Deck 10: Pathology

Question 1

A 34-year-old woman comes to the emergency department because of sharp chest pain that radiates to the left shoulder.  The pain increases with inspiration and is partially relieved by sitting up and leaning forward.  Review of her outpatient medical records shows that she was seen for a facial rash 6 months ago.  She is also being evaluated for proteinuria that was identified during her last clinic appointment.  Which of the following is the most likely cause of this patient's chest pain?

A)Aortic dissection
B)Cardiac tamponade
C)Intimal hyperplasia of pulmonary arteries
D)Non-infectious cardiac valve vegetations
E)Pericardial inflammation

Answer 1

E

This patient's chest pain is characteristic of acute pericarditis, which along with her facial rash and proteinuria is suggestive of underlying systemic lupus erythematosus (SLE).  SLE is a chronic autoimmune disease predominantly affecting women age 20-40 that causes constitutional and multisystemic symptoms.  Serosal inflammation is common in SLE and most often manifests as pleuritis or pericarditis.
Pericarditis presents with severe and constant middle or left chest pain that may radiate to the neck and shoulders (particularly the trapezius ridge).  The pain increases on inspiration (pleuritic) and is relieved by sitting up and leaning forward (postural).  Auscultation of the chest reveals a scratchy sound called a pericardial friction rub that is best heard when the patient is leaning forward or lying prone.  Additional cardiovascular manifestations in SLE include pericardial effusion, verrucous (Libman-Sacks) endocarditis, and increased risk of coronary artery disease.
(Choice A)  Aortic dissection occurs in patients with long-standing hypertension and those with Marfan and Ehlers-Danlos syndromes (the 2 latter conditions cause cystic medial necrosis of the aortic wall).  It presents with abrupt-onset, severe, tearing chest pain that radiates to the back.
(Choice B)  Cardiac tamponade is caused by accumulation of fluid in the pericardial space that prevents the heart from filling properly in diastole.  It presents with dyspnea and tachypnea.  Physical examination shows distended neck veins, hypotension, diminished heart sounds, and pulsus paradoxus (drop in systolic blood pressure >10 mm Hg on inspiration).
(Choice C)  Intimal hyperplasia of the pulmonary arteries is characteristic of both primary and secondary pulmonary hypertension.  It presents with dyspnea, malaise, and findings associated with right ventricular failure (eg, jugular venous distention, pedal edema, hepatomegaly).  Severe symptomatic pulmonary hypertension is a rare complication of SLE.
(Choice D)  Non-bacterial endocarditis (verrucous or Libman-Sacks endocarditis) refers to small, wart-like fibrinous lesions and generalized thickening that can affect the heart valves of patients with SLE.  These are typically asymptomatic but may lead to valvular insufficiency or embolism.
Educational objective:
Pericarditis is the most common cardiovascular manifestation associated with systemic lupus erythematosus.  It presents with sharp pleuritic chest pain that is relieved by sitting up and leaning forward.
__________
References:
Cardiac involvement in systemic lupus erythematosus.
(http://www.ncbi.nlm.nih.gov/pubmed/10089998)

Question 2

A 21-year-old woman comes to the office due to recurrent episodes of self-limited, colicky abdominal pain.  She also had an episode of facial swelling that resolved spontaneously.  The patient has no other significant past medical history and takes no medications.  Examination is unremarkable.  Evaluation shows that her complement protein C1, even when not attached to an antigen-antibody complex, is excessively cleaving C2 and C4.  Which of the following is most likely increased in this patient?

A)Antinuclear antibody titer
B)Antistreptolysin O titer
C)Bradykinin
D)Free hemoglobin
E)Renin

Answer 2

C
This patient with recurrent episodes of abdominal pain and an episode of facial swelling likely has angioedema due to C1 inhibitor (C1INH) deficiency.  C1INH prevents C1-mediated cleavage of C2 and C4, thereby limiting activation of the complement cascade.  It also blocks kallikrein-induced conversion of kininogen to bradykinin, a potent vasodilator that also causes increased vascular permeability.
Acquired or hereditary C1INH deficiency (due to the complete absence of C1INH or the presence of a dysfunctional variant or an anti-C1INH antibody) leads to elevated levels of bradykinin, and patients can develop bradykinin-associated angioedema.  Symptoms include facial swelling (without urticaria), life-threatening laryngeal edema, and gastrointestinal manifestations (eg, nausea/vomiting, colicky pain, diarrhea).  Management of acute attacks involves supportive care and the administration of C1INH concentrate or a kallikrein inhibitor.
(Choice A)  Increased antinuclear antibody (ANA) titers are seen in a number of autoimmune conditions, including systemic lupus erythematosus, which is associated with hypocomplementemia but would have different clinical manifestations (eg, butterfly rash, arthritis, oral ulcers).  ANA production is not a result of excessive C1 activity.
(Choice B)  Poststreptococcal glomerulonephritis is associated with increased antistreptolysin O titers, complement activation, and low levels of C3.  The typical presentation is hematuria or nephritic syndrome following respiratory infection.
(Choice D)  Complement-mediated intravascular hemolysis can result from autoantibodies (autoimmune hemolytic anemia) or from direct complement activation (paroxysmal nocturnal hemoglobinuria); however, neither condition results from excessive C1 activity.
(Choice E)  Angiotensin-converting enzyme (ACE) inhibitors, which are associated with idiopathic angioedema, function by blocking ACE and result in increased renin levels.  ACE also degrades bradykinin.
Educational objective:
C1 inhibitor (C1INH) deficiency causes increased cleavage of C2 and C4 and results in inappropriate activation of the complement cascade.  C1INH also blocks kallikrein-induced conversion of kininogen to bradykinin, a potent vasodilator associated with angioedema.
__________
References:
C1 inhibitor deficiency: consensus document.
(http://www.ncbi.nlm.nih.gov/pubmed/15730382)
Hereditary angio-oedema.
(http://www.ncbi.nlm.nih.gov/pubmed/22305226)
Pathophysiology of hereditary angioedema.
(http://www.ncbi.nlm.nih.gov/pubmed/25538858)

Question 3

A 32-year-old man comes to the emergency room due to severe headaches and vomiting.  Soon after, he becomes comatose and expires despite extensive resuscitation efforts.  Autopsy shows a ruptured cerebral aneurysm with extensive intracranial hemorrhage and a congenital heart defect.  This patient's presentation is most likely associated with which of the following abnormalities?

A)Coarctation of the aorta
B)Patent ductus arteriosus
C)Primum-type atrial septal defect
D)Secundum-type atrial septal defect
E)Tetralogy of Fallot
F)Ventricular septal defect

Answer 3

A

This patient died from an intracranial hemorrhage due to a ruptured cerebral aneurysm.  Hypertension, genetic conditions (eg, Ehlers-Danlos syndrome), and lifestyle factors (eg, cigarette smoking) increase the risk of aneurysm development.  Coarctation of the aorta is a congenital cardiac defect associated with secondary hypertension and is a risk factor for cerebral aneurysm development.
Coarctation of the aorta is a narrowing of the aortic arch near the ligamentum arteriosum (remnant of ductus arteriosus) with localized medial and intimal hyperplasia.  Critical coarctation (severe narrowing) often presents in the neonatal period with heart failure and cardiogenic shock.  However, less severe narrowing allows for distal (but decreased) perfusion to the lower extremities and may present with only asymptomatic upper extremity hypertension in children or adults.  Additional symptoms due to this differential in blood pressure include headache, epistaxis, chest pain, and lower extremity claudication.
Complications of aortic coarctation include cerebral and aortic aneurysm.  Cerebral aneurysm with life-threatening rupture causing subarachnoid hemorrhage is likely due to chronic hypertension.  Aortic aneurysms usually develop near the site of coarctation due to embryologic vessel wall abnormalities and may lead to dissection or fatal rupture.
(Choices B, C, D, and F)  Patent ductus arteriosus and atrial and ventricular septal defects create a left-to-right intracardial shunt that, if untreated, may lead to right-to-left shunting (ie, Eisenmenger syndrome) due to pulmonary hypertension.  This may increase risk of a paradoxical thromboembolism from the venous to arterial circulation directly to the brain, causing an ischemic stroke.  However, neither condition is associated with cerebral aneurysm or intracranial hemorrhage.
(Choice E)  Tetralogy of Fallot is characterized by pulmonary stenosis, ventricular septal defect, an overriding aorta, and right ventricular hypertrophy.  Almost all patients have cyanosis in infancy or hypercyanotic episodes (ie, tet spells) in early childhood.  This patient's age makes this diagnosis unlikely.  In addition, risk of intracranial hemorrhage is not increased with tetralogy of Fallot, regardless of age.
Educational objective:
Adults with coarctation of the aorta often have chronic hypertension and are at increased risk of developing life-threatening aneurysms of the aorta (eg, dissection, rupture) and cerebral vessels (ie, intracranial hemorrhage).
__________
References:
Coarctation of the aorta.
(http://www.ncbi.nlm.nih.gov/pubmed/28613663)
Subarachnoid aneurysmal hemorrhage associated with coarctation of the aorta: case report and review of the literature.
(http://www.ncbi.nlm.nih.gov/pubmed/28978900)

