Path Heart

A 17-year-old boy complains of pain in his legs when he runs more than 400
meters. Physical examination shows +1 dorsalis pedis pulses and +4 radial
pulses. Blood pressure is 160/96 mm Hg in the upper extremity and 130 mm
Hg in the lower extremity. A high-pitched, blowing early diastolic murmur is
heard in the right second intercostal space, and it does not increase in
intensity with deep, held inspiration. An arterial blood gas shows a normal
Pao2 and oxygen saturation. Which of the following lesions is most likely
present?

A Aortic dissection
.
B Aortic valve stenosis
.
C Patent ductus arteriosus
.
D Postductal coarctation of
. the aorta
E. Takayasu’s arteritis

QID: 108249

Option D (Postductal coarctation of the aorta) is correct. Strong

pulses in the upper extremities and diminished pulses in the lower
extremities associated with pain in the legs with running (claudication) are
indicative of postductal coarctation of the aorta. Proximally there may be
dilation of the aorta leading to aortic regurgitation (early diastolic murmur).
The blood pressure and pulse amplitude is increased in the upper extremities
from increased blood flow into the upper extremity vessels. There is at least
a 10 mm Hg drop in blood pressure in the lower extremities. Systemic
hypertension is due to reduced renal blood flow leading to activation of the
renin-angiotensin-aldosterone system. Aldosterone causes renal retention of
sodium, and angiotensin II constricts the peripheral resistance arterioles.
There is no shunting of blood between chambers of the heart and arterial
blood gases are normal. Collateral vessels develop between the anterior and
posterior intercostal arteries and between the superior epigastric artery and
inferior epigastric arteries to bypass the area of constriction in aorta. The
collateral circulation between the anterior and posterior intercostal arteries
leads to rib notching, which is visible on chest radiographs (see Fig. 10-12F
in Rapid Review Pathology, 3rd edition).

Option A (Aortic dissection) is incorrect. Aortic dissection occurs in

elderly men with hypertension or in young individuals with defects in
collagen (e.g., Ehlers-Danlos syndrome) or elastic tissue (Marfan syndrome).
Blood enters an intimal tear and dissects proximally and/or distally through
the weakened media of the aorta. Aortic dissections produce pain that
radiates to the back and are often associated with absent (not increased)
upper extremity pulses.

Option B (Aortic valve stenosis) is incorrect. Aortic valve stenosis

causes a systolic ejection murmur (not diastolic murmur). The pulse
amplitude in the upper extremities is the same as that in the lower
extremities.

Option C (Patent ductus arteriosus) is incorrect. Patent ductus

arteriosus causes a continuous machinery-like murmur. The pulse amplitude
in the upper extremities is the same as that in the lower extremities.

Option E (Takayasu’s arteritis) is incorrect. Takayasu’s arteritis is an

elastic artery vasculitis that occurs in young Asian women. Pulses in the
upper extremities are absent (not increased).

2. A 65-year-old man on the 5th day of hospitalization for an acute anterior

myocardial infarction has recurrence of chest pain and an increase in both
creatine kinase (CK)-MB and troponin I and T. Examination of the heart and
lungs is normal. Which of the following is most likely responsible for the
laboratory test abnormalities?

A Myocardial rupture
.
B Papillary muscle
. dysfunction
C Reinfarction
.
D Right ventricular
. infarction
E. Ventricular aneurysm
QID: 108387

Option C (Reinfarction) is correct. CK-MB isoenzymes, a marker for acute

myocardial infarction, are usually gone by 3 days. Therefore, reappearance
of CK-MB after 3 days indicates reinfarction or further extension of an
existing myocardial infarction. Troponins I and T are the gold standard for
diagnosing an acute myocardial infarction. However, troponin I remains
increased for a week, while troponin T remains increased for 10 to 14 days;
therefore, they cannot be used to diagnose a reinfarction.

Option A (Myocardial rupture) is incorrect. Rupture of the myocardium

either produces a murmur (e.g., mitral regurgitation from posteromedial
papillary muscle infarction) or cardiac tamponade with muffling of the heart
sounds and jugular neck vein distention and muffled heart sounds. Neither of
these findings are present in the patient.

Option B (Papillary muscle dysfunction) is incorrect. The

posteromedial papillary muscle is supplied by the right coronary artery.
Dysfunction or infarction due to thrombosis of the artery should produce the
pansystolic murmur of mitral valve regurgitation. Cardiac examination is
normal in the patient.

Option D (Right ventricular infarction) is incorrect. The right coronary

artery supplies the right ventricle. Infarction of the right ventricle produces
signs of right-sided heart failure, which include neck vein distention and the
murmur of tricuspid valve regurgitation. These findings are not present in the
patient.

Option E (Ventricular aneurysm) is incorrect. Ventricular aneurysms are

a late finding in an acute myocardial infarction. They produce a precordial
bulge with systole due to blood entering the aneurysm and expanding it out.
They do not produce reappearance of CK-MB.

3. The figure shows a transverse section of the left and right ventricle from
the enlarged heart of a 55-year-old man. Which of the following clinical
disorders is most likely responsible for the changes in the left ventricle?
From Kumar V, Fausto N, Abbas A: Robbins and Cotran’s Pathologic Basis of
Disease, 7th ed. Philadelphia, WB Saunders, 2004, pp 561, Fig. 12-3A.

A Acute myocardial
. infarction (MI)
B Aortic valve regurgitation
.
C Dilated cardiomyopathy
.
D Essential hypertension
.
E. I Mitral stenosis

QID: 107612

Option D (Essential hypertension) is correct. Cardiac muscle undergoes

hypertrophy (increase in size) as an adaptation to an increase in workload.
This occurs when muscle contracts against an increase in afterload
(resistance the heart must overcome to pump blood out of the heart) or
preload (increased volume of blood that the ventricle must eject). Sustained
pressure in the ventricles increases wall stress, which produces changes in
gene expression. Changes in gene expression result in duplication of
sarcomeres, which are the contractile elements of muscle. Changes in gene
expression related to an increase in afterload result in concentric thickening
of the ventricular wall, which is present in this patient. Muscle thickening is
due to sarcomere duplication parallel to the long axes of the cells. Essential
hypertension is the most common cause of concentric hypertrophy. The
increase in afterload is in the peripheral resistance arterioles, which are
vasoconstricted in hypertension.
Option A (Acute myocardial infarction (MI)) is incorrect. The gross
manifestation of an acute MI is a pale infarction of cardiac muscle, which is
not present in this heart.

Option B (Aortic valve regurgitation) is incorrect. Improper closing of

the aortic valve leads to volume overload in the left ventricle as blood drips
back into the left ventricle in diastole. This increases preload in the left
ventricle, which causes dilation and hypertrophy of the left ventricle.
Changes in gene expression related to an increase in preload result in
lengthening of the muscle due to sarcomeres replicating in series.

Option C (Dilated cardiomyopathy) is incorrect. Dilated

cardiomyopathy is characterized by generalized enlargement of the heart
with dilation of atria and ventricles, which are not evident in this heart.

Option E (I Mitral stenosis) is incorrect. Stenosis of the mitral valve

leads to problems in opening the valve. Because less blood enters the left
ventricle, the workload on the ventricle is reduced; hence, there is no
stimulus for left ventricular hypertrophy.

4. A 62-year-old man complains of fatigue. Physical examination shows a

harsh systolic ejection murmur, grade 4/6, that radiates into the carotid
arteries. Laboratory studies show a mild microcytic anemia. A urine dipstick
test is positive for blood. The photograph shows a representative section of
the peripheral blood smear. Which of the following laboratory studies would
most likely identify the cause of the microcytic anemia?

From Goldman L, Ausiello, D: Cecil's Medicine, 23rd ed, Saunders Elsevier,

2008, p 1174, Fig. 161-10.

A Direct Coombs’ test

.
B Enzyme assay for pyruvate
. kinase
 C Hemoglobin
. electrophoresis
D Osmotic fragility test
.
E. Serum ferritin test

QID: 108047

Option E (Serum ferritin test) is correct. The systolic ejection murmur

indicates aortic stenosis, and the fragmented RBCs (schistocytes) in the
peripheral blood indicate the presence of a microangiopathic hemolytic
anemia due to intravascular destruction of the RBCs as they hit the stenotic
and dystrophically calcified valve. The damaged cells release hemoglobin
directly into the blood. Haptoglobin, a protein synthesized in the liver,
combines with the free hemoglobin to form complexes that are
phagocytosed by macrophages in the spleen and completely degraded
causing very low to absent serum haptoglobin levels. The excess hemoglobin
in the plasma is then filtered into the urine producing a red color and a
positive dipstick for blood. Chronic hemoglobinuria eventually causes iron
deficiency and a microcytic anemia. Serum ferritin is the best screening test
for iron deficiency. Since ferritin is a soluble iron-binding protein, some
ferritin leaks out of bone marrow macrophages and directly reflects the
ferritin stores in the bone marrow. In iron deficiency, ferritin stores are
decreased; therefore, serum ferritin is also decreased. Aortic stenosis is the
most common cause of a hemolytic anemia associated with schistocytes.

Option A (Direct Coombs’ test) is incorrect. The direct Coombs’ test

detects the presence of IgG and/or C3b on the surface of RBCs. It is useful
when autoimmune hemolytic anemia is suspected. Autoimmune hemolytic
anemia is usually normocytic; however, it would not explain the presence of
schistocytes in the peripheral blood and aortic stenosis.

Option B (Enzyme assay for pyruvate kinase) is incorrect. When

anemia is caused by pyruvate kinase deficiency, the peripheral blood smear
shows dehydrated RBCs with thorny projections, unlike the cells shown in the
photograph. In addition, pyruvate kinase deficiency produces a normocytic
(not microcytic) anemia.

Option C (Hemoglobin electrophoresis) is incorrect. Hemoglobin

electrophoresis detects changes in the concentration of normal and
abnormal forms of hemoglobin, such as hemoglobin S in sickle cell disease.

Option D (Osmotic fragility test) is incorrect. The osmotic fragility test

is used to confirm a diagnosis of hereditary spherocytosis, in which the
osmotic fragility of RBCs is increased. Hereditary spherocytosis is a
normocytic anemia and spherocytes are present in the peripheral blood not
schistocytes.

