XI - The Heart

Question 2

The morphologic and clinical effects of this condition primarily result from progressive damming of blood within the pulmonary circulation. The left ventricle is hypertrophied and dilated, with secondary left atrial dilation. The lungs are heavy and boggy, with perivascular and interstitial transudate, alveolar septal edema, and intra-aleolar edema. Hemosiderin-laden macrophages are present.

Answer 2

Left sided heart failure(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 381

Question 3

Hemosiderin laden macrophages are also called _______

Answer 3

Heart failure cells(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 381

Question 4

This is usually the earliest and most significant compaint of patients in Left sided HF.

Answer 4

Dyspnea(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 381

Question 5

Most common cause of right sided HF.

Answer 5

Left sided HF(TOPNOTCH)

Question 6

This is a particularly dramatic form of breathlessness, awakening patients from sleeo with attacks of extreme dyspnea bordering on suffocation.

Answer 6

Paroxysmal nocturnal dyspnea(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 381

Question 7

Isolated right sided HF occuring in patients with intrinsic lung disease that result in chronic pulmonary hypertension.

Answer 7

Cor Pulmonale(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 381

Question 8

Long standing severe right-sided HF leads to fibrosis of centrilobular areas, creating this condition.

Answer 8

Cardiac cirrhosis(TOPNOTCH)

Question 9

The liver is increased in size and weight, a cut section reveals congested red centers of liver lobules surrounded bybpaler, sometimes fatty peripheral regions.

Answer 9

Nutmeg liver (CPC of the liver)(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 381

Question 10

Right-sided HF produces a tense, enlarged spleen, achieving weights of 300-500 grams. Sinusoidal dilation present.

Answer 10

Congestive splenomegaly(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 381

Question 11

This is a hallmark of right sided HF.

Answer 11

Pedal and pretibial edema(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 382

Question 12

Generalized, massive edema is called ______.

Answer 12

Anasarca(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 382

Question 13

Most congenital heart disease arise from faulty embryogenesis during what AOG?

Answer 13

3 - 8 weeks AOG(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 382

Question 14

An abnormal communication between chambers of the heart or blood vessels.

Answer 14

Shunt(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 383

Question 15

These a typically smooth-walled defects near the foramen ovale, usually without associated cardiac abnormalities. Accompanied by right atrial and ventricular dilation, right ventricular hypertrophy and dilation of the pulmonary artery.

Answer 15

Ostium secundum ASD(TOPNOTCH)

Question 16

Reversal of blood flow through a prolonged (left-to-right shunt) due to pulmonary hypertension, yielding right-sided pressures that exceed those on the left side. This causes unoxygenated blood to go into circulation, causing cyanosis.

Answer 16

Eisenmenger syndrome(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 383

Question 17

These occur at the lowest part of the atrial septum and can extend to the mitral and tricuspid valves. Abnormalities of the AV Valves are usually present, forming a cleft in the anterior leaflet of the mitral valve or septal leaflet of the tricuspid valve.

Answer 17

Ostium primum ASD(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 384

Question 18

Incomplete closure of the ventricular septum leading to left-to-right shunting. The right ventricle is hypertrophied and often dilated. Diameter of pulmonary artery is increased because of the increased volume by the right ventricle.

Answer 18

Ventricular Septal Defect(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 385

Question 19

This arises from the left pulmonary artery and joins the aorta just distal to the origin of the left subclavian artery.

Answer 19

Ductus arteriosus(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 385

Question 20

In this condition, some of the oxygenated blood flowing from the left ventricle is shunted back to the lungs. Proximal pumonary arteries, left atrium and ventricle can become dilated.

Answer 20

Patent ductus arteriosus(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 385

Question 21

The most common cause of cyanotic congenital heart disease. Heart is large and “boot shaped” as a result of right ventricular hypertrophy.

Answer 21

Tetralogy of Fallot(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 385

Question 22

Components of Tetralogy of Fallot.

Answer 22

Pulmonary valve stenosisOverriding of aortaRight ventricular hypertrophyVentricular septal defect(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 386

Question 23

It is a discordant connection of the ventricles to their vascular outflow. The defect is an abnormal formation of the truncal and aortopulmonary septa. Right ventricular hypertrophy becomes prominent, while the left ventricle becomes somewhat atrophic.

Answer 23

Transposition of the Great Arteries(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 386

Question 24

Predominant manifestation of TGA?

Answer 24

Early cyanosis(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 387

Question 25

Characterized by tubular narrowing of the aortic segment between the left subclavian artery and the ductus arteriosus. DA is usually patent and is the main source of blood to the distal aorta. RV is hypertrophied and dilated, pulmonary trunk is also dilated.

