UMSLE STEP I
1 A 72 year old female with no significant past medical history passes out while exercising. He has intermittent exertional chest pains and dyspnea on exertion as well. Her physical examination reveals a III/VI late-peaking creshendo-decreshendo murmur at the right upper sternal border and a III/VI holosystolic murmur at the apex. Her S2 heart sound is very soft and her carotid upstroke is weak and delayed. Which of the following is most likely causing her symptoms? A. Aortic valve regurgitation B. Aortic valve stenosis C. Mitral valve regurgitation D. Mitral valve stenosis E. Mitral valve prolapse 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. An 18 year old male basketball player passes out during a game. An echocardiogram reveals that he has hypertrophic obstructive cardiomyopathy a.k.a. HOCM (formerly IHSS or idiopathic hypertrophic subaortic stenosis). Which of the following patterns of inheritance are most consistent with HOCM? A.
Answer: A - Autosomal dominant The image for choice A depicts an autosomal dominant inheritance pattern which is present in about half of cases of hypertrophic 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 56 year old African American female with a history of asthma is diagnosed with hypertension. Laboratory studies reveal a creatinine of 3.0 mg/dL and a potassium level of 5.6 mg/dL. Which of the following medications is appropriate to treat her hypertension? A. amlodipine B. hydrochlorothiazide C. enalapril D. propranolol E. spironolactone 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 noncardioselective 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. A 45 year old male with a history of hypertension and hyperlipidemia complains of generalized weakness. His laboratory studies are below. Serum Na+ 144 mEq/L K+ 5.9 mEq/L Cl- 101 mEq/L HCO3- 16 mEq/L Urea nitrogen 66 mEq/L Cr 2.8 mEq/L Uric acid 13.1 mEq/L Calcium 6.1 mEq/L Urine dipstick Specific gravity 1.030 Blood - positive Nitrates – negative Leukocyte esterase – negative Urine microscopic examination No WBCs No RBCs Which of the following medications is likely the cause of his symptoms? A. lisinopril B. verapamil C. digoxin
D. rosuvastatin E. hydralazine 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. A 29 year old male with a history of ectopia lentis presents for a routine physical examination. He is noted to have pectus excavatum, pes planus, a high arched palate, and a positive wrist and thumb sign. Which of the following cardiac disorders is associated with his condition? A. Aortic valve stenosis B. Coarctation of the aorta C. Mitral valve prolapse D. Ventricular septal defect E. Ebstein’s anomaly 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 xray. Ventricular septal defects (D) and Ebstein’s anomaly (E) are not associated with Marfan’s. 6. A 52 year old male with a history of hypertension, dyslipidemia, and congestive heart failure presents to the emergency department with acute onset swelling of the lips, eyes, and tongue causing airway obstruction and respiratory distress. He also has an urticarial rash. Which of the following medications is the likely culprit of his current symptoms? A. pindolol B. clonidine C. felodipine D. captopril E. methyldopa 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 blocker which 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. A 2 day old full term male infant is noted to have a systolic and diastolic continuous murmur at the right upper sternal border. His vital signs are normal and he is overall doing well. Which of the following interventions should be done at this time? A. Administration of a diuretic B. Administration of indomethacin
C. Surgical ligation D. Observation 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. A 45 year old female with a history of hypertension previously controlled with diet and lifestyle modifications presents to here primary care physician with increasing headaches. Her blood pressure is 160/90. She is diagnosed with worsening migraine headaches. Which of the following medications is appropriate to treat her hypertension and headaches simultaneously? A. hydrochlorothiazide B. metoprolol C. clonidine D. methyldopa E. lisinopril 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. A 67 year old male suffers a large myocardial infarction complicated by sustained ventricular tachycardia. He is started on a lidocaine infusion. He subsequently develops shock liver from hypotension during the ventricular tachycardia and his serum lidocaine levels become significantly elevated. Which of the following is a manifestation of lidocaine toxicity? A. Stroke B. Seizures C. Renal failure D. Congestive heart failure E. Hyperkalemia 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. Which of the following describes the cardiac anomalies associated with the tetralogy of Fallot? A. Atrial septal defect, ventricular septal defect, a common atrioventricular valve, and pulmonic valve stenosis B. Atrial septal defect, right ventricular hypertrophy, pulmonic valve stenosis, and an overriding aorta C. Ventricular septal defect, left ventricular hypertrophy, aortic valve stenosis, and an overriding aorta
