A 40-year-old man received an orthotopic cardiac transplant 7 years ago to treat a dilated cardiomyopathy. Since that time he has been healthy, with no episodes of rejection or infection. Over the next year, however, he develops fatigue with exercise. He has worsening pedal edema and orthopnea. On physical examination, his vital signs are T 37.0 C, P 78/minute, RR 16/minute, and BP 130/70 mm Hg. There are no murmurs, rubs, or gallops audible. Bibasilar crackles in the lungs are audible. Which of the following conditions is most likely to account for these findings?

A Myocarditis

B Pulmonary hypertension

C Mitral valvular stenosis

D Coronary arteriopathy

E Angiosarcoma

F Constrictive pericarditis

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An 18-year-old man was born with a membranous ventricular septal defect that was never repaired. Over the past year his exercise tolerance has become greatly reduced. On physical examination he is afebrile, with blood pressure 110/65 mm Hg. A chest radiograph now reveals a prominent right heart border. His arterial oxygen saturation is now only 88%. Which of the following is the most likely diagnosis?

A Eisenmenger complex

B Restrictive cardiomyopathy

C Infective endocarditis

D Coronary atherosclerosis

E Tetralogy of Fallot

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A 65-year-old woman, a heavy smoker for many years, has had worsening dyspnea for the past 5 years, without a significant cough. On physical examination her vital signs show T 37.1 C, P 77/minute, R 15/minute, and BP 115/70 mm Hg. A chest x-ray shows increased chest size, increased lucency of lung fields, and flattening of the diaphragms. Over the next 3 years she develops worsening peripheral edema. Which of the following cardiac abnormalities is most likely to be present in this woman?

A Mitral valve stenosis

B Constrictive pericarditis

C Right ventricular hypertrophy

D Left ventricular aneurysmm

E Non-bacterial thrombotic endocarditis

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A 21-year-old primigravida has a screening ultrasound scan performed at 18 weeks gestation. The scan reveals a male fetus with an endocardial cushion defect (atrio-ventricular canal defect). Other abnormalities noted include increased nuchal thickening and a "double bubble" sign suggesting duodenal atresia. Which of the following congenital problems is the most likely diagnosis?

A Maternal use of phentermine-dexfenfluramine

B Marfan syndrome

C Maternal folate deficiency

D Trisomy 21

E Congenital syphilis

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A 61-year-old man comes to his physician for a routine check of his health status. On physical examination his blood pressure is 183/100 mmHg while sitting and 190/105 while standing. He has a heart rate of 81/minute, with an irregularly irregular rhythm. On auscultation of the chest, there are no murmurs, but he has bibasilar crackles in lower lung fields. Pulses are equal and full in all extremities. Which of the following pathologic findings is most likely to be present in his heart?

A Left ventricular hypertrophy

B Left atrial myxoma

C Occlusive coronary atherosclerosis

D Cor pulmonale

E Mitral regurgitation

F Aortic coarctation

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A 59-year-old man who has been active all his life develops sudden severe anterior chest pain that radiates to his back. Within minutes, he is unconscious. He has a history of hypertension, but a recent treadmill test had revealed no evidence for cardiac disease. On physical examination now shows that he is afebrile, with pulse 101/minute and blood pressure 80/40 mm Hg. Which of the following is the most likely diagnosis for his acute problem?

A Tear in the aortic intima

B Group A streptococcal infection

C Right middle cerebral artery embolus

D Acute viral myocarditis

E Pulmonary embolus

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A 27-year-old man with a history of intravenous drug abuse develops a fever over the past 3 days. Physical examination reveals his temperature to be 39.1 C. He has a heart murmur. His spleen tip is palpable. Echocardiography reveals large vegetations involving the aortic valve. Which of the following complications is most likely to develop during the next week?

