Robbin's Review - Ch.12 (Heart)

Two weeks after having a mild upper respiratory tract infection, a 14-year-old girl has fever and chest pain. On physical examination, her temperature is 37°C, pulse is 90/min, respirations are 20/min, and blood pressure is 85/45 mm Hg. A friction rub is audible on auscultation of the chest. A chest radiograph shows pulmonary edema. An echocardiogram shows small vegetations at the closure line of the mitral and aortic valves. An endocardial biopsy shows focal interstitial inflammation with Aschoff nodules and Anichkov cells. Her condition improves over the next month. The representative gross appearance of the disease process 20 years later is shown in the figure. Which of the following additional complications of this illness is most likely to be seen in the patient? □ (A) Aortic stenosis □ (B) Constrictive pericarditis □ (C) Left ventricular aneurysm □ (D) Mitral valve prolapse □ (E) Dilated cardiomyopathy

A. Aortic stenosis

An 86-year-old man has had increasing dyspnea and reduced exercise tolerance for the past 7 years. On physical examination, he is afebrile and has a blood pressure of 135/85 mm Hg. An irregularly irregular heart rate averaging 76/min is audible on auscultation of the chest. Crackles are heard at the bases of the lungs. A chest radiograph shows mild cardiomegaly and mild pulmonary edema. Echocardiography shows slight right and left ventricular wall thickening with reduced left and right ventricular wall motion, reduced left ventricular filling, and an ejection fraction estimated to be 25%. An endomyocardial biopsy specimen shows amorphous pink-staining deposits between myocardial fibers, but no inflammation and no necrosis. Which of the following is the most likely diagnosis? □ (A) Cardiac amyloidosis □ (B) Rheumatic heart disease □ (C) Constrictive pericarditis □ (D) Mitral valve prolapse □ (E) Left ventricular aneurysm

A. Cardiac amyloidosis

A 25-year-old man dies suddenly and unexpectedly. At autopsy his heart is enlarged from right ventricular dilation. The left ventricle is normal. There is no coronary atherosclerosis. Sectioning of the myocardium shows marked thinning of the right ventricle, and microscopic findings include extensive myocardial fatty infiltration and fibrosis, but no inflammation. Which of the following is the most likely cause for his sudden death? □ (A) Cardiomyopathy □ (B) Chagas disease □ (C) Hypertension □ (D) Long QT syndrome □ (E) Radiation therapy

A. Cardiomyopathy

A 27-year-old woman has had a fever for 5 days. On physical examination, her temperature is 38.2°C, pulse is 100/min, respirations are 19/min, and blood pressure is 90/60 mm Hg. A cardiac murmur is heard on auscultation. The sensorium is clouded, but there are no focal neurologic deficits. Laboratory findings include hemoglobin, 13.1 g/dL; platelet count, 233,300/mm3; and WBC count, 19,200/mm3. Blood cultures are positive for Staphylococcus aureus. Urinalysis shows hematuria. An echocardiogram shows a 1.5-cm vegetation on the mitral valve. Which of the following conditions is this patient most likely to develop? □ (A) Cerebral arterial mycotic aneurysm □ (B) Dilated cardiomyopathy □ (C) Abscess of the left upper lobe □ (D) Myxomatous degeneration of the mitral valve □ (E) Polyarteritis nodosa □ (F) Polycystic kidneys

A. Cerebral arterial mycotic aneurysm

In the third match of a volleyball tournament, a 15-year-old girl jumps up for a block and collapses. Despite cardiopulmonary resuscitation, she cannot be revived. She had been healthy all her life and complained only of limited episodes of chest pain in games during the current school year. Which of the following pathologic findings of the heart is the medical examiner most likely to find? □ (A) Haphazardly arranged hypertrophied septal myocytes □ (B) Extensive myocardial hemosiderin deposition □ (C) Tachyzoites within foci of myocardial necrosis and inflammation □ (D) Mitral valvular stenosis with left atrial enlargement □ (E) Large, friable vegetations with destruction of aortic valve cusps

A. Haphazardly arranged hypertrophied septal myocytes

For the past 2 years, a 49-year-old woman has had a chronic cough that produces a small amount of whitish sputum. The sputum occasionally is blood-streaked. On physical examination, her temperature is 37.9°C, pulse is 71/min, respirations are 17/min, and blood pressure is 125/80 mm Hg. Crackles are heard on auscultation over the upper lung fields. Heart sounds are faint, and there is a 15 mm Hg inspiratory decline in systolic arterial pressure. The chest radiograph shows prominent heart borders with a "water bottle" configuration. Pericardiocentesis yields 200 mL of bloody fluid. Infection with which of the following organisms is most likely to produce these findings? □ (A) Mycobacterium tuberculosis □ (B) Group A streptococcus □ (C) Coxsackievirus B □ (D) Candida albicans □ (E) Staphylococcus aureus