5. A 28-year-old man with a history of intravenous drug abuse died of

complications related to septicemia. He had a history of fever and a
pansystolic murmur at the apex that did not increase in intensity on deep,
held inspiration. The photograph shows a valvular lesion that was present at
autopsy. Which of the following valvular disorders and pathogen is the most
likely cause of death?

From Damjanov I, Linder J: Pathology: A Color Atlas. St. Louis, Mosby, 2000, p
11, Fig. 1-16.

A Aortic regurgitation and Staphylococcus

. aureus
B Mitral regurgitation and S. aureus
.
C Mitral stenosis and Streptococcus viridans
.
D Tricuspid regurgitation and S. aureus
.
E. Tricuspid regurgitation and Staphylococcus
epidermidis

QID: 108027
Option B (Mitral regurgitation and S. aureus) is correct. The patient
had acute bacterial endocarditis (ABE; fever, septicemia, heart murmur)
involving the mitral valve. Mitral valve regurgitation produces a pansystolic
murmur that does not increase in intensity with deep, held inspiration. The
mitral valve in the photograph shows bulky vegetations (see arrow) located
along the margin or line of closure of the valve. S. aureus is the most
common pathogen in acute infective endocarditis associated with
intravenous drug abuse. The tricuspid valve is the most common valve
involved followed by the aortic and mitral valve.

Option A (Aortic regurgitation and Staphylococcus aureus) is

incorrect. Aortic regurgitation is associated with a high-pitched diastolic
blowing murmur that occurs immediately after S2. As with all left-sided
valvular murmurs and abnormal heart sounds, it does not increase in
intensity with deep, held inspiration.

Option C (Mitral stenosis and Streptococcus viridans) is incorrect.

The heart sounds associated with mitral stenosis begin with an opening snap
in early of mid diastole followed by a mid-diastolic rumbling murmur.
Bacterial endocarditis that is not associated with intravenous drug abuse is
most often due to S. viridans, which is a pathogen that can only seed a
previously damaged valve.

Option D (Tricuspid regurgitation and S. aureus) is incorrect.

Although the tricuspid valve is the most common valve involved in ABE due
to intravenous drug abuse, the pansystolic murmur most often located along
the left parasternal border increases on deep, held inspiration, which
distinguishes it from mitral regurgitation.(The negative intrathoracic pressure
increases on deep inspiration and draws blood into the right side of the heart
causing all heart murmurs and abnormal heart sounds to increase in
intensity.)

Option E (Tricuspid regurgitation and Staphylococcus epidermidis) is

incorrect. S. epidermidis is the most common cause of ABE associated with
prosthetic heart valves.

6. A mother notes in her 1-month-old baby girl clear fluid in the umbilicus.
She brings this to the attention of her pediatrician, who not only verifies the
presence of the fluid but also palpates a midline mass. At surgery a cystic
mass is noted that connects to the urinary bladder. What is the most likely
diagnosis?
 A. A

B. B

C. C

D. D

E. E

F. F

QID: 108417

Option D is correct. In congestive heart failure, the cardiac output is

decreased and the kidney reabsorbs a slightly hypotonic fluid producing
hyponatremia (↓ serum Na+ = ↑TBNa+/↑↑TBW). An increase in total
body sodium (TBNa+) is clinically manifested by weight gain, dependent
pitting edema, and body effusions. Since POsm and serum Na+ parallel each
other, hyponatremia produces a decreased POsm; therefore, the overall height
of the interrupted squares is decreased. Since excess fluid is being added to
the ECF compartment (primarily the interstitial space), the ECF compartment
is expanded. Hyponatremia establishes an osmotic gradient causing water to
shift from the ECF into the ICF compartment causing expansion of that
compartment as well. This emphasizes the importance of restricting water
and sodium in heart failure and using diuretics to eliminate excess sodium
and water. Other edema states that can be associated with schematic D are
cirrhosis and the nephrotic syndrome.

Option A is incorrect. A represents an isotonic loss of fluid (e.g., secretory

diarrhea in cholera and traveler’s diarrhea; loss of whole blood). Serum
sodium remains normal (also POsm) when equal amounts of water and sodium
are lost (↔serum Na+ = ↓TBNa+/↓TBW). Only the ECF compartment is
contracted. A decrease in TBNa+ produces signs of volume depletion
(hypotension, dry mucous membranes, tenting of the skin). There is no
osmotic gradient; therefore, the ICF compartment is normal.

Option B is incorrect. Schematic B occurs when there is a hypertonic loss

of fluid (e.g., diuretics, Addison’s disease, 21-hydroxylase deficiency). Loss of
hypertonic fluid produces a hyponatremia (↓serum Na+ =
↓↓TBNa+/↓TBW). Since fluid is lost, the ECF compartment is contracted.
Hyponatremia establishes an osmotic gradient; therefore, water shifts into
the ICF compartment.

Option C is incorrect. Schematic C occurs when there is an isotonic gain of

fluid (e.g., excessive infusion of normal (0.9%) saline. The serum
sodium remains normal (↔serum Na+ = ↑TBNa+/↑TBW); therefore,
the POsm is normal. Since fluid is being added to the ECF, it is expanded.
There is no osmotic gradient; therefore, the ICF remains normal. The
treatment is diuretics.

Option E is incorrect. Schematic E occurs when there is a hypotonic loss of

sodium (e.g., sweating, osmotic diuresis, glucosuria). The serum sodium is
increased (hypernatremia; ↑ serum Na+ = ↓TBNa+/↓↓TBW), causing
an increase in POsm and an increase in the height of the interrupted squares.
Since fluid is being lost, the ECF compartment is contracted. Hypernatremia
establishes an osmotic shift; therefore, water moves out of the ICF
compartment.
F is incorrect. Schematic F occurs when there is a hypertonic gain of sodium
(e.g., excessive infusion of sodium bicarbonate; infusion of a sodium
containing antibiotic). The serum sodium is increased (hypernatremia; ↑
serum Na+ = ↑↑TBNa+/↑TBW), causing an increase in POsm and an
increase in the height of the interrupted squares. Since fluid is being added,
the ECF compartment is expanded (pitting edema; body effusions).
Hypernatremia establishes an osmotic shift; therefore, water moves out of
the ICF compartment.
7. A 48-year-old man is admitted to the hospital for an acute myocardial
infarction (MI). Four days later, he suddenly becomes short of breath.
Physical examination shows a pansystolic murmur at the apex that does not
increase in intensity with deep, held inspiration. Bibasilar inspiratory crackles
are heard in both lung bases. There is no neck vein distention. Laboratory
studies show absence of serum creatine kinase MB (CK-MB) and increased
serum troponin-I (cTnI) and troponin-T (cTnT). Which of the following
complications has most likely occurred in this patient?

A Anterior wall rupture

.
B Interventricular septum rupture
.
C Posterior wall rupture
.
D Posteromedial papillary muscle
. rupture
E. Rupture of a ventricular
aneurysm

QID: 108244

Option D (Posteromedial papillary muscle rupture) is correct. The

posteromedial papillary muscle in the left ventricle is supplied by the right
coronary artery (RCA) (see Fig. 10-5, Rapid Review Pathology, 3rd edition).
Rupture of this muscle causes acute mitral regurgitation (pansystolic
murmur at the apex that does not increase in intensity with deep, held
inspiration), which leads to left-sided heart failure (pulmonary edema with
bibasilar inspiratory crackles). Serum CK-MB peaks in 24 hours and returns to
normal within 72 hours; therefore, it is not expected to be increased on day
4. Both cTnI and cTnT peak in 24 hours and return to normal within 7 to 10
days, which is an expected finding.

Option A (Anterior wall rupture) is incorrect. The entire anterior portion

of the heart and the anterior two-thirds of the interventricular septum is
supplied by the left anterior descending (LAD) coronary artery. Rupture of
the anterior wall causes cardiac tamponade and rapid death. There are no
murmurs with this type of rupture.

Option B (Interventricular septum rupture) is incorrect. The entire

anterior portion of the heart and the anterior two-thirds of the
interventricular septum are supplied by the LAD coronary artery. Rupture of
the interventricular septum causes a systolic murmur with a left-to-right
shunt, resulting in right-sided heart failure. Signs of right-sided heart failure
are not present in this patient (e.g., neck vein distention, dependent pitting
edema).

Option C (Posterior wall rupture) is incorrect. The entire posterior

portion of the left ventricle, the posterior one-third of the interventricular
septum, and the right ventricle are supplied by the RCA. Rupture of the
posterior wall of the heart is uncommon and causes cardiac tamponade and
rapid death. It is not associated with heart murmurs.

Option E (Rupture of a ventricular aneurysm) is incorrect. Ventricular

aneurysms occur 4 to 8 weeks after a myocardial infarction. They produce a
precordial bulge that correlates with systole (not present in this patient). The
aneurysms rarely rupture because their walls are composed of scar tissue.
Congestive heart failure is the most common complication.

8. The photograph is a histologic section of a pathologic finding in the left

anterior descending coronary artery of a 56-year-old man. Which of the
following drugs is most frequently used to prevent the pathologic finding in
the coronary artery?

From Damjanov I, Linder J: Pathology: A Color Atlas. St. Louis, Mosby, 2000, p
21, Fig. 1-44.

A. Abciximab
 B. Alteplase

C. Aspirin

D. Ticlopidin
e
E. Warfarin

QID: 108035

Option C (Aspirin) is correct. The histologic section shows a thrombus

(red-colored) occluding the lumen of the coronary artery. Directly
underneath the thrombus is a fibrous cap (blue-colored lesion), the
pathognomonic lesion of atherosclerosis. Directly beneath the blue fibrous
cap is a core of necrotic material containing cholesterol (clear, needle-
shaped spaces). There is a small fissure at the edge of the fibrous cap
(disrupted plaque) that contains necrotic atheromatous debris. This debris
was responsible for initiating the formation of a platelet thrombus in the
lumen of the vessel. Aspirin is the most frequently used medication to
prevent platelet aggregation. It does so by irreversibly inhibiting platelet
cyclooxygenase activity. This prevents the production of prostaglandin H2
and its conversion to thromboxane A2 by the enzyme thromboxane synthase
in the platelet. Thromboxane A2 is a potent vasoconstrictor and platelet
aggregator.