Question 4

A 42-year-old woman comes to the emergency department due to worsening jaw pain over the past week.  She is unable to chew solid foods on the left side because of severe throbbing pain.  The patient has had several "tooth infections" over the last year despite meticulous oral hygiene.  Physical examination shows facial edema over the left mandible.  Multiple teeth have decay and gingival recession, and gentle tapping of the left second molar elicits pain.  This patient's current disease process is most likely to develop secondary to which of the following underlying conditions?

A)Acromegaly
B)Crohn disease
C)Riboflavin deficiency
D)Selective IgA deficiency
E)Sjögren syndrome

Question 5

A 62-year-old man is brought to the emergency department by a friend due to severe shortness of breath.  He is agitated and gasping for air and is unable to provide history.  His friend says the patient has a history of chronic alcohol use and attends Alcoholics Anonymous meetings.  He is unaware of any other medical history.  Temperature is 36.7 C (98 F), blood pressure is 110/70 mm Hg, pulse is 104/min, and respirations are 32/min.  Due to worsening respiratory distress, immediate endotracheal intubation is performed and mechanical ventilation is begun.  The patient is admitted to the intensive care unit and, despite appropriate therapy, dies several hours later.  Autopsy examination is performed, and histopathology of the lungs reveals engorged pulmonary capillaries and intraalveolar, acellular, pink material that is more prominent at the bases.  This patient most likely had which of the following conditions?

A)Aspiration pneumonia
B)Centriacinar emphysema
C)Hypersensitivity pneumonitis
D)Pulmonary arterial hypertension
E)Pulmonary edema
F)Pulmonary infarction

Question 6

A 78-year-old man dies of advanced esophageal cancer.  The man's family donates his body for teaching purposes and an autopsy is performed.  Gross examination of the heart reveals left ventricular chamber size and wall thickness within normal limits.  No significant atherosclerosis is seen in the coronary arteries.  On microscopic examination, myocardial cells demonstrate prominent yellow-brown intracytoplasmic granules as shown in the exhibit.
  Which of the following most likely accounts for the observed microscopic changes?

A)Exogenous pigment endocytosis
B)Glucose polymerization
C)Iron overload
D)Lipid peroxidation
E)Protein accumulation
F)Tyrosine oxidation

Question 7

An 18-year-old woman comes to the emergency department for evaluation of a rash.  The patient developed mild aches involving her knees and ankles 2 days ago.  Before going to sleep last night, she noticed purplish spots around her right knee.  Today, the rash involves both of the lower extremities.  She has had no fever, weight loss, sore throat, abdominal pain, vomiting, or diarrhea.  The patient has no chronic medical conditions and takes no medications.  Temperature is 37 C (98.6 F), blood pressure is 146/90 mm Hg, pulse is 90/min, and respirations are 20/min.  Cardiopulmonary examination is unremarkable.  The patient has pain with passive range of motion of the knees and ankles.  Skin examination is seen in the exhibit.Laboratory results are as follows: Histologic examination of the rash is most likely to show which of the following?

A)Abundant intravascular fibrin without inflammatory cells
B)Obliterative endarteritis with lymphocytes and plasma cells
C)Perivascular necrotizing granulomas with eosinophilic infiltration
D)Small vessels damaged by perivascular neutrophil accumulation
E)Vessels with transmural granulomatous infiltration and fragmentation of elastic fibers

Question 8

A 66-year-old man comes to the office for a routine visit.  He has a history of hypertension and osteoarthritis.  The patient has smoked a pack of cigarettes daily for 40 years and occasionally drinks 1 or 2 glasses of wine but does not use illicit drugs.  Blood pressure is 142/82 mm Hg and pulse is 80/min.  Cardiopulmonary examination is normal.  There is a pulsating, central abdominal mass on physical examination.  Which of the following pathologic conditions is the most likely underlying cause of this patient's abnormal findings?

A)Chronic transmural inflammation
B)Cystic medial necrosis
C)Focal intimal tear
D)Malignant endothelial proliferation
E)Microbial infection of aortic wall
F)Vasa vasorum endarteritis

Question 9

A 74-year-old man is brought to the emergency department due to acute-onset lower abdominal and back pain that was followed by an episode of syncope 2 hours ago.  The pain is sharp and constant.  He has had no changes in bowel or bladder function.  Other medical conditions include hypertension and hyperlipidemia.  The patient has a 50-pack-year history of smoking cigarettes.  Temperature is 36.9 C (98.4 F), blood pressure is 90/60 mm Hg, pulse is 108/min and regular, and respirations are 18/min.  BMI is 34 kg/m².  The patient is diaphoretic and appears pale.  Heart and lung sounds are normal.  Abdominal examination reveals moderate tenderness to deep palpation in the lower abdomen; there is no guarding or rebound tenderness.  Bowel sounds are normal.  Which of the following is the most likely diagnosis?

A)Acute mesenteric ischemia
B)Colonic perforation
C)Renal colic
D)Ruptured abdominal aortic aneurysm
E)Vertebral compression fracture

Question 10

A 62-year-old man is found dead in his apartment.  An autopsy shows diffuse cerebral edema, laryngeal edema, and hyperinflated lungs.  Which of the following is the most likely cause of death in this patient?

A)Acute myocardial infarction
B)Anaphylaxis
C)Chronic obstructive pulmonary disease exacerbation
D)Meningococcal meningitis
E)Seizures

Question 11

A 65-year-old man is admitted to the hospital with an acute ST-segment elevation myocardial infarction.  The patient undergoes coronary angiography with stent placement in the left circumflex artery, and he is started on therapy with a beta blocker and antiplatelet agents.  On day 3 of hospitalization he experiences chest pain.  The pain, described as sharp and radiating to his neck and shoulders, is exacerbated by coughing and swallowing.  He has no shortness of breath, lightheadedness, or abdominal pain.  Temperature is 37.5 C (99.5 F), blood pressure is 130/80 mm Hg, pulse is 90/min and regular, and respirations are 20/min.  The lungs are clear on auscultation.  There is no peripheral edema.  Which of the following is the most likely cause of this patient's chest pain?

A)Pericardial inflammation due to autoimmune reaction to necrotic tissue
B)Pericardial inflammation due to concomitant viral infection
C)Pericardial inflammation overlying the necrotic segment of myocardium
D)Recurrent thrombosis of the culprit coronary vessel
E)Thrombosis of a nonculprit coronary vessel

Question 12

A 24-year-old man comes to the urgent care clinic due to painful swelling in his left groin.  The patient noticed the swelling 2 days ago, and it has progressively become more tender and painful.  He has no chronic medical conditions, but he recently sustained a puncture wound to the sole of his left foot.  Physical examination shows an enlarged, tender, and nonfluctuant left inguinal lymph node with erythematous overlying skin.  There is a small puncture wound on the sole of the left foot, which expresses pus upon mild pressure.  Which of the following histologic findings is most likely responsible for this patient's groin mass?

A)Atypical B-cell proliferation
B)Diffuse granulomatous reaction
C)Extensive lymph node necrosis
D)Marked paracortical cell expansion
E)Formation of multiple germinal centers

Question 13

An 80-year-old man is brought to the hospital due to increasing chest pain.  Over the past 6 weeks, he has had dry cough, dysphagia, and a change in voice quality.  The patient has a long history of hypertension.  He is an ex-smoker with a 35-pack-year history.  On physical examination, the patient appears anxious, pale, and diaphoretic.  Blood pressure is 80/60 mm Hg, pulse is 120/min, and respirations are 20/min.  While being evaluated in the emergency department, he develops cardiac arrest with pulseless electrical activity and cannot be resuscitated.  Autopsy is most likely to show which of the following findings?