A. M.tuberculosis

A 10-year-old girl who is normally developed has chronic progressive exercise intolerance. On physical examination, temperature is 37.1°C, pulse is 70/min, respirations are 14/min, and blood pressure is 100/60 mm Hg. A chest radiograph shows cardiomegaly and mild pulmonary edema. An echocardiogram shows severe left ventricular hypertrophy and a prominent interventricular septum. The right ventricle is slightly thickened. During systole, the anterior leaflet of the mitral valve moves into the outflow tract of the left ventricle. The ejection fraction is abnormally high, and the ventricular volume and cardiac output are both low. Which of the following is the most likely cause of the cardiac abnormalities in this patient? □ (A) Mutations in β-myosin heavy chain □ (B) Autoimmunity against myocardial fibers □ (C) Excessive iron accumulation □ (D) Deposition of amyloid protein □ (E) Latent enterovirus infection

A. Mutations in beta-myosin heavy chain

A 50-year-old man has sudden onset of severe substernal chest pain that radiates to the neck. On physical examination, he is afebrile, but has tachycardia, hyperventilation, and hypotension. No cardiac murmurs are heard on auscultation. Emergent coronary angiography shows a thrombotic occlusion of the left circumflex artery and areas of 50% to 70% narrowing in the proximal circumflex and anterior descending arteries. Which of the following complications of this disease is most likely to occur within 1 hour of these events? □ (A) Ventricular fibrillation □ (B) Pericarditis □ (C) Myocardial rupture □ (D) Ventricular aneurysm □ (E) Thromboembolism

A. Ventricular fibrillation

A 19-year-old man has had a low-grade fever for 3 weeks. On physical examination, his temperature is 38.3°C, pulse is 104/min, respirations are 28/min, and blood pressure is 95/60 mm Hg. A tender spleen tip is palpable. There are splinter hemorrhages under the fingernails and tender hemorrhagic nodules on the palms and soles. A heart murmur is heard on auscultation. Which of the following infectious agents is most likely to be cultured from this patient's blood? □ (A) Viridans streptococci □ (B) Trypanosoma cruzi □ (C) Coxsackievirus B □ (D) Candida albicans □ (E) Mycobacterium tuberculosis □ (F) Pseudomonas aeruginosa

A. Viridans streptococci

A 77-year-old woman sees her physician for a routine health maintenance examination. On physical examination, she is afebrile. Her pulse is 66/min, respirations are 14/min, and blood pressure is 125/85 mm Hg. On auscultation, a systolic ejection murmur is heard. There are a few crackles over the lung bases posteriorly. From the representative gross appearance of the aortic valve shown in the figure, which of the following most likely contributed to the development of this lesion? □ (A) Chromosomal aneuploidy □ (B) Aging □ (C) Tertiary syphilis □ (D) Atherosclerosis □ (E) Systemic lupus erythematosus

B. Aging

A 15-year-old boy complains of pain in his legs when he runs more than 300 m. Physical examination shows temperature, 36.8°C; pulse, 76/min; respirations, 22/min; and blood pressure, 165/90 mm Hg. The radial pulses are 4+, and the dorsalis pedis pulses are 1+. Arterial blood gas measurement shows a normal oxygen saturation level. Which of the following lesions is most likely to be present in this patient? □ (A) Tricuspid atresia □ (B) Coarctation of the aorta □ (C) Aortic valve stenosis □ (D) Patent ductus arteriosus □ (E) Transposition of the great arteries

B. Coarctation of the aorta

In a clinical study of tetralogy of Fallot, patients are examined before surgery to determine predictors observed on echocardiography that correlate with the severity of the disease and the need for more careful monitoring. A subset of patients is found to have more severe congestive heart failure, poor exercise tolerance, and decreased arterial oxygen saturation levels. Which of the following is most likely to predict a worse clinical presentation for these patients? □ (A) Size of the left ventricle □ (B) Degree of pulmonary stenosis □ (C) Size of the ventricular septal defect □ (D) Diameter of the tricuspid valve □ (E) Presence of an atrial septal defect

B. Degree of pulmonary stenosis

A 49-year-old, previously healthy woman reports having suddenly lost consciousness four times in the past 6 months. In three instances, she was unconsciousness for only a few minutes. After the fourth episode 1 month ago, she was unconscious for 6 hours and had weakness in her left arm and difficulty speaking. On physical examination, she is afebrile, and her blood pressure is normal. She has good carotid pulses with no bruits. Which of the following cardiac lesions is most likely to be present? □ (A) Pericardial effusion □ (B) Left atrial myxoma □ (C) Bicuspid aortic valve □ (D) Mitral valve stenosis □ (E) Left anterior descending artery thrombosis