Option A (Abciximab) is incorrect. Abciximab is a monoclonal antibody

that is directed against the glycoprotein (Gp) IIb/IIIa fibrinogen receptor on
platelets. Fibrinogen, which is later converted into fibrin, is responsible for
causing the platelet to aggregate to form a thrombus. Abciximab is very
expensive and is most often used after angioplasty or stenting to prevent
thrombus formation.

Option B (Alteplase) is incorrect. Alteplase is a recombinant tissue

plasminogen activator that is used to lyse a preexisting platelet thrombus.
Plasminogen activators cause the release of plasmin within the thrombus
which leads to cleavage of the fibrin strands holding the thrombus together.
It is not used to prevent a platelet thrombus.

Option D (Ticlopidine) is incorrect. Ticlopidine (or clopidogrel) inhibits

ADP-induced expression of platelet GpIIb:IIIa receptors, which prevents
fibrinogen binding and platelet aggregation. It is expensive and is most often
used if patients are allergic to aspirin.

Option E (Warfarin) is incorrect. Warfarin is an anticoagulant that inhibits

the formation of venous clots. Venous clots frequently develop in areas of
stasis (e.g., deep veins of the lower leg) and are produced by localized
activation of the coagulation system leading to the formation of fibrin
strands that entrap the blood that is present in the vessel lumen. Hence, the
thrombus is composed of RBCs, WBCs, and platelets held together by fibrin
strands. Hence, an anticoagulant like warfarin or heparin prevents activation
of the coagulation system and the formation of fibrin, which, in turn,
prevents a venous clot from forming. Warfarin and heparin do not prevent
platelet aggregation and the formation of a platelet thrombus.

9. A 22-year-old woman saw her physician because of a severe sore throat.

Three weeks later, she complained of fever, pains in the knees, and a rash
on the arms that consisted of a circular ring of erythema around normal skin.
Other findings on physical examination included bibasilar inspiratory
crackles, an S3 and S4 heart sound, and a pansystolic murmur at the apex
that radiated into the axilla. Both murmur and S3 and S4 heart sounds did
not increase in intensity on deep, held inspiration. Which of the following test
results is most likely present in this patient?

A Increased anti-streptokinase O (ASO)

. titer
B Normal serum anti-DNAase B titers
.
C Positive blood culture
.
D Positive serum antinuclear antibody
. test
E. Positive throat culture for
Staphylococcus aureus

QID: 108030
Option A (Increased anti-streptokinase O (ASO) titer) is correct. This
patient developed acute rheumatic fever (ARF) several weeks after a group A
streptococcal (Streptococcus pyogenes) pharyngitis. Clinical findings in ARF
include sore throat, followed within several weeks by fever, joint pain,
subcutaneous nodules, and skin changes (erythema marginatum, as in this
case). The pansystolic murmur at the apex is caused by mitral regurgitation
due to a sterile endocarditis (see Fig. 10-13 in Rapid Review Pathology, 3rd
edition), and the bibasilar inspiratory crackles and S3 and S4 heart sounds
are caused by left-sided heart failure due to myocarditis, which decreases
the force of contraction leading to a systolic dysfunction type of heart failure.
Valvular regurgitation and the myocarditis produce left ventricular volume
overload, which is responsible for the abnormal heart sounds. The S3 heart
sound is due to blood entering a volume overloaded chamber in early
diastole, while the S4 heart sound is due to blood entering a noncompliant
left ventricle in late diastole. Cardiac damage is caused by antibodies
directed against the streptococcal M proteins (virulence factor), which cross-
react with antigens in the heart and other tissues (i.e., type II
hypersensitivity reaction). Type IV hypersensitivity has also been implicated
in the disease. Blood cultures are negative, because rheumatic fever causes
immunologic damage (fibrinoid necrosis) to the heart and tissues. The serum
antistreptolysin O titers are increased and are useful in confirming the
diagnosis.

Option B (Normal serum anti-DNAase B titers) is incorrect. In ARF,

anti-DNAase B antibodies are increased, because DNAase B is an important
inflammatory enzyme associated with Streptococcus pyogenes infections
involving the skin and throat.

Option C (Positive blood culture) is incorrect. The blood culture is

negative in ARF. It is not an example of infective endocarditis.

Option D (Positive serum antinuclear antibody test) is incorrect. In

ARF, antibodies are directed against the M protein (virulence factor) of
Streptococcus pyogenes and not against nuclear antigens (e.g., DNA,
histones, acidic nuclear proteins, nucleolar antigens).

Option E (Positive throat culture for Staphylococcus aureus) is

incorrect. In ARF, the throat culture is positive for Streptococcus pyogenes,
a group A streptococcus.

10. A 62-year-old man was admitted to the hospital with severe retrosternal
chest pain that radiated down the left arm. Three days later, he developed a
ventricular arrhythmia and died. In the hours shortly after the patient first
experienced chest pain, which of the following biochemical changes would
have marked the beginning of irreversible damage to the cardiac muscle?
 A Decreased intracellular pH
.
B Decreased Na+, K+-ATPase activity
.
C Increased activity of
. phosphofructokinase
D Increased concentration of
. cytosolic Ca2+
E. Increased conversion of pyruvate
to lactate

QID: 107617

Option D (Increased concentration of cytosolic Ca2+) is correct. An

increase in cytosolic Ca2+ concentration marks the beginning of irreversible
damage to the cardiac muscle. Normally, a Ca2+ ATPase pump pumps Ca2+
out of the cytosol into the interstitial fluid. Calcium within the cytosol
activates phospholipase in the cell membrane, proteases in the cytosol, and
endonucleases in the nucleus, which produces irreversible injury and death
of the cell.

Option A (Decreased intracellular pH) is incorrect. Decreased

intracellular pH marks hypoxic change that is still reversible if O2 becomes
available to hypoxic tissue, in this case, cardiac muscle. The nicotinamide
adenine dinucleotide plus hydrogen (NADH) that is normally shuttled into the
mitochondria for adenosine-5'-triphosphate (ATP) synthesis is used in
anaerobic glycolysis to convert pyruvate to lactate, which reduces the
intracellular pH.

Option B (Decreased Na+, K+-ATPase activity) is incorrect. Decreased

Na+, K+-ATPase activity marks hypoxic change that is reversible if O2
becomes available to hypoxic tissue. During the early stages of an
myocardial infarction (MI), lack of O2 in the muscle leads to reduced
synthesis of ATP, which normally drives the ATPase pump. A dysfunctional
Na+, K+ ATPase pump causes Na+ and H2O to move into cells, causing cellular
swelling.
Option C (Increased activity of phosphofructokinase) is incorrect.
Increased phosphofructokinase activity marks hypoxic changes that are
reversible if O2 becomes available to hypoxic tissue. In the early stages of an
MI, lack of O2 in cardiac muscle causes ATP synthesis to shift to anaerobic
glycolysis in the cytosol. This triggers increased activity of
phosphofructokinase, the rate-limiting reaction of glycolysis.

Option E (Increased conversion of pyruvate to lactate) is incorrect.

Increased conversion of pyruvate to lactate occurs in anaerobic glycolysis,
which is a reversible finding if O2 becomes available to the hypoxic tissue.

11. The photograph shows the heart of a 25-year-old woman who died in a
car accident. Which of the following best describes the valvular lesion
located in the left side of the heart?

From Kumar V, Fausto N, Abbas A: Robbins and Cotran’s Pathologic Basis of

Disease, 7th ed. Philadelphia, WB Saunders, 2004, p 592, Fig. 12-23.

A Diastolic blowing murmur after S2

.
B Midsystolic click followed by a murmur
.
C Opening snap followed by a mid-diastolic rumbling
. murmur
D Pansystolic murmur located at the apex
.
E. Systolic ejection murmur located in the right second
intercostal space
QID: 108028

Option B (Midsystolic click followed by a murmur) is correct. The

photograph shows the posterior leaflet of the mitral valve being prolapsed
(projected into) the left atrium. Mitral valve prolapse (MVP) is the most
common valvular lesion in the United States. A systolic click occurs when the
valve prolapses into the left atrium during systole and is suddenly restrained
by the chordae tendineae. The murmur following the click is caused by mitral
regurgitation as blood leaks underneath the valve cusps into the left atrium.
Most patients with MVP are asymptomatic. Redundancy of the valve leaflet
(which looks like a parachute) is due to an increase of dermatan sulfate in
the valve causing myxomatous degeneration. The most serious complication
of MVP is rupture of the chordae leading to acute mitral regurgitation and
left-sided heart failure.

Option A (Diastolic blowing murmur after S2) is incorrect. A diastolic

blowing murmur in the left side of the heart that occurs after S2
characterizes aortic regurgitation. Regurgitation murmurs are problems with
closing of the valve; hence, blood dripping back into the left ventricle in early
diastole is responsible for the murmur.

Option C (Opening snap followed by a mid-diastolic rumbling

murmur) is incorrect. An opening snap followed by mid-diastolic rumbling
murmur characterizes mitral stenosis. Recall that stenosis murmurs are
problems with opening of the valve; hence, localizing the murmur of mitral
stenosis in diastole. In mitral stenosis, the leaflets of the mitral valve are
fibrotic and frequently calcified. It is most often caused by recurrent attacks
of rheumatic fever over a period of time ranging from 2–10 years.

Option D (Pansystolic murmur located at the apex) is incorrect. A

pansystolic murmur located at the apex characterizes mitral regurgitation.
Since there is no obstruction to blood flow, the blood redirected into the left
atrium produces a murmur throughout systole; hence the term pansystolic.
Both the left atrium and left ventricle become volume overloaded. The mitral
regurgitation in MVP follows a systolic click and does occur throughout
systole unless there is rupture of the chordae.

Option E (Systolic ejection murmur located in the right second

intercostal space) is incorrect. A systolic ejection murmur in the right
second intercostal space characterizes aortic stenosis, in which the area of
the valvular orifice is reduced. Ejection murmurs have a diamond-shaped
appearance (crescendo-decrescendo) on a phonocardiogram. The aortic
valve is normal in this heart.