A)Fat globules in the pulmonary vasculature
B)Left ventricular free-wall rupture
C)Longitudinal tear in the esophagus
D)Ruptured pulmonary bleb
E)Ruptured thoracic aortic aneurysm

Question 14

A 43-year-old man is hospitalized with recent-onset oliguria and a high serum creatinine level.  He has been seen in clinic several times for an intranasal ulcer that has failed to heal.  This patient's condition is most likely associated with antibodies against which of the following?

A)Glomerular basal membrane
B)Smooth muscle cells
C)Neutrophils
D)Erythrocytes
E)Platelets
F)Mitochondria

Question 15

A 34-year-old woman is evaluated in the emergency department due to 1-day of chest and left shoulder pain.  The patient describes the pain as constant and worse in certain positions.  A month ago, she was treated for a skin rash and joint pain.  Her medical history is otherwise unremarkable.  She is a lifetime nonsmoker and does not use alcohol or illicit drugs.  Her father has hypertension and had a stroke at age 64.  Temperature is 37.5 C (99.5 F), blood pressure is 122/70 mm Hg, and pulse is 97/min and regular.  A triphasic, scratchy sound is heard over the left sternal border with the patient sitting up.  The lungs are clear on auscultation.  ECG shows sinus rhythm with ST-segment elevation in all leads except avR and V1.  Which of the following is the most likely diagnosis?

A)Eosinophilic myocarditis
B)Fibrinous pericarditis
C)Hemorrhagic pericarditis
D)Nonbacterial endocarditis
E)Purulent pericarditis

Question 16

A 65-year-old man is evaluated for progressive shortness of breath.  He has had exertional dyspnea for the past year and has been experiencing weight gain and peripheral edema for the last several weeks.  He has no previous history of cardiac or pulmonary disease.  Cardiac biopsy of the left ventricle shows the histopathologic findings on the slide below. Which of the following are the most likely echocardiographic findings in this patient?

Question 17

An 82-year-old man comes to the office due to progressive dyspnea and fatigue over the last year, which now limit his daily activities.  He has also noticed bilateral swelling of his feet.  The patient has hypertension, which is controlled with amlodipine.  His blood pressure is 122/72 mm Hg and pulse is 55/min.  Physical examination reveals elevated jugular venous pressure with rapid 'y' descent and a prominent S4.  Abdominal examination shows moderate ascites.  The patient has 3+ bilateral lower extremity pitting edema.  Echocardiogram reveals left atrial enlargement with marked left ventricular hypertrophy and normal left ventricular ejection fraction.  Complete blood count and basic metabolic panel are within normal limits.  Endomyocardial biopsy findings are shown below. Which of the following is the most likely diagnosis?

A)Cardiac myxoma
B)Cardiac sarcoidosis
C)Chagas disease
D)Hemochromatosis
E)Senile amyloidosis

Question 18

A 39-year-old man comes to the emergency department with anterior chest pain.  He had felt well until the pain developed 4 days ago.  The patient says the pain is sharp and makes it difficult to take a deep breath.  Since yesterday, he has also felt out of breath.  Medical history is unremarkable.  The patient's father died of a heart attack at age 52, and his mother suffers from rheumatoid arthritis.  He does not use alcohol or tobacco.  Temperature is 37.4 C (99.3 F), blood pressure is 112/65 mm Hg, and pulse is 103/min and regular.  Bedside ultrasound examination demonstrates a moderate pericardial effusion.  Which of the following is the most likely cause of this patient's current condition?

A)Autoimmune disease
B)Coronary artery disease
C)Gram-positive cocci
D)Malignancy
E)Viral infection

Question 19

A 54-year-old man comes to the emergency department due to worsening shortness of breath for the last 3 days.  His symptoms initially occurred with exertion but are now present at rest.  The patient could not sleep last night because of a suffocating cough each time he tried to lie down.  He considers himself generally healthy and states, "I've never had to see a doctor for any problems."  Family history is remarkable for asthma and hypertension.  Blood pressure is 162/86 mm Hg, pulse is 92/min, and respirations are 26/min.  An x-ray of the chest is shown in the exhibit. 
Which of the following is the most likely diagnosis?

A)Acute decompensated heart failure
B)Acute respiratory distress syndrome
C)Idiopathic pulmonary fibrosis
D)Obstructive pulmonary disease
E)Pulmonary arterial hypertension
F)Tension pneumothorax

Question 20

A research center studying cardiovascular pathology is conducting trials in which experimental rabbits are fed sweet peas containing substances that alter connective tissue synthesis and maturation.  The animals are monitored for several weeks prior to euthanasia.  Autopsy shows myxomatous degeneration with pooling of proteoglycans in the media layer and an intact intima layer in large arteries.  The pathologic findings seen in the experiment are most similar to which of the following conditions?

A)Aortic aneurysm
B)Atherosclerosis
C)Berry aneurysm
D)False aneurysm
E)Giant cell arteritis
F)Malignant hypertension

Question 21

A 68-year-old man comes to the office due to exertional shortness of breath and fatigue, which have progressed over the past year.  The patient has a history of hypertension, but medical history is otherwise unremarkable.  He is a lifetime nonsmoker.  His father died of a "heart attack" at age 70.  Blood pressure is 144/74 mm Hg, and pulse is 72/min and regular.  Cardiac auscultation reveals a 3/6 ejection-type, late-peaking systolic murmur and a barely audible S2.  The murmur diminishes in intensity during the straining phase of the Valsalva maneuver.  Which of the following processes underlies this patient's current condition?

A)Cardiomyocyte hypertrophy and disarray causing asymmetric septal thickening
B)Differentiation of valve fibroblasts into osteoblast-like cells
C)Myxomatous valve thickening and elastin fragmentation
D)Subendocardial granulomatous lesions with fibrinoid necrosis and subsequent fibrosis
E)Thrombus formation on inflamed valvular endothelium

Question 22

A 58-year-old man comes to the emergency department with abrupt-onset, severe chest pain that radiates to his back.  His blood pressure is 220/130 mm Hg in the left arm and 180/100 mm Hg in the right.  His heart rate is 100/min.  Initial laboratory studies show normal serum troponin levels.  Electrocardiogram is negative for ST-segment changes.  This patient's acute condition was most likely triggered by which of the following events?

A)Intimal fatty streak formation
B)Intimal tearing
C)Medial calcification
D)Medial inflammation
E)Vasa vasorum obliteration

Question 23

A 44-year-old man is evaluated in the clinic for occasional chest discomfort that is not consistently related to exertion.  The patient's past medical history is significant for hypertension and hyperlipidemia.  His grandfather experienced a myocardial infarction at age 50.  Coronary CT angiography reveals several nonobstructive atherosclerotic plaques in the coronary arteries.  One plaque in the proximal left anterior descending artery appears extensive, has a large hypodense core, and occupies 40% of the lumen.  No intervention is performed.  One year later, the patient comes to the emergency department with acute severe chest pain and is found to have thrombotic occlusion of the proximal left anterior descending artery.  High intraplaque activity of which of the following enzymes most likely resulted in this patient's myocardial infarction?

A)Hydroxymethylglutaryl CoA reductase
B)Lysyl oxidase
C)Metalloproteinases
D)Procollagen peptidases
E)Prolyl hydroxylase

Question 24

A 75-year-old man comes to the office due to worsening dyspnea and fatigue on exertion over the last 6 months.  Recently, he has had severe lightheadedness during physical activity.  Blood pressure is 125/65 mm Hg and pulse is 65/min and regular.  Physical examination reveals a harsh ejection-type systolic murmur at the base of the heart radiating to the neck.  The second heart sound is diminished in intensity.  A fourth heart sound is heard at the cardiac apex.  Which of the following is the most likely cause of this patient's heart condition?

A)Extensive valve calcification with impaired leaflet mobility
B)Fusion of valve commissures due to repetitive inflammation
C)Hypertrophy of the basal interventricular septum with subaortic obstruction
D)Infectious vegetations attached to the valve cusps
E)Myxomatous valve degeneration with leaflet prolapse

Question 25

A 60-year-old man comes to the office with a 6-month history of exertional chest pain that remits with rest.  His other medical conditions include hypertension, diabetes mellitus, and hypercholesterolemia.  An exercise stress test is positive for inducible ischemia.  Cardiac catheterization shows 80% occlusion of the right coronary artery and 60% occlusion of the left coronary artery.  The first step in the pathogenesis of this patient's coronary artery disease most likely involved which of the following cell types?