D. Ventricular septal defect, right ventricular hypertrophy, pulmonic valve stenosis, and an overriding aorta 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. A 62 year old female with a history of hypertension presents with increasing shortness of breath, abdominal pain, and diarrhea. Computed tomography reveals a mass in the appendix and multiple nodules in the liver. Chest x-ray is normal. Serum 5hydroxyindoleacetic acid levels are elevated. Which of the following cardiac disorders is she likely to have? A. Aortic valve stenosis B. Aortic valve regurgitation C. Mitral valve stenosis D. Mitral valve regurgitation E. Tricuspid valve stenosis 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. A 48 year old male with a history of hypertension and high cholesterol presents to the emergency department with chest pains for 60 minutes. He describes a substernal chest
pressure “like an elephant on my chest” associated with shortness of breath and diaphoresis. His ECG shows ST elevations consistent with myocardial infarction. Which of the following laboratory results would be expected? A. Elevated myoglobin, elevated troponin I, and elevated CK-MB B. Normal myoglobin, elevated troponin I, and normal CK-MB C. Elevated myoglobin, normal troponin I, and normal CK-MB D. Normal myoglobin, normal troponin I, and elevated CK-MB E. Normal myoglobin, normal troponin I, and normal CK-MB 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. A 29 year old male with no past medical history has been experiencing headaches for the past few months. His blood pressure is noted to be 210/110. Physical examination reveals an S4 heart sound and reduced femoral pulses. Which of the following is associated with his condition?
A. Atrial septal defect B. Wolff-Parkinson-White syndrome C. Bicuspid aortic valve D. Mitral valve regurgitation 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. A 61 year old female with a history of hypertension presents with jaundice and generalized fatigue. Her laboratory studies are below: Serum Na+ 144 mEq/L K+ 5.9 mEq/L Cl- 101 mEq/L HCO3- 25 mEq/L Urea nitrogen 15 mEq/L Cr 1.1 mEq/L
Indirect bilirubin 4.2 mEq/L Hemoglobin 9.8 g/dL Haptoglobin undetectable Which of the following medications could potentially cause this patient’s presentation? A. lisinopril B. minoxidil C. clonidine D. methyldopa E. valsartan 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 56 year old obese female presents for a routine physical examination. Her lipid profile reveals a significantly elevated triglyceride level of 355 mg/dL. Which of the following medications can act to lower her triglyceride level by stimulating the synthesis of lipoprotein lipase? A. gemfibrozil B. rosuvastatin
C. cholestyramine D. ezetimibe E. ketoconazole 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. A 68 year old male suffers a myocardial infarction. Six weeks later he begins to have sharp substernal chest pains radiating to his left neck worse with laying flat and better while sitting up and leaning forward. His electrocardiogram is below. Which of the following is his likely diagnosis?
A. Ventricular free wall rupture
B. Acute mitral valve regurgitation C. Dressler’s syndrome D. Left ventricular aneurysm E. Aortic dissection 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 in cardiac 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. A 72 year old male with a history of dementia and hypertension presents to the emergency room with profound bradycardia and altered mental status. His temperature is 34.8 C, blood pressure is 80/40, and heart rate 30 beats per minute. His laboratory studies are below: Serum Na+ 141 mEq/L K+ 4.1 mEq/L
Cl- 103 mEq/L HCO3- 28 mEq/L Urea nitrogen 19 mEq/L Cr 1.2 mEq/L Glucose 44 mEq/L Which of the following is the appropriate therapy at this time? A. Methylene blue B. Dantrolene C. Glucagon D. Magnesium E. Flumazenil 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. A 76 year old female with a history of coronary artery disease and prior inferior wall myocardial infarction presents with gradual onset of increased shortness of breath on exertion and lower extremity swelling. Her physical examination reveals a IV/VI holosystolic murmur at the 5th intercostals space at the mid-clavicular line. Which of the following is likely the cause of her symptoms? A. Tricuspid valve regurgitation B. Mitral valve regurgitation C. Ventricular septal defect D. Mitral valve stenosis
