Heart

Impaired cardiac function → unable for the cardiac muscles to contract → unable to propel the blood to the systemic circulation → unable to supply oxygen to metabolic processes

Pathophysiologic state resulting from impaired cardiac function that renders the heart muscle unable to maintain an output sufficient for the metabolic requirements of the tissues & organs

In order to maintain the CO, the heart muscles must stretch → dilatation of the heart muscles → increase in the preload and increase in the contractility of the heart → increased cardiac output (volume pumped out).

Increased cross-sectional areas of the myocytes; increase wall thickness , reduced cavity diameter

deposition of new sarcomeres;
cell length and width and muscle mass and wall thickness are increased in proportion to chamber diameter

CHF: Fetal gene program

Mechanism: direct impairment of myocardial contractility
(CHF)

Mechanism: excessive pressure
(CHF)

Mechanism: excessive volume
(CHF)

Mechanism: high output states
(CHF)

Mechanism: reduced myocardial expansion
(CHF)

Mechanism: disrupted electrical function
(CHF)

Mechanism: unknown
(CHF)

Mechanism: electrical conduction disruption
(CHF)

Mechanism: Inflammation, Degenerative, Congenital
(CHF)

Mechanism: Congenital
(CHF)

CHF: Impaired contractile function → dec CO;
S/S: pulmonary congestion;
progressive deterioration of myocardial contractile function

CHF: reduced ventricular compliance;
Inability of the heart to relax and expand → dec ventricular filling → dec CO

Left-Sided Heart Diseases

Pulmonary congestion, edema;
Peripheral edema, hypoxic encephalopathy

dyspnea, exertional dyspnea, orthopnea, paroxysmal nocturnal dyspnea , tachy, cardiomegaly, blood-tinged sputum, cyanosis, rales

Causes: pulmonary embolism, intrinsic lung disease (COPD, cystic fibrosis), pulmonary HPN, kyphoscoliosis, pneumoconiosis, schistosomiasis

Mechanism: Increased pulmonary vascular resistance due to fibrosis and/or hypoxic vascular response

CPC of liver, centrilobular necrosis, sclerosis; congestive splenomegaly, congestion in kidneys, hypoxic encephalopathy, ascites, pleural effusion, edema

splanchnic congestion: hepato-splenomegaly, hepatojugular reflex, jugular venous distention, dependent edema, transudative effusions (ascites, pleural effusion), cyanosis

heart failure secondary to pumping excessive volume of blood

High-Output Failure: causes

Secondary to ischemic heart disease (IHD), hypertension, dilated cardiomyopathy, and vulvar/pericardial disease

CRITERIA FOR DX OF CHF: Major Criteria

CRITERIA FOR DX OF CHF: Minor Criteria

imbalance between blood supply/perfusion to the heart and its demand for oxygenated blood; limits oxygenation, ATP generation, reduces availability of nutrients and removal of metabolic wastes

Syndromes: AMI, angina pectoris, chronic ischemic heart disease, sudden cardiac death

dec coronary artery perfusion relative to myocardial demand; chronic, progressive atherosclerotic narrowing of the epicardial coronary arteries, variable degrees of superimposed acute plaque change, thrombosis, and vasospasm

70% of lumen of one or more CA is/are obstructed with atherosclerotic plaque; compensatory CA vasodilatation is insufficient to meet moderate increases in myocardial O2 demand

acute plaque changes

myocardial infarct where the whole wall of the muscle has undergone cell death

Paroxysmal, recurrent precordial or substernal chest pain, caused by transient (15s to 15min) myocardial ischemia that falls short of infarction

chest pain: transient (<15 min), precipitated by exertion and emotion, relieved by rest, vasodilators

Episodic, recurrent chest pain at rest; caused by coronary artery spasm; ECG: suggestive of transmural ischemia (ST segment elevation); responds promptly to vasodilators

90% vessel block; pain progressiveljy increasing in frequency, duration (>15 min), intensity, occurs at rest

local coagulative necrosis of myocardium due to ischemia; irreversible damage to myocardium

Severe Ischemia 20-40 mins. or longer + <10% blood flow + initial damage to sarcolemma membrane