A Lung abscess

B Hemopericardium

C Mycotic aneurysm

D Valvular stenosis

E Annular calcification

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A 57-year-old man has noted worsening exercise tolerance for the past 2 years. On physical examination he is afebrile with heart rate of 81/minute and blood pressure 130/85 mm Hg. He has distant heart sounds on auscultation of the chest. A chest radiograph reveals that there is a thin rim of calcification surrounding the cardiac outline. Which of the following conditions is most likely to cause these findings?

A Sarcoidosis

B Tuberculosis

C Rheumatic heart disease

D Uremia

E Metastatic carcinoma

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A 20-year-old man has had reduced exercise tolerance since childhood, but he is still able to perform activities of daily living. On physical examination he is afebrile and normotensive. An echcoardiogram shows that he has a single atrioventricular valve. Which of the following additional structural abnormalities involving his heart most likely allowed him to survive to this age?

A Aortic coarctation

B Single coronary artery

C Truncus arteriosus

D Transposition of great vessels

E Pulmonic stenosis

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A 68-year-old woman with Parkinson disease dies from pneumonia. At autopsy, she is found to have a heart that is enlarged, predominantly the left atrium and left ventricle. The mitral valve shows shortening and thickening with fusion of the chordae tendineae, resulting in diminished size of the valvular orifice. The aortic valve cusps are also thickened, with partial fusion of the commissures that produces a narrowed aortic valvular orifice. Which of the following underlying diseases is most likely to produce these findings?

A Diabetes mellitus

B Systemic lupus erythematosus

C Group A streptococcal infections

D Hereditary hemochromatosis

E Blunt force trauma to the chest

F Parkinson disease

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A 7 month old infant has developed worsening congestive heart failure for the past 2 months. The infant was born at term with no anomalies noted and did well for the first 5 months. There is no family history of heart disease or birth defects. The baby's condition worsens and death occurs. At autopsy, the enlarged heart is found to have extensive left ventricular endocardial thickening with obliteration of the chordae tendineae. The cardiac valves appear normal. Which of the following is the most likely diagnosis?

A Infection with group A streptococcus occurred earlier in infancy

B There was maternal use of cocaine during pregnancy

C An inborn error of metabolism is present

D Gestational diabetes was present

E This condition is idiopathic

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A study of embryologic defects involving the heart is conducted. Results of ultrasound scans are compared to autopsy findings with dissection of the heart. It is observed that, in some cases, a complete failure of development of the spiral septum of the developing fetal heart occurs. Which of the following congenital heart defects is most likely to result from this developmental abnormality?

A Transposition of the great vessels

B Aortic arch interruption

C Persistent truncus arteriosus

D Atrioventricular septal defect

E Overriding aorta

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A 50-year-old woman is bothered by unsightly subcutaneous veins over her lower legs that have gotten more prominent over the past 5 years. On physical examination the skin of her calves appears roughened and brownish, but is not ulcerated. She has no leg swelling. The veins are removed surgically. Her sister developed a similar condition at the age of 22. Which of the following is the most likely mechanism for development of her condition?

A Venous valve incompetence

B Recurrent thrombophlebitis

C Systemic hypertension

D Exercise

E Local trauma

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A 64-year-old man has a fall from a ladder. He experiences pain in his right upper thigh immediately thereafter. On physical examination he has shortening of the right leg. A radiograph reveals a right femoral neck fracture. The radiograph also shows prominent calcified vessels. Which of the following is the most appropriate course of action to take regarding this vascular finding?

A Heparinize the patient

B Ignore it

C Get a CK-MB test

D Start the patient on a nitroprusside drip

E Order a pulmonary ventilation-perfusion scan

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A 51-year-old man has a sudden change in mental status. On examination his sensorium is clouded. A head CT scan shows a right subarachnoid hemorrhage. There is subsequent pressure effect with herniation and death. At autopsy, a peripheral right parietal cerebral artery branch shows acute inflammation with necrosis and aneurysmal dilation with rupture of the arterial wall. This is most likely to be a complication of which of the following diseases?