12. A febrile 30-year-old woman with systemic lupus erythematosus (SLE)

has a 4-day history of precordial chest pain that increased when she leaned
back and decreased when she leans forward. She now complains of
progressively worsening dyspnea. Physical examination shows muffled heart
sounds and neck vein distention during inspiration. Blood pressure and pulse
amplitude both decrease during inspiration. What is the most likely
diagnosis?

A Aortic dissection
.
B Constrictive
. pericarditis
C Dilated
. cardiomyopathy
D Pericarditis with
. effusion
E. Restrictive
cardiomyopathy

QID: 108242

Option D (Pericarditis with effusion) is correct. Pericarditis with

effusion is the most common cardiac manifestation of SLE. Inflammation of
serosal epithelium (e.g., pericardium, pleura) is a characteristic finding in
SLE. Immunocomplexes directed against the pericardium cause increased
vessel permeability and leakage of a fibrinous exudate onto the serosal
surface, producing a friction rub heard over the precordium during filling and
emptying of the heart (see Fig. 2-7 in Rapid Review Pathology, 3rd edition).
Fluid accumulates in the pericardial sac producing a “water bottle”
configuration on a chest x-ray (see Fig. 10-24 in Rapid Review Pathology, 3rd
edition). This prevents filling of the right side of the heart during inspiration
(neck vein distention), when venous blood is normally drawn into the right
side of the heart by the increasing negative intrathoracic pressure.
Decreased right-sided filling causes a decrease in cardiac output, resulting in
decreased pulse amplitude and blood pressure during inspiration as well; this
is called pulsus paradoxus. The pain caused by inflammation of the
pericardium is aggravated by leaning back and is relieved by leaning
forward. Fluid must be removed from the pericardial sac by paracentesis or
death will occur.

Option A (Aortic dissection) is incorrect. Aortic dissection occurs in

elderly men with hypertension or young individuals with defects in collagen
(Ehlers-Danlos syndrome) or elastic tissue (Marfan syndrome). Blood enters
an intimal tear and dissects proximally and/or distally through the weakened
media of the aorta. Pain radiates into the back and is often associated with
absent upper extremity pulses.

Option B (Constrictive pericarditis) is incorrect. Constrictive

pericarditis is most often idiopathic or a complication of tuberculosis.
Incomplete filling of the cardiac chambers is caused by thickening of the
parietal pericardium. Constrictive pericarditis is not a complication of the
pericarditis associated with SLE.

Option C (Dilated cardiomyopathy) is incorrect. Dilated

cardiomyopathy is associated with an enlarged heart with dilation of atria
and ventricles as well as biventricular congestive heart failure. Signs of right-
sided heart failure (e.g., dependent pitting edema) or left-sided heart failure
(e.g., pulmonary edema) are not present in this patient.

Option E (Restrictive cardiomyopathy) is incorrect. Restrictive

cardiomyopathy is characterized by decreased ventricular compliance
(decreased filling of the heart) caused by an infiltrative disease of the
myocardium (e.g., amyloid). Pericardial effusions are not usually present.

13. A 28-year-old man has fever, fatigue, difficulty breathing, and substernal
chest pain while walking or at rest. The patient has a history of alcohol
abuse. Physical examination shows bibasilar inspiratory crackles, distention
of the jugular neck veins, hepatomegaly, and dependent pitting edema.
Pansystolic murmurs along the left parasternal border and apex are present
as well as S3 and S4 heart sounds. A chest radiograph shows generalized
enlargement of all chambers and alveolar infiltrates in the lungs. The
ejection fraction was 10% (normal ≥ 55%). Laboratory studies
reveal an increase in cardiac-specific troponins and creatine kinase (CK)-MB.
The photograph shows a histologic section of myocardial tissue from a
subendocardial biopsy. Which of the following is the most likely cause of the
heart disease?
From Damjanov I, Linder J: Pathology: A Color Atlas. St. Louis, Mosby, 2000, p
15, Fig. 1-28.

A Acute rheumatic fever

.
B Coronary artery
. thrombosis
C Ischemic heart
. disease
D Toxin-induced
. myocarditis
E. Viral myocarditis

QID: 108026

Option E (Viral myocarditis) is correct. The histologic section shows an

extensive lymphocytic infiltrate (round nuclei) and dissolution of myocardial
fibers, which are characteristic of a viral-induced acute myocarditis. Clinical
findings include left-sided heart failure (dyspnea, bibasilar inspiratory
crackles, alveolar infiltrates); right-sided heart failure (neck vein distention,
hepatomegaly, dependent pitting edema); tricuspid and mitral valve
regurgitation (pansystolic murmur due to dilated valve rings) with S3 and S4
heart sounds related to volume increases in both ventricles; a decreased
ejection fraction (systolic dysfunction due to decreased contractility); and
myocardial damage (increased cardiac-specific troponin levels and CK-MB).
Coxsackievirus is the most common viral cause of myocarditis. In this
patient, the myocarditis has produced dilated (congestive) cardiomyopathy.
This patient will likely require a cardiac transplantation.
Option A (Acute rheumatic fever) is incorrect. A patient with rheumatic
fever would have a history of group A streptococcal infection (usually
pharyngitis) and other features of acute rheumatic fever including
polyarthritis, subcutaneous nodules, and erythema marginatum.

Option B (Coronary artery thrombosis) is incorrect. Histologic features

of thrombosis leading to a myocardial infarction include coagulation necrosis
(loss of nuclei and cross-striations) and a neutrophilic infiltrate, neither of
which are present in the histologic section.

Option C (Ischemic heart disease) is incorrect. Chronic ischemic heart

disease is associated with patchy replacement of myocardial tissue by
collagen.

Option D (Toxin-induced myocarditis) is incorrect. There is nothing in

the history to suggest toxin exposure (e.g., diphtheria toxin, alcohol, drugs);
hence, this is an unlikely cause of the dilated cardiomyopathy.

14. A 55-year-old woman with a history of chronic rheumatic heart disease

suddenly developed weakness and sensory changes in the right side of the
face and right upper extremity. On auscultation, an opening snap and mid-
diastolic rumble located at the apex of the chest was heard. Pulse was
irregular, and there was a loss of a wave in the jugular venous pulse. On day
3 of hospitalization, the patient had cardiorespiratory arrest and died. The
photograph shows the gross appearance of the brain at autopsy. Which of
the following underlying conditions best explains the cause of the lesion?

From Damjanov I, Linder J: Pathology: A Color Atlas. St. Louis, Mosby, 2000, p
408, Fig. 19-25B.
 A Cerebral
. atherosclerosis
B Embolism from the
. heart
C Metastatic
. carcinoma
D Ruptured berry
. aneurysm
E. Systemic
hypertension

QID: 108174

Option B (Embolism from the heart) is correct. The patient’s mitral

stenosis (opening snap followed by a mid-diastolic rumble) with atrial
fibrillation (irregularly irregular pulse) secondary to left atrial dilation. A
thrombus most likely developed in the left atrium and embolized to the
brain, causing an infarction in the left cerebral cortex in the distribution of
the middle cerebral artery. The photograph shows a wedge-shaped
hemorrhagic infarction that extends to the surface of the brain. It is
hemorrhagic because the embolus was broken down by the fibrinolytic
system, causing reperfusion of the infarcted area of the brain.

Option A (Cerebral atherosclerosis) is incorrect. The majority of

cerebral infarctions are due to thrombosis overlying an atheromatous plaque
located in the middle cerebral artery. This produces a pale (not hemorrhagic)
infarction that extends up to the surface of the brain.

Option C (Metastatic carcinoma) is incorrect. Metastases to the brain

usually are multiple, nonhemorrhagic, and peripherally located at the
junction of the white and gray matter. The history does not suggest the
presence of an underlying cancer in this patient.

Option D (Ruptured berry aneurysm) is incorrect. Most ruptured berry

(saccular) aneurysms are located at the junction of the anterior cerebral
artery and anterior communicating artery. Rupture usually is into the
subarachnoid space rather than the brain parenchyma.
Option E (Systemic hypertension) is incorrect. Hypertension causes
vascular changes in small vessels, causing rupture of a vessel into the brain
parenchyma. Most intracerebral hemorrhages are located in the basal
ganglia, particularly the putamen and external capsule rather than the
periphery of the brain.

15. A 60-year-old man with angina pectoris and a 35-pack-year history of

smoking cigarettes suddenly clutches his chest and dies. He had no previous
history of a heart murmur. Which of the following findings is most likely
present at autopsy?

A Aortic dissection
.
B Coronary artery thrombosis
.
C Hypertrophic cardiomyopathy
.
D Rupture of the anterior wall of the left
. ventricle
E. Severe coronary artery atherosclerosis

QID: 108246

Option E (Severe coronary artery atherosclerosis) is correct. Sudden

cardiac death is defined as death within 1 hour after the onset of symptoms
(e.g., chest pain). Severe coronary artery atherosclerosis, often involving
multiple vessels, is present at autopsy. Coronary artery thrombosis is
present in <20&percnt; of cases. Smoking is one of the most common
environmental contributors to sudden cardiac death. It often precipitates
ventricular arrhythmias.

Option A (Aortic dissection) is incorrect. Aortic dissections cause pain

radiating into the back and are not a common cause of sudden death.

Option B (Coronary artery thrombosis) is incorrect. Severe coronary

artery atherosclerosis, often involving multiple vessels, is present at autopsy
in sudden cardiac death. Coronary artery thrombosis occurs in <20&percnt;
of cases.

Option C (Hypertrophic cardiomyopathy) is incorrect. Hypertrophic

cardiomyopathy is characterized by asymmetric hypertrophy of the
interventricular septum. The conduction system in the septum is abnormal,
which may precipitate fatal arrhythmias. Although there is a variant that
occurs in the elderly, it would not be the most common cause of sudden
cardiac death.

Option D (Rupture of the anterior wall of the left ventricle) is

incorrect. Rupture of the anterior wall leading to cardiac tamponade and
death usually occurs 3 to 7 days following an acute anterior myocardial
infarction, not immediately after the onset of symptoms of chest pain.

