  (C) Polyarteritis nodosa ment of one of these diseases?  (D) Filariasis  (E) Micronodular cirrhosis  (A) Anti-proteinase 3 (PR3)  (B) C-reactive protein (CRP) 35 A clinical study is performed that includes a group of  (C) Cryoglobulin subjects whose systemic blood pressure measurements are  (D) Erythrocyte sedimentation rate (ESR) consistently between 145/95 mm Hg and 165/105 mm Hg.  (E) Platelet count 140 UNIT II Systemic Pathology: Diseases of Organ Systems 39 A 67-year-old woman with glucose intolerance, hyper-  (A) Angiosarcoma tension, central obesity, and hyperlipidemia has increasing  (B) Bacillary angiomatosis dyspnea from worsening congestive heart failure. Echocar-  (C) Cystic medial degeneration diography shows a left ventricular ejection fraction of 25%.  (D) Giant cell arteritis Percutaneous transluminal coronary angioplasty is performed  (E) Proliferative restenosis with placement of a left anterior descending arterial stent  (F) Thromboangiitis obliterans containing paclitaxel. Which of the following long-term pathologic complications in her coronary artery is this drug- eluting stent most likely to prevent? Answers lis can produce endaortitis and aortic root dilation, but hypertension is not a likely sequela. BP7 339 BP8 355–356 PBD7 526 PBD8 495 1 (A) Atheromatous plaques can be complicated by various pathologic alterations, including hemorrhage, ulceration, 4 (B) In children, Henoch-Schönlein purpura is the mul- thrombosis, and calcification. These processes can increase tisystemic counterpart of the IgA nephropathy seen in adults. the size of the plaque and narrow the residual arterial lumen. The immune complexes formed with IgA produce the vas- Although atherosclerosis is a disease of the intima, in culitis that affects mainly arterioles, capillaries, and venules advanced disease, the expanding plaque compresses the in skin, gastrointestinal tract, and kidney. In older adults, media. This causes thinning of the media, which weakens giant-cell arteritis is seen in external carotid branches, prin- the wall and predisposes it to aneurysm formation. cipally the temporal artery unilaterally. Polyarteritis nodosa BP7 331 BP8 348–351 PBD7 516, 518–519 is seen most often in small muscular arteries and sometimes PBD8 503–504 veins, with necrosis and microaneurysm formation followed by scarring and vascular occlusion. This occurs mainly in the 2 (B) The figure shows an arteriole with marked hyaline kidney, gastrointestinal tract, and skin of young to middle- thickening of the wall, indicative of hyaline arteriolosclero- aged adults. Takayasu arteritis is seen mainly in children and sis. Diabetes mellitus also can lead to this finding. Sepsis can involves the aorta (particularly the arch) and branches such produce disseminated intravascular coagulopathy with arte- as coronary and renal arteries, with granulomatous inflam- riolar hyaline thrombi. The debilitation that accompanies mation, aneurysm formation, and dissection. Telangiecta- cancer tends to diminish the vascular disease caused by ath- sias are small vascular arborizations seen on skin or mucosal erosclerosis. Syphilis can cause a vasculitis involving the vasa surfaces. Wegener granulomatosis, seen mainly in adults, vasorum of the aorta. Polyarteritis can involve large to involves small arteries, veins, and capillaries and causes medium-sized arteries in many organs, including the mixed inflammation and necrotizing and non-necrotizing kidneys; the affected vessels show fibrinoid necrosis and granulomatous inflammation with geographic necrosis sur- inflammation of the wall (vasculitis). rounded by palisading epithelioid macrophages and giant BP7 341 BP8 356–357 PBD7 529–530 cells. PBD8 495 BP7 525 BP8 366 PBD7 535, 541–542 PBD8 512, 517 3 (F) This is a classic example of a secondary form of hypertension for which a cause can be determined. In this 5 (D) Takayasu arteritis leads to “pulseless disease” because case, the renal artery stenosis reduces glomerular blood flow of involvement of the aorta (particularly the arch) and and pressure in the afferent arteriole, resulting in renin branches such as coronary, carotid, and renal arteries, with release by juxtaglomerular