Answer 25

Preductal “infantile” coarctation of the aorta(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 387

Question 26

Aorta is sharply constricted by a ridge of tissue at or just distal to the ligamentum arteriosum. Constricted segment is made of smooth muscle and elastic fibers that are continuous with the aortic media, and lined by thickened intima. Ductus arteriosus is closed. Proximally, the aortic arch and its vessels are dilated, LV is hypertrophic.

Answer 26

Postductal “adult” coarctation of the aorta(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 387

Question 27

There is upper extremity hypertension, due to poor perfusion of the kidneys, but weak pulses and low blood pressure in the lower extremities. Claudication and coldness of the lower extremities also present. Enlarged intercostal and internal mammary arteries due to collateral circulation, seen as rib “notching” on xray.

Answer 27

Postductal coarctation of the aorta (without a PDA)(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 388

Question 28

Left-to-right or Right-to-Left shunt?Atrial septal defect

Answer 28

Left-to-right(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 388

Question 29

Left-to-right or Right-to-Left shunt?TOF

Answer 29

Right-to-Left(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 388

Question 30

Left-to-right or Right-to-Left shunt?VSD

Answer 30

Left-to-right(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 388

Question 31

Left-to-right or Right-to-Left shunt?Eisenmenger syndrome

Answer 31

Right-to-Left (TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 388

Question 32

Left-to-right or Right-to-Left shunt?Transposition of great arteries

Answer 32

Right-to-Left(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 388

Question 33

A condition wherein ischemia causes pain but is insufficient to lead to death of myocardium.

Answer 33

Angina pectoris(TOPNOTCH)

Question 34

A condition wherein ischemia causes pain but is insufficient to lead to death of myocardium.

Answer 34

Angina pectoris(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 388

Question 35

A condition wherein the severity or duration of ischemia is enough to cause cardiac muscle death.

Answer 35

Acute Myocardial Infarction(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 388

Question 36

This refers to progressive cardiac decompensation (heart failure) following myocardial infarction.

Answer 36

Chronic Ischemic Heart Disease(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 388

Question 37

This can result from a lethal arrythmia following myocardial ischemia.

Answer 37

Sudden Cardiac Death(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 388

Question 38

How many percent should the lumen of a blood vessel be obstructed for it to be symptomatic, in the setting of increased demand?

Answer 38

70-75% (critical stenosis)(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 389

Question 39

How many percent should the lumen of a blood vessel be obstructed for it to be symptomatic at rest?

Answer 39

90%(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 389

Question 40

Episodic chest pain associated with exertion or some other form of increased myocardial oxygen demand. Pain described as crushing or squeezing substernal sensation which can radiate to left arm. Relieved by rest or vasodilators.

Answer 40

Stable angina(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 390

Question 41

Increasing frequency of pain, precipitated by progressively less exertion, episodes tend to be more intense and longer lasting.

Answer 41

Unstable angina(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 390

Question 42

Angina occuring at rest due to coronary artery spasm.

Answer 42

Variant or Prinzmetal angina(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 390

Question 43

Infarct involving >= 50% of the myocardial wall thickness.

Answer 43

Transmural infarcts(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 391

Question 44

Most common blood vessel involved in myocardial infarction?

Answer 44

Left anterior descending artery (40-50%)(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 392

Question 45

Electron microscope findings 30 minutes after an ischemic event.

Answer 45

Microfibril relaxation, glycogen loss and mitochondrial swelling(TOPNOTCH)

Question 46

An infarct can be readily identified by a reddish blue discoloration after how many hours after MI?

Answer 46

12-24 hours(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 393

Question 47

Coagulation necrosis ensues how many hours after MI?

Answer 47

4-12 hrs after an irreversible injury(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 394

Question 48

Injury to infarcts mediated in part by oxygen free radicals generated by increased number of infiltrating leukocytes facilitated by reperfusion.

Answer 48

Reperfusion injury(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 394

Question 49

Cardiac enzymes that become detectable 2-4 hours post-infarct peaks at 48 hours and remains elevated for 7-10 days.

Answer 49

Troponin I and Troponin T(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 395

Question 50

This cardiac enzyme is detectable in the blood within 2-4 hrs of MI, peaks at 24-48 hrs and returns to normal within approximately 72 hrs.

Answer 50

CKMB(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 395

Question 51

Myocardial rupture may occur how many days after MI?

Answer 51

3-7 days after infarction(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 397

Question 52

This occurs within 2-3 days of a transmural infarct and typically resolves within time. It is the epicardial manifestation of the underlying myocardial inflammation.

Answer 52

Pericarditis(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 397

Question 53

A late complication of MI, most commonly results from a large transmural anteroseptal infarct that heals with formation of a thin scar tissue.

Answer 53

Ventricular aneurysm(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 397

Question 54

This type of hypertrophy develops in pressure-overloaded ventricles, with an increase in wall thickness, and reduced cavity diameter.

Answer 54

Concentric hypertrophy(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 399

Question 55

Type of hypertrophybthat develops in patients with volume overload such as aortic valve insufficiency. Characterized by hypertrophy associated with ventricular dilation.

Answer 55

Eccentric hypertrophy(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 399

Question 56

In this disease the left ventricle may exceed 2.0cm in thickness and the heartvmay weigh >500 grams. Microscopically, myocyte diameter increases, associated with irregular nuclear enlargement and hyperchromasia (“box-car nuclei”), and increased interstitial fibrosis.

Answer 56

Sytemic Hypertensive heart disease(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 399

Question 57

It is the failure of a valve to open completely, obstructing forward flow.

Answer 57

Stenosis(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 401

Question 58

This results from failure of a valve to close completely, thereby allowing reversed flow.

Answer 58

Insufficiency(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 401

Question 59

The hallmark of this disease is heaped-up calcified masses on the outflow side of the cusps, which protrude intonthe sinuses of Valsalva and mechanically impede valve opening. Cusps may become secondarily fibrosed and thickened.

Answer 59

Calcific aortic stenosis(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 401

Question 60

This is characterized by ballooning or hooding of the mitral leaflets. Affected leaflets are enlarged, redundant, thick and rubbery. The tendinous cords are elongated, thinned and occasionally ruptured. Histologically, there is thinning of the fibrosa layer of the valve, accompanied by expansion of the middle spongiosa layer with increased deposition of mucoid material.

Answer 60

Myxomatous degeneration of the mitral valve(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 402

Question 61

Patients with this disease may complain of palpitations, dyspnea or atypical chest pain. Auscultation shows a midsystolic click associated with a regurgitant murmur.

Answer 61

Mitral valve prolapse(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 402

Question 62

Pathognomonic sign for rheumatic fever,consisting of of a cental zone of degenerating, hypereosinophilic ECM infiltrated by lymphocytes, ocassional plasma cells and plump, activated macrophages.

Answer 62

Aschoff bodies(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 402

Question 63

These cells have abundant cytoplasm and central nuclei with chromatin arrayed in a slender, wavy ribbon (caterpillar cells) which can be found in all three layers of the heart in rheumatic fever. A component of Aschoff bodies.

Answer 63

Anitschkow cells(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 403

Question 64

Characterized by organization and subsequent scarring, as a sequelae of rheumatic fever. The mitral (or tricuspid) valve is involved, with leaflet thickening, commisural fission and shortening, thickening and fusiong of the chordae tendinae. Fibrous bridging across valvular commisures create “fishmouth” or “ buttonhole” deformity.

Answer 64

Chronic Rheumatic Heart Disease(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 403

Question 65

Most common valve involved in RHD.

Answer 65

Mitral valve (upto 70% of cases with RHD)(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 405

Question 66

Major components Jones Criteria for RF.

Answer 66

CarditisMigratory polyarthritisSubcutaneous nodulesErythema marginatumSyndenham chorea(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 406

Question 67

Minor components Jones criteria for RF

Answer 67

FeverArthralgiaElevated acute phase reactants (e.g. CRP)(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 406

Question 68

How many major and/or minor manifestations are needed to diagnose RF?

Answer 68

Remember: 20122 major 0 minor or1 major 2 minor(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 406

Question 69

Endocarditis of previously normal valves, the most common causative agent is S. aureus.

Answer 69

Acute bacterial endocarditis(TOPNOTCH)

Question 70

Serious infection characterized by microbial invasion of heart valves or mural endocardium, often with destruction of the underlying cardiac tissues. The heart valves are friable, bulky and potentially destructive.

Answer 70

Infective endocarditis(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 406

Question 71

Endocarditis affecting previously damaged or abnormal valves, commonly caused by viridans Streptococci.

Answer 71

Subacute bacterial endocarditis(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 406

Question 72

Most consistent sign of infective endocarditis.

Answer 72

Fever(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 407

Question 73

Characterized by deposition of sterile, non-inflammatory, nondestructive and small (1mm) masses of fibrin, platelets and other blood components on cardiac valves, along the line of closure of leaflets or cusps.

Answer 73

Nonbacterial thrombotic endocarditis(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 407

Question 74

Sterile vegetations thatvcan develop on the valves of patients with SLE. These are small, granular, pinkish vegetations 1-4mm in diameter and can be located on the undersurface of AV valves, on the cords or endocardium.

Answer 74

Libman-Sacks endocarditis(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 408

Question 75

The lesions of this disease are distinctive, glistening white intimal plaquelike thickenings on the endocardial surfaces of the cardiac chambers and valve leaflets seen in patients with carcinoid tumors. The lesions are composed of muscle cells and sparse collagen fibers embedded in an acid mucopolysaccharide-rich matrix.

Answer 75

Carcinoid heart disease(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 408

Question 76

Cardiomyopathy which is characterized by progressive cardiac dilation and contractile dysfunction. The heart is characteristically enlarged and flabby, with dilation of all chambers,the ventricular thickness may be less than, equal to or greater than normal.

Answer 76

Dilated cardiomyopathy(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 411

Question 77

Alcohol intake and infection with coxsackie B virus are some of the causes of this cardiomyopathy.

Answer 77

Dilated cardiomyopathy(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 411

Question 78

Characterized by myocardial hypertrophy, abnormal diastolic filling and ventricular outflow obstruction. The heart is thick-walled, heavy and hypercontracting. There is an assymetrical septal hypertrophy described as “banana-like”. Histologically, there is severe myocyte hypertrophy and disarray with interstitial fibrosis.

Answer 78

Hypertrophic cardiomyopathy(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 412

Question 79

Mechanism of heart failure in hypertrophic cardiomyopathy.

Answer 79

Diastolic dysfunction (impaired compliance)(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 412

Question 80

A common cause of sudden death in young athlethes.

Answer 80

Hypertrophic cardiomyopathy(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 412

Question 81

The ventricles are of approximately normal size or slightly enlarged, the cavities not dilated, and the myocardium is firm. Biatrial dilation is common. Microscopically, there is interstitial fibrosis, varying from minimal to patchy to extensive and diffuse.

Answer 81

Restrictive cardiomyopathy(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 413

Question 82

Mechanism of heart failure in restrictive cardiomyopathy.

Answer 82

Diastolic dysfunction or impaired compliance(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 413

Question 83

Inflammation of the myocardium.

Answer 83

Myocarditis(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 414

Question 84

Most common type of myocarditis wherein lymphocytes infiltrate the interstitium. This may resolve or heal by progressive fibrosis.

Answer 84

Lymphocytic myocarditis(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 414

Question 85

Myocarditis that has interstitial and perivascular infiltrates composed of lymphocytes, macrophages and a high proportion of eosinophils.

Answer 85

Hypersensitivity myocarditis(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 414

Question 86

Myocarditis characterized by widespread inflammatory infoltrates containing multinucleated giant cells interspresed with lymphocytes, eosinophils and plasma cells. Poor prognosis.

Answer 86

Giant-cell myocarditis(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 414

Question 87

Myocarditis distinctive by virtue of parasitization of scattered myofibers by trypanosomes accompanied by an inflammatory infiltrate of neutrophils, lymphocytes, macrophages and occasional eosinophils.

Answer 87

Chagas myocarditis(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 414

Question 88

Viruses which account for most cases of myocarditis.

Answer 88

Coxsackie A and B(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 414

Question 89

Type of pericarditis found in patients with uremia or viral infection. The exudate imparts an irregular apperance to the pericardial surface (bread and butter pericarditis).

Answer 89

Fibrinous pericarditis(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 416

Question 90

Bacterial pericarditis manifests with this type of exudate.

Answer 90

Fibrinopurulent (suppurative)(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 416

Question 91

Heart is completely encased by dense fibrosis that it cannot expand normally during diastole.

Answer 91

Constrictive pericarditis(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 416

Question 92

Normal amount of pericardial fluid in pericardial sac.

Answer 92

30 - 50 mL of thin, straw-colored fluid(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 417

Question 93

Serous pericardial effusion can be caused by _________

Answer 93

CHF, hypoalbuminemia(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 417

Question 94

Chylous pericardial fluid can be caused by _______

Answer 94

Mediastinal lymphatic obstruction(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 417

Question 95

Rapidly developing collections of fluid within the pericardial sac can restrict diastolic cardiac filling producing this fatal sequelae.

Answer 95

Cardiac tamponade(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 417

Question 96

The most common tumor of the heart.

Answer 96

Metastatic tumor(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 417

Question 97

Most common primary tumor of the adult heart.

Answer 97

Myxoma(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 417

Question 98

Major clinical manifestations of this cardiac tumor are due to valvular “ball-valve” obstruction, embolization or a syndrome of constitutional symptoms.

Answer 98

Myxoma(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 418

Question 99

The most frequent primary tumor of the heart in infants and children. These are generally small gray-white myocardial masses composed of a mixed population of cells, the most characteristic of which are large, rounded or polygonal cells containing numerous glycogen-laden vacuoles separated by strands of cytoplasm, so-called spider cells.

Answer 99

Rhabdomyomas(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 418

Question 100

Serosanguinous pericardial effusion can be caused by ________

Answer 100

Blunt chest trauma, malignancy, ruptured MI, aortic dissection(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 418