16. A 52-year-old woman had a 10-year history of chronic left-sided and

right-sided heart failure and, most recently, pulmonary hypertension. As a
child and young adult, she had numerous episodes of pharyngitis. At
autopsy, the heart showed thickening of the mitral valve leaflets and fusion
of the commissures. There was thickening of the right ventricular wall and
atherosclerosis of the pulmonary artery. The pulmonary vessels prominently
protruded from the cut surface of the lung parenchyma. Which of the
following is the most likely cause of the valvular disease?

A Ischemic heart disease

.
B Libman-Sacks
. endocarditis
C Mitral valve prolapse
. (MVP)
D Recurrent bacterial
. endocarditis
E. Recurrent rheumatic
fever

QID: 108038
Option E (Recurrent rheumatic fever) is correct. The patient had
chronic recurrent rheumatic fever and developed mitral stenosis. Recall that
in rheumatic fever antibodies directed against the M proteins of certain
strains of group A streptococci cross-react with antigens present in cardiac
valves (most commonly the mitral valve) and other tissues. Repeated valve
inflammation leads to repair by fibrosis, dystrophic calcification, and
eventual stenosis of the valve. This leads to atrial dilation and hypertrophy
and an increase in pulmonary venous pressure resulting in pulmonary edema
and pulmonary venous hypertension. Increased pulmonary vein pressure
caused right ventricular hypertrophy and right-sided heart failure. The
combination of pulmonary hypertension and right ventricular hypertrophy is
called cor pulmonale. Gross signs of pulmonary hypertension are
atherosclerosis of the pulmonary artery and prominent vessels on cut
sections of the lungs.

Option A (Ischemic heart disease) is incorrect. Ischemic heart disease

produces heart failure with dilation of the mitral and tricuspid valve rings
leading to regurgitation murmurs; however, there is no damage to the valves
leading to mitral or tricuspid stenosis.

Option B (Libman-Sacks endocarditis) is incorrect. Libman-Sacks

endocarditis is the most common valvular disease in systemic lupus
erythematosus. Sterile vegetations occur on the mitral valve and chordae
leading to valve dysfunction characterized by mitral regurgitation rather than
mitral stenosis.

Option C (Mitral valve prolapse (MVP)) is incorrect. MVP, where there

is redundancy of the valve tissue due to myxomatous degeneration, is more
likely to be associated with mitral regurgitation than mitral stenosis.

Option D (Recurrent bacterial endocarditis) is incorrect. Recurrent

bacterial endocarditis is more likely to produce mitral regurgitation rather
than mitral stenosis, because the bacteria destroy the valve leaflet and often
cause rupture of the chordae tendineae.

17. A 55-year-old man with a lengthy history of chronic ischemic heart

disease has difficulty breathing. Physical findings include bibasilar inspiratory
crackles, distention of the neck veins, and tender hepatomegaly. The
photograph shows the left ankle after finger pressure was applied to the
skin. Which of the following chemical alterations is most likely operative in
this patient?
From Forbes C, Jackson W: Color Atlas and Text of Clinical Medicine, 2nd ed.
St. Louis, Mosby, 2003, Fig. 5-8.

A Decreased plasma renin activity

.
B Decreased serum concentration of antidiuretic
. hormone (ADH)
C Decreased serum concentration of atrial natriuretic
. peptide (ANP)
D Decreased total body sodium concentration
.
E. Increased serum concentration of aldosterone

QID: 108004

Option E (Increased serum concentration of aldosterone) is correct.

Increased serum concentration of aldosterone (secondary aldosteronism) is
partly responsible for retention of sodium in this patient and the presence of
pitting edema (slide). Pitting edema is due to an alteration in Starling’s
pressure (increased in plasma hydrostatic pressure and/or decrease in
oncotic pressure). Sodium retention increases plasma hydrostatic pressure
and decreases oncotic pressure (dilutes serum albumin due to an excess in
plasma volume). The fluid that accumulates in the interstitial tissue is
protein-poor and cell-poor (i.e., transudate). The patient has signs and
symptoms of both left-sided heart failure (dyspnea and bibasilar inspiratory
crackles) and right-sided heart failure (distention of neck veins,
hepatomegaly, and dependent pitting edema). Right-sided heart failure
causes a backup of blood into the venous system, which increases the
hydrostatic pressure and is the primary factor causing the pitting edema. In
biventricular heart failure, decreased cardiac output decreases renal blood
flow, which activates the renin-angiotensin-aldosterone (RAA) system, which
leads to an increase in the serum concentration of aldosterone.

Option A (Decreased plasma renin activity) is incorrect. In

biventricular heart failure, decreased cardiac output activates the RAA
system and leads to an increase (not decrease) in plasma renin activity.

Option B (Decreased serum concentration of antidiuretic hormone

(ADH)) is incorrect. In biventricular heart failure, a decrease in cardiac
output automatically stimulates the release of ADH from the posterior
pituitary gland, leading to an increase (not decrease) in serum concentration
of the hormone. ADH increases renal reabsorption of free water (water
without electrolytes) and produces hyponatremia, which would have been
present in this patient.

Option C (Decreased serum concentration of atrial natriuretic

peptide (ANP)) is incorrect. Biventricular failure causes volume overload
in both atria. When either atrium is dilated with blood, ANP is released, with
a subsequent increase (not decrease) in the serum concentration. ANP has a
diuretic effect in the kidneys, which helps the kidneys eliminate sodium.
Brain natriuretic peptide would also be increased because of distention of the
ventricles.

Option D (Decreased total body sodium concentration) is incorrect.

In biventricular heart failure, decreased cardiac output activates the RAA
system. The increase in aldosterone is partly responsible for retention of
sodium in this patient and contributes to an increase (not decrease) in total
body sodium, which is invariably present in the pitting edema states (e.g.,
right-sided heart failure, cirrhosis, nephrotic syndrome). The primary source
for the increase in total body sodium is increased renal reabsorption of
sodium in the proximal tubules of the kidneys.

18. A 58-year-old man had a sudden onset of difficulty breathing and

substernal chest pain with radiation down the inner aspects of both arms
that lasted for a few hours. The patient subsequently died in a coronary care
unit. The photograph shows a cross-section of the left and right ventricles.
The anterior portion of the heart is at the top. Based on the gross
appearance of the infarction and location, which of the following would most
likely have been present on the day of his death?

From Damjanov I, Linder J: Pathology: A Color Atlas. St. Louis, Mosby, 2000, p
22, Fig 1-47.

A Increased serum creatine kinase

. (CK)-MB
B Increased serum troponins I and
. T
C Q waves in leads I and aVL
.
D Q waves in leads I, V4−V6, and
. aVL
E. Q waves in leads V1−V4

QID: 108029

Option B (Increased serum troponins I and T) is correct. The patient

had an acute myocardial infarction (MI) involving the posterior wall of the left
ventricle. Note the bright yellow area of infarction (coagulation necrosis) and
the rim of dark red granulation tissue (blood vessels + collagen formation)
surrounding the infarction (white arrow in photograph). These findings are
present approximately 1 week after a transmural (Q-wave) infarction. The
posterior wall of the left ventricle is supplied by the right coronary artery;
therefore, a right coronary artery thrombosis is most likely responsible for
the infarction. The serum concentration of troponins I and T begin to increase
3 to 12 hours after cardiac injury, peak within 24 hours, and return to normal
within 7 to 10 days; hence, these would have been increased at the time of
death.
Option A (Increased serum creatine kinase (CK)-MB) is incorrect.
Levels of CK-MB begin to increase 4 to 8 hours after infarction, peak in 24
hours, and return to normal within 1.5 to 3 days. Therefore, CK-MB would not
have been present at the time of death.

Option C (Q waves in leads I and aVL) is incorrect. Q waves in leads I

and aVL are present in an infarction involving the lateral wall of the left
ventricle, which is supplied by the left circumflex coronary artery.

Option D (Q waves in leads I, V4−V6, and aVL) is incorrect. Q waves in

leads I, V4–V6, and aVL occur in an anterolateral wall infarction of the left
ventricle due to thrombosis of the midportion of the left anterior descending
coronary artery or the circumflex coronary artery.

Option E (Q waves in leads V1−V4) is incorrect. Q waves in leads V1–V4

are present in an anterior wall infarction of the left ventricle and are most
often due to thrombosis of the left anterior descending coronary artery.

19. The photograph shows the cut surface of the liver removed at autopsy
from a 65-year-old woman with hepatomegaly. Which of the following is the
most likely cause of this disorder?

From Damjanov I, Linder J: Pathology: A Color Atlas. St. Louis, Mosby, 2000, p
146, Fig. 8-8.

A Alcohol abuse
.
B Biventricular heart
. failure
C Metastatic disease
.
D Portal vein
. thrombosis
 E. Viral hepatitis

QID: 108101

Option B (Biventricular heart failure) is correct. The photograph shows

a mottled, cut surface of the liver. The dark areas represent congested
central veins. These findings are indicative of centrilobular necrosis
(“nutmeg liver”), which is most often caused by left-sided heart failure (LHF)
and right-sided heart failure (RHF). LHF decreases cardiac output causing
hypoperfusion of the liver. This results in ischemic necrosis of hepatocytes
located around the central vein. RHF causes a backup of systemic venous
blood into the central veins and sinusoids, which produces congestion of
central veins and sinusoids and necrosis of hepatocytes around the central
vein. Clinical findings include painful hepatomegaly with or without jaundice.
Increased transaminases are caused by ischemic necrosis. Left untreated, it
may progress to cardiac cirrhosis with fibrosis around the central veins.

Option A (Alcohol abuse) is incorrect. Alcohol abuse most often causes a

fatty liver, which has a fatty, yellow appearance (not a mottled, cut surface).

Option C (Metastatic disease) is incorrect. Liver metastasis is

associated with nodular masses surrounded by a normal-appearing liver.

Option D (Portal vein thrombosis) is incorrect. Portal vein thrombosis

does not cause liver congestion, because the portal vein normally empties
blood into the liver. However, patients develop portal hypertension, ascites,
and splenomegaly.

Option E (Viral hepatitis) is incorrect. Viral hepatitis causes generalized

hepatomegaly; however, it is not associated with vessel congestion.