B. Left atrial myxoma

A 48-year-old woman has had increasing dyspnea for the past 2 days. She experiences sudden cardiac arrest and cannot be resuscitated. The light microscopic appearance of the left ventricular free wall at autopsy is shown in the figure. Which of the following is the most likely diagnosis? □ (A) Viral myocarditis □ (B) Myocardial infarction □ (C) Acute rheumatic myocarditis □ (D) Septic embolization □ (E) Restrictive cardiomyopathy

B. MI

A 45-year-old man has had increasing fatigue, exertional dyspnea, and episodes of mild chest pain for the past 7 months. On physical examination, he is afebrile. His pulse is 79/min, respirations are 15/min, and blood pressure is 125/75 mm Hg. Laboratory studies show normal levels of serum troponin I, glucose, creatinine, and total cholesterol. The representative microscopic appearance of the myocardium with a Prussian blue stain is shown in the figure. Echocardiography would most likely show which of the following functional cardiac disturbances? □ (A) Dynamic obstruction to left ventricular outflow □ (B) Reduced ventricular compliance resulting in impaired ventricular filling in diastole □ (C) Mitral and tricuspid valvular insufficiency □ (D) Lack of ventricular expansion during diastole □ (E) Reduced ejection fraction from decreased contraction

B. Reduced ventricular compliance resulting in impaired ventricular filling in diastole

A 44-year-old woman who has rheumatic heart disease with aortic stenosis undergoes valve replacement with a bioprosthesis. She remains stable for the next 8 years and then develops diminished exercise tolerance. Which of the following complications involving the bioprosthesis has most likely occurred? □ (A) Paravalvular leak □ (B) Stenosis □ (C) Hemolysis □ (D) Embolization □ (E) Myocardial infarction

B. Stenosis

A 59-year-old man has experienced chronic fatigue for the past 18 months. On physical examination, he is afebrile. A chest radiograph shows bilateral pulmonary edema and a prominent border on the left side of the heart. The representative gross appearance of the heart is shown. Laboratory studies show serum glucose, 74 mg/dL; total cholesterol, 189 mg/dL; total protein, 7.1 g/dL; albumin, 5.2 g/dL; creatinine, 6.1 mg/dL; and urea nitrogen, 58 mg/dL. What is the most likely diagnosis? □ (A) Chronic alcoholism □ (B) Systemic hypertension □ (C) Pneumoconiosis □ (D) Hemochromatosis □ (E) Diabetes mellitus

B. Systemic hypertension

A 68-year-old man has had progressive dyspnea for the past year. On physical examination, extensive rales are heard in all lung fields. An echocardiogram shows that the left ventricular wall is markedly hypertrophied. A chest radiograph shows pulmonary edema and a prominent left-sided heart shadow. Which of the following conditions has most likely produced these findings? □ (A) Centrilobular emphysema □ (B) Systemic hypertension □ (C) Tricuspid valve regurgitation □ (D) Chronic alcoholism □ (E) Silicosis

B. Systemic hypertension

A 60-year-old man visits the physician because of worsening cough and orthopnea. On physical examination, he has dullness to percussion at the lung bases and diffuse crackles in the upper lung fields. He is afebrile. Echocardiography shows marked left ventricular hypertrophy and severe aortic stenosis. The remaining cardiac valves are normal. A coronary angiogram shows no significant coronary arterial narrowing. Which of the following conditions best accounts for these findings? □ (A) Diabetes mellitus □ (B) Marfan syndrome □ (C) Bicuspid aortic valve □ (D) Systemic hypertension □ (E) Infective endocarditis

C. Bicuspid aortic valve

A 45-year-old man experiences crushing substernal chest pain after arriving at work one morning. Over the next 4 hours, the pain persists and begins to radiate to his left arm. He becomes diaphoretic and short of breath, but waits until the end of his 8-hour shift to go to the hospital. An elevated serum value of which of the following laboratory tests would be most useful for diagnosis of this patient on admission to the hospital? □ (A) Lipase □ (B) AST □ (C) CK-MB fraction □ (D) ALT □ (E) LDH-1 □ (F) C-reactive protein

C. CK-MB fraction

A 73-year-old man has developed worsening congestive heart failure over the past year. On physical examination, he has pitting edema to the thighs. There is dullness to percussion at the lung bases. He develops pneumonia and dies. At autopsy, there is marked right ventricular and right atrial dilation and hypertrophy. The aorta shows minimal atherosclerosis, and the pulmonary trunk shows moderate atherosclerosis. Which of the following conditions is most likely to have produced these findings? □ (A) Saddle pulmonary thromboembolism □ (B) Ventricular septal defect □ (C) Chronic obstructive pulmonary disease □ (D) Rheumatic heart disease □ (E) Hypertrophic cardiomyopathy