A)Endothelial cells
B)Interstitial fibroblasts
C)Macrophages
D)Mast cells
E)Pericytes
F)Platelets
G)Smooth muscle cells

Question 26

A 69-year-old man comes to the clinic due to a squeezing pressure in his midline chest and neck that occurs when walking.  He has a history of hypertension and type 2 diabetes mellitus.  The patient is an ex-smoker with a 20-pack-year history.  Coronary angiography shows extensive atherosclerosis and near-total occlusion of the left anterior descending artery.  Further testing shows normal resting left ventricular ejection fraction with no regional wall motion abnormalities.  The absence of myocardial necrosis and scarring despite vessel occlusion in this patient can be best explained by which of the following features of the occluding plaque?

A)Active peripheral inflammation
B)Lipid-rich core
C)Low calcium content
D)Ostial location
E)Slow growth rate
F)Thin fibrous cap

Question 27

A 35-year-old woman is brought to the emergency department due to severe right leg pain.  She is a concert pianist with no known medical issues.  During rehearsal, she began experiencing cramping of the right foot that did not improve with stretching or rest.  The pain quickly spread to her calf, and the patient now rates the pain as 9 out of 10 in intensity.  On examination, the right foot and calf appear pale and cool compared with the left, and sensation is diminished.  Right-sided dorsalis pedis and posterior tibial pulses are absent, and the popliteal pulse is barely palpable.  Emergency embolectomy is performed and a gelatinous mass causing arterial occlusion is successfully removed.  Histopathologic section of the mass is shown in the exhibit. 
Which of the following is the most likely origin of this patient's embolus?

A)Aortic valve
B)Descending aorta
C)Left atrium
D)Left ventricle
E)Mitral valve

Question 28

A group of forensic pathologists are analyzing tissue samples of adolescents age 13-18 to study the aging process.  Autopsy of a 14-year-old boy who died in a motor vehicle accident shows several minimally raised yellow spots on the inner surface of the abdominal aorta.  The rest of the cardiovascular findings during the autopsy are unremarkable.  He had no known medical problems.  There was no family history of cardiovascular disease or sudden cardiac death.  Which of the following is most likely to be the predominant cell type in these lesions on light microscopy?

A)Eosinophils
B)Fibroblasts
C)Macrophages
D)Mast cells
E)Neutrophils

Question 29

A 64-year-old man dies suddenly while playing tennis.  In the preceding months, he experienced fatigue and some exertional dyspnea.  Autopsy is performed.  The heart examination shows left ventricular septal thickness of 1.6 cm (normal: <1.1), posterior wall thickness of 1.6 cm (normal: <1.1), and an internal left ventricular diameter of 3.2 cm (normal: 3.5-5.9).  No focal myocardial scarring is seen.  Which of the following is the most likely cause of the cardiac findings seen in this individual?

A)Aortic stenosis
B)Dilated cardiomyopathy
C)Mitral regurgitation
D)Obstructive coronary artery disease
E)Rheumatic mitral stenosis

Question 30

A 16-year-old boy is evaluated in the office due to leg cramps.  He plays soccer but frequently stops in the middle of practice due to cramps in both legs.  The patient is afebrile.  Blood pressure is 170/90 mm Hg bilaterally in upper extremities.  Point of maximal impulse is nondisplaced.  S1 is normal, and S2 is physiologically split.  An S4 is heard, as well as a continuous murmur best auscultated over the back at the left paravertebral area.  Distal pulses in the radial arteries are prominent.  Dorsalis pedis and posterior tibial pulses are difficult to palpate.  The rest of the physical examination is unremarkable.  Which of the following is the most likely cause of this patient's continuous murmur?

A)Increased blood flow across the tricuspid valve
B)Increased blood flow through collateral blood vessels
C)Increased cardiac output due to low systemic vascular resistance
D)Increased pulmonary arterial blood flow
E)Persistence of the ductus arteriosus

Question 31

A 64-year-old man dies suddenly while playing tennis.  In the preceding months, the patient experienced fatigue and some exertional dyspnea.  Autopsy reveals rupture of an unsuspected ascending aortic aneurysm.  Heart examination shows a septal thickness of 1.1 cm (normal: ?1.1), a posterior wall thickness of 1.1 cm (normal: ?1.1), and an internal left ventricular diameter of 6.8 cm (normal: 3.5-5.9).  No focal myocardial scarring is seen.  Which of the following is the most likely cause of the cardiac findings seen in this individual?

A)Aortic regurgitation
B)Aortic stenosis
C)Hypertrophic cardiomyopathy
D)Obstructive coronary artery disease
E)Restrictive cardiomyopathy

Question 32

A 56-year-old man comes to the clinic for a routine checkup.  Medical history includes hypertension, type 2 diabetes mellitus, hyperlipidemia, and mild intermittent asthma.  The patient currently takes no medications and has not seen a physician in 7 years.  He reports feeling well.  Blood pressure is 152/101 mm Hg and pulse is 87/min.  Waist circumference is 110 cm (43 in).  Laboratory results are as follows: Which of the following vascular beds is most likely to carry the highest atherosclerotic burden in this patient?

A)Coronary arteries
B)Internal thoracic arteries
C)Mesenteric arteries
D)Popliteal arteries
E)Renal arteries

Question 33

A 68-year-old man is brought to the emergency department with chest pain.  For the last year, the patient has had exertional chest pain that has progressively worsened.  He was shoveling snow this morning when the pain became unbearable.  He has smoked a pack of cigarettes daily for 40 years.  Blood pressure is 140/100 mm Hg and pulse is 90/min.  Examination showed mild diaphoresis.  The lungs are clear to auscultation.  ECG shows ST-and T-wave changes suggestive of ischemia.  Cardiac enzymes are elevated.  Emergent coronary angiography is performed, which demonstrates significant atherosclerotic involvement of the left anterior descending and circumflex arteries.  Development of these vascular lesions most likely involved growth factors released from which of the following sources?

A)B lymphocytes
B)Eosinophils
C)Erythrocytes
D)Mast cells
E)Neutrophils
F)Platelets

Question 34

A 52-year-old man comes to the office due to periodic substernal chest heaviness.  The pain is precipitated by fast walking, especially uphill and against the wind, and remits after 5 minutes of rest.  The patient has a history of hypertension and smokes a pack of cigarettes a day.  Blood pressure is 140/80 mm Hg and pulse is 80/min.  The lungs are clear to auscultation, and no heart murmurs are heard.  There is no peripheral edema.  Which of the following pathologic states is most likely to be present in this patient?

A)Atherosclerotic plaque obstructing 50% of the coronary artery lumen, no thrombus
B)Atherosclerotic plaque obstructing 80% of the coronary artery lumen, no thrombus
C)Ruptured atherosclerotic plaque with fully obstructive thrombus
D)Significant coronary artery vasospasm causing flow obstruction
E)Ulcerated atherosclerotic plaque with partially obstructive thrombus

Question 35

A 74-year-old man comes to the office for a follow-up visit for hypertension.  His last visit was a year ago.  He has no chest pain, shortness of breath, leg swelling, or dizziness.  The patient is compliant with his medications and can tolerate a moderate level of physical activity.  Blood pressure is 145/75 mm Hg and pulse is 67/min and regular.  Auscultation findings at the base of the heart are depicted in the exhibit below. Which of the following is the most likely cause of this patient's physical findings?

A)Ankylosing spondylitis
B)Chordal rupture
C)Coronary artery disease
D)Cystic medial necrosis
E)Infective endocarditis
F)Rheumatic fever
G)Right ventricular failure
H)Valvular calcification

Question 36

A 50-year-old man is brought to the emergency department due to chest pain.  He passed out briefly after the pain started.  Medical history includes hypertension, hyperlipidemia, and early-stage chronic kidney disease.  The patient reports that his father had a heart attack at a young age.  Despite treatment, the patient dies during hospitalization.  An autopsy is performed.  Light microscopy of the aortic wall is shown on the slide below. Which of the following underlying mechanisms is most likely responsible for this patient's disease process?