20. A 50-year-old man had an acute anterior myocardial infarction (MI) 4

days ago, and he now complains of substernal chest pain with radiation into
the left arm and jaw. Physical examination shows no cardiac or pulmonary
abnormalities. Laboratory studies show increases in serum creatine kinase
MB (CK-MB), serum troponin-I (cTnI), and serum troponin-T (cTnT). Which of
the following is the most likely cause of the chest pain?
 A Angina pectoris
.
B Pericarditis
.
C Reinfarction
.
D Right ventricular
. infarction
E. Rupture of the
anterior wall

QID: 108240

Option C (Reinfarction) is correct. After an uncomplicated acute MI,

serum CK-MB peaks in 24 hours and returns to normal within 72 hours.
Therefore, the presence of CK-MB on day 4, after an MI, indicates
reinfarction. Both cTnI and cTnT peak in 24 hours and return to normal within
7 to 10 days following an uncomplicated MI. The presence of cTnI and cTnT
on day 4 is expected.

Option A (Angina pectoris) is incorrect. Angina pectoris causes

myocardial ischemia without causing myocardial cell injury. Angina pectoris
does not lead to an increase in CK-MB, troponin-I, and troponin-T.

Option B (Pericarditis) is incorrect. Pericarditis is inflammation of the

surface lining of the heart, causing precordial chest pain and a pericardial
friction rub. Pericarditis does not damage the myocardial tissue and lead to
an increase in the cardiac enzymes.

Option C (Right ventricular infarction) is incorrect. Isolated right

ventricular infarction is extremely rare, because the blood vessels perfusing
the right ventricle are usually too small to develop occluding atheromas.
Right ventricular infarction produces signs of right-sided heart failure (neck
vein distention, dependent pitting edema), which are not present in the
patient.

Option D (Rupture of the anterior wall) is incorrect. Rupture caused by

necrosis of the myocardial wall usually occurs on days 3 to 7 following an MI.
Rupture of the anterior wall produces cardiac tamponade with muffled heart
sounds and neck vein distention followed by rapid death. The patient has a
normal cardiac exam.

21. A 55-year-old man with chronic ischemic heart disease complains of

muscle weakness. The photograph shows the ECG in the patient. Which of
the following drugs is most likely to be responsible for these findings?

From Goldman L, Bennet JC: Cecil Textbook of Medicine, 21st ed.

Philadelphia, WB Saunders, 1999, Fig 102-7.

A Aldosterone blocker
.
B β-Adrenergic
. blocker
C Calcium channel
. blocker
D Digitalis
.
E. Loop diuretic

QID: 108005

Option E (Loop diuretic) is correct. Loop diuretics are associated with

hypokalemia (decreased serum K+ concentration), which can produce both
muscle weakness and the characteristic ECG pattern of a prominent U wave
(positive wave) following the T wave. Loop diuretics cause hypokalemia by
blocking the Na+-K+-2 Cl− cotransport system in the thick ascending limb in
the renal medulla. This causes increased exchange of Na+ for K+ in the late
distal tubules and collecting tubules and increased urinary loss of K+
resulting in hypokalemia. Hypokalemia causes muscle weakness by
interfering with the normal repolarization of muscle.

Option A (Aldosterone blocker) is incorrect. An aldosterone blocker

(e.g., spironolactone) interferes with the normal exchange of Na+ for K+ in
the late distal and collecting tubules, resulting in increased urinary loss of
Na+ (diuretic effect) and retention of K+ (hyperkalemia). Hyperkalemia
produces a peaked T wave on an ECG.

Option B (β-Adrenergic blocker) is incorrect. β-Adrenergic blockers

inhibit the Na+, K+-ATPase pump, causing Na+ to move into cells and K+ out
of cells (may produce hyperkalemia). Hyperkalemia produces peaked T
waves.

Option C (Calcium channel blocker) is incorrect. Calcium channel

blockers produce sinus bradycardia. They do not produce electrolyte
abnormalities (e.g., hypokalemia).

Option D (Digitalis) is incorrect. Digitalis inhibits the Na+, K+-ATPase

pump in cardiac muscle, which normally enhances the movement of Na+ into
the muscle and the opening of calcium channels resulting in an increase in
muscle contraction. Since K+ is pumped out of the cell, hyperkalemia may
occur in digitalis toxicity.

22. The heart shown in the photograph is from a 22-year-old man, who died
suddenly while playing softball. Physical examination prior to death revealed
a systolic ejection type murmur that decreased in intensity when the patient
was supine and increased in intensity when he stood up. What is the most
likely cause diagnosis?
From Schoen FJ: Interventional and Surgical Cardiovascular Pathology:
Correlations and Basic Principles, Saunders, 1989.

A Aortic regurgitation
.
B Aortic stenosis
.
C Hypertrophic
. cardiomyopathy
D Mitral stenosis
.
E. Mitral valve prolapse
(MVP)

QID: 108229

Option C (Hypertrophic cardiomyopathy) is correct. The patient had

hypertrophic cardiomyopathy, the most common cause of sudden cardiac
death in young people. The familial form (most common) is autosomal
dominant with nearly complete penetrance. Abnormal genes are mapped to
chromosome 14, where there is usually a mutation in the myosin heavy
chain gene. There is hypertrophy of the myocardium with disproportionately
greater hypertrophy of the interventricular septum (IVS) than the free left
ventricular wall, as evident in the photograph. IVS hypertrophy may obstruct
blood flow through the outflow tract; however, most patients do not have
severe obstruction of the outflow tract. As blood exits the left ventricle, the
anterior leaflet of the mitral valve is drawn against the asymmetrically
hypertrophied IVS causing the obstruction. Aberrant myofibers are present in
the conduction system. Because the left ventricle is hypertrophied, it is
noncompliant and restricts filling. A harsh systolic ejection murmur is best
heard along the left sternal border and has a palpable double apical impulse.
Murmur intensity increases (obstruction worsens) with decreased preload
(e.g., standing up) and decreases (obstruction lessens) with increased
preload (e.g., lying supine). Increasing preload opens the outflow track.
Sudden death is due to ventricular tachycardia/fibrillation, most likely related
to the aberrant myofibers in the conduction system. β-Blockers are the
mainstay of therapy, because they prolong diastole allowing for more blood
to enter the left ventricle.
Option A (Aortic regurgitation) is incorrect. Aortic regurgitation is
characterized by an early diastolic murmur directly after the second heart
sound. The left ventricle would be dilated and hypertrophied.

Option B (Aortic stenosis) is incorrect. Aortic stenosis is characterized

by a systolic ejection murmur due to a stenotic aortic valve (normal in this
patient). Murmur intensity increases with an increase in preload (decreases
in hypertrophic cardiomyopathy) and decreases in intensity with a decrease
in preload (increases in hypertrophic cardiomyopathy). Aortic stenosis is
associated with syncope but not sudden cardiac death.

Option D (Mitral stenosis) is incorrect. Mitral stenosis is characterized

by the presence of an opening snap followed by a diastolic rumble. The
mitral valve is normal in this patient.

Option E (Mitral valve prolapse (MVP)) is incorrect. MVP produces a

midsystolic ejection click followed by a murmur. There is no redundancy of
the mitral valve leaflets in the photograph. MVP does not cause sudden
cardiac death except when associated with Marfan syndrome, where
conduction defects are often present.

23. A 65-year-old man with a history of coronary artery disease complains of

severe substernal chest pain for the past 24 hours. He states that the pain
also radiates down the left arm. A blood sample is drawn for electrolytes and
serum creatine kinase (CK) isoenzyme MB. Owing to technical difficulties in
collecting the blood, the sample is visibly hemolyzed. The serum potassium
and CK isoenzyme MB are both increased. An ECG shows a Q wave in the
anterior leads; however, there are no T-wave abnormalities present. Which of
the following correctly describes the test results?

Serum Serum CK-

potassium MB
A False True
. negative positive
B False positive False
. positive
C False positive True
. positive
D True positive False
. positive
E True positive True
. positive
 A false negative, true
. positive
B false positive, false
. positive
C false positive, true
. positive
D true positive, false
. positive
E. true positive, true
positive

QID: 108334

Option C (false positive, true positive) is correct. A true positive (TP)

test result is a positive test result in a person with disease, while a false
positive (FP) test result is a positive test result in a person without disease.
The patient has chest pain for 12 hours with radiation of the pain down the
arm and into the jaw and a Q wave in an ECG. These changes are consistent
with an acute myocardial infarction (AMI). A hemolyzed blood sample falsely
increases the serum potassium (pseudohyperkalemia), because potassium is
the major intracellular cation. Further confirmation that it is a FP test result is
that the ECG does not show peaked T waves, which is a sign of pathologic
hyperkalemia. The CK-MB is a true positive test result, because it begins
increasing approximately 6 to 8 hours after an AMI and peaks in 24 hours. It
is not falsely increased by hemolysis.

Option A (false negative, true positive) is incorrect. A false negative

(FN) test result is a negative test result in a person with disease. The serum
potassium is a FP test result (not a FN) and the serum CK-MB is correctly
classified as a TP test result.

Option B (false positive, false positive) is incorrect. The serum

potassium is correctly classified as a FP test result; however, the serum CK-
MB is a TP test result (not a FP).

Option D (true positive, false positive) is incorrect. The serum

potassium is a FP test result (not a TP), and the serum CK-MB is a TP test
result (not a FP).
Option E (true positive, true positive) is incorrect. The serum
potassium is a FP test result (not a TP), and the serum CK-MB is correctly
classified as a TP test result.

24. The photograph shows the superior view of an unopened aortic valve in a
55-year-old man. Which of the following complications commonly occurs with
this type of aortic valve lesion?

From Silver MD, Gotlieb AI, Schoen FJ: Cardiovascular Pathology, 3rd edition,
Churchill Livingstone, 2001, p. 424, Fig. 13.26B.

A Acute myocardial
. infarction (MI)
B Aortic dissection
.
C Hemolytic anemia
.
D Hypertension
.
E. Ischemic stroke

QID: 108034

Option C (Hemolytic anemia) is correct. The photograph shows a

bicuspid aortic valve with severe aortic stenosis due to fibrocalcific
involvement of the two valve cusps. A common complication of severe aortic
stenosis is an intravascular hemolytic anemia with schistocytes (fragmented
RBCs; microangiopathic hemolytic anemia), which may lead to chronic iron-
deficiency anemia from loss of hemoglobin in the urine. Serum haptoglobin
levels are often zero, because haptoglobin combines with free hemoglobin in
the plasma and is removed from the circulation by splenic macrophages.
Absent haptoglobin levels is a sign for cardiovascular surgeons to replace the
valve.