A Disseminated aspergillosis

B Aortic valve endocarditis with Staphylococcus aureus

C Metastatic adenocarcinoma

D Marfan syndrome

E Polyarteritis nodosa

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An 8-year-old boy was born with a large ventricular septal defect (VSD) that was never surgically corrected. Now he has increasing dyspnea along with hypoxia and cyanosis. Which of the following mechanisms is most likely to cause these findings?

A Natural closure of the VSD

B Pulmonary hypertension

C Acute myocardial infarction

D Endocardial fibroelastosis

E Left atrial thrombosis

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A 35-year-old healthy woman has a routine check of her health status. On physical examination she is afebrile. She has a faint systolic murmur. An echocardiogram shows a bicuspid aortic valve. In explaining the meaning of this finding to her, which of the following is the most appropriate statement to make?

A She should be treated with cholesterol-lowering pharmacologic agents

B Other family members may have the same condition

C An aortic valve prosthesis may eventually need to be placed

D This is one manifestation of an underlying autoimmune disease process

E The problem resulted from past injection drug usage

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A 45-year-old woman has not received regular medical care. At a health screening fair she is found to have a serum glucose of 243 mg/dL. Ten years later she is hospitalized with worsening shortness of breath, and laboratory studies show a CK-MB of 15% with total CK of 524 mg/dL. Five years after that hospitalization she develops steadily worsening exercise tolerance and orthopnea, and by echocardiography her cardiac ejection fraction is measured at only 20%. Which of the following abnormalities is most likely to explain her low ejection fraction?

A Septal hypertrophy with myofiber disarray

B Calcific aortic valvular stenosis

C Left ventricular aneurysm

D Constrictive pericarditis

E Endocardial fibroelastosis

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A 68-year-old man has been very ill for 6 months following the onset of chronic liver disease with hepatitis C infection. He experiences a sudden loss of consciousness and then exhibits paraplegia on the right. A cerebral angiogram reveals lack of perfusion in the left middle cerebral artery distribution. Which of the following cardiac lesions is most likely to be associated with these events?

A Non-bacterial thrombotic endocarditis

B Paradoxical thromboembolus

C Acute rheumatic fever

D Left atrial myxoma

E Libman-Sacks endocarditis

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A 59-year-old man has had decreasing exercise tolerance for 4 years. On physical examination motor strength in his extremities is normal. Peripheral pulses are equal and full. His blood pressure is 110/80 mm Hg. On echocardiography he has decreased cardiac output with diminished diastolic filling, but the heart does not appear to be hypertrophied or dilated. Which of the following conditions is he most likely to have?

A Amyloidosis

B Rheumatic heart disease

C Alcoholic cardiomyopathy

D Coxsackie B viral infection

E Constrictive pericarditis

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A 69-year-old woman has noted swelling of legs for the past 3 months. On physical examination she has 4+ pitting edema involving her lower extremities to a level just above the knees. Her jugular venous pulse is visible to the angle of the jaw. A chest radiograph reveals bilateral pleural effusions along with a prominent right heart border. Which of the following cardiac valvular abnormalities is the most likely cause her findings?

A Mitral stenosis

B Tricuspid insufficiency

C Pulmonic atresia

D Aortic regurgitation

E Common atrioventricular valve

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An 18-year-old man has had reduced exercise tolerance for the past 3 years. On physical examination his blood pressure is 160/80 mm Hg in upper extremities but 110/70 mm Hg in lower extremities. Echocardiography reveals narrowing of his aorta just distal to the left subclavian artery.. Which of the following findings is he most likely to have?

A Positive serologic test for syphilis

B Elevated hemoglobin A1C markedly elevated

C Chest x-ray with rib notching

D Mid-systolic "click" heart murmur

E Arachnodactyly

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A 62-year-old man has had episodes of chest pain during the past 5 years, with a myocardial infarction documented 2 years ago. He has had episodes of abdominal pain over the past month. On physical examination, bowel sounds are present. On palpation of the abdomen there is a midline pulsatile mass. Dorsalis pedis and posterior tibial pulses are barely palpable. An abdominal CT scan reveals a 6 cm diameter enlargement of the abdominal aorta below the renal arteries that is partially filled with thrombus. Bright areas representing calcifications are seen in the aortic wall. Which of the following laboratory test findings is he most likely to have?