C. COPD

A study of ischemic heart disease analyzes cases of individuals hospitalized with acute chest pain in which myocardial infarction was documented at autopsy. The gross and microscopic appearances of the hearts are correlated with the degree of coronary atherosclerosis and its complications, clinical symptoms, and therapies given before death. Hemorrhage and contraction bands in necrotic myocardial fibers are most likely to be seen with infarction in which of the following settings? □ (A) Acute vasculitis □ (B) Angioplasty with stent □ (C) Coronary thrombosis □ (D) Septic embolization □ (E) Thrombolytic therapy

C. Coronary thrombosis

A 32-year-old woman who lives in Pensacola, Florida, goes to the physician because of increasingly severe dyspnea, orthopnea, and swelling of the legs for the past 2 weeks. She has no previous history of serious illness or surgery. On physical examination, her temperature is 37.8°C, pulse is 83/min, respirations are 20/min, and blood pressure is 100/60 mm Hg. An ECG shows episodes of ventricular tachycardia. An echocardiogram shows right and left ventricular dilation, but no valvular deformities. An endomyocardial biopsy shows focal myocyte necrosis and lymphocytic infiltrate. Which of the following organisms most likely caused the infection? □ (A) Trypanosoma cruzi □ (B) Viridans streptococci □ (C) Coxsackievirus A □ (D) Toxoplasma gondii □ (E) Staphylococcus aureus □ (F) Mycobacterium kansasii

C. Coxsackievirus A

A 60-year-old man has experienced angina on exertion for the past 6 years. A coronary angiogram performed 2 years ago showed 75% stenosis of the left anterior descending coronary artery and 50% stenosis of the right coronary artery. For the past 3 weeks, the frequency and severity of the anginal attacks have increased, and pain sometimes occurs even when he is lying in bed. On physical examination, his blood pressure is 110/80 mm Hg, and pulse is 85/min with irregular beats. Laboratory studies show serum glucose, 188 mg/dL; creatinine, 1.2 mg/dL; and troponin I, 1.5 ng/mL. Which of the following is most likely to explain these findings? □ (A) Hypertrophy of ischemic myocardium with increased oxygen demands □ (B) Increasing stenosis of right coronary artery □ (C) Fissuring of plaque in left coronary artery with superimposed mural (partial) thrombosis □ (D) Sudden complete thrombotic occlusion of right and left coronary arteries □ (E) Reduction in oxygen-carrying capacity owing to pulmonary congestion

C. Fissuring of plaque in left coronary artery w/ superimposed mural thrombosis

A 50-year-old man with a lengthy history of diabetes mellitus and hypertension has had pain in the left shoulder and arm for the past 12 hours. He attributes the pain to arthritis and takes acetaminophen. Over the next 6 hours, he develops shortness of breath, which persists for 2 days. On day 3, he visits the physician. On physical examination, his temperature is 37.1°C, pulse is 82/min, respirations are 18/min, and blood pressure is 160/100 mm Hg. Laboratory studies show that the total creatine kinase (CK) activity is within reference range, but the troponin I level is elevated. The patient is admitted to the hospital and continues to experience dyspnea for the next 3 days. On day 7 after the onset of shoulder pain, he has a cardiac arrest. Resuscitative measures are unsuccessful. Postmortem examination shows a large transmural infarction of the left anterior free wall with rupture and hemopericardium. Which of the following statements is best supported by these clinical and autopsy data? □ (A) Infarction did not develop until day 5 or day 6 after the episode of chest pain □ (B) The normal CK level obtained on day 3 excludes the possibility of infarction within the preceding 72 hours □ (C) He had an acute infarction occurring on the day he developed shoulder pain □ (D) A CK-MB fraction determination would have detected acute infarction on day 3 □ (E) A second acute infarction on day 6 or day 7 caused myocardial rupture within several hours

C. He had an acute infarction occurring on the day he developed shoulder pain

A 68-year-old man has become increasingly lethargic and weak for the past 7 months. On physical examination, his temperature is 36.9°C, pulse is 70/min, respirations are 15/min, and blood pressure is 150/90 mm Hg. On auscultation, the physician notes a friction rub. There are no other remarkable findings. The representative gross appearance of the heart is shown in the figure. Which of the following laboratory findings is most likely to be reported for this patient? □ (A) Positive ANA □ (B) Elevated antistreptolysin O titer □ (C) Increased urea nitrogen level □ (D) Elevated renin level □ (E) Increased serum CK level

C. Increased urea nitrogen level

A 2-year-old child is brought to the physician for a routine examination. Physical examination shows a low-pitched cardiac murmur. An echocardiogram shows the presence of ostium secundum, with a 1-cm defect. Which of the following abnormalities is most likely to be found in this child? □ (A) Pulmonary hypertension □ (B) Pericardial effusion □ (C) Left-to-right shunt □ (D) Mural thrombosis □ (E) Cyanosis