Option A (Acute myocardial infarction (MI)) is incorrect. Aortic

stenosis is the most common valvular lesion associated with angina with
exercise due to concentric hypertrophy of the left ventricle. The thickened
muscle wall does not receive sufficient blood flow during exercise resulting in
angina from subendocardial ischemia. There is no increased risk for a
myocardial infarction.

Option B (Aortic dissection) is incorrect. Aortic dissection is associated

with hypertension and connective tissue disorders leading to cystic medial
degeneration. It is not a complication of aortic stenosis.

Option D (Hypertension) is incorrect. In aortic stenosis, the diminished

area of the stenotic valve orifice eventually leads to a decrease in stroke
volume and cardiac output, which reduces systolic pressure but has no effect
on diastolic pressure. It is not a cause of hypertension.

Option E (Ischemic stroke) is incorrect. Aortic stenosis is the most

common valvular lesion associated with syncope with exercise. However, it is
not associated with an ischemic stroke, which is most often related to
atherosclerosis or embolic disease to the middle cerebral artery.

25. A 59-year-old man had an acute anterior myocardial infarction (MI). Six
weeks later, he saw his physician because of fever and precordial chest pain
that was less severe when he leaned forward. On physical examination, a
friction rub was heard over the precordium. Which of the following
mechanisms is most likely involved in the pathogenesis of abnormality in the
heart?

A Alteration in Starling’s
. pressure
B Immunologic reaction
.
C Rupture of the anterior
. wall
 D Ventricular aneurysm
.
E. Viral infection

QID: 108031

Option B (Immunologic reaction) is correct. The patient has fibrinous

pericarditis producing a pericardial friction rub (see Fig. 2-7 in Rapid Review
Pathology, 3rd edition). Because of the MI 6 weeks earlier, an immunologic
reaction is the likely cause. In Dressler’s syndrome (post–MI syndrome), the
patient develops antibodies against the pericardial tissue, which is frequently
damaged in a transmural (Q wave) infarction. Antibodies are directed against
pericardial antigens (type II hypersensitivity reaction) leading to acute
inflammation with a subsequent increase in vessel permeability and
production of a fibrinous exudate that covers the surface of the heart.
Clinical manifestations of Dressler’s syndrome include fever, precordial
friction rub, and pain that increases on inspiration but lessens when the
patient leans forward. Fibrinous pericarditis also may occur in the first week
of an acute transmural infarction. However, the pericarditis in this time
frame is due to increased vessel permeability not related to immunologic
damage of the pericardium.

Option A (Alteration in Starling’s pressure) is incorrect. An alteration

in Starling’s pressure refers to increased hydrostatic pressure or decreased
oncotic pressure within the vascular compartment. The transudate produced
by this change is poor in proteins and cells, unlike the fibrinous exudate that
is associated with a fibrinous pericarditis.

Option C (Rupture of the anterior wall) is incorrect. A rupture of the

anterior wall occurs 3 to 7 days after an acute MI, causing cardiac
tamponade and death.

Option D (Ventricular aneurysm) is incorrect. A ventricular aneurysm is

clinically recognized within 4 to 8 weeks after a transmural infarction. It
presents with a precordial bulge during systole as blood enters the aneurysm
causing anterior chest wall movement. The most common complications are
heart failure and embolization of clot material. There is no association with
fibrinous pericarditis.

Option E (Viral infection) is incorrect. Coxsackievirus is the most

common cause of pericarditis. This patient’s history of a previous MI
indicates that an immunologic cause for the pericarditis is more likely than
an infection.

26. The photograph shows a skin lesion on a mentally retarded 25-yr-old

man who also has reddish-brown papules on his nose, cheeks, and chin.
What additional findings may be present in this patient?

Courtesy of The Honickman Collection of Medical Images in memory of

Elaine Garfinkel and The Jefferson Clinical Images Collection (through the
generosity of JMB, AKR, LKB and DA)

A Acoustic neuroma
.
B Arteriovenous malformation in
. meninges
C Meningioma
.
D Renal cell carcinoma
.
E. Rhabdomyoma of the heart

QID: 108198

Option E (Rhabdomyoma of the heart) is correct. The patient has

tuberous sclerosis, an autosomal-dominant neurocutaneous disorder. It is
characterized by mental retardation, seizures beginning in infancy, adenoma
sebaceum (red-brown papules on the face), hypopigmented skin lesions
(“ash leaf” lesions; see photograph) and various hamartomatous lesions.
Astrocyte proliferations occur in the subependyma and are the cause for
seizure activity. Angiomyolipomas commonly occur in the kidneys, and
rhabdomyomas may occur in the heart (almost 100&percnt; predictive of
tuberous sclerosis).

Option A (Acoustic neuroma) is incorrect. Neurofibromatosis is also a

neurocutaneous syndrome like tuberous sclerosis and the type 2 variant is
associated with acoustic neuromas, often bilateral.

Option B (Arteriovenous malformation in meninges) is incorrect.

Sturge Weber syndrome is a neurocutaneous syndrome with a vascular
malformation of the face in a trigeminal nerve distribution and ipsilateral
arteriovenous malformation in the meninges.

Option C (Meningioma) is incorrect. Neurofibromatosis has an increase

in meningiomas in the brain. The lesions in the brain in tuberous sclerosis
are astrocyte hamartomas.

Option D (Renal cell carcinoma) is incorrect. Angiomyolipomas of the

kidney do occur in tuberous sclerosis; however, they are hamartomas and do
not progress into a renal cell carcinoma.

27. A 55-year-old man has an anterior myocardial infarction. He is placed on

aspirin, heparin, and warfarin. Which of the following sets of hemostasis
studies is most likely to be present in this patient?

Platelet Bleeding PT PT
Count Time T
A Normal · Norm ·
. al
B Normal Normal Norm ·
. al
C· · · ·
.
D Normal ↑ ↑ ↑
.
E Normal Normal ↑ ↑
.

PT, prothrombin time; PTT, partial thromboplastin time.

 A platelet count normal, bleeding time increased, PT normal, PTT
. increased
B platelet count normal, bleeding time normal, PT normal, PTT
. increased
C platelet count decreased, bleeding time increased, PT
. increased, PTT increased
D platelet count normal, bleeding time increased, PT increased,
. PTT increased
E. platelet count normal, bleeding time normal, PT increased, PTT
increased

QID: 108378

Option D (platelet count normal, bleeding time increased, PT

increased, PTT increased) is correct. In an acute anterior myocardial
infarction, aspirin is used to prevent thrombus formation either in the
coronary arteries or the damaged endothelium in the left ventricle. Aspirin
prevents platelet aggregation causing prolongation of the bleeding time
without affecting the platelet count. Patients are frequently anticoagulated
with heparin and warfarin. Heparin enhances antithrombin III activity leading
to neutralization of many of the coagulation factors including thrombin and X
in the final common pathway. This prolongs the PT and the PTT, although the
latter test is a better test to follow heparin therapy. Warfarin inhibits further
activation of the vitamin K–dependent coagulation factors (II, VII, IX, and X).
Since factors II and X are in the final common pathway, the PT and PTT are
both prolonged; however, the former test is a better test to follow warfarin
therapy when it is converted into the international normalized ratio.

Option A (platelet count normal, bleeding time increased, PT

normal, PTT increased) is incorrect. This set of studies is most often
associated with classic von Willebrand disease. In this autosomal dominant
disorder, there is a deficiency of von Willebrand factor (vWF), which is
necessary for platelet adhesion to areas of endothelial injury. This prolongs
the bleeding time without affecting the platelet count. In the circulation, vWF
also complexes with factor VIII coagulant (VIII:C), which prevents degradation
of VIII:C. Therefore, deficiency of vWF automatically leads to decreased
factor VIII:C activity and prolongation of the PTT. The PT is normal because it
does not evaluate VIII:C in the intrinsic pathway.

Option B (platelet count normal, bleeding time normal, PT normal,

PTT increased) is incorrect. PT normal, PTT increased) is incorrect. This
set of studies is consistent with a coagulation factor deficiency in the intrinsic
coagulation pathway: XII, XI, IX, or VIII. Of the four choices, factor VIII would
be the most common deficiency in a patient with hemophilia A.

Option C (platelet count decreased, bleeding time increased, PT

increased, PTT increased) is incorrect. This set of data in which all the
tests are abnormal is most often seen in disseminated intravascular
coagulation (DIC). Tissue thromboplastin activates the extrinsic coagulation
system causing the formation of fibrin thrombi within the microcirculation.
Factors I, II, V, and VIII are consumed in the fibrin clots, which results in
anticoagulation (PT and PTT are both increased). Fibrin thrombi also trap
platelets causing thrombocytopenia, which, in turn, is responsible for
producing a prolonged bleeding time.

Option E (platelet count normal, bleeding time normal, PT

increased, PTT increased) is incorrect. This set of data is most
consistent with a patient that is taking warfarin and/or heparin. Rat poison
contains warfarin, which blocks epoxide reductase rendering vitamin K
inactive. This prevents further γ-carboxylation of the vitamin K–dependent
coagulation factors: factors II (prothrombin), VII, IX, and X. Since factors X
and II are in the final common pathway, both the PT and PTT are prolonged.
The PT evaluates factors VII, X, V, II, and I (fibrinogen), while the PTT
evaluates factors XII, XI, IX, VIII, X, V, II, and I. The platelet count and
bleeding time are not affected by warfarin. Heparin enhances antithrombin III
activity, which neutralizes activated serine proteases.

28. A 75-year-old man was admitted to the hospital with severe substernal
chest pain that radiated into the left arm and jaw. On day 5 of
hospitalization, he developed acute mitral valve regurgitation and died. The
photograph shows a transverse section of the heart at autopsy with the
anterior portion of the heart at the top. Which of the following coronary
arteries was most likely responsible for the gross changes in the heart?

From Damjanov I, Linder J: Anderson’s Pathology, 10th ed. St. Louis, Mosby,
1996, p 374, Fig. 17-13.
 A Left anterior descending coronary
. artery (LAD)
B Left circumflex coronary artery
.
C Left main stem coronary artery
.
D Right coronary artery
.

QID: 107618

Option D (Right coronary artery) is correct. The patient had an acute

myocardial infarction secondary to a coronary artery thrombosis. An infarct
is the gross manifestation of coagulation necrosis that is present in
underlying tissue. The gross specimen shows an extensive pale yellow infarct
in the left ventricle, which involves the posterior wall and posteromedial
papillary muscle (the round structure projecting into the ventricular lumen)
of the mitral valve. The posteromedial papillary muscle most likely ruptured
producing the acute mitral valve regurgitation and death. This area of the
left ventricle is in the distribution of the right coronary artery. The infarct is
pale because of the increased density of myocardial tissue, which prevents
the infiltration of blood into surrounding dead tissue from necrotic blood
vessels.

Option A (Left anterior descending coronary artery (LAD)) is

incorrect. The LAD supplies the anterior portion of the left ventricle and
anterior two-thirds of the interventricular septum.

Option B (Left circumflex coronary artery) is incorrect. In general, the

left coronary artery supplies the anterior portion of the heart, not the
posterior portion of the heart.

Option C (Left main stem coronary artery) is incorrect. The left

circumflex coronary artery supplies the lateral portion of the left ventricle.

29. A 48-year-old man complains of fever and several fainting spells over the
past few months. He states that he faints when he stands up and not when
he is lying down. He also complains of pain in the left upper quadrant that is
aggravated by inspiration as well as pain in the right flank. Physical
examination shows a normal blood pressure when lying down and sitting up.
A late diastolic murmur is heard. The spleen is enlarged and tender, and a
splenic friction rub is present. There is right flank pain on percussion. A urine
dipstick is positive for blood, and RBCs are present in the urine sediment.
What is the most likely diagnosis?

A Calcific aortic stenosis

.
B Hypertrophic
. cardiomyopathy
C Left atrial myxoma
.
D Mitral stenosis
.
E. Pericardial effusion

QID: 108247

Option C (Left atrial myxoma) is correct. Cardiac myxomas are the most
common primary cardiac tumors in adults (see Fig. 10-28 in Rapid Review
Pathology, 3rd edition). Symptoms include nonspecific complaints such as
fever and malaise. The tumor has a ball-valve effect that causes sudden
blockage of blood flow through the mitral valve, resulting in episodic fainting
spells. A diastolic murmur similar to that of mitral stenosis is also present.
Peripheral embolization of tumor also occurs. Infarctions of the spleen cause
pain in the left upper quadrant and friction rubs. Infarctions of the kidneys
cause flank pain and hematuria.

Option A (Calcific aortic stenosis) is incorrect. Calcific aortic stenosis is

associated with a systolic ejection murmur. Angina with exercise occurs
because of ischemia of the subendocardium in the concentrically
hypertrophied left ventricle. The decreased cardiac output through the
stenotic valve causes syncope with exercise. Peripheral embolization does
not occur.

Option B (Hypertrophic cardiomyopathy) is incorrect. Hypertrophic

cardiomyopathy is characterized by asymmetric hypertrophy of the
interventricular septum causing findings similar to those described for
calcific aortic stenosis. Peripheral embolization does not occur.

Option D (Mitral stenosis) is incorrect. Mitral stenosis is a complication

of chronic rheumatic fever. Left atrial dilation and thrombus formation with
embolization is a common finding. The patient has no history of recurrent
rheumatic fever and physical exam does not reveal an opening snap in early
diastole in this patient.

Option E (Pericardial effusion) is incorrect. A pericardial effusion

produces muffled heart sounds and is not associated with syncope or with
peripheral embolization.

30. A 2-day-old newborn male infant with respiratory distress syndrome

(RDS) has a continuous harsh murmur that is heard over the entire
precordium. Which of the following sets of oxygen saturation (Sao2) values in
the cardiac chambers and vessels is most likely present in this patient?

R R P P L A
A V A V V 0
Normal 7 7 7 9 9 9
SaO2 5 5 5 5 5 5
A 7 7 7 9 9 9
5 5 5 5 5 5
B 7 8 8 9 9 9
5 0 0 5 5 5
C 8 8 8 9 9 9
0 0 0 5 5 5
D 7 7 8 9 9 9
5 5 5 5 5 5
E 7 7 7 9 8 8
5 5 5 5 0 0

RA, right atrium; RV, right ventricle; PA, pulmonary artery;

PV, pulmonary vein; LV, left ventricle; Ao, aorta.

A Aortic regurgitation
.
B Aortic stenosis
.
C Hypertrophic
. cardiomyopathy
 D step-up of SaO2 in pulmonary
. artery
E. Mitral valve prolapse (MVP)

QID: 108230

Option D (step-up of SaO2 in pulmonary artery) is correct. The patient

has the classic machinery murmur (continuous murmur) of a patent ductus
arteriosus (PDA). This neonate has hypoxemia (decreased arterial Po2)
secondary to RDS; therefore, closure of the ductus is not stimulated. When
oxygenated blood (Sao2 95&percnt;) is shunted into a chamber or vessel with
venous blood (Sao2 75&percnt;), there is a step-up of Sao2 (approximately
80&percnt;) in the venous blood; this is called a left-to-right shunt. Similarly,
when venous blood is shunted into a chamber or vessel with oxygenated
blood, there is a step-down of the Sao2 (˜80&percnt;) leading to clinical
cyanosis; this is called a right-to-left shunt. In PDA, there is a left-to-right
shunt causing blood to flow from the aorta (where pressure is high) through
the PDA to the pulmonary artery (where pressure is low), which causes a
step-up of Sao2 in the pulmonary artery (85&percnt; versus normal of
75&percnt;).

Option A (Aortic regurgitation) is incorrect. The patient has a heart

murmur consistent with a PDA; therefore, there should be a step-up of SaO2
in the pulmonary artery.

Option B (Aortic stenosis) is incorrect. A step-up of Sao2 in the right

ventricle (80&percnt; versus 75&percnt;) and pulmonary artery (80&percnt;
versus 75&percnt;) characterizes a ventricular septal defect (VSD), a left-to-
right shunt. VSD is the most common type of congenital heart disease. If a
VSD is not corrected, cyanosis, or Eisenmenger’s syndrome, may eventually
occur. This is due to volume overload in the right side of the heart from the
left-to-right shunt causing pulmonary hypertension and right ventricular
hypertrophy. When pressure in the right heart is greater than the pressure in
the left heart, the shunt reverses and becomes right-to-left, causing a step-
down in SaO2 in the left ventricle and aorta leading to cyanosis if SaO2 <
80&percnt;.

Option C (Hypertrophic cardiomyopathy) is incorrect. A step-up of

Sao2 in the right atrium (80&percnt;), right ventricle (80&percnt;), and
pulmonary artery (80&percnt;) characterizes an atrial septal defect (ASD,
left-to-right shunt), which is most often the result of a patent foramen ovale.
If an ASD is not corrected, cyanosis and Eisenmenger’s syndrome may
eventually occur.

Option E (Mitral valve prolapse (MVP)) is incorrect. A step-down of

Sao2 in the left ventricle (80&percnt;) and aorta (80&percnt;) characterizes
tetralogy of Fallot (right-to-left shunt), which is the most common type of
cyanotic congenital heart disease. It consists of an overriding aorta (least
common defect), VSD, pulmonary stenosis, and right ventricular
hypertrophy. The degree of pulmonic stenosis determines the severity of the
right-to-left shunt. If the stenosis is not severe, then most of the venous
blood enters the pulmonary artery and is oxygenated; hence, the patient is
often acyanotic (SaO2 > 80&percnt;). However, when the stenosis is severe,
most of the venous blood is shunted through the VSD into the left ventricle
(right-to-left shunt), leading to cyanosis. Note that the pulmonary vein has
normally oxygenated blood (95&percnt;), because once blood enters the
lungs through the stenotic pulmonic valve it is oxygenated.

31. A 50-year-old man with ischemic heart disease has signs of both left- and
right-sided heart failure. Which of the following is characteristic of both types
of heart failure?

A Bibasilar inspiratory
. crackles
B Decreased cardiac
. output
C Dependent pitting
. edema
D Jugular neck vein
. distention
E. Paroxysmal nocturnal
dyspnea

QID: 108241

Option B (Decreased cardiac output) is correct. The heart fails when it

cannot pump blood delivered to it by the venous system. Therefore, cardiac
output is decreased whether the heart failure is left-sided or right-sided.

Option A (Bibasilar inspiratory crackles) is incorrect. Bibasilar

inspiratory crackles are a sign of left-sided heart failure, a “forward” heart
failure causing a decreased cardiac output and backup of blood in the left
ventricle, left atrium, and pulmonary capillaries. Increased pulmonary
capillary hydrostatic pressure causes fluid (transudate) to enter the
interstitium of the lung and eventually the alveoli (pulmonary edema). Air
entering alveoli containing fluid produces inspiratory crackles that are best
heard at the base of both lungs.

Option C (Dependent pitting edema) is incorrect. Dependent pitting

edema is a sign of right-sided heart failure, a “backward” heart failure
causing systemic venous congestion. The increase in hydrostatic pressure in
the venous system causes fluid (transudate) to leak into the interstitial space
through the venules, leading to dependent pitting edema.

Option D (Jugular neck vein distention) is incorrect. Jugular neck vein

distention is a sign of right-sided heart failure. In right-sided heart failure,
blood builds up in the venous system causing distention of the jugular veins
(see Fig. 10-3 in Rapid Review Pathology, 3rd edition).

Option E (Paroxysmal nocturnal dyspnea) is incorrect. Paroxysmal

nocturnal dyspnea is a sign of left-sided heart failure, which occurs primarily
at night when the patient is supine in bed. At this time, gravity does not
impede blood flow to the right side of the heart, and fluid from the interstitial
space enters the venous system. Excess blood enters the failed left ventricle
and backs up into the lungs, causing pulmonary edema and dyspnea, which
awakens the patient. Symptoms resolve when the patient stands up and
gravity decreases venous return to the right side of the heart.