A)Atherosclerosis
B)Calciphylaxis
C)Cystic degeneration of the media
D)Granulomatous inflammation
E)Monckeberg sclerosis

Question 37

A 27-year-old man comes to the emergency department due to acute-onset chest pain and shortness of breath.  The pain started suddenly an hour ago while he was lifting a box of books.  The patient has no chronic medical conditions, and he was in his usual state of health prior to the onset of symptoms.  He does not use tobacco, alcohol, or illicit drugs.  Height is 182 cm (71.7 in) and weight is 72 kg (158.7 lb).  On physical examination, the patient is in acute respiratory distress.  Temperature is 37 C (98.6 F), blood pressure is 90/60 mm Hg, pulse is 120/min and regular, and respirations are 26/min.  Pulse oximetry shows 86% saturation on room air.  Cardiopulmonary examination reveals pectus excavatum, bilateral pulmonary crackles, and a decrescendo diastolic murmur at the right upper sternal border.  The abdomen is soft and nontender.  There is no peripheral edema.  Subsequently, the patient develops cardiac arrest and dies.  Which of the following histologic changes is most likely to be seen on autopsy?

A)Atherosclerotic thickening of the intima
B)Coagulative necrosis of the left ventricular wall
C)Fragmentation of the elastic lamellae in the aortic media
D)Necrosis of the papillary muscles
E)Perivascular inflammation around the vasa vasorum

Question 38

A 22-year-old woman comes to the office due to worsening dyspnea.  The patient has also experienced low-grade fevers, a 6.8-kg (15-lb) weight loss, and syncopal episodes over the last 3 months.  Her shortness of breath worsens when sitting and improves when lying down.  Medical history is insignificant.  The patient does not use tobacco, alcohol, or illicit drugs.  Temperature is 37.4 C (99.3 F), blood pressure is 115/72 mm Hg, pulse is 75/min and regular, and respirations are 12/min.  The lungs are clear.  Cardiac auscultation reveals a low-pitched, mid-diastolic rumble at the cardiac apex.  The remainder of the physical examination is normal.  ECG reveals left atrial enlargement, and echocardiography shows a large, pedunculated mass in the left atrium.  Histologic analysis of this mass will most likely reveal which of the following?

A)Coarse, filamented, branching septate hyphae
B)Gram-positive cocci in clusters
C)Malignant myeloid cells
D)Scattered cells within a mucopolysaccharide stroma
E)Solid mass of platelets and fibrin

Question 39

A 42-year-old man comes to the emergency department due to severe chest pain that started abruptly 2 hours ago.  The pain is midline, constant, and 10/10 in intensity.  He has had no fever, cough, or shortness of breath.  His only medical condition is hypertension.  On examination, the patient is diaphoretic and is in severe distress due to pain.  Temperature is 37 C (98.6 F), pulse is 116/min, and respirations are 24/min.  Systolic blood pressure is 82 mm Hg in the right arm and 60 mm Hg in the left arm.  Jugular veins are distended with an estimated pressure of 13 cm H₂O.  The lungs are clear to auscultation.  The point of maximal impulse is not palpable.  The extremities are cold with no peripheral edema.  The patient develops cardiac arrest and dies soon after arrival.  Autopsy would most likely reveal which of the following findings?

A)Air within the pleural space
B)Blood in the pericardial space
C)Ruptured posteromedial papillary muscle
D)Thrombus at the pulmonary artery bifurcation
E)Transmural necrosis of the anterolateral myocardium

Question 40

A 56-year-old man comes to the emergency department with acute, severe chest pain.  His electrocardiogram is within normal limits.  Contrast-enhanced computed tomography of his chest is shown below. Which of the following is the single most important risk factor for this patient's current condition?

A)Diabetes mellitus
B)High cholesterol
C)Hypertension
D)Limited physical activity
E)Smoking
F)Spirochete infection

Question 41

A 68-year-old man comes to the office to establish care.  He has type 2 diabetes mellitus complicated by chronic kidney disease and peripheral sensory neuropathy.  Medical history is notable for hypertension and coronary artery disease.  The patient works for an agricultural supply company and spends most of his day walking and standing on the warehouse floor.  Vital signs are normal.  BMI is 34 kg/m².  Examination of the lower extremities shows decreased sensation to monofilament testing below the knees bilaterally, with 2+ pitting edema, varicosities, and extensive discoloration, as shown below. Which of the following is the most likely cause of the discoloration in this patient's skin?

A)Arteriolar calcium phosphate deposition
B)Cutaneous fungal infection
C)Glycosaminoglycan accumulation
D)Hemosiderin deposition
E)Increased melanin production

Question 42

A 35-year-old man is evaluated for progressive fatigue and shortness of breath.  Recently, he has noticed bilateral leg swelling and abdominal distension despite overall weight loss.  He does not use tobacco, alcohol, or illicit drugs.  Despite treatment, the patient dies several weeks later.  Autopsy reveals significant endocardial thickening with dense fibrous deposits around the tricuspid and pulmonary valves as well as moderate pulmonary valve stenosis.  The left-sided cardiac chambers and valves are normal.  Measuring the levels of which of the following substances would have helped in diagnosing this patient?

A)Plasma cortisol
B)Plasma homocysteine
C)Plasma phenylalanine
D)Urinary 5-hydroxyindoleacetic acid
E)Urinary porphobilinogen
F)Urinary vanillylmandelic acid

Question 43

A 32-year-old man dies suddenly in his sleep.  He had been experiencing easy fatigability for the past 6 months and mentioned that he was feeling tired "'all the time"' and needed to take naps during the day.  He did not drink alcohol or use illicit drugs.  He smoked a pack of cigarettes per day for 10 years.  His father died from a "heart problem" at age 40.  On autopsy, the heart appears grossly enlarged.  A layered mural thrombus is seen in the left ventricular apex.  Coronary atherosclerosis is present, with 20% narrowing of the mid-left anterior descending artery and 25% narrowing of the left circumflex artery.  Which of the following is the most likely cause of death of this patient?

A)Antiphospholipid antibody syndrome
B)Dilated cardiomyopathy
C)Hypertrophic cardiomyopathy
D)Ischemic heart disease
E)Restrictive cardiomyopathy

Question 44

A 37-year-old man is found unresponsive under a tree during a thunderstorm.  He is not breathing when paramedics arrive on the scene.  On examination, his pupils are fixed and dilated.  Several cutaneous erythematous marks in a fern-leaf pattern are seen on his lower extremities.  Second-degree burns are present on both arms.  Cardiopulmonary resuscitation is started; however, the patient does not respond and is pronounced dead on arrival at the hospital.  Which of the following is most likely to be the primary cause of his death?

A)Cardiac arrhythmia
B)Extensive deep-tissue burns
C)Intracranial hemorrhage
D)Myocardial contusion
E)Pulmonary barotrauma

Question 45

A 38-year-old woman comes to the office due to worsening shortness of breath over the past 4 weeks.  She has no chest pain or lightheadedness but has noticed some ankle puffiness.  The patient can hardly do chores without having to stop to catch her breath.  Medical history is unremarkable.  She delivered a healthy child 2 months ago without complications.  The patient is a lifetime nonsmoker and does not use alcohol.  She has no family history of early heart disease or sudden cardiac death.  Blood pressure is 96/60 mm Hg and pulse is 92/min and regular.  The apical impulse is palpated along the anterior axillary line, and S3 is heard at the apex.  There are no heart murmurs.  Which of the following best describes the left ventricular changes in this patient?

Question 46

A 42-year-old man comes to the emergency department due to fatigue and exertional dyspnea.  Over the last 2 weeks his symptoms have progressively worsened to the point that he can no longer walk across his living room without becoming short of breath.  He is admitted to the hospital but dies suddenly despite aggressive treatment.  An autopsy is performed, and a cross-section of his heart is shown in the image below. This patient's symptoms were most likely caused by which of the following mechanisms?