A Anti-streptolysin O titer of 1:512

B Plasma homocysteine of 20 micromol/L

C HDL cholesterol of 65 mg/dL

D Coxsackie B serologic titer of 1:80

E Positive serum VDRL

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A 53-year-old woman has had increasing malaise for the past 3 months. On physical examination she is afebrile, with blood pressure 165/95 mm Hg. Laboratory studies show her anti-neutrophil cytoplasmic autoantibody (ANCA) test is positive at 1:160. A renal biopsy is performed and on microscopic examination shows a focal necrotizing vasculitis. Which of the following complications is she most likely to have?

A Angina

B Hemorrhagic pericarditis

C Hemoptysis

D Endocarditis

E Hemothorax

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A clinical study of untreated systemic hypertension is performed. The subjects are adults from 50 to 90 years of age who had blood pressures recorded consistently above 165/105 mm Hg for more than 10 years but who elected not to receive antihypertensive therapy. The medical problems developing in these patients are analyzed. Which of the following problems are they most likely to develop from their untreated hypertension?

A Cerebral hemorrhage (stroke)

B Aortic aneurysm rupture

C Congestive heart failure

D Chronic renal failure

E Intracranial arterial aneurysm rupture

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An 11-year-old girl is brought to your office by her mother who is concerned that the child exhibits irregular, uncontrolled movements some of the time, but mostly does not want to get out of bed. On physical examination the child has a fever. The skin shows erythema marginatum and subcutaneous nodules. Laboratory studies show leukocytosis and a positive anti-DNAse B titer. Which of the following is the most likely long-term risk from this girl's disease process?

A Sudden death

B Ischemic cardiomyopathy

C Constrictive pericarditis

D Valvular prolapse

E Cerebral embolization

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A 70-year-old woman, a non-smoker, has been bothered by left sided headaches for 3 months. She does not seek medical attention. A month later she notices sudden decreased visual acuity in her left eye. On physical examination her vital signs show T 36.9 C, P 74/minute, R 14/minute, and BP 125/85 mm Hg. Laboratory studies show an erythrocyte sedimentation rate of 94 mm/hr. Her CBC shows a WBC count of 7540/microliter. Which of the following is the most likely diagnosis?

A Wegener granulomatosis

B Polyarteritis nodosa

C Raynaud disease

D Giant cell arteritis

E Thromboangitis obliterans

F Atherosclerosis

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A 33-year-old man develops a low grade fever over the past 4 days. During the next week he develops dyspnea and palpitations. He dies suddenly and unexpectedly. The medical examiner finds a diffusely enlarged, flabby heart with no focal grossly apparent lesions. The coronary arteries show minimal atherosclerosis with no narrowing. Which of the following is the most likely cause of death?

A Cocaine overdose

B Acute rheumatic fever

C Viral myocarditis

D Systemic lupus erythematosus

E Endomyocardial fibrosis

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A 60-year-old woman has the sudden onset of severe chest pain. She has had anginal pain for the past 5 years, but this time the pain is much worse and not relieved by nitroglycerin. On physical examination her vital signs include T 37.1 C, P 100/minute, R 24/minute, and BP 155/100 mm Hg. A chest radiograph shows a widened mediastinum, with no pulmonary edema or pleural effusion. She loses consciousness. An emergent pericardiocentesis yields blood. Which of the following is the most likely diagnosis?

A Aortic dissection

B Mitral valve prolapse

C Polyarteritis nodosa

D Rheumatic myocarditis

E Pericardial metastases

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A clinical study of complications of aortic porcine bioprostheses is conducted. It is observed that, on average, within 5 to 10 years of their initial surgery to place the prostheses, the subjects have had a second surgery to replace the original bioprosthesis. These excised bioprostheses were examined to determine the pathologic findings. Which of the following abnormalities is most likely to be found in the excised bioprostheses?