E. Left ventricular aneurysm 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. An 82 year old male with a history of long standing atrial fibrillation and hypertension presents with increasing dyspnea on exertion. Chest x-ray shows a honeycoming pattern and pulmonary function testing shows a severe restrictive defect. Which of the following is likely causing his symptoms? A. Congestive heart failure B. amiodarone C. ramipril D. sotalol E. diltiazem 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. A 67 year old female with a history of breast cancer and tobacco use complains of dizziness and dyspnea on exertion. Her heart sounds are distant and her systolic blood pressure is noted to markedly decrease with inspiration. Which of the following is the likely diagnosis? A. Restrictive cardiomyopathy B. Mitral valve regurgitation C. Congestive heart failure D. Pulmonary embolus E. Cardiac tamponade 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. A 22 year old female is noted to have a reduced upper to lower body segment ratio, positive Walker and Steinberg signs, and pectus carinatum. Her father died suddenly at the age of 34. She is subsequently diagnosed with Marfan’s syndrome. Which of the following was the likely cause of death of her father? A. Mitral valve prolapse B. Aortic valve regurgitation C. Myocardial infarction D. Aortic dissection E. Congestive heart failure 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. A 60 year old male with a history of congestive heart failure and atrial fibrillation states his vision has been mostly yellow recently. He has noted lack of appetite and weight loss. Laboratory studies reveal and elevated potassium level. Which of the following is the likely causing his symptoms? A. Nitroglycerine B. Digoxin C. Amiodarone D. Spironolactone E. Sildenafil
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 45 year old male with severe asthma is having a myocardial infarction. Which of the following beta-blockers can potentially treat his myocardial infarction while causing minimal bronchoconstriction? A. metoprolol B. propranolol C. carvedilol D. nadolol 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. A 72 year old female complains of increasing lower extremity swelling. Her cardiac testing and laboratory studies are normal. Cessation of which of the following medications would improve her symptoms? A. benzapril B. felodipine
C. propranolol D. clopidrogel E. procainamide 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 52 year old female is experiencing difficulty hearing. Which of the following drugs may be the cause? A. bumetinide B. clonidine C. minoxidil D. triamterene 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. A 55 year old female is treated for an acute myocardial infarction with alteplase and experiences severe gastrointestinal bleeding. Which of the following can be administered to reverse the action of alteplase? A. Platelet transfusion B. Protamine sulfate C. Vitamin K D. aminocaproic acid 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 59 year old male with congestive heart failure has noted increased dyspnea on exertion. Swan-Ganz catheterization reveals the following (pressures in mmHg): Right atrium - 22 Right ventricle – 44/20 Pulmonary artery – 49/24 Pulmonary capillary wedge pressure – 23 Cardiac index – 2.6 L/min/BSA2 (normal 2.4-4.4 L/min/BSA2) Administration of which of the following will likely improve his symptoms? A. Furosemide B. Carvedilol C. Lisinopril
D. Intravenous fluids 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. An 18 year old male dies suddenly during a track and field event. During a recent sports physical he was noted to have a II/VI systolic crescendo-decreshendo murmur at the right upper sternal border that became louder with Valsalva. A paradoxical split S2 heart sound was heard. Which of the following is his likely diagnosis? A. Congenital coronary anomaly B. Comotio cordis C. Idiopathic hypertrophic subaortic stenosis D. Dilated cardiomyopathy 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 34 year old male experiences shortness of breath with minimal exertion. Physical examination reveals elevated jugular venous pressure markedly worse with inspiration, a regular rhythm with an S4 heart sound and 2+ lower extremity pitting edema. Laboratory studies are normal. Cardiac biopsy