Pathognomonic sign: ischemic coagulative necrosis; central damage at subendocardium progresses into transmural in a waveform fashion

involves >2/3 or full thickness of ventricular wall; ischemia due to superimposed thrombus in atherosclerosis (chronic obstruction + acute plaque change + complete thrombosis)

inner 1/3 or 1/2 of ventricular wall; ischemia due to dec blood volume (e.g. hypovolemic shock, hypotension, diffuse stenosing coronary artery atherosclerosis w/o thrombosis and acute plaque change)

triphenyl tetrazolium chloride: unstained pale zone (due to the lactate dehydrogenase leakage that follows cell death)

S/S: hypotension, rapid weak pulse, diaphoretic & nauseous, dyspnea; ECG: ST elevation, new Q waves, T wave inversion

progressive heart failure as a consequence of ischemic myocardial damage → fibrosis → reduce cardiac activity → ischemia

Gross: left ventricular dilation and hypertrophy, often with discrete areas of gray-white scarring; moderate to severe atherosclerosis;
Histo: diffuse subendocardial myocyte vacuolization, fibrosis from previous infarction

SA node damaged → AV node takes over

atrial dilation → irritable atrial myocytes →independent & sporadic depolarization of each atrial myocytes → variable signal transmission to AV node

dysfunctional AV node:
inc PR interval on ECG

dysfunctional AV node:
intermittent signal transmission

dysfunctional AV node:
complete failure

S/S: palpitations, lightheadedness, syncope, sudden cardiac death

adaptive response to pressure overload on the heart that can lead to myocardial dysfunction, cardiac dilatation, CHF and in some cases sudden death

Concentric left ventricular hypertrophy in the absence of other cardiovascular pathology that might induce cardiac hypertrophy

Gross: thickened left ventricular wall (concentric), cardiomegaly, decreased luminal size;
Histo: inc transverse diameter of the myocytes, boxcar nuclei, intercellular fibrosis

pressure overload in the right ventricle due to increased pulmonary resistance usually caused by chronic lung disease that can lead to pulmonary vasculature fibrosis

right ventricular dilatation after massive pulmonary embolization

right ventricular (and often right atrial) hypertrophy; result of chronic RV pressure overload; seen in COPD, CRPD, long standing pulmonary artery disease, chest wall motion impairment

Gross: marked RV dilation without hypertrophy

Gross: RV wall thickens and RV dilation may lead to tricuspid regurgitation

heart disease resulting from a primary abnormality in the myocardium; attributed to intrinsic myocardial disease

biventricular and bi-atrial dilatation; hypo-contracting heart muscle; systolic dysfunction; low LV ejection fraction, low CO = congestion

Causes: idiopathic, previous viral myocarditis, alcohol or other toxic exposure, peripartum cardiomyopathy/pregnancy, genetic causes, iron overload

cardiomegalic heart, global enlargement, generalized chamber dilatation, annular ring dilatation, valvular defects, functional regurgitation, mural thrombus

ages of 20-50, slowly progressive CHF, including dyspnea, easy fatigability, poor exertional capacity; secondary mitral regurgitation and abnormal cardiac rhythms are common, and embolism from intracardiac (mural) thrombi

Idiopathic Hypertrophic Subaortic Stenosis (IHSS), Hypertrophic Obstructive CM, Asymmetric Septal Hypertrophy

myocardial hypertrophy in the interventricular septum w/o ventricular dilatation, abnormal diastolic filling and LV outflow obstruction; heavy, muscular, hypercontracting heart

missense mutation in sarcomere (B-MHC gene); inc myofilament activation = myocyte hypercontractility

Asymmetric septal hypertrophy → outflow obstruction; septal wall ratio >1.3; massive myocardial hypertrophy w/o ventricular dilatation; banana-like ventricular cavity

Impaired diastolic filling of the massively hypertrophied LV; dec CO and increase in pulmonary venous pressure -> exertional dyspnea with harsh systolic ejection murmur

Gross: non-dilated ventricular chamber; bi-atrial dilatation due to poor ventricular filling and pressure overload;
Histo: patchy or diffuse interstitial fibrosis, identifiable depositions/infiltrations

Dense diffuse fibrosis of ventricular endocardium and subendocardium, often involving the tricuspid and mitral that extends from the apex upward; linked to nutritional deficiencies and/or inflammation related to helminthic infections

Hypereosinophilia and eosinophilic tissue infiltrates, endomyocardial fibrosis, large mural thrombosis