A)Decreased ventricular contraction force
B)Dynamic left ventricular outflow obstruction
C)Impaired left ventricular filling
D)Increased pulmonary vascular resistance
E)Poor ventricular wall compliance

Question 47

A 35-year-old previously healthy man is brought to the emergency department after being involved in a motor vehicle collision.  He was an unrestrained driver and hit his chest against the steering wheel during the collision.  When the paramedics reached him, he reported chest discomfort but was hemodynamically stable.  Shortly after arrival at the emergency department, the patient experiences worsening respiratory distress and becomes hypotensive.  On physical examination, the patient appears anxious and is tachycardic and tachypneic.  The trachea is midline; the jugular veins are distended.  The anterior chest wall is bruised and tender to palpation.  Vesicular breath sounds are present bilaterally.  The abdomen is soft and nontender.  Which of the following is the most likely cause of this patient's clinical deterioration?

A)Aortic rupture
B)Cardiac tamponade
C)Diaphragmatic rupture
D)Hemothorax
E)Tension pneumothorax

Question 48

A 30-year-old man is brought to the emergency department due to left-sided weakness.  His left arm was sluggish after awakening, and he had difficulty walking to the bathroom.  The patient has no known medical conditions but has a 2-month history of positional dyspnea.  Vital signs are within normal limits.  On examination, strength of the left upper and left lower extremity is reduced, and touch/temperature sensation is similarly diminished.  Cardiovascular examination reveals an intermittent murmur at the apex without carotid bruits.  Echocardiography shows a mobile, 3-cm left atrial mass obstructing the mitral opening during diastole.  Histologic examination of this mass would most likely show which of the following findings?

A)Amorphous extracellular matrix
B)Malignant epithelial cells with necrotic debris
C)Malignant vascular spindle cells
D)Organized thrombus
E)Well-organized, mature adipocytes

Question 49

A 32-year-old man dies suddenly in his sleep.  An autopsy is performed and the heart is examined for signs of structural cardiac disease.  The heart shows a left ventricle with a large area of apical thinning composed of fibrotic scar tissue.  The mid and basal left ventricular segments show normal myocardial thickness.  The coronary arteries show no evidence of obstructive atherosclerosis.  The right ventricle appears normal in size.  No apparent valve damage is present.  Dilation and wall-thickening of the esophagus is also noted.  Which of the following factors in this patient's medical history would be most helpful in determining the cause of death?

A)Alcohol consumption
B)Location of residence
C)Childhood vaccinations
D)Family history of cardiomyopathy
E)Occupational chemical exposure

Question 50

A 32-year-old man is brought to the emergency department with a several-day history of high-grade fever, dyspnea, and fatigue.  The patient's temperature is 39.4 C (102.9 F), blood pressure is 122/70 mm Hg, and pulse is 102/min and regular.  Physical examination reveals a new holosystolic murmur with a blowing quality that is best heard over the cardiac apex.  Skin examination shows nontender lesions on the sole of the feet, as shown in the image below. Which of the following do these lesions most likely represent?

A)Areas of local vascular growth
B)Foci of granulomatous inflammation
C)Immune complex-mediated vascular lesions
D)Microemboli to skin vessels
E)Primary infection foci

Question 51

A 24-year-old woman comes to the physician with a 3-week history of progressive fatigue.  Physical examination shows lesions involving the nail beds, as seen in the photograph below. Further evaluation would most likely reveal additional abnormalities during which part of the physical examination?

A)Neck palpation
B)Lung auscultation
C)Cardiac auscultation
D)Abdominal palpation
E)Rectal examination

Question 52

A 54-year-old man comes to the emergency department due to severe fatigue and dyspnea.  He has a long history of progressively worsening heart failure that has been resistant to treatment with medications, including diuretics.  He was treated with chest radiation 10 years ago for non-Hodgkin lymphoma and has been in remission since then.  The patient is admitted to the hospital, but his condition continues to deteriorate despite aggressive therapy.  He dies 3 days later, and an autopsy is performed.  Gross inspection of the heart shows dense, thick, fibrous tissue in the pericardial space between the visceral and parietal pericardium.  Which of the following signs would most likely have been detected during a physical examination of this patient just prior to his death?

A)Kussmaul sign
B)Loud P2
C)Paradoxical splitting of S2
D)S3 gallop
E)Sustained left parasternal lift

Question 53

A 42-year-old woman comes to the emergency department for evaluation of chest pain.  She was moving furniture in her summer house 2 days ago when she experienced sharp pain in the left side of the sternum that quickly subsided.  Since then, the patient has had episodic pain with deep inspiration or trunk movement.  She has no fever or cough.  The patient has a history of hypertension.  Her father died of myocardial infarction at age 67.  She does not use tobacco or illicit drugs.  Blood pressure is 146/85 mm Hg in the right arm and 142/80 mm Hg in the left arm, pulse is 86/min, and respirations are 12/min.  She has localized tenderness to palpation at the left sternal border.  Lungs are clear to auscultation, and cardiac examination reveals normal heart sounds without gallops or murmurs.  The abdomen is soft and nontender.  There is no peripheral edema.  Which of the following is the most likely cause of this patient's symptoms?

A)Acute pericarditis
B)Aortic dissection
C)Costochondritis
D)Esophageal spasm
E)Gastroesophageal reflux disease
F)Panic disorder
G)Pneumonia
H)Pulmonary arterial hypertension
I)Pulmonary embolism
J)Unstable angina

Question 54

A 65-year-old man dies while hospitalized for severe breathing difficulty.  The patient had several prior episodes of dyspnea and cough requiring hospitalization.  He had a history of hypertension and chronic kidney disease.  The patient smoked a pack of cigarettes daily for 38 years and immigrated to the United States 20 years ago.  Autopsy is performed, and microscopic examination of the lungs reveals alveolar macrophages containing aggregates of golden-brown cytoplasmic granules that turn dark blue with Prussian blue staining.  Which of the following conditions is most likely associated with this patient's microscopic findings?

A)Airway hyperreactivity
B)Coal particle inhalation
C)Granulomatous microbial infection
D)Left ventricular dysfunction
E)Pulmonary arterial hypertension
F)Pulmonary thromboembolism

Question 55

A 36-year-old woman is brought to the emergency department with sudden-onset right side weakness and speech difficulty.  During the last 3 weeks, the patient has experienced progressive fatigue, malaise, and low-grade fevers.  Despite the symptoms, she did not seek medical attention and did not take any medications.  She had a dental extraction 5 weeks ago, which was uncomplicated.  The patient has never previously been significantly ill or hospitalized.  She works as a receptionist at a legal firm and has never traveled outside the United States.  She does not use tobacco, alcohol, or illicit drugs.  The patient is admitted to the hospital, but despite adequate resuscitative measures, she dies 2 hours later.  Gross autopsy shows large, friable irregular masses attached to the atrial surface of a valve.  Which of the following underlying conditions most likely predisposed this patient to developing these cardiac changes?

A)Atrial septal defect
B)Coronary artery disease
C)Hypertrophic cardiomyopathy
D)Mitral annular calcification
E)Regurgitant mitral valve prolapse

Question 56

A 42-year-old man is found dead at home.  His medical problems included hypertension and dyslipidemia, but he had been noncompliant with his medications.  The patient had a lengthy smoking history and, despite constant urging from his physicians to stop smoking, he had only quit recently.  An autopsy is requested by the family.  Pathological examination shows complete thrombotic occlusion of the left main coronary artery and diffuse atherosclerotic vascular disease characterized by multiple atheromas.  Along with a lipid core, these atheromas have a fibrous cap formed from dense deposition of collagen.  Which of the following cells are directly responsible for synthesizing this fibrous cap?

A)Endothelial cells
B)Interstitial fibroblasts
C)Macrophages
D)Smooth muscle cells
E)T lymphocytes

Question 57

A 5-week-old boy is being evaluated for a week-long history of rapid breathing and tiring with feeds.  The infant was born at home after an uneventful pregnancy.  The mother declined all prenatal testing and ultrasound evaluations.  His temperature is 36.7 C (98 F), blood pressure is 76/38 mm Hg, pulse is 124/min, and respirations are 66/min.  The patient's cardiovascular examination is notable for a hyperdynamic precordium, a mid-diastolic rumble at the left sternal border, and a 3/6 holosystolic murmur in the apex that radiates to the left axilla.  An echocardiogram shows defects in the lower part of the interatrial septum and the interventricular septum.  This patient's condition is most likely associated with which of the following genetic conditions?