cells. The renin initiates angio- granulomatous inflammation, aneurysm formation, and tensin II–induced vasoconstriction, increased peripheral dissection. Fibrosis is a late finding, and the pulmonary vascular resistance, and increased aldosterone, which pro- arteries also can be involved. Aortic dissection is an acute motes sodium reabsorption in the kidney, resulting in problem that, in older adults, is driven by atherosclerosis increased blood volume. Anti–double-stranded DNA is a and hypertension, although this patient is within the age specific marker for systemic lupus erythematosus. ANCAs range for complications of Marfan syndrome, which causes are markers for some forms of vasculitis, such as micro- cystic medial necrosis of the aorta. Kawasaki disease affects scopic polyangiitis or Wegener granulomatosis. Some children and is characterized by an acute febrile illness, coro- patients with hepatitis B or C infection can develop a mixed nary arteritis with aneurysm formation and thrombosis, skin cryoglobulinemia with a polyclonal increase in IgG. Renal rash, and lymphadenopathy. Microscopic polyangiitis affects involvement in such patients is common, and cryoglobulin- arterioles, capillaries, and venules with a leukocytoclastic emic vasculitis then leads to skin hemorrhages and ulcer- vasculitis that appears at a similar stage in multiple organ ation. Hyperglycemia is a marker for diabetes mellitus, sites (in contrast to classic polyarteritis nodosa, which causes which accelerates the atherogenic process and can involve varying stages of acute, chronic, and fibrosing lesions in the kidneys, promoting the development of hypertension. small to medium-sized arteries). Tertiary syphilis produces HIV infection is not related to hypertension. Tertiary syphi- an endaortitis with proximal aortic dilation. Thromboangi-  12 The Heart PBD7 and PBD8 Chapter 12: The Heart BP7 and BP8 Chapter 11: The Heart 1 A 50-year-old man experiences episodes of severe sub- and the need for more careful monitoring. A subset of patients sternal chest pain every time he performs a task that requires is found to have more severe congestive heart failure, poor moderate exercise. The episodes have become more frequent exercise tolerance, and decreased arterial oxygen saturation and severe over the past year, but they can be relieved by sub- levels. Which of the following is most likely to predict a worse lingual nitroglycerin. On physical examination, he is afebrile, clinical presentation for these patients? his pulse is 78/min and regular, and there are no murmurs  (A) Size of the left ventricle or gallops. Laboratory studies show creatinine, 1.1 mg/dL;  (B) Degree of pulmonary stenosis glucose, 130 mg/dL; and total serum cholesterol, 223 mg/dL.  (C) Size of the ventricular septal defect Which of the following cardiac lesions is most likely to be  (D) Diameter of the tricuspid valve present?  (E) Presence of an atrial septal defect  (A) Rheumatic mitral stenosis  (B) Serous pericarditis 4 A 12-year-old boy was brought to the physician with a  (C) Restrictive cardiomyopathy sore throat and fever 3 weeks ago, and a throat culture was  (D) Calcific aortic stenosis positive for group A β-hemolytic streptococcus. On the follow-  (E) Coronary atherosclerosis up examination, the child is afebrile. His pulse is 85/min, res-  (F) Viral myocarditis pirations are 18/min, and blood pressure is 90/50 mm Hg. On 2 A 44-year-old woman who has rheumatic heart disease auscultation, a murmur of mitral regurgitation is audible, and with aortic stenosis undergoes valve replacement with a bio- there are diffuse rales over both lungs. The child is admitted prosthesis. She remains stable for the next 8 years and then to the hospital and over the next 2 days has several episodes develops diminished exercise tolerance. Which of the follow- of atrial fibrillation accompanied by signs of acute left ven- ing complications involving the bioprosthesis has most likely tricular failure. Which of the following pathologic changes occurred? occurring in this child’s heart during hospitalization is most likely to be the cause of the left ventricular failure?  (A) Paravalvular leak  (B) Stenosis  (A) Amyloidosis  (C) Hemolysis  (B) Endocardial fibroelastosis  (D) Embolization  (C) Fibrinous pericarditis  (E) Myocardial infarction  (D) Fibrosis of mitral valve with fusion of commissures 3 In a clinical study of tetralogy of Fallot, patients are  (E) Myocarditis examined before surgery to determine predictors observed on  (F) Tamponade echocardiography that correlate with the severity of the disease  (G) Verrucous endocarditis 146  CHAPTER 12 The Heart 147 130/80 mm Hg. Diffuse crackles are heard on auscultation of the lungs. The representative gross appearance of the heart is shown in the figure. Which of the following complications of this disease is the patient most likely to develop?  (A) Atrial myxoma  (B) Cardiac tamponade  (C) Constrictive pericarditis  (D) Hypertrophic cardiomyopathy  (E) Infective endocarditis  (F) Systemic thromboembolism Courtesy of Arthur Weinberg, MD, Department of Pathology, University of Texas Southwestern Medical School, Dallas, TX. 5 The parents of a 5-year-old child notice that he is not as active as other children his age. During the past 9 months, the child has had several episodes of respiratory difficulty fol- lowing exertion. On physical examination, his temperature is 36.9°C, pulse is 81/min, respirations are 19/min, and blood pressure is 95/60 mm Hg. On auscultation, a loud holosystolic murmur is audible. There are diffuse crackles over the lungs bilaterally, with dullness to percussion at the bases. A chest radiograph shows a prominent border on the left side of the heart, pulmonary interstitial infiltrates, and blunting of the costodiaphragmatic recesses. The representative gross appear- ance of the child’s heart is shown in the figure. Which of the 7 A 25-year-old man was found dead at home by the following additional pathologic conditions would most likely apartment manager, who had been called by the decedent’s develop in this child? employer because of failure to report to work for the past 3 days. An external examination by the medical examiner  (A) Aortic regurgitation showed splinter hemorrhages under the fingernails and no  (B) Coronary atherosclerosis signs of trauma. The gross appearance of the heart at autopsy  (C) Nonbacterial thrombotic endocarditis is shown in the figure. Which of the following laboratory find-  (D) Pulmonary hypertension ings is most likely to provide evidence of the cause of the  (E) Restrictive cardiomyopathy disease?  (A) Elevated antistreptolysin O titer  (B) Positive ANCA determination  (C) Increased creatine kinase-MB fraction (CK-MB)  (D) High double-stranded DNA autoantibody titer  (E) Positive blood culture for Staphylococcus aureus 8 A 10-year-old girl who is normally developed has chronic progressive exercise intolerance. On physical exami- nation, temperature is 37.1°C, pulse is 70/min, respirations are 14/min, and blood pressure is 100/60 mm Hg. A chest radio- graph shows cardiomegaly and mild pulmonary edema. An echocardiogram shows severe left ventricular hypertrophy and a prominent interventricular septum. The right ventricle is slightly thickened. During systole, the anterior leaflet of the mitral valve moves into the outflow tract of the left ventricle. The ejection fraction is abnormally high, and the ventricular volume and cardiac output are both low. Which of the follow- 6 A 68-year-old man with a history of diabetes mellitus ing is the most likely cause of the cardiac abnormalities in this had chest pain and an elevated serum troponin I level 1 year patient? ago. He was treated in the hospital with antiarrhythmic agents  (A) Mutations in β-myosin heavy chain for 1 week. An echocardiogram showed an ejection fraction  (B) Autoimmunity against myocardial fibers of 28%. He now has markedly reduced exercise tolerance.  (C) Excessive iron accumulation On physical examination, his temperature is 37°C, pulse is  (D) Deposition of amyloid protein 68/min, respirations are 17/min, and blood pressure is  (E) Latent enterovirus infection 148 UNIT II Systemic Pathology: Diseases of Organ Systems 9 For the past 2 years, a 49-year-old woman has had a  (A) Ventricular fibrillation chronic cough that produces a small amount of whitish  (B) Pericarditis sputum. The sputum occasionally is blood-streaked. On phys-  (C) Myocardial rupture ical examination, her temperature is 37.9°C, pulse is 71/min,  (D) Ventricular aneurysm respirations are 17/min, and blood pressure is 125/80 mm Hg.  (E) Thromboembolism Crackles are heard on auscultation over the upper lung fields. Heart sounds are faint, and there is a 15 mm Hg inspiratory 13 One week ago, a 72-year-old woman had an episode in decline in systolic arterial pressure. The chest radiograph which she became disoriented, had difficulty speaking, and shows prominent heart borders with a “water bottle” configu- had weakness on the right side of the body. On physical exam- ration. Pericardiocentesis yields 200 mL of bloody fluid. Infec- ination, she is afebrile with pulse of 68/min, respirations of tion with which of the following organisms is most likely to 15/min, and blood pressure of 130/85 mm Hg. On ausculta- produce these findings? tion, the lungs are clear, the heart rate is irregular, and there  (A) Mycobacterium tuberculosis is a midsystolic click. An echocardiogram shows nodular  (B) Group A streptococcus deposits with the density of calcium around the mitral valve.  (C) Coxsackievirus B One leaflet of the mitral valve appears to balloon upward. The  (D) Candida albicans ejection fraction is estimated to be 55%. Laboratory findings  (E) Staphylococcus aureus show Na+, 141 mmol/L; K+, 4.1 mmol/L; Cl−, 98 mmol/L; CO2, 25 mmol/L; glucose, 77 mg/dL; creatinine, 0.8 mg/dL; calcium, 10 A 27-year-old woman has had a fever for 5 days. On 8.1 mg/dL; and phosphorus, 3.5 mg/dL. Which of the follow- physical examination, her temperature is 38.2°C, pulse is ing is the most likely diagnosis? 100/min, respirations are 19/min, and blood pressure is 90/60 mm Hg. A cardiac murmur is heard on auscultation.  (A) Carcinoid heart disease The sensorium is clouded, but there are no focal neurologic  (B) Hyperparathyroidism deficits. Laboratory findings include hemoglobin, 13.1 g/dL;  (C) Infective endocarditis platelet count, 233,300/mm3; and WBC count, 19,200/mm3.  (D) Infiltrative cardiomyopathy Blood cultures are positive for Staphylococcus aureus. Urinaly-  (E) Mitral annular calcification sis shows hematuria. An echocardiogram shows a 1.5-cm  (F) Rheumatic heart disease vegetation on the mitral valve. Which of the following condi-  (G) Senile calcific stenosis tions is this patient most likely to develop?  (A) Cerebral arterial mycotic aneurysm 14 An 82-year-old woman has had increasing fatigue for  (B) Dilated cardiomyopathy the past 2 years. During this time, she has experienced parox-  (C) Abscess of the left upper lobe ysmal dizziness and syncope. On physical examination, she is  (D) Myxomatous degeneration of the mitral valve afebrile. Her pulse is 44/min, respirations are 16/min, and  (E) Polyarteritis nodosa blood pressure is 100/65 mm Hg. On auscultation, the lungs  (F) Polycystic kidneys are clear, and no murmurs are heard. An echocardiogram shows a normal-sized heart with normal valve motion and 11 A 19-year-old man has had a low-grade fever for 3 estimated ejection fraction of 50%. After being treated with weeks. On physical examination, his temperature is 38.3°C, digoxin, the heart rate slows and becomes irregular. An abnor- pulse is 104/min, respirations are 28/min, and blood pressure mality involving which of the following is most likely to be is 95/60 mm Hg. A tender spleen tip is palpable. There are present in this patient? splinter hemorrhages under the fingernails and tender hemor-  (A) Atrioventricular node rhagic nodules on the palms and soles. A heart murmur is  (B) Bundle of His heard on auscultation. Which of the following infectious  (C) Left bundle branch agents is most likely to be cultured from this patient’s blood?  (D) Parasympathetic ganglion  (A) Viridans streptococci  (E) Right bundle branch  (B) Trypanosoma cruzi  (F) Sinoatrial node  (C) Coxsackievirus B  (G) Sympathetic ganglion  (D) Candida albicans  (E) Mycobacterium tuberculosis  (F) Pseudomonas aeruginosa 15 A 50-year-old man with a history of infective endocar- ditis has increasing fatigue. On physical examination, he is 12 A 50-year-old man has sudden onset of severe subster- afebrile.