C. Left-to-right shunt

A 70-year-old woman sees her physician because she has had episodes of chest pain during the past week. She is afebrile. Her pulse is 80/min, respirations are 16/min, and blood pressure is 110/70 mm Hg. On auscultation of the chest, heart sounds seem distant, but the lung fields are clear. Neck veins are distended to the angle of the jaw, even while sitting. There is a darkly pigmented, 1.2-cm skin lesion on the right shoulder. A chest radiograph shows cardiomegaly and prominent borders on the left and right sides of the heart. Pericardiocentesis yields bloody fluid. Laboratory findings include a serum troponin I level of 0.3 ng/mL. Which of the following is the most likely cause of these findings? □ (A) Calcific aortic stenosis □ (B) Coronary atherosclerosis □ (C) Metastases □ (D) Rheumatic heart disease □ (E) Tuberculosis

C. Metastases

During the past year, a 34-year-old woman has had palpitations, fatigue, and worsening chest pain. On physical examination, she is afebrile. Her pulse is 75/min, respirations are 15/min, and blood pressure is 110/70 mm Hg. Auscultation of the chest indicates a midsystolic click with late systolic murmur. A review of systems indicates that the patient has one or two anxiety attacks per month. An echocardiogram is most likely to show which of the following? □ (A) Aortic valvular vegetations □ (B) Pulmonic stenosis □ (C) Mitral valve prolapse □ (D) Patent ductus arteriosus □ (E) Tricuspid valve regurgitation

C. Mitral valve prolapse

A 21-year-old primigravida gives birth at term to a 2800-g infant with no apparent external anomalies. The next day, the infant develops increasing respiratory distress and cyanosis and dies. At autopsy, a slitlike left ventricular chamber, small left atrium, and atretic aortic and mitral valves are seen. Through which of the following structures could oxygenated blood most likely have reached the infant's systemic circulation? □ (A) Anomalous venous return □ (B) Foramen ovale □ (C) Patent ductus arteriosus □ (D) Right fourth aortic arch □ (E) Truncus arteriosus □ (F) Ventricular septal defect

C. Patent ductus arteriosus

While touring a 17th century European mansion, you notice that one of the antique beds was designed so that the occupant slept while sitting up. What cardiac disease in the 40-year-old wife of the mansion's owner at that time would best explain this bed design? □ (A) Libman-Sacks endocarditis □ (B) Giant cell myocarditis □ (C) Rheumatic heart disease □ (D) Atrial myxoma □ (E) Fibrinous pericarditis

C. Rheumatic heart disease

A 50-year-old man with a history of infective endocarditis has increasing fatigue. On physical examination, he is afebrile. His pulse is 80/min, respirations are 17/min, and blood pressure is 110/70 mm Hg. On auscultation, diffuse rales are heard in the lungs. A chest radiograph shows bilateral pulmonary edema. An echocardiogram shows mitral regurgitation. He receives a bileaflet tilting disk mechanical mitral valve prosthesis. After surgery, he is stable, and an echocardiogram shows no abnormal valvular or ventricular function. Which of the following pharmacologic agents should he receive regularly after this surgical procedure? □ (A) Amiodarone □ (B) Ciprofloxacin □ (C) Warfarin □ (D) Cyclosporine □ (E) Digoxin □ (F) Propranolol

C. Warfarin

A 50-year-old man has had increasing abdominal discomfort and swelling of his legs for the past 2 years. On physical examination, he has jugular venous distention, even when sitting up. The liver is enlarged and tender and can be palpated 10 cm below the right costal margin. Pitting edema is observed on the lower extremities. A chest radiograph shows bilateral diaphragmatic flattening, pleural effusions, and increased lucency of lung fields. Thoracentesis on the right side yields 500 mL of clear fluid with few cells. Which of the following is most likely to produce these findings? □ (A) Tricuspid valve stenosis □ (B) Acute myocardial infarction □ (C) Pulmonary valve stenosis □ (D) Chronic obstructive pulmonary disease □ (E) Primary pulmonary hypertension

D. COPD

A 37-year-old woman dies suddenly. Investigation of the scene of death in her bedroom at home and external examination of the body show no evidence of trauma. The microscopic appearance of the proximal left anterior descending artery at autopsy is shown in the figure. Which of the following conditions is most likely to be the underlying cause of death? □ (A) Marfan syndrome □ (B) Acute leukemia □ (C) Polyarteritis nodosa □ (D) Diabetes mellitus □ (E) Chronic alcoholism

D. DM

For the past 2 years, a 56-year-old man has experienced increased fatigue and decreased exercise tolerance. On physical examination, his temperature is 36.9°C, pulse is 75/min, respirations are 17/min, and blood pressure is 115/75 mm Hg. On auscultation, diffuse crackles are audible. The abdomen is distended with a fluid wave, and there is bilateral pitting edema to the knees. A chest radiograph shows pulmonary edema, pleural effusions, and marked cardiomegaly. An echocardiogram shows mild tricuspid and mitral regurgitation and reduced right and left ventricular wall motion, with an ejection fraction of 30%. He experiences cerebral, renal, and splenic infarctions over the next year. Chronic use of which of the following substances is most likely to produce these findings? □ (A) Acetaminophen □ (B) Cocaine □ (C) Digoxin □ (D) Ethanol □ (E) Lisinopril □ (F) Nicotine □ (G) Propranolol