A)22q11.2 deletion
B)Autosomal trisomy
C)Fibrillin mutation
D)Frataxin mutation
E)Hamartin gene mutation
F)Sex chromosome monosomy

Question 58

A 59-year-old African American male presents to the emergency room with crushing chest pain, sweating, and lightheadedness.  His blood pressure is 90/60 mm Hg and his heart rate is 48 beats per minute.  Electrocardiogram (ECG) shows sinus bradycardia and ST segment elevation in leads II, III, and aVF.  Occlusion of which of the following coronary arteries is most likely responsible for this patient's symptoms?

A)Left main coronary artery
B)Left anterior descending artery
C)Left circumflex artery
D)Right coronary artery
E)Intramural arteries

Question 59

A 62-year-old woman comes to the office due to a vague feeling of heaviness in her legs, especially when standing for long periods.  She has no history of trauma to the legs.  The patient's other medical problems include osteoarthritis and gastroesophageal reflux disease.  She has worked as a cashier at a department store for the last 20 years.  Vital signs are normal.  Jugular venous pressure is normal.  The heart has regular rate and rhythm with no murmurs or extra sounds.  Lung sounds are clear with normal air movement.  The abdomen is soft and without masses.  Examination of the lower extremities shows dilated, tortuous veins at the calves and ankles bilaterally, with no erythema or edema.  Upper extremity examination is unremarkable.  This patient's current condition increases the risk for which of the following?

A)Intermittent claudication
B)Ischemic stroke
C)Myocardial infarction
D)Phlegmasia alba dolens
E)Pulmonary embolism
F)Skin ulcerations

Question 60

A 17-year-old girl is evaluated due to decreased exercise tolerance and fatigue.  She has no known medical conditions and takes no medications.  Blood pressure is 110/60 mm Hg and pulse is 88/min.  The lungs are clear on auscultation.  Cardiac examination reveals a parasternal heave, and a widely split second heart sound is heard throughout the respiratory cycle.  A systolic ejection murmur is present at the left upper sternal border.  Distal pulses are normal.  Echocardiography of this patient is most likely to demonstrate which of the following findings?

Question 61

A 53-year-old woman comes to the office due to progressive distension and tightness in her right lower extremity for the past year.  She has had no shortness of breath, fever, or skin rash.  The patient was diagnosed with melanoma of the right thigh approximately 2 years ago and underwent surgical resection.  Two of her inguinal lymph nodes tested positive for metastasis, and she subsequently underwent inguinal lymphadenectomy and received adjuvant therapy.  Recent imaging revealed no evidence of recurrent malignancy.  The patient's medical history also includes hypertension and hyperlipidemia.  Vital signs are within normal limits.  Physical examination shows clear lungs and normal heart sounds without murmurs or gallops.  No jugular venous distension is present.  The right lower extremity is significantly larger in diameter than the left.  Edema is present up to the thigh, with mild skin indentation after application of pressure.  The skin overlying the involved area is thickened and dry.  Laboratory testing, including complete blood count, serum metabolic panel, and urinalysis, is within normal limits.  Treatment of this patient's symptoms should include which of the following strategies?

A)Blockade of estrogen receptors
B)Compression to increase lymphatic flow
C)Dissolution of intravascular thrombus
D)Inhibition of vascular endothelial growth factor
E)Reduction of intravascular volume

Question 62

A 21-year-old Caucasian male presents to the emergency department following an episode of syncope.  The syncopal episode was not provoked by any activity or circumstance, nor was it preceded by lightheadedness.  The patient has no significant past medical history and he is not taking any medications.  An ECG obtained in the ER reveals QT-interval prolongation but is otherwise unremarkable.  Assuming this is an inherited condition, the relevant mutation most likely affects which of the following structures?

A)Cardiac cell cytoskeleton proteins
B)Cardiac cell sarcomere proteins
C)Mitochondrial enzymes of oxidative phosphorylation
D)Calcium-binding sarcoplasmic reticulum protein
E)Membrane potassium channel proteins

Question 63

An 18-year-old man suddenly collapses during a high school soccer game.  There was no preceding collision or trauma.  Immediate resuscitation is initiated but he dies before the emergency medical service arrives.  The patient had recently experienced occasional exertion-related chest discomfort and dyspnea.  Otherwise, he had no significant medical history.  He took no medications and did not use tobacco, alcohol, or illicit drugs.  Which of the following is most likely to be seen on autopsy?

A)Atherosclerotic narrowing of the left anterior descending artery
B)Dilation of all cardiac chambers with thin ventricular walls
C)Extracellular deposits and stiff ventricular walls
D)Myocardial hypertrophy with patchy interstitial fibrosis
E)Thrombus at the bifurcation of the main pulmonary artery

Question 64

A 6-year-old boy is evaluated in the office for difficulty hearing.  The patient has no ear pain, discharge, or upper respiratory symptoms.  Initial testing suggests that he has bilateral sensorineural hearing loss.  He has no motor deficits or cerebellar signs.  His paternal uncle died suddenly at age 12.  Examination of the ears, nose, and throat is normal.  ECG shows normal sinus rhythm with a prolonged QT interval (520 msec).  Echocardiogram shows normal left and right ventricular function with no significant valvular disease.  A genetic defect affecting which of the following is most likely present in this patient?

A)Calcium channels
B)Membrane anchoring protein
C)Potassium channels
D)Sodium channels
E)Sodium-potassium ATPase

Question 65

The blood cultures obtained from this patient on admission grow Streptococcus species.  Which of the following processes was the most likely initiating step in the pathogenesis of this patient's condition? A)Endocardial fibrosis B)Fibrin deposition C)Liquefactive necrosis D)Myxomatous degeneration E)White blood cell infiltration

Question 66

A 4-year-old boy is brought in by his parents for evaluation of a fever that has persisted for the past 5 days.  He has also been more irritable than usual and had 2 or 3 episodes of vomiting.  The patient has no prior medical problems and takes no medications.  He has received all recommended vaccinations.  He traveled to China last year to visit his grandparents and cousins but has not traveled outside of the country this year.  Temperature is 38.9 C (102 F).  Physical examination shows bilateral conjunctival injection with no exudates.  His tongue is bright red and lips are cracked.  Nonpitting edema is present on his hands and feet.  Which of the following complications is this patient at greatest risk for developing?

A)Aortic dissection
B)Coronary artery aneurysm
C)Monocular blindness
D)Mononeuritis multiplex
E)Proliferative glomerulonephritis
F)Pulmonary cavitation

Question 67

A 78-year-old man is brought to the emergency department due to lightheadedness that began earlier in the day.  This morning, the patient felt unsteady and almost passed out when he tried to get out of bed.  He has not had similar symptoms before and he has had no chest pain or shortness of breath.  He has a history of coronary artery disease requiring percutaneous coronary intervention.  The rhythm strip of the ECG obtained in the emergency department is shown in the exhibit. 
Which of the following is the most likely cause of this patient's current symptoms?

A)Degenerative changes involving the atrioventricular node
B)Degenerative changes involving the sinus node
C)Ectopic electrical foci in left ventricular myocardium
D)Reentrant circuit involving the cavotricuspid isthmus
E)Sporadic depolarization of atrial cardiomyocytes

Question 68

A 17-year-old boy collapses while jogging and dies despite resuscitation efforts.  He has no medical history; however, his family history is significant for an uncle who died suddenly at age 20.  The boy is 185.4 cm (6 ft 1 in) tall and weighs 80 kg (176.4 lb).  Autopsy reveals significant left ventricular hypertrophy predominantly affecting the interventricular septum.  There are no valvular abnormalities.  Assuming the boy died of an inherited condition, which of the following proteins was most likely affected by the relevant mutation?

A)Beta-myosin heavy chain
B)Dystrophin
C)Fibrillin-1
D)Membrane potassium channel
E)Transthyretin

Question 69

A 34-year-old Asian female is hospitalized with progressive exertional dyspnea, lower extremity edema and cough.  She also describes frequent nocturnal episodes of breathlessness and recent hoarseness.  She does not use tobacco, alcohol or drugs.  Auscultation reveals loud first and second heart sounds and a mid-diastolic rumble best heard at the cardiac apex.  This patient's hoarseness is most likely caused by:

A)Laryngeal edema
B)Impaired arterial supply
C)Nerve impingement
D)Epithelial sloughing
E)Vocal cord polyps

Question 70

A 36-year-old man comes to the emergency department after briefly losing consciousness while watching television half an hour ago.  The patient had no preceding chest pain or shortness of breath, but he has been having recurrent palpitations over the past several days.  Two weeks ago, he noticed a non-itchy rash on his thigh that he treated with an over-the-counter steroid cream.  Medical history is otherwise unremarkable.  There is no family history of heart disease or sudden cardiac death.  The patient recently began working as a forest ranger in Pennsylvania.  He does not use tobacco, alcohol, or illicit drugs.  Blood pressure is 130/80 mm Hg and pulse is 46/min.  The lungs are clear on auscultation and heart sounds are normal.  The remainder of the examination shows no abnormalities.  ECG reveals complete atrioventricular block.  Echocardiography shows normal ventricular function with no major valvular disease.  Which of the following is the most likely underlying cause of this patient's current condition?