A Infective endocarditis

B Thrombosis with occlusion

C Deterioration with calcification

D Chronic rejection

E Paravalvular leakage with insufficiency

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A 45-year-old woman has had worsening congestive heart failure for 5 years and increasing difficulty swallowing. On physical examination she has diffuse crackles in all lung fields. She is afebrile and normotensive. An echocardiogram shows mitral stenosis. She undergoes open heart surgery with mitral valve replacement with a mechanical prosthesis. The excised valve grossly shows thickened and fused chordae tendineae. Which of the following underlying conditions is she most likely to have?

A Marfan syndrome

B Congenital heart disease

C Rheumatic heart disease

D Systemic lupus erythematosus

E Diabetes mellitus

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A 32-year-old man has the sudden onset of chest pain. On physical examination his vital signs include T 37.2 C, P 90/minute, R 20/minute, and BP 100/60 mm Hg. MR imaging of the chest shows a tear in the aortic intima. Which of the following inherited conditions is he most likely to have?

A Turner syndrome

B Neurofibromatosis

C Familial hypercholesterolemia

D Marfan syndrome

E Dilated cardiomyopathy

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An autopsy study of causes for hemopericardium is conducted. Cases are selected in which the pericardial cavity was found to contain over 300 cc of clotted and liquid blood at the time of autopsy and in which heart was normal in size, with coronary arteries that showed no atherosclerosis. It is found that the median age for this group of patients is 25 years. Which of the following is the most likely cause for hemopericardium in these cases?

A Disseminated Mycobacterium tuberculosis infection

B Chronic rheumatic valvulitis

C Vasculitis associated with systemic lupus erythematosus

D Penetrating trauma to the chest

E Small cell anaplastic carcinoma of the right lung with metastases

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A 63-year-old woman has experienced a series of transient ischemic attacks (TIAs) over the past year. During these episodes she loses consciousness for several minutes. On physical examination her vital signs include T 37 C, P 77/minute, RR 15/minute, and BP 140/85 mm Hg. Her BMI is 30. Laboratory studies show a serum glucose of 125 mg/dL with creatinine 0.9 mg/dL and total cholesterol 224 mg/dL. An echocardiogram shows no valvular or lumenal lesions, and her ejection fraction is 46%. Which of the following cardiovascular abnormalities is show most likely to have?

A Atrial myxoma

B Carotid stenosis

C Syphilitic aortitis

D Aortic aneurysm

E Rheumatic heart disease

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A 28-year-old woman with a history of intravenous drug use has had a progressive dementia for the past 6 months. On physical examination she shows cachexia. She is afebrile and normotensive. Laboratory studies show a positive HIV test and a CD4 lymphocyte count of 100/microliter. Which of the following pathologic conditions is most likely to involve her heart?

A Endocardial fibroelastosis

B Kaposi sarcoma

C Interatrial septal rhabdomyoma

D Left atrial myxoma

E High grade B-cell lymphoma

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A 22-year-old woman has a 3 year history of systemic lupus erythematosus. She now presents with increasing dyspnea over the past day. On physical examination her heart sounds are distant on auscultation. She is noted to have distended neck veins to the angle of the jaw while sitting. She is afebrile. Her chest radiograph reveals a globally enlarged cardiac shadow. An echocardiogram shows no valvular lesions, but there is a large pericardial effusion. Laboratory findings include a normal serum urea nitrogen and creatinine, normocytic anemia, and normal serum creatine kinase. A pericardiocentesis is performed. Which of the following laboratory findings is most likely to be present in the fluid obtained?

A A low protein

B Numerous RBC's

C Chylomicrons

D Malignant cells

E Gram positive cocci

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A 12-year-old child who lives in a mud hut in northeastern Brazil presents to the clinic with increasing malaise for the past 2 weeks. On physical examination, she has right conjunctival edema, preauricular lymphadenopathy, and exophthalmos. She has periorbital and presacral edema. Which of the following diseases is she most likely to have?