D. Ethanol

A 41-year-old man has had increasing dyspnea for the past week. On physical examination, temperature is 37.3°C, pulse is 85/min, respirations are 20/min, and blood pressure is 150/95 mm Hg. There is dullness to percussion over the lung bases. A chest radiograph shows large bilateral pleural effusions and a normal heart size. Laboratory findings include serum creatinine, 3.1 mg/dL; urea nitrogen, 29 mg/dL; troponin I, 0.1 ng/mL; WBC count, 3760/mm3; hemoglobin, 11.7 g/dL; and positive ANA and anti-double-stranded DNA antibody test results. Which of the following cardiac lesions is most likely to be present in this patient? □ (A) Calcific aortic stenosis □ (B) Constrictive pericarditis □ (C) Ischemic cardiomyopathy □ (D) Libman-Sacks endocarditis □ (E) Rheumatic mitral valvulitis □ (F) Rhabdomyoma

D. Libman-Sacks endocarditis

An infant born at term is noted to have cyanosis during the first week of life. On examination a heart murmur is auscultated. Abnormal findings with echocardiography include an overriding aorta, ventricular septal defect, right ventricular thickening, and pulmonic stenosis involving the fetal heart. This infant is most likely to have an inherited mutation involving which of the following genes? □ (A) β-myosin heavy chain (β-MHC) □ (B) Fibrillin-1 □ (C) KCNQ1 □ (D) NOTCH2 □ (E) Transthyretin

D. NOTCH2

A 40-year-old woman has had a 10-kg weight loss accompanied by severe nausea and vomiting of blood for the past 8 months. On physical examination, she is afebrile. Her pulse is 91/min, respirations are 19/min, and blood pressure is 90/50 mm Hg. Laboratory studies show hemoglobin, 8.4 g/dL; platelet count, 227,100/mm3; and WBC count, 6180/mm3. Biopsy specimens obtained by upper gastrointestinal endoscopy show adenocarcinoma of the stomach. CT scan of the abdomen shows multiple hepatic masses. CT scan of the head shows a cystic area in the right frontal lobe. The patient's condition is stable until 2 weeks later, when she develops severe dyspnea. A pulmonary ventilation-perfusion scan shows a high probability of pulmonary thromboembolism. Which of the following cardiac lesions is most likely to be present? □ (A) Epicardial metastases □ (B) Left ventricular mural thrombosis □ (C) Constrictive pericarditis □ (D) Nonbacterial thrombotic endocarditis □ (E) Calcific aortic valvular stenosis

D. Nonbacterial thrombotic endocarditis

A 31-year-old man died suddenly after a week-long illness. He experienced chest pain, became increasingly dyspneic and nauseated, and lost consciousness multiple times. On the day of his death, he did not get up from his bed. External examination of the body by the medical examiner shows no evidence of trauma. The body is 166 cm (5 ft 5 in) in height and weighs 75 kg (BMI 27). The gross appearance of the chest cavity at autopsy with the pericardial sac opened as shown in the figure is most consistent with what underlying cause of death? □ (A) Marfan syndrome □ (B) Disseminated tuberculosis □ (C) Scleroderma □ (D) Occlusive coronary atherosclerosis □ (E) Malignant melanoma □ (F) Dilated cardiomyopathy

D. Occlusive coronary atherosclerosis

The parents of a 5-year-old child notice that he is not as active as other children his age. During the past 9 months, the child has had several episodes of respiratory difficulty following exertion. On physical examination, his temperature is 36.9°C, pulse is 81/min, respirations are 19/min, and blood pressure is 95/60 mm Hg. On auscultation, a loud holosystolic murmur is audible. There are diffuse crackles over the lungs bilaterally, with dullness to percussion at the bases. A chest radiograph shows a prominent border on the left side of the heart, pulmonary interstitial infiltrates, and blunting of the costodiaphragmatic recesses. The representative gross appearance of the child's heart is shown in the figure. Which of the following additional pathologic conditions would most likely develop in this child? □ (A) Aortic regurgitation □ (B) Coronary atherosclerosis □ (C) Nonbacterial thrombotic endocarditis □ (D) Pulmonary hypertension □ (E) Restrictive cardiomyopathy

D. Pulmonary hypertension

A 55-year-old man undergoes orthotopic cardiac transplantation. One month later, an endomyocardial biopsy specimen shows focal myocardial cell death with scattered lymphocytes and plasma cells. Which of the following pathologic processes best accounts for the biopsy findings? □ (A) Autoimmunity □ (B) Ischemia □ (C) Infection □ (D) Rejection □ (E) Autophagy