A)Disseminated spirochetal infection
B)Granulomatous myocardial inflammation
C)Missense mutation of a sarcomere protein
D)Myocyte potassium channel mutation
E)Viral infection-induced cardiac injury

Question 71

A 24-year-old man is evaluated due to an episode of syncope.  He was jogging when he felt light-headed and passed out, but he did not sustain a head injury.  The patient has had 2 similar episodes of light-headedness while jogging over the last year, but this was the first time he passed out.  He considers himself in good health and has no other medical conditions.  The patient does not use tobacco, alcohol, or recreational drugs.  His father died suddenly at age 30.  Vital signs are within normal limits.  On physical examination, the patient has a harsh systolic murmur.  The lungs are clear to auscultation.  There is no peripheral edema.  Transthoracic echocardiography shows asymmetric interventricular septal hypertrophy.  This patient's symptoms are most likely explained by left ventricular outflow obstruction created by which of the following structures?

A)Aortic valve cusp and interventricular septum
B)Aortic valve cusp and papillary muscle
C)Aortic valve cusps
D)Ascending aorta and interventricular septum
E)Mitral valve leaflet and interventricular septum

Question 72

A 21-year-old man comes to the office due to multiple episodes of syncope.  The patient has no chest discomfort or dyspnea.  He has no known medical problems and does not use tobacco, alcohol, or illicit drugs.  The patient is a computer analyst and leads a mostly sedentary lifestyle.  He reports that several family members have died of sudden cardiac death.  Genetic analysis reveals an ion channel defect.  Due to the defect, cardiac cells show decreased outward potassium flow and resultant prolongation of the action potential.  Which of the following is the most likely consequence of this patient's disease?

A)Abnormal anatomic communication between cardiac chambers
B)Asymmetric hypertrophy of the left ventricle
C)Ischemic myocardial necrosis followed by scarring
D)Left ventricular dilation and systolic dysfunction
E)Ventricular tachycardia and sudden death

Question 73

A 56-year-old woman with unknown medical history is brought to the emergency department in an unresponsive state.  Paramedics were called for sudden-onset, left-sided weakness, and when they arrived at the patient's house she was having a generalized tonic-clonic seizure.  Antiseizure medications were given and the patient was subsequently intubated.  Evaluation shows a comatose woman with left hemiplegia.  Neuroimaging reveals a large infarction in the right middle cerebral artery territory with cerebral edema.  Despite aggressive interventions, the patient dies from brain herniation.  Autopsy reveals multiple small, nondestructive masses attached to the edges of the mitral valve leaflets.  Microscopy shows that these masses are composed of platelet-rich thrombi, but no organisms are present.  Which of the following is most closely associated with this patient's heart valve findings?

A)Advanced malignancy
B)Chronic liver failure
C)Dilated cardiomyopathy
D)Intravenous drug use
E)Large-vessel vasculitis

Question 74

A 19-year-old man comes to the office to establish medical care.  He is transitioning care from his pediatrician.  The patient states that he recently changed his glasses for myopia; he otherwise has no symptoms.  He takes no medications.  Vital signs are within normal limits.  On physical examination, the patient is tall with long upper extremities and fingers.  The face appears narrow with down-slanted palpebral fissures, flattened malar bones, and a small jaw.  The lungs are clear on auscultation.  A late-systolic murmur is present at the cardiac apex.  The abdomen is soft and nontender with no organomegaly.  Which of the following is the most likely cause of this patient's murmur?

A)Aortic root dilation
B)Aortic valve cusp fusion
C)Endocardial fibrous deposition
D)Myxomatous mitral degeneration
E)Papillary muscle dysfunction

Question 75

A 42-year-old man comes to the emergency department after a syncopal episode preceded by palpitations and lightheadedness.  The patient has no significant medical history and takes no medications.  He does not use tobacco, alcohol, or illicit drugs.  The patient is a computer programmer and spends most of his time indoors.  Vital signs are within normal limits with no orthostatic changes.  Heart and lung sounds are normal.  The liver span is increased with no tenderness.  The skin appears darkly tanned.  ECG demonstrates sinus node dysfunction, and echocardiography reveals abnormal diastolic relaxation of the left ventricle.  Which of the following is the most likely cause of this patient's condition?

A)Amyloid deposition
B)Cortisol deficiency
C)Granuloma formation
D)Human leukocyte antigen gene mutation
E)Iron accumulation
F)Lysosomal glycogen accumulation

Question 76

A 35-year-old previously healthy woman comes to the hospital due to increasing shortness of breath and cough.  Over the past few months, she has had arthralgias, fatigue, low-grade fever, and weight loss.  Laboratory results are as follows: CT scan of chest is shown in the exhibit. 
Histologic examination of the lung would most likely reveal which of the following findings?

A)Fibrinoid degeneration of the vessel wall with luminal narrowing
B)Necrotizing arteritis and focal collections of epithelioid histiocytes
C)Necrotizing granuloma with a predominance  of eosinophils
D)Proliferation of irregularly shaped glands and pleomorphic cells

Question 77

A 20-year-old student collapses suddenly in class and dies, despite resuscitation efforts.  Review of the medical chart is conducted-including symptoms, review of systems, medical history, and substance use-and is unremarkable except for a family history of sudden cardiac death.  Autopsy is performed and myocardial histology is shown in the image.  Complete genotyping is most likely to reveal a pathologic mutation that affects a protein belonging to which of the following structures?

A)Cell junction complex
B)Ion channel
C)Mitochondria
D)Nuclear envelope
E)Sarcomere

Question 78

A 42-year-old woman is brought to the hospital due to right-sided weakness and difficulty speaking.  She has a longstanding history of a diastolic murmur, but her medical follow-up has been poor.  She does not use tobacco, alcohol, or illicit drugs.  A CT scan of the brain reveals a large ischemic stroke involving the left middle cerebral artery distribution.  The patient dies 2 days later due to progressive neurologic deterioration.  At autopsy, exploration of the left atrium shows diffuse fibrous thickening and distortion of the mitral valve leaflets, commissural fusion at the leaflet edges, and narrowing of the mitral valve orifice.  This finding is most likely the result of which of the following conditions?

A)Congenital heart disease
B)Degenerative valvular calcinosis
C)Infective endocarditis
D)Late syphilis
E)Rheumatic fever
F)Rheumatoid arthritis
G)Rupture of the papillary muscle

Question 79

A 55-year-old man comes to the office for follow-up of abnormal serum chemistries found on routine laboratory testing.  He has a history of hypertension, for which he is being treated with pharmacologic therapy, weight loss, and dietary salt restriction.  Blood pressure is 130/80 mm Hg and pulse is 80/min.  BMI is 27 kg/m².  Physical examination reveals no abnormalities.  Laboratory studies are as follows: Which of the following is most likely responsible for these findings?

A)Familial hypocalciuric hypercalcemia
B)Hypothyroidism
C)Medication effect
D)Primary hyperparathyroidism
E)Secondary hyperparathyroidism

Question 80

A 53-year-old man comes to the emergency department due to fever and progressive weakness over the last 2 weeks.  Yesterday, he also developed shortness of breath.  The patient emigrated from Eastern Europe 2 years ago and says he was diagnosed with "heart disease" in the distant past, but he does not recall any details.  He does not use tobacco, alcohol, or illicit drugs.  Despite receiving appropriate medical care, the patient expires during hospitalization.  On autopsy, gross examination of his heart shows large, friable masses on the mitral valve with extensive destruction of cuspal tissue. Which of the following is the most likely predisposing factor for this patient's presenting condition? A)Myocardial hypertrophy B)Myocardial thinning and fibrosis C)Pericardial effusion D)Rupture of chordae tendineae E)Valvular inflammation and scarring