revealed apple green birefringence with congo red staining. Genetic testing reveals a mutation in the transthyretin gene. Which of the following is the correct diagnosis? A. Restrictive cardiomyopathy B. Dilated cardiomyopathy C. Constrictive pericarditis D. Hypertrophic obstructive cardiomyopathy E. Chagas cardiomyopathy 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. A 35 year old female with a history of anxiety and panic attacks presents for a routine physical examination. She intermittently experiences palpitations but in general feels well. Physical examination reveals a mid-systolic click at the cardiac apex which moves earlier in systole with standing from a squatting position. No murmur is present. Which of the following is the likely diagnosis? A. Mitral valve regurgitation B. Mitral valve stenosis C. Mitral valve prolapse D. Normal mitral valve 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. See the heart sounds and heart murmurs review for more details. 31. A 7 year old male with a history of a seizure disorder and developmental delay is found to have cortical tubers on brain magnetic resonance imaging. Which of the following cardiac tumors is he likely to develop? A. Left atrial myxoma
B. Cardiac sarcoma C. Rhabdomyoma D. Cardiac lymphoma 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. A 56 year old female with a history of mitral valve prolapse has been experiencing fevers and joint pains for 3 weeks. She recently underwent a tooth extraction. Physical examination reveals a III/VI holosystolic murmur at the cardiac apex which was not present on prior examinations. Her erythrocyte sedimentation rate is markedly elevated. A painful nodule on the pad of her left index finger has developed. Which of the following is the most likely culprit? A. Staphalococcus aureus B. Pseudomonas auriginosa C. Streptococcus viridins D. Candida albicans
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. A 45 year old male presents with substernal chest pressure and is found to have elevated troponin levels consistent with a large myocardial infarction. Which of the following interventions can best decrease myocardial oxygen demand and potentially reduce the size of the infarction? A. Nitroglycerine infusion B. Beta-blocker administration C. Aspirin D. Loop diuretic administration E. Dobutamine infusion 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. A 64 year old male is being treated for with intravenous nitroprusside for hypertensive emergency. On day 3 of therapy he experiences a severe headache, nausea, and vomiting and becomes lethargic. The next day he experiences a seizure. Which of the following is the appropriate therapy at this time? A. Methylene blue B. Sodium thiosulfate C. Sodium EDTA D. Hemodialysis 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. A 29 year old female with a history of migraine headaches is seen in the emergency room for substernal chest pains of acute onset that woke her up in the morning. During an episode of chest pain her ECG shows ST elevations. Coronary angiography was normal without any significant coronary artery disease, however ergotamine infusion reproduced her symptoms. Which of the following medications could potentially cause or worsen her condition? A. nifedipine
B. sumatriptan C. ibuprofen D. estrogen E. paroxetine 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 58 year old female has been diagnosed with systolic congestive heart failure. She currently takes lisinopril, hydralazine, and furosemide. Which of the following medications should be added to improve her symptoms and reduce her mortality in the long-term? A. carvedilol B. nitroglycerine C. amlodpine D. verapamil E. digoxin 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 toxicitypotential). 37. A 55 year old female with a history of congestive heart failure is found to have breast cancer requiring chemotherapy. Which of the following chemotherapeutic agents should be avoided? A. doxorubicin B. bleomycin C. paclitaxel D. cyclophosphamide 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 preexisting 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 70 year old male presents with chest pain and is found to have an anterior wall myocardial infarction. Appropriate therapy is initiated, however he becomes profoundly hypotensive. His Swan-Ganz catheterization pressures are below (pressures in mmHg): Right atrium - 20 Right ventricle – 44/19 Pulmonary artery – 46/24 Pulmonary capillary wedge pressure – 20 Cardiac index – 1.9 L/min/BSA2 (normal 2.4-4.4 L/min/BSA2) Which of the following medications will siginficantly improve his cardiac index (cardiac output)?