D. Rejection

A 27-year-old woman gives birth to a term infant after an uncomplicated pregnancy and delivery. The infant is cyanotic at birth. Two months later, physical examination shows the infant to be at the 37th percentile for height and weight. The representative gross appearance of the infant's heart is shown in figure. What is the most likely diagnosis? □ (A) Tetralogy of Fallot □ (B) Pulmonic stenosis □ (C) Truncus arteriosus □ (D) Transposition of the great vessels □ (E) Aortic stenosis

D. Transposition of the great vessels

A 50-year-old man experiences episodes of severe substernal chest pain every time he performs a task that requires moderate exercise. The episodes have become more frequent and severe over the past year, but they can be relieved by sublingual nitroglycerin. On physical examination, he is afebrile, his pulse is 78/min and regular, and there are no murmurs or gallops. Laboratory studies show creatinine, 1.1 mg/dL; glucose, 130 mg/dL; and total serum cholesterol, 223 mg/dL. Which of the following cardiac lesions is most likely to be present? □ (A) Rheumatic mitral stenosis □ (B) Serous pericarditis □ (C) Restrictive cardiomyopathy □ (D) Calcific aortic stenosis □ (E) Coronary atherosclerosis □ (F) Viral myocarditis

E. Coronary atherosclerosis

A 10-year-old girl develops subcutaneous nodules over the skin of her arms and torso 3 weeks after a bout of acute pharyngitis. She manifests choreiform movements and begins to complain of pain in her knees and hips, particularly with movement. A friction rub is heard on auscultation of the chest. Which of the following serum laboratory findings is most characteristic of the disease affecting this patient? □ (A) Elevated cardiac troponin I level □ (B) Positive ANA test □ (C) Elevated creatinine level □ (D) Positive rapid plasma reagin test □ (E) Elevated antistreptolysin O level

E. Elevated antistreptolysin O level

A 19-year-old man suddenly collapses and is brought to the emergency department. His vital signs are temperature, 37.1°C; pulse, 84/min; respirations, 18/min; and blood pressure, 80/40 mm Hg. Laboratory findings include hemoglobin, 13.5 g/dL; platelet count, 252,000/mm3; WBC count, 7230/mm3; serum glucose, 73 mg/dL; and creatinine, 1.2 mg/dL. The total creatine kinase (CK) level is elevated, with a CK-MB fraction of 10%. Which of the following underlying conditions is most likely to be present in this patient? □ (A) Hereditary hemochromatosis □ (B) Marfan syndrome □ (C) Down syndrome □ (D) DiGeorge syndrome □ (E) Familial hypercholesterolemia

E. Familial hypercholesterolemia

A 44-year-old, previously healthy man has experienced worsening exercise tolerance accompanied by marked shortness of breath for the past 6 months. On physical examination, he is afebrile. His pulse is 78/min, respirations are 22/min, and blood pressure is 110/70 mm Hg. He has diffuse rales in all lung fields and pitting edema to the knees. Laboratory studies show serum sodium, 130 mmol/L; potassium, 4 mmol/L; chloride, 102 mmol/L; CO2, 25 mmol/L; creatinine, 2 mg/dL; and glucose, 120 mg/dL. A 100-mL urine sample is collected. There is 1.3 mmol of sodium and 40 mg of creatinine in the urine sample. A chest radiograph shows cardiomegaly and pulmonary edema with pleural effusions. An echocardiogram shows four-chamber cardiac dilation and mitral and tricuspid valvular regurgitation, with an ejection fraction of 30%. A coronary angiogram shows less than 10% narrowing of the major coronary arteries. Which of the following is the most likely diagnosis? □ (A) Rheumatic heart disease □ (B) Hereditary hemochromatosis □ (C) Chagas disease □ (D) Diabetes mellitus □ (E) Idiopathic dilated cardiomyopathy

E. Idiopathic dilated cardiomyopathy

One week ago, a 72-year-old woman had an episode in which she became disoriented, had difficulty speaking, and had weakness on the right side of the body. On physical examination, she is afebrile with pulse of 68/min, respirations of 15/min, and blood pressure of 130/85 mm Hg. On auscultation, the lungs are clear, the heart rate is irregular, and there is a midsystolic click. An echocardiogram shows nodular deposits with the density of calcium around the mitral valve. One leaflet of the mitral valve appears to balloon upward. The ejection fraction is estimated to be 55%. Laboratory findings show Na+, 141 mmol/L; K+, 4.1 mmol/L; Cl−, 98 mmol/L; CO2, 25 mmol/L; glucose, 77 mg/dL; creatinine, 0.8 mg/dL; calcium, 8.1 mg/dL; and phosphorus, 3.5 mg/dL. Which of the following is the most likely diagnosis? □ (A) Carcinoid heart disease □ (B) Hyperparathyroidism □ (C) Infective endocarditis □ (D) Infiltrative cardiomyopathy □ (E) Mitral annular calcification □ (F) Rheumatic heart disease □ (G) Senile calcific stenosis