Focal or diffuse thickening usually involving the mural LV endocardium; heart is infiltrated by fibroelastic tissue

Inherited disease; defective cell adhesion proteins in desmosomes that link adjacent myocytes; right ventricular failure and rhythm disturbances (particularly ventricular tachycardia or fibrillation) with sudden death

Disorder characterized by arrhythmogenic right ventricular cardiomyopathy and hyperkeratosis of plantar palmar skin surfaces

RV wall: severely thinned because of loss of myocytes, extensive fatty infiltration and fibrosis

infectious agents and/or inflammatory processes primarily target the myocardium; result in injury to cardiac myocytes (necrosis and degeneration) not typical of ischemic heart disease

Gross: beefy red myocardium due to vascular congestion; ventricular myocardium is flabby and mottled by either pale foci or minute hemorrhagic lesions

Histo: interstitial inflammatory myocardial necrosis near the inflammatory cells; vascular congestion, edema; viral inclusions and parasitic organisms may be present

edema, interstitial inflammatory infiltrates, and myocyte injury; diffuse lymphocytic infiltrate; self-limiting

presence of multi-nucleated giant cells; infiltrates: Lymphocytes, eosinophils, necrosis, patchy myocitic necrosis in muscle cell

Interstitial infiltrate: macrophages, a lot of eosinophils with little or no necrosis; no granulomas; associated with adverse drugs effects of methyldopa

ASD, VSD, PDA, AVSD; acyanotic, right ventricular hypertrophy and eventually failure

early cyanosis; hypertrophic osteoarthropathy, polycythemia, paradoxical embolization;
Tetralogy of Fallot, Transposition of Great Vessels, Truncus Arteriosus, Tricuspid Atresia, TAPVR

untreated congenital cardiac defect with intracardiac communication that leads to pulmonary hypertension, reversal of flow, and cyanosis

Basic defect: Abnormal opening at the level of the atrial septum; most common CHD that is
 asymptomatic until adulthood; S/S may manifest in 3rd decade

90% of ASD
; defect in the fossa ovale


defect occurs (low-lying) near the AV valve; associated with a clefted anterior mitral valve/ abnormal tricuspid valve

Located at the top, near the entrance of SVC; associated with total anomalous pulmonary venous return to the right atriu

Located in the upper portion of the interventricular septum; affects membranous portion

VSD: located below the pulmonary valve

VSD: found in the muscular wall of the interventricular septum

Presence of holes in the atrial and the ventricular level; abnormal development of atrio-ventricular canal; incomplete closure of AV septum and inadequate formation of the tricuspid valve and mitral valve

Failure of fusion of superior and inferior endocardial cushions with the mid portion of the atrial septum and the muscular (trabecular) portion of the ventricular septum

primum atrial septal defect and a cleft in the mitral valve

Defects of both the primum atrial septum and inlet ventricular septum and the presence of a common atrioventricular valve; free communication of all 4 chambers

AVSD: mitral and tricuspid annuli are separate; usually associated with a primum ASD

AVSD: single atrioventricular valve annulus; defect of the inlet ventricular septum

Persistence of communication between the aorta (a high pressure chamber) and the pulmonary artery (a relatively low pressure chamber) via a non-closure of the ductus arteriosus

continuous, harsh murmur: “Machinery murmur”

inability of the great vessel to
rotate to its normal location

VSD; subpulmonic stenosis (right ventricular outflow tract obstruction); overriding of the VSD by the aorta; right ventricular hypertrophy

Heart is large and “boot-shaped” as a consequence of right ventricular hypertrophy; proximal aorta is dilated; hypoplastic pulmonary trunk

Hemodynamic consequences: R-to-L shunting, dec pulmonary blood flow, inc aortic volume; polycythemia w/ attendant hyperviscosity and hypertrophic osteoarthropathy

with good APGAR score (pink complexion, without cyanosis); mild pulmonary stenosis; may present with L→R shunt

severe pulmonary stenosis, greater resistance to right ventricular outflow, R→L shunt (blood cannot go to the
lungs → cyanotic)

Abnormal formation of the truncal and aortopulmonary septa

ventrico-aterial discordance: aorta from RV, PA from LV; RV Hypertrophy, LV Hypoplasia/Atrophy

failure of separation of truncus arteriosus into aorta and pulmonary artery; single great artery or a single great vessel that receives blood from both ventricles