A. milrinone B. furosemide C. epoprostenol D. vasopressin 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 84 year old male with a history of severe emphysema and a prior myocardial infarction becomes short of breath with exertion. Physical examination reveals a III/VI holosystolic murmur at the cardiac apex, an S3 heart sound, and rales in the lower lung fields. No lower extremity edema is present. Which of the following is the likely diagnosis? A. Left-sided congestive heart failure B. Right-sided congestive heart failure C. Left and right sided congestive heart failure D. Cor pulmonale 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. An 82 year old male with a history of hypertension and congestive heart failure presents with palpitations. His heart rate is 140 beats per minute and his physical examination reveals an irregularly irregular rhythm. He is diagnosed with atrial fibrillation. Which of the following medications can reduce his heart rate while improving left ventricular systolic function? A. Propranolol B. Verapamil C. Digoxin D. Amiodarone 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. A 56 year old female has a history of hypertension, diabetes mellitus type II, elevated low density lipoprotein levels, and smokes tobacco. Which of the above is the most significant risk factor for the development of atherosclerotic heart disease? A. Hypertension
B. Diabetes mellitus type II C. Elevated low density lipoprotein D. Tobacco use 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 28 year old female with no prior past medical history becomes markedly short of breath and hypotensive over a 3 day time period. She had been suffering from an upper respiratory tract infection starting 1 week prior. Physical examination reveals no murmurs, an S3 heart sound is present, elevated jugular venous pressure, pulmonary rales, and lower extremity edema. An echocardiogram confirms an ejection fraction of 10%. Which of the following is the most common culprit of her condition? A. Coxsackie B virus B. Human immunodeficiency virus C. Epstein-Barr virus D. Influenza A virus 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. A 65 year old male with a history of congestive heart failure and severe chronic obstructive pulmonary disease complains of increasing dyspnea on exertion, lower extremity edema, paroxysmal nocturnal dyspnea, and orthopnea. His ejection fraction is noted to be 15%. Physical examination reveals an S3 heart sound, pulmonary rales, and lower extremity pitting edema. Which of the following elevates in the serum with congestive heart failure, reduces preload and afterload and causes diuresis? A. Aldosterone
B. Carbon dioxide C. B-type naturitic peptide D. endothelin 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. A 23 year old male presents to the emergency room with sharp chest pains radiating to his left neck and altered mental status. For the past 3 days he has had severe nausea and vomiting attributed to viral gastroenteritis. Physical examination reveals a loud abnormal scratching sound in end systole and all of diastole located near the cardiac apex. Chest xray is normal. His laboratory studies are below: Serum Na+ 145 mEq/L K+ 5.4 mEq/L Cl- 103 mEq/L HCO3- 22 mEq/L Urea nitrogen 112 mEq/L Cr 5.2 mEq/L Troponin I - negative Which of the following is likely causing his chest pain? A. Myocardial ischemia B. Aortic dissection C. Esophageal rupture D. Pericarditis
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 pressurelike) 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. A 26 year old male is noted to have a V/VI holosystolic murmur associated with a thrill at the left lower sternal border. He has no health complaints and is able to exercise regularly without difficulty. Which of the following is the likely diagnosis? A. Atrial septal defect B. Ventricular septal defect C. Mitral valve regurgitation D. Tricuspid valve regurgitation 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 S2heart 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. A 81 year old female with a history of pulmonary embolus is taking warfarin for anticoagulation. She is given antibiotics for pneumonia and has noted blood in her stool. Which of the following laboratory abnormalities would be expected? A. Elevated activated partial thromboplastin time (PTT) B. Elevated international normalized ratio (INR) C. Elevated bleeding time D. Elevated factor Xa assay 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. A 36 year old female with no significant past medical history is being evaluated for increasing shortness of breath. Her physical examination reveals a II/IV early diastolic decrescendo murmur occurring after an early diastolic opening snap. Which of the following is the ideal therapy for her cardiac disorder? A. Observation B. Angiotensin converting enzyme inhibitor C. Surgical mitral valve repair D. Mitral valve balloon valvotomy
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. A 55 year old male complains of increasing dyspnea on exertion and orthopnea. His physical examination reveals an S3 heart sound, pulmonary rales, jugular venous distension, and lower extremity edema. Coronary angiography is normal. An echocardiogram confirms an ejection fraction of 5% indicating severe congestive heart failure and dilated cardiomyopathy. Which of the following could explain the above findings? A. A history of heroin abuse B. Heavy alcohol use C. Tuberculosis infection D. Prior chemotherapy with cisplatin 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. A 44 year old male with a history of diabetes mellitus type II has been experiencing a severe dry cough for 2 months. Chest x-ray is normal as are laboratory studies. He has had no recent travel or sick contacts. Which of the following medications could be contributing to his current symptoms? A. clonidine B. methyldopa C. losartan
D. benzapril E. nicardipine 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. An infant becomes cyanotic soon after birth. Emergent echocardiogram reveals the aorta originating from the right ventricle and the pulmonary artery originating from the left ventricle. Which of the following interventions can be done to improve the cyanosis until surgical correction is performed? A. Prostaglandin E2 B. Hyperbaric oxygen administration C. Indomethacin D. No intervention is needed 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.