E. Mitral annular calcification

A 12-year-old boy was brought to the physician with a sore throat and fever 3 weeks ago, and a throat culture was positive for group A β-hemolytic streptococcus. On the follow-up examination, the child is afebrile. His pulse is 85/min, respirations are 18/min, and blood pressure is 90/50 mm Hg. On auscultation, a murmur of mitral regurgitation is audible, and there are diffuse rales over both lungs. The child is admitted to the hospital and over the next 2 days has several episodes of atrial fibrillation accompanied by signs of acute left ventricular failure. Which of the following pathologic changes occurring in this child's heart during hospitalization is most likely to be the cause of the left ventricular failure? □ (A) Amyloidosis □ (B) Endocardial fibroelastosis □ (B) Endocardial fibroelastosis □ (C) Fibrinous pericarditis □ (D) Fibrosis of mitral valve with fusion of commissures □ (E) Myocarditis □ (F) Tamponade □ (G) Verrucous endocarditis

E. Myocarditis

One year ago, a 2-year-old child had an illness characterized by a high fever. Staphylococcus epidermidis was cultured from the blood. The child was given antibiotic therapy and recovered. Now on physical examination, a harsh, waxing and waning, machinery-like murmur is heard on auscultation of the upper chest. A chest radiograph shows prominence of the pulmonary arteries. Echocardiography shows all valves to be normal in configuration. Laboratory studies show normal arterial oxygen saturation level. Which of the following congenital heart diseases is most likely to explain these findings? □ (A) Atrial septal defect □ (B) Tetralogy of Fallot □ (C) Aortic coarctation □ (D) Total anomalous pulmonary venous return □ (E) Patent ductus arteriosus □ (F) Aortic atresia

E. Patent ductus arteriosus

A 25-year-old man was found dead at home by the apartment manager, who had been called by the decedent's employer because of failure to report to work for the past 3 days. An external examination by the medical examiner showed splinter hemorrhages under the fingernails and no signs of trauma. The gross appearance of the heart at autopsy is shown in the figure. Which of the following laboratory findings is most likely to provide evidence of the cause of the disease? □ (A) Elevated antistreptolysin O titer □ (B) Positive ANCA determination □ (C) Increased creatine kinase-MB fraction (CK-MB) □ (D) High double-stranded DNA autoantibody titer □ (E) Positive blood culture for Staphylococcus aureus

E. Positive BC for S.aureus

A 4-year-old girl who is below the 5th percentile for height and weight for age becomes easily fatigued. On physical examination, she appears cyanotic. Her temperature is 37°C, pulse is 82/min, respirations are 16/min, and blood pressure is 105/65 mm Hg. Arterial blood gas measurement shows decreased oxygen saturation. One month later, she has fever and obtundation. A cerebral CT scan shows a right parietal, ring-enhancing, 3-cm lesion. Which of the following congenital heart diseases is the most likely diagnosis? □ (A) Atrial septal defect □ (B) Bicuspid aortic valve □ (C) Coarctation of the aorta □ (D) Patent ductus arteriosus □ (E) Truncus arteriosus □ (F) Ventricular septal defect

E. Truncus arteriosus

An 82-year-old woman has had increasing fatigue for the past 2 years. During this time, she has experienced paroxysmal dizziness and syncope. On physical examination, she is afebrile. Her pulse is 44/min, respirations are 16/min, and blood pressure is 100/65 mm Hg. On auscultation, the lungs are clear, and no murmurs are heard. An echocardiogram shows a normal-sized heart with normal valve motion and estimated ejection fraction of 50%. After being treated with digoxin, the heart rate slows and becomes irregular. An abnormality involving which of the following is most likely to be present in this patient? □ (A) Atrioventricular node □ (B) Bundle of His □ (C) Left bundle branch □ (D) Parasympathetic ganglion □ (E) Right bundle branch □ (F) Sinoatrial node □ (G) Sympathetic ganglion

F. Sinoatrial node

A 68-year-old man with a history of diabetes mellitus had chest pain and an elevated serum troponin I level 1 year ago. He was treated in the hospital with antiarrhythmic agents for 1 week. An echocardiogram showed an ejection fraction of 28%. He now has markedly reduced exercise tolerance. On physical examination, his temperature is 37°C, pulse is 68/min, respirations are 17/min, and blood pressure is 130/80 mm Hg. Diffuse crackles are heard on auscultation of the lungs. The representative gross appearance of the heart is shown in the figure. Which of the following complications of this disease is the patient most likely to develop? □ (A) Atrial myxoma □ (B) Cardiac tamponade □ (C) Constrictive pericarditis □ (D) Hypertrophic cardiomyopathy □ (E) Infective □ (F) Systemic thromboembolism

F. Systemic thromboembolism