Complete absence of the development of the tricuspid valve; cyanosis is present virtually from birth; blood cannot flow into the right ventricle

results from unequal division of AV canal; mitral valve is larger than normal and almost always underdevelopment (hypoplasia) of RV

Pulmonary veins do not drain into left atrium, but into left innominate vein or coronary sinus

Results embryologically when the common pulmonary vein fails to develop or becomes atretic; needs PFO or VSD

common pulmonary vein fails to develop or becomes atretic; right atrial and ventricular dilatation, pulmonary trunk dilation
, hypoplastic left atrium

Tubular hyperplasia of aortic arch proximal to Patent Ductus Arteriosus (PDA)

Circumferential narrowing of the aortic segment between the left subclavian artery and the ductus arteriosus; leads to the inc of pressure in the pulmonary trunk proximal to the obstruction

Ridge-like coarctation of aorta at the level of or after ligamentum arteriosus; aortic arch and its vessels are dilated and presence of LVH

disparity in the blood pressure of the upper extremities (increased) and of the lower extremities (decreased)

pulmonary artery and valve becomes tight and stenotic; patient presents with a sinus disorder

Narrowing/obstruction of aortic valve at the level of the valve

Narrowing/obstruction of aortic valve below the aorta

Narrowing/obstruction of aortic valve above the aorta

Failure of valve to open completely, impeding forward flow

Failure of valve to close completely, leading to reversal of flow of blood

No anatomic defect in the valve;
dilated cardiomyopathy – the ventricular chambers dilate → annular ring dilates → annular ring cannot close completely → regurgitation of blood from ventricle to atrium

Most often caused by age-related “wear and tear"; involves the aortic valve which becomes obstructed by the calcium deposits at the roof of the valve

Heaped calcified masses within aortic cusps; protrudes into the sinuses of Valsalva, mechanically impeding valve opening; no commissural fusion

Unequal sized bicuspid valve with midline “raphe”; only two valvular leaflets

Calcific nodular deposits in the annular ring; common in elderly women with myxomatous mitral valve, or mitral valve prolapse

Irregular, stony hard nodules behind the mitral valve leaflets; ballooning of the mitral leaflets; affected leaflets are enlarged, redundant, thick, and rubbery; tendinous cords also tend to be elongated, thinned, and occasionally rupture

Thinning of the valve layer known as the fibrosa layer of the valve; middle spongiosa layer expands, owing to increased deposition of myxomatous (mucoid) material

auscultatory finding caused by abrupt tension on the redundant valve leaflets at chordae tendinae as the valve attempts to close

Colonization of heart valves and mural endocardium by microbiologic agents, forming bulky vegetations; most common valves involved are mitral and aortic

Fibrin deposits on injured endothelium; circulating bacteria are trapped by the forming thrombus, infecting the microthrombi (hematogenous seeding)

Highly virulent organism; arises in normal valve; necrotizing, ulcerative and destructive lesions; bulky, friable vegetations; more severe clinical signs and symptoms, higher morbidity

occurs in previously diseased valve; low virulent organism; insidious clinical onset

. Irregular reddish tan vegetations overlie valve cusps;
Fever, weight loss, fatigue, anorexia, changing murmurs, embolic phenomenom: Roth spots, sub-ungal hemorrhages, CVA, embolic infarcts, focal/diffuse GN, abscesses

Sterile vegetations; associated with debilitated patients, carcinoma, sepsis, and cachexia; nondestructive

Valvular damage is not a prerequisite for NBTE; usually found on previously normal valves; hypercoagulable states are the precursor

Small nodules along lines of closure, may be bulky & friable, single or multiple, leaflets often normal; free of inflammation

Associated with SLE; affects MV and TV; sterile, small vegetations located in the under-surface of cords

finely grandular, fibrinous eosinophilic material containing "hematoxylin bodies"; • Intense valvulitis may be present characterized by fibrinoid necrosis of valve

Caused by carcinoid tumors which secrete vasoactive amines; neuroendocrine tumor;
Clinical findings: episodic skin flushing, cramps, nausea, vomiting and diarrhea

Distinctive, glistening white intimal plaque-like endocardial fibrous thickening on the inside surfaces of the cardiac chambers and both TV and PV; tricuspid insufficiency and pulmonic stenosis

Minor Criteