JAWABAN USMLE Cardiology STEP 1

1.(B) Aortic valve stenosis (AS) presents with one of the classic triad: syncope (passing out),
exertional angina, or exertional dyspnea (from heart failure). Over the age of 70 the most
likely cause is degenerative calcific aortic stenosis while under the age of 70 a bicuspid aortic
valve is the likely culprit. Rheumatic heart disease is the third leading cause. Physical
examination reveals a crescendo-decrescendo murmur at the aortic listening post (right upper
sternal border) which radiates to the carotids. The more severe the aortic stenosis the later the
peak of the murmur in systole and the softer the A2 component of the S2 heart sound. The
murmur is depicted below:

“Pulses parvus et tardus” is present upon carotid artery examination. Remember parvus
means weak and tardus means late. The murmur can radiate to the apex and sound
holosystolic mimicking mitral regurgitation (this is known as the Galiverdin phenomenon).
No medical treatment is available for aortic stenosis. Aortic valve replacement surgically is
indicated for anyone who is symptomatic.
Aortic valve regurgitation (A) would cause a diastolic murmur, but may also present with
exertional dyspnea from congestive heart failure (but not syncope or angina). Mitral valve
regurgitation (C) also can manifest as heart failure, but there would again be no syncope or
angina. The murmur of mitral regurgitation is holosystolic at the apex (remember the only
two other causes of holosystolic murmurs is tricuspid regurgitation or a ventricular sepetal
defect). Isolated mitral valve prolapse (E) causes a mid-systolic click, but no murmur unless
mitral regurgitation is also present. Mitral valve prolapse is usually asymptomatic, but can be
associated with anxiety/panic attacks and palpitations.

2. A)

Answer: A - Autosomal dominant

The image for choice A depicts an autosomal dominant inheritance pattern which is present
in about half of cases ofhypertrophic obstructive cardiomyopathy (HOCM). The other half
are sporadic. Choice B depicted an autosomal recessive pattern. Choice C an X-linked
recessive pattern.
HOCM is associated with mostly exertional symptoms. During exercise (when the heart
contracts harder), the abnormally thickened interventicular septum obstructs blood from
flowing out of the left ventricular outflow tract and aortic valve resulting in a markedly
reduced cardiac output. This leads to syncope (loss of consciousness). It can also lead to life-
threatening arrhythmias such as ventricular tachycardia and ventricular fibrillation, thus
HOCM is the most common cause of sudden death in young athletes. On histological
examination you would see the myocardial myocytes in a chaotic pattern commonly
described as "myocardial disarray", not a normal organized pattern.

3. (A) Selecting the appropriate antihypertensive regimen requires knowledge of side-effects

and contraindications to each drug class. Dihyropyridine calcium channel blockers anything
that ends in “dipine” such as amlodipine or nifedipine, work to block vascular calcium
channels resulting in vascular smooth muscle relaxation and have no specific
contraindications. They can cause peripheral edema (due to venous dilation) and dizziness.
Non-dihydropyridine calcium channel blockers (verapamil and diltiazem) work mostly on
cardiac calcium channels and thus decrease the heart rate and inotropy of the heart. They can
also be used to treat hypertension and are contraindicated in systolic congestive heart failure.
Hydrochlorothiazide (B) is not effective to treat hypertension when renal insufficiency is
present. Enalapril (C), an ACE inhibitor, is contraindicated in chronic kidney disease with a
creatinine greater than 2.5 mg/dL or a potassium greater than 5.5 mg/dL. Propranolol (D) is a
non-cardioselective beta-blocker (blocks both beta-1 and beta-2 receptors) which can worsen
asthmatic bronchoconstriction (cardioselective beta-blockers do this to a lesser degree).
Spironolactone (E), an aldosterone antagonist, is also contraindicated in renal insufficiency
and with elevated potassium levels.

4. (D) This patient has rhabdomyolysis, a condition in which the myocytes lyse releasing
their contents into the blood stream including potassium. The myoglobin causes the urine
dipstick to be positive for blood despite no red blood cells seen on microscopic examination.
Other laboratory abnormalities seen with rhabdomyolysis include elevated uric acid, low
calcium, elevated phosphorus (remember the pneumonic PUcK) and a markedly elevated
creatine kinase (CK). The only medication listed that can cause rhabdomyolysis is
rosuvastatin, a HMG-CoA reductase inhibitor used for the treatment of hyperlipidemia.
Lisinopril (A) can cause angioedema, elevated Cr, and hyperkalemia. Verapamil (B) can
cause bradycardia, congestive heart failure, and constipation. Digoxin (C) causes
gastrointestinal symptoms and cardiac arrhythmias. Hydralazine (E) causes dizziness, a reflex
tachycardia (should be used concomitantly with a beta-blocker), and rarely drug-induced
lupus erythematosis (causing positive anti-histone antibodies).

5. (C) Marfan’s syndrome is associated with mitral valve prolapse and aortic aneurysms.
Specifically, the ascending aorta may dilated and predispose patient’s to acute aortic
dissection which can be fatal. Also, when the ascending aorta dilates, the aortic valve annulus
stretches causing the valve leaflets to fail to coapt which results in aortic regurgitation.
Aortic valve stenosis (A) is not associated with Marfan’s syndrome and is caused be either
senile calcific degeneration of the valve or from a congenital bicuspid aortic valve.
Coartaction of the aorta (B) is associated with Turner’s syndrome and presents with
hypertension in the upper extremities and hypotension in the lower extremities. “Rib
notching” is seen on the chest x-ray. Ventricular septal defects (D) and Ebstein’s anomaly (E)
are not associated with Marfan’s.

6. (D) This patient has angioedema, an acute allergic reaction that frequently occurs along
with urticaria (hives). Angioedema involves the face, lips, eyes, tongue and ears and can
cause respiratory failure from obstruction of the airway. Angiotensin converting enzyme
inhibitors (ACE inhibitors) are the most common cause, however opiates, aspirin,
nonsteroidal anti-inflammatory drugs, and radiocontrast agents can cause it as well.
Pindolol (A) is a beta-blocker with intrinsic sympathomimetic activity. Pindolol can cause
bradycardia, fatigue, hypotension, and can worsen asthma. Clonidine (B) is a central active
alpha-2 agonist and can cause bradycardia, dry mouth, hypotension, and rebound
hypertension. Felodipine (C) is a non-dihydropyridine calcium channel blockerwhich can
cause dizziness, hypotension, and lower extremity swelling. Methyldopa (E) is also a
centrally active alpha-2 agonist commonly used to treat hypertension in pregnancy.
Methyldopa can cause hemolytic anemia (Coombs positive).

7. (D) This infant has a patent ductus arteriosus (PDA) which is a communication between
the pulmonary artery and the aorta. Since the blood pressure in the aorta is always higher than
that in the pulmonary artery (in both systole and diastole), blood is continuously flowing
from left (aorta) to right (pulmonary artery) causing a continuous murmur.

Most PDAs will close spontaneously within weeks to months and no intervention is needed.
If symptoms are present, a diuretic (A) or indomethacin (B) can be given to close the PDA.
Indomethacin, a nonsteroidal anti-inflammatory drug, blocks the production of prostaglandins
which are needed to keep the PDA open.

8. (B) Metoprolol is a lipid soluble beta-blocker which can cross the blood-brain barrier
easily and have been shown to be effective in the prophylaxis of migraine headaches as well
as the treatment of hypertension. They have slight sedating effects and therefore can also be
used for stage freight or panic attacks as well.
Hydrochlorothiazide (A), clonidine (B), methyldopa (D), and lisinopril (E) can treat
hypertension but have not been shown to be effective for migraine prophylaxis.

9. (B) Lidocaine (see also lidocaine toxicity), which is used to treat ventricular arrhythmias
such as ventricular tachycardia or ventricular fibrillation, can easily reach toxic levels and can
cause seizures and may progress to coma and death. Lidocaine at high levels first inhibits the
inhibitory neurons in the brain resulting in seizures. Eventually all neurons are inhibited and
coma ensues. No specific treatment or antidote exists.
Stroke (A), renal failure (B), congestive heart failure (D), and hyperkalemia (E) do not occur
with lidocaine toxicity.

10. (D) Tetralogy of fallot occurs from embryologic anterior and superior displacement of the
infundibular septum resulting in a ventricular septal defect, pulmonic valve stenosis which
leads to right ventricular hypertrophy, and an aorta which is large and accepts blood from the
right ventricle “overriding” the stenotic pulmonic valve. This results in right to left shunting
and early cyanosis (in infancy or early childhood). Chest x-ray would show a “boot shaped”
heart due to the right ventricular hypertrophy. Affected individuals may have “tet spells” in
which they may suddenly become cyanotic and pass out. Frequently affected children will
squat during these spells to increase venous return and improve right ventricular filling
resulting in more blood ejecting into the pulmonic artery to become oxygenated.

11. (E) This patient has carcinoid syndrome which consists of diarrhea, facial flushing,
reactive airways causing shortness of breath, and cardiac valvular disease specifically of
right-sided heart valves since the toxins produced by the tumor are filtered by the lungs and
never reach the left sided heart valves (unless pulmonary metastasis are present).
Aortic valve stenosis (A), aortic valve regurgitation (B), mitral valve stenosis (C), and mitral
valve regurgitation (D) are all left-sided heart valves which would not be affected in
carcinoid syndrome unless pulmonary metastasis are present (which is rare).

12. (C) During myocardial infarction, certain cardiac biomarkers are released into the
bloodstream early and others late. Myoglobin is non-specific enzyme which only takes 30
minutes to elevate in the serum after the onset of myocardial infarction. Troponin I and CK-
MB elevate 3-4 hours after onset. Troponin I will stay elevated for 7-10 days and CK-MB for
only 3-4 days, thus CK-MB is the preferred test to check for re-infarction (for example 5 days
after a prior MI).

13. (C) Coarctation of the aorta occurs when the congenital narrowing of the aorta occurs.
About two thirds of patients with coarctation of the aorta have a bicuspid aortic valve as well.
Depending on the location of the narrowing differing presentations may occur.
Infantile coarctation of the aorta presents when the stenosis is proximal or next to the ductus
arteriosus. When the ductus arteriosus closes (as it should in normal infants), a severe
increase in afterload occurs resulting in congestive heart failure (since blood was normally
able to traverse the patent ductus arteriosus resulting in lower resistance, then suddenly is
unable to).
Adult coarctation of the aorta occurs distal to the ductus arteriosus and is usually diagnosed
in the 2nd or 3rd decade of life. Patients present with hypertension and diastolic congestive
heart failure. The blood pressure in the legs (and hence pulses) are markedly lower than in the
arms. Collateral arterial circulation develops to allow blood to reach the lower extremities,
mostly in the internal mammary arteries (which give rise to the intercostals arteries). The
intercostals arteries then become enlarged due to the pressure overload and can be visibly
seen on chest x-ray as small boney deficits in the ribs termed “rib notching”.
14. (D) Methyldopa can cause a Coombs positive hemolytic anemia. Remember that
hemolytic anemia would result in elevated LDH levels, elevated indirect bilirubin levels, and
reduced haptoglobin levels. Historically, methyldopa has been frequently used to treat
hypertension in pregnancy due to experience indicating its safety.
Lisinopril (A) is an ACE inhibitor which can cause renal failure, hyperkalemia, a dry cough,
or angioedema. Minoxidil (B) is a direct arterial vasodilator (similar to hydralazine) which
can cause excessive hair growth (it is the active ingredient in Rogaine) and pericardial
effusions. Clonidine (C) is a central alpha-2 receptor agonist which causes bradycardia, dry
mouth, and rebound hypertension. Valsartan is an angiotensin receptor blocker that can cause
renal failure and hyperkalemia.

15. (A) Gemfibrozil acts by stimulating the synthesis of lipoprotein lipase to

degrade triglycerides into fatty acids increasing their metabolism and lowering blood levels.
Elevated triglyceride levels can lead to atherosclerosis and coronary artery disease.
Rosuvastatin (B) is an HMG-CoA reductase inhibitor which can cause rhabdomyolysis or
hepatic dysfunction (elevation in AST and ALT levels). Cholestyramine (C) is a bile acid
binding resin used to treat elevated low-density lipoprotein levels (LDL). Ezetimibe (D) is
also used to treat elevated LDL levels and acts by inhibiting cholesterol absorption at the
brush border of the small intestine. Ketoconazole (E), an anti-fungal medication, significantly
reduces LDL levels as well.

16. (C) Dressler's syndrome is an autoimmune pericarditis what occurs weeks to months after
myocardial infarction. The typical ECG changes of pericarditis occur (diffuse ST segment
elevation in a concave upward shape with PR depression). Symptoms of pericarditis include
sharp chest pain worse with laying flay and better with leaning forward and pain that radiates
to the left trapizius muscle. Dressler's syndrome is thought to be due to antibodies produced
against an unknown myocyte protein. Those antibodies crossreact with pericardial antigens
resulting in inflammation and pericarditis. The physical exam findings of pericarditis include
a pericardial friction rub, however it is not always present. Treatment includes NSAIDs such
as ibuprofen and if needed corticosteroids. Avoiding anticoagulation is recommended due to
the risk of spontaneous hemorrhage into the pericardium in Dressler's syndrome resulting
incardiac tamponade.
Ventricular free wall rupture (A) occurs as a complication of myocardial infarction that
occurs within a few days of infarction and results in cardiac tamponade which can be fatal.
Acute mitral valve regurgitation (B) is a complication of an inferior wall myocardial
infarction due to papillary muscle dysfunction or rupture which also occurs a few days after
MI. Left ventricular aneurysm (D) takes weeks to develop, usually after an anterior wall
myocardial infarction and does result in ST segement elevation on the ECG in leads V1 – V3
(not diffuse like in pericarditis). Left ventricular aneurysms cause heart failure, ventricular
arrhythmias, and increase the risk of rupture, but do not cause chest pains. Aortic dissection
(E) is not a complication of myocardial infarction, but can actually result in infarction due to
concomitant dissection of a coronary artery.

17 (C) Beta-blocker overdose results in bradycardia, hypotension, hypothermia,

hypoglycemia, and in severe cases seizures. Treatment includes intravenous glucagon which
stimulates heart rate, contractility, and raises blood glucose through non-adrenergic
pathways.
Methylene blue (A) is the antidote for methemoglobinemia. Dantrolene (B) is used in
malagnient hyperthermia or neuroleptic malignant syndrome, both of which have elevated
temperatures. Magnesium (D) is used to treat a prolonged QT interval and prevent Torsades
de Pointes. Flumazenil (E) reverses the actions of benzodiazepines and barbiturates.

18. (B) Mitral regurgitation occurs as a complication of an inferior wall myocardial infarction
due to papillary muscle dysfunction resulting in failure of the mitral valve leaflets to coapt
normally. Recall the cardiac anatomy of the mitral valve, specifically that there are two
papillary muscles, the anterolateral and posteromedial. The anterolateral papillary muscle is
perfused by the left anterior descending AND the left circumflex coronary arteries, thus
dysfunction of the anterolateral papillary muscle is uncommon (since it would require 2
major artery occlusions). The posteromedial papillary muscle receives its sole blood supply
from the right coronary artery (which also supplies the inferior wall in 80% of people). Thus
a right coronary artery occlusion resulting in inferior wall myocardial infarction frequently
causes mitral regurgitation due to concomitant papillary muscle infarction. Rarely, rupture of
a papillary muscle can cause acute mitral regurgitation and cardiogenic shock which requires
emergent surgical correction.
Tricuspid regurgitation (A) is another cause of a holosystolic murmur, however does not
result from inferior infarction and does not commonly cause congestive heart failure. A
ventricular septal defect (C) likewise can cause a holosystolic murmur and can also be a
complication of inferior wall myocardial infarction, but presents more acutely a few days
after the MI. Mitral valve stenosis (D) causes a diastolic murmur and is most commonly due
to rheumatic heart disease. Left ventricular aneurysm (E) can cause heart failure, however
does not cause a murmur and results more commonly from anterior myocardial infarction.

19. (B) Amiodarone (see also amiodarone toxicity), used to treat atrial fibrillation and
ventricular arrhythmias, can cause pulmonary fibrosis after long-term use. Amiodarone also
causes hypothyroidism, hyperthyroidism, and on rare occasion liver failure. Remember to
check PFTs (pulmonary function tests), LFTs (liver function tests), and TFTs (thyroid
function tests) on all patients on amiodarone. Blue man syndrome can occur as well due to
deposition of amiodarone metabolites in the skin resulting in a blue hue.
Congestive heart failure (A) can show a restrictive defect on pulmonary function testing,
however pulmonary edema would be seen on the chest x-ray and not honeycoming. Ramipril
(C), an ACE inhibitor, causes angioedema, renal failure, hyperkalemia, and a dry cough, but
not pulmonary fibrosis. Sotalol (D) is a class III antiarrhythmic drug that blocks sodium
channels and is used to treat atrial fibrillation. The beta-blocking properties of sotalol can
worsen asthma. Sotalol also prolongs the QT interval. Diltiazem (E), a dihydropyradine
calcium channel blocker, causes bradycardia, congestive heart failure, and constipation.

20. (E) Cancer is the most common cause of pericardial effusion and when enough fluid
accumulates in the pericardial space, cardiac tamponade occurs. “Pulses paradoxus” is when
there is a decrease in systolic blood pressure during inspiration due to failure of the right
ventricle to accept the normal increased venous return that occurs with inspiration. This also
results in a “Kussmal’s sign” or elevated jugular venous distension during inspiration
(normally the opposite occurs). Treatment is with emergent pericardiocentesis.
A restrictive cardiomyopathy (A) occurs from infiltrative diseases such as amyloidosis,
sarcoidosis, or hemachromatosis. Mitral valve regurgitation (B) should cause a holosystolic
murmur and does not cause pulsus paradoxus, but can present with congestive heart failure.
Pulmonary embolus (D) is possible given her history of breast cancer (which causes a
hypercoaguable state), however no chest pains were mentioned.
21. (D) The leading cause of death in patient’s with Marfan’s syndrome is acute ascending
aortic dissection and/or aortic rupture. Aortic dissection presents with sudden onset tearing
chest and upper back pain and can result in aortic rupture, cardiac tamponade, coronary artery
dissection resulting in myocardial infarction, acute aortic insufficiency, or stroke all of which
can be fatal.
Mitral valve prolapse (A) is common in patients with Marfan’s syndrome, but does not cause
sudden cardiac death. Likewise, aortic regurgitation (B) occurs as a result of a dilated aortic
annulus and is common with Marfan’s, however this would more commonly result in
congestive heart failure (E) and not sudden death. There is no increased risk of myocardial
infarction (C) in Marfan’s syndrome.

22. (B) Digoxin toxicity causes non-specific gastrointestinal symptoms (nausea, vomiting,
lack of appetite), heart rhythm disturbances, and xanthopsia (yellow vision). Remember the
mechanism of digoxin is to block the Na+/K+ ATPase pump in the cell membrane preventing
sodium from leaving the cells and preventing potassium from entering cells. Because of this,
digoxin toxicity itself produces hyperkalemia. Remember that digoxin increases inotropy via
the above mechanism ultimately by causing calcium to influx into myocardial cells. Thus
giving intravenous calcium (which is the normal treatment for severe hyperkalemia) will
cause an excessive amount of calcium to enter the cells, severely raising the myocyte
threshold potential, and worsening any bradyarrhythmias potentially causing cardiac arrest
and death.
Nitroglycerine (A) can cause headache and dizziness. Amiodarone (C) can cause thyroid
dysfunction, pulmonary fibrosis, and hepatic failure. Spironolactone (D) can cause
hyperkalemia and gynecomastia. Sildenafil (E) can cause dizziness, headache, and blue
vision (remember that digoxin and sildenafil together make green vision, although not
really).

23. (A) Metoprolol is a cardioselective beta-blocker that will block beta-1 receptors much
more that beta-2 receptors thus minimizing any bronchoconstriction and worsening of
asthmatic symptoms. Remember that despite being cardioselective, some beta-2 receptor
blockade does still occur, so patients have to be monitored closely for worsening asthma.
Propranolol (B), carvedilol (C), and nadolol (D) are all non-selective beta-blockers

24. (B) Dihydropyridine calcium channel blockers (ending in dipine) cause significant
venodilation resulting in lower extremity edema which resolves upon cessation of the
medication. Dihydropyridine calcium channel blockers are first line therapy for hypertension
and do not cause many side effects or complications.
Benzapril (A) is an ACE inhibitor which can cause angioedema. hyperkalemia, renal failure,
and a dry cough. Propranolol (C) is a lipid soluble, non-cardioselective beta-blocker which
can worsen asthma, cause bradycardia, congestive heart failure, and
hypotension. Clopidrogel (D) is an ADP receptor blocker on platelets and can cause
bleeding, Procainamide (E) is a class I antiarrhythmic agent which can cause a prolonged QT
interval or drug induced lupus (indicated by elevated anti-histone antibodies).

25. (A) Ototoxicity occurs with high dose loop diuretics such as furosemide, bumetinide, or
torsemide. Aminoglycosides can cause similar hearing loss (also nephrotoxicity). Remember
that congenital ear malformations are associated with congenital kidney problems to help
remember the connection between medications that act on the kidney and cause hearing loss
such as loop diuretics.
Clonidine (B) is a central alpha-2 agonist which can cause bradycardia, dry mouth, and
rebound hypertension.Minoxidil (C) is a direct arterial vasodilator which can cause excessive
hair growth and pericardial effusions. Triamterene (D) is a potassium sparing diuretic which
can cause hyperkalemia and can precipitate in the renal pelvis causing nephrolithiasis.

26. (D) Thrombolytic drugs such as tissue plasminogen activator (tPA or alteplase),
streptokinase, and urokinase can be reversed using aminocaproic acid.
A platelet transfusion (A) will not work since thrombolytics act by converting plasminogen to
plasmin causing fibrinolysis and clot destruction. This mechanism is independent of platelet
function. Proamine sulfate (B) is used to reverse the actions of heparin. Vitamin K (C) or
fresh frozen plasma transfusions can be used to reverse the actions of warfarin (coumadin).

27. (A) This patient has elevated preload based on the elevation on the right atrial pressure
(normal 5 mmHg) and pulmonary capillary wedge pressure (normal 12 mmHg). Remember
that preload is mostly determined by the total body fluid volume. Too much fluid in the body
pools in the venous system increasing the preload. Diuresis with medications such as
furosemide with reduce the preload and improve his symptoms.
Carvedilol (B) is frequently used for systolic congestive heart failure, however does not
acutely improve symptoms. Lisinopril (C) reduces preload (by reducing aldosterone
secretion) and afterload (by decreasing conversion of angiotensin I to angiotensin II which
vascoconstricts), but does not reduce symptoms acutely in congestive heart failure.
Intravenous fluids (D) would increase the preload and worsen his symptoms.

28. (C) Idiopathic hypertrophic subaortic stenosis (IHSS) is otherwise known as hypertrophic
obstructive cardiomyopathy(HOCM) and is an autosomal dominant inherited disorder in
about 50% of cases (the rest are sporadic). HOCM is associated with mostly exertional
symptoms. During exercise (when the heart contracts harder), the abnormally large
interventicular septum obstructs blood from flowing out of the aortic valve resulting in a
markedly reduced cardiac output. This leads to syncope (loss of consciousness). It can also
lead to life-threatening arrhythmias such as ventricular tachycardia and ventricular
fibrillation, thus HOCM is the most common cause of sudden death in young athletes. The
classic murmur may mimic aortic stenosis and is a systolic creshendo-decreshendo murmur at
the right upper sternal border that gets louder with Valsalva due to lessened blood return to
the left ventricle allowing more obstruction to occur. On histologic examination you would
see the myocardial myocytes in a chaotic pattern commonly described as "myocardial
disarray", not a normal organized pattern.
Congenital coronary anomalies (A) can cause sudden cardiac death, but would not cause a
murmur. Comotio cordis (B) is sudden death from ventricular fibrillation after chest wall
trauma (such as getting struck by a ball in the chest or a hard tackle in football). Dilated
cardiomyopathy (D) can lead to sudden death, but again there would be no murmur and the
patient would likely not be athletic (due to reduced cardiac output).

29. (A) Amyloidosis of the heart causes a restrictive cardiomyopathy and a majority of the
cases are due to a mutation in the transthyretin gene resulting in the abnormal deposition of
this protein in the myocardial tissue. The typical stain for amyloid is the congo red stain
which displays an “apple green birefringence”. Restrictive cardiomyopathy can also occur
from sarcoidosis or hemachromotisis. Physical examination reveals an S4 heart sound due to
impaired relaxation and a Kussmal’s sign which is marked elevation in the jugular venous
pressure with inspiration (the opposite of what usually happens).
Dilated cardiomyopathy (B) can occur from viral myocarditis, alcohol, pregnancy, or can be
idiopathic. An S3 heart sound would be present. Constrictive pericarditis (C) occurs after
prior heart surgery or if many episodes of pericarditis has occurred. A Kussmal’s sign may
also be present, but congo red staining would be negative.Hypertrophic obstructive
cardiomyopathy or HOCM (D) presents with exertional symptoms such as syncope or sudden
death. An S4 heart sound may also be present, but again congo red staining would be
negative. Chagas cardiomyopathy (E) is due to infection with Tympanosoma cruzi and is
associated with dilated cardiomyopathy, megaesophagus, and megacolon. Parasites may be
seen on the biopsy.

30. (C) Mitral valve prolapse (MVP) is usually a benign disorder very common in young
females and has been associated with anxiety and panic attacks. Also known as “Barlowe
syndrome” or “floppy mitral valve”, histologic examination shows myxomatous degeneration
of the valve and papillary muscles. Severe cases of MVP can be associated with mitral
regurgitation in which a holosystolic murmur would be heard. Patients with connective tissue
disorders such as Marfan’s syndrome are more likely to have MVP. No specific treatment is
needed unless heart failure develops from mitral regurgitation.

Mitral valve regurgitation (A) would cause a holosytolic murmur at the apex. Mitral valve
stenosis (B) would cause a diastolic murmur with an opening snap.

31. (C) This patient has tuberous sclerosis, an autosomal dominant genetic disorder due to
mutations in the tumor suppressor gene hamartin or tuberin. Cardiac tumors are most
commonly rhabdomyomas in this disorder. Seizures and developmental delay are common.
Multiple other tumors may also develop.
Left atrial myxoma (A) is more common in older individuals and can cause a mitral stenosis
picture as the tumor obstructs the valve. Cardiac sarcomas (B) and cardiac lymphomas (D)
are extremely rare.

32. (C) Subacute bacterial endocarditis is most commonly due to Streptococcus viridins
which is a normal flora of the mouth and thus frequently enters the blood stream after dental
procedures. Pre-existing valvular heart disease increases the risk of endocarditis and a new
regurgitant murmur should raise suspicion as the pathogen can destroy valve leaflets.
Remember that if Streptococcus bovis is the culprit, concominant colon cancer may be
present. Osler’s nodes (painful lesions on the pads of the fingers, remember Osler’s and
Ouch), Janeway’s lesions (painless lesions on the palms and soles), splinter hemorrhages in
the fingernails, and Roth spots on fundoscopic examination (retinal hemorrhages with
white/pale centers) are all a result of peripheral embolization or immune complex deposition
related to endocarditis. Also, endocarditis elevated the erythrocyte sedimentation rate (as all
inflammatory conditions do) and can cause a false positive RPR test for syphilis (similar to
systemic lupus).
Staphalococcus aureus (A) causes a more acute picture and is less common (2nd leading
cause) and may be seen on the tricuspid valve of intravenous drug users. Pseudomonas
aurginosa (B) is also acute and uncommon. Fungal endocarditis such as from Candida
albicans (D) is uncommon and occurs in immunocomprimised patients and can be subacute
or chronic.

33. (B) Beta-blockers decrease heart rate and inotropy, two major determinants of myocardial
oxygen demand. All acute coronary syndromes (myocardial infarctions or unstable angina)
should be given beta-blockers such as metoprolol immediately unless an obvious
contraindication exists (bradycardia, hypotension, severe congestive heart failure, severe
asthma or obstructive pulmonary disease). Beta-blocker administration during acute
myocardial infarction has been definitively shown to reduce mortality rates.
Nitroglycerine infusion (A) and loop diuretics (D) will reduce preload by venodilation and
decreased total body volume respectively which will have some reduction in myocardial
oxygen demand, but not profound. Nitroglycerine has never been shown to reduce mortality
in myocardial infarction. Aspirin (C) which does reduce mortality rather dramatically, does
not effect myocardial oxygen demand but rather inhibits platelets to prevent thrombus
propagation. Dobutamine infusion (D), which can be used in myocardial infarction if severe
cardiogenic shock is present, actually increases heart rate and inotropy resulting in increased
myocardial oxygen demand by stimulating beta-1 receptors.

34. (B) Cyanide toxicity can result from prolonged nitroprusside infusion. When cyanide
accumulates, a severe metabolic acidosis occurs resulting in gastrointestinal symptoms,
headache, lethargy/coma, seizures, and eventually death. The antidote is sodium thiosulfate
which converts cyanide to a renally excreted thiocyanate.
Methylene blue (A) is the antidote for methemaglobinemia. Sodium EDTA (ethylene diamine
tetraacetic acid) is used for some heavy metal toxicities. Hemodialysis (D) is not effective for
cyanide toxicity.

35. (B) Coronary vasospasm (Pritzmetal’s angina) occurs most commonly in young females
and in the early morning hours. Electrocardiography at the time of the chest pains will show
ST elevations, but will be normal when vasospasm is not occurring. Certain medications are
known to cause or worsen vasospasm which include the triptans (for migraine treatment),
ergotamine or ergonavine (also for migraine treatment), alpha agonists such as phenylephrine
or ephedrine, and cocaine. Treatment is with a dihydropyradine calcium channel blocker such
as nifedipine (A) to induce vascular smooth muscle relaxation.
Ibuprofen (C), estrogen (D), and proxetine (E) do not cause vasospasm.

36. (A) Systolic congestive heart failure patients benefit from long-term beta-blocker therapy.
The overactivation of the sympathetic nervous system that occurs in heart failure causes a
negative remodeling of the myocardium which actually worsens the cardiac output in the
long-term. Thus, using beta-blockers will block this negative remodeling and eventually
improve symptoms. A significant mortality benefit has been demonstrated only with
carvedilol, long-active metoprolol (succinate), and bisoprolol. No other beta-blockers are
FDA approved for systolic congestive heart failure. ACE inhibitors and spironolactone are
other important medications to reduce mortality in heart failure. Loop diuretics can be used to
reduce preload and improve symptoms as well.
Nitroglycerine (B) will reduce preload by venodilation and may improve symptoms, but it is
not used first line for heart failure. Amlodipine (C) is safe in heart failure, but does not
reduce symptoms or improve mortality. Verapamil (D) can worsen heart failure by
decreasing cardiac output (by its negative inotropic and chronotropic effect). Digoxin (E)
reduced symptoms, but not mortality (due to its significant toxicity potential).

37. (A) Doxorubicin and daunorubicin are anthracycline chemotherapeutic agents that are
well known to cause systolic congestive heart failure especially at higher doses and should be
avoided if pre-existing heart failure is present.
Bleomycin (B) can cause pulmonary fibrosis. Paclitaxel (C) also causes pulmonary toxicity.
Cyclophosphamide (D) can cause hemorrhagic cystitis (resulting in hematuria and bladder
pain).

38. (A) This patients has cardiogenic shock and a low cardiac output. Milrinone is a
phosphodiesterase 3 inhibitor that works by increasing contractility (inotropy), heart rate
(chronotropy) and vasodilating. While milrinone does indeed increased cardiac output, it also
increases myocardial oxygen demand which is not good in the setting of a myocardial
infarction. Nevertheless, inotropes may be required when cardiogenic shock is present such
as this situation. Intraaortic balloon counterpulsation and emergency percutaneous coronary
intervention would also be appropriate.
Furosemide (B) is a loop diuretic and would not improve the hemodynamic state of shock
(hypotension). Epoprostenol (C) is a vasodilator but has no evidence to support its use for any
cardiac disease state. Vasopressin (D), a.k.a. antidiuretic hormone (ADH) will increase blood
pressure, but also is not used in cardiogenic shock. It is used in refractory septic shock.

39. (A) Left-sided congestive heart failure (CHF) occurs when the left ventricle is not able to
produce adequate cardiac output to meet the demands of the body resulting in increases in left
ventricular pressure which are then transmitted to the pulmonary veins resulting in pulmonary
edema and shortness of breath. Essentially any cardiac disorder can reach the endpoint of left
ventricular failure (valve disease such as mitral regurgitation, prior myocardial infarctions,
cardiomyopathies etc…)
Right-sided congestive heart failure (B) presents with lower extremity edema. Physical exam
findings include elevated jugular venous pressure, hepatojugular reflux (increased jugular
venous pressure with deep palpation of the liver due to “hepatic congestion”), and lower
extremity pitting edema. Remember that left-sided heart failure is the most common cause of
right-sided heart failure (eventually the pressure overload of the failing left ventricle gets
transmitted to the right ventricle causing it to fail as well). Cor pulmonale (D) occurs when
severe lung disease elevated pulmonary artery pressures which transmits back to the right
ventricle causing right ventricular failure.

40. (C) Atrial fibrillation is an irregularly irregular tachyarrhythmia which is the most
common chronic rhythm disorder. Goals of therapy include reducing the heart rate which can
be done by any medication that blocks AV nodal conduction. Remember the mneumonic
“ABCD” for adenosine or amiodarone, beta-blockers, calcium channel blockers (non-
dihydropyridine), and digoxin. Digoxin also increases inotropy (contractility) augmenting
cardiac output and thus is ideal in the setting of both atrial fibrillation and systolic congestive
heart failure.
Beta-blockers (A) and non-dihyropyridine calcium channel blockers (B) can decrease
inotropy and worsen systolic congestive heart failure in the short term, although beta-
blockers in the long term prevent negative remodeling of the myocardium from chronic
sympathetic stimulation which is beneficial. Amiodarone (D) does not directly affect overall
cardiac output.
41. (B) Diabetes mellitus (DM) type II is considered an atherosclerotic heart disease
equivalent meaning when diabetes type II is present, so is atherosclerotic heart disease. The
other choices to significantly increase the risk of developing atherosclerotic heart disease, but
not as much as diabetes mellitus type II.

42. (A) This patient has the typical presentation of a viral myocarditis leading to a dilated
cardiomyopathy. About 1/3 of cases recover left ventricular function spontaneously, 1/3
remain unchanged, and 1/3 worsen. The most common pathogen is coxsackie virus B. Other
causes include the influenza viruses (D), adenoviruses, hepatitis C virus, cytomegalovirus,
Epstein-Barr virus (C), and human immunodeficiency virus (B).

43.(C) B-type naturic peptide (BNP) and A-type naturitic peptide (ANP) get released in high
concentrations with the myocardial stretch that occurs in congestive heart failure. The
physiologic properties of BNP and ANP include vasodilation (reducing afterload), naturesis
(excretion of sodium reducing preload). ANP and BNP are the bodies natural mechanism to
maintain a normal volume status in the setting of heart failure, but frequently are not enough.
Exogenous BNP can be administered (a.k.a. nesiritide) to enhance the
preload/afterload/naturesis effects and improve heart failure symptoms. Measuring BNP
levels in the serum is helpful to diagnose heart failure as a cause of dyspnea. A newer assay
called NT-pro BNP is more sensative.

44. (D) Etiologies of pericarditis include uremia (this patient), viral, tuberculosis,
autoimmune, and iatrogenic (post-heart surgery). Symptoms include a sharp chest pain worse
with laying flat and better sitting-up and leaning forward. The pain radiates to the left
trapizius muscle. Electrocardiogram findings include diffuse ST segment elevation and PR
segment depression. A pericardial friction rub is frequently auscultated near the cardiac apex
and can be quite loud and overbear the normal heart sounds. Treatment is aimed at the cause
and symptoms can be relieved with nonsteroidal anti-inflammatory drugs.
Myocardial ischemia (A) is ulikely given his age, sharp nature of the pain (ischemia is
pressure-like) and negative troponin I levels. Aortic dissection (B) presents with sudden onset
“tearing” chest and back pain and is a surgical emergency. Esophageal rupture (C) can occur
with significant emesis, however fevers, septic shock, and pleural effusion on chest x-ray
(from esophageal and gastric secretions in the pleural space).

45. (B) A ventricular septal defect (VSD) can range from small and asymptomatic to large
and life threatening. The smaller the VSD the louder the murmur as is seen in this patient.
Many VSDs will close spontaneously and require no intervention. Recall that a VSD is a left
to right shunt. A large VSD would eventually cause right ventricular overload and pulmonary
hypertension. As the right-sided heart pressures exceed that of the left ventricle, the shunt can
change to right to left and severe symptoms of heart failure can develop. This is known as
Eisenmenger’s syndrome.
An atrial septal defect (A) usually causes symptoms at some point and requires closure. The
murmur is due to increased flow across the pulmonic valve and thus is a creshendo-
decreshendo murmur at the left upper sternal border. A fixed split S2 heart sound is
present. Mitral valve regurgitation (C) and tricuspid valve regurgitation (D) are the other two
causes of holosystolic murmurs. The mitral regurgitation murmur is located at the apex and
radiates to the axilla. If severe it can frequently to heart failure. The tricuspid regurgitation
murmur is located at the left lower sternal border and increases in intensity with inspiration
(Carvallo’s sign). Right-sided heart failure can develop from tricuspid regurgitation.
46. (B) Warfarin (coumadin) elevates the protrombin time (PT) by inhibition of vitamin K
dependent clotting factors II, VII, IX, and X. The international normalized ratio (INR) is
another measure of PT which was standardized due to inconsistencies between different PT
assays used in different hospitals. Many drug interactions exist with warfarin which include
antibiotics (since the eradicate normal gastrointestinal flora which produce vitamin K),
verapamil, cimetidine, and foods rich in vitamin K.
The activated partial thromboplastin time (A) is elevated with heparin sulfate use or in the
presence of lupus anticoagulant. Bleeding time (C) is elevated with platelet dysfunction.
Factor Xa assay (D) would be elevated in the presence of low molecular weight heparin such
as enoxaparin.

47. (D) Mitral stenosis occurs most commonly due to rheumatic heart disease and the mitral
valve is the most common valve affected. Only half of patients will recall an initial episode of
rheumatic fever. Medications (B) are not effective to treat mitral stenosis since the problem
itself is anatomical, thus relieving the stenosis is key. The less invasive procedure of mitral
valve balloon valvotomy is preferred over open surgical repair (C) if possible.

48. (B) Alcoholic cardiomyopathy is a form of dilated cariomyopathy (causing systolic

congestive heart failure) which can occur in genetically susceptible individuals from as little
as 2 alcoholic drinks per day. A majority of cases resolve with alcohol cessation, but some
never recover left ventricular function. Other causes of dilated cardiomyopathy include
viruses (most commonly coxsackie B), pregnancy, and idiopathic or genetic.
Heroin (A) and cisplatin (D) do not have any cardiotoxicity. Tuberculosis infection (C) can
cause pericarditis and pericardial effusion, but is not a cause of dilated cardiomyopathy.

49. (D) Angiotensin converting enzyme inhibitors (ACE inhibitors) can cause a dry cough
related to accumulation of bradykinin. Recall that the ACE enzyme, in addition to converting
angiotensin I to the more active angiotensin II, also degrades bradykinin. Accumulation of
bradykinen in the lungs is thought to be the cause of the dry cough that occurs in up to 20%
of patients. When an ACE inhibitor is absolutely needed (for diabetic nephropathy or
congestive heart failure), angiotensin receptor blockers like losartan (C) are thought to be an
acceptable alternative due to similar blockade of the renin-angiotensin-aldosterone system
without the bradykinin effects.
Clonidine (A) can cause dry mouth, bradycardia, and rebound hypertension. Methyldopa (B)
can cause drug induced hemolysis (Coombs positive) and is frequently used in pregnancy due
to its safety. Nicardipine (E) is a dihydropyridine calcium channel blocker that can cause
peripheral edema and dizziness.

50. (A) The anomaly described is transposition of the great vessels in which the aorta and the
pulmonary artery arise from the incorrect ventricle resulting in two closed circuits of blood
flow. The first circuit (right ventricle to aorta to organs to right atrium and back to right
ventricle) delivers only deoxygenated blood to the organs resulting in cyanosis. The second
circuit (left ventricle to pulmonary artery to lungs for oxygenation to left atrium back to left
ventricle) oxygenates the blood but does not allow it to get to the systemic circulation. Vital
to the survival of these infants is a left to right shunt of some kind such as an atrial or
ventricular septal defect or a patent ductus arteriosis. Prostaglandin E2 (A) helps to keep the
ductus arteriosus open until surgical correction can be done.
Hyperbaric oxygen administration (B), used for carbon monoxide poisoning or for wound
healing, will not help since there is still no communication between the blood oxygenated by
the lungs and the systemic circulation. Indomethacin (C) actually causes the ductus arteriosus
to close which could potentially be fatal to this infant.