A Idiopathic dilated cardiomyopathy

B Chagas disease

C Endomyocardial fibroelastosis

D Toxoplasmosis

E Beri beri

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A 60-year-old woman with a 100 pack year history of smoking has had increasing dyspnea for the past 10 years. On physical examination she is using accessory muscles for respirations. Auscultation reveals expiratory wheezes. She has 2+ pitting edema to the knees. She is afebrile. A chest CT scan shows no masses, but there are multiple bullae, particularly of upper lobes, and pleural effusions are present. Thoracentesis is performed. Which of the following laboratory test findings is most likely to be present in the fluid obtained?

A Albumin of 0.8 g/dL

B Malignant cells

C Neutrophils

D Chylomicrons

E Hemosiderin-laden macrophages

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A 49-year-old man with a history of diabetes mellitus develops sudden dyspnea. On physical examination vital signs show T 37 C, P 95/minute, R 22/minute, and BP 90/50 mm Hg. Emergent angiography reveals a thrombosis of the left anterior descending artery. Which of the following serum analytes is most likely to be increased in this man?

A Amylase

B Creatine kinase

C Lactate dehydrogenase

D Aspartate aminotransferase

E Bilirubin

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A 60-year-old man has had worsening dyspnea and orthopnea for the past year. On physical examination he is afebrile and normotensive. He has no peripheral edema. A chest x-ray shows pulmonary congestion and edema with a prominent left heart border. Laboratory studies show a serum glucose of 95 mg/dl with total serum cholesterol 185 mg/dl. His serum creatine kinase is not elevated. Which of the following cardiac abnormalities is he most likely to have?

A Aortic dissection

B Alcoholic cardiomyopathy

C Tricuspid valve endocarditis

D Mitral valve annulus calcification

E Calcified bicuspid aortic valve

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A 6 month old male infant has exhibited decreased activity and increasing peripheral edema for the past 3 months. On physical examination no gross congenital anomalies are noted. No cardiac murmurs are auscultated. A radiograph shows marked cardiomegaly. There is no history of serious infections. Which of the following conditions is this infant most likely to have?

A Hemochromatosis

B Rheumatoid heart disease

C Pompe disease

D Idiopathic dilated cardiomyopathy

E Sarcoidosis

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A 45-year-old woman has noted increasing dyspnea and orthopnea for the past month. On physical examination vital signs show T 37 C, P 76/minute, RR 16/minute, and BP 140/90 mm Hg. There is 2+ pitting edema to her knees. Her liver span is increased. There is jugular venous distension to the angle of the jaw while sitting. She has a greater than normal (10 mmHg) inspiratory decline in systolic arterial pressure. A chest x-ray shows an enlarged cardiac shadow. Laboratory studies show a CBC with hemoglobin 14.1 g/dL, WBC count 7780/microliter, and platelet count 291,000/microliter. Her serum urea nitrogen is 16 mg/dL, glucose 84 mg/dL, and anti-DNA topoisomerase I positive. Which of the following is the most likely mechanism to produce these findings?

A Myocardial ischemia

B Pericardial effusion

C Valvular insufficiency

D Right-to-left shunt

E Outflow obstruction

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A 32-year-old man with a 40 pack year history of smoking has experienced increasing pain with ambulation for the past 5 months. Over the past month he has had pain even at rest. On physical examination his vital signs include T 37.1 C, P 66/minute, RR 16/minute, and blood pressure 110/75 mm Hg. There is a shallow ulceration over his right great toe. Motor strength is normal in all extremities. Dorsalis pedis and posterior tibial pulses are diminished. Angiography reveals narrowing with corkscrew outlines of posterior tibial and peroneal arteries. Laboratory studies show a serum glucose of 70 mg/dL, creatinine 1.1 mg/dL, and total cholesterol of 189 mg/dL. Which of the following is the most likely diagnosis?

A Giant cell arteritis

B Kawasaki syndrome

C Microscopic polyangiitis

D Polyarteritis nodosa

E Takayasu arteritis

F Thromboangiitis obliterans

G Wegener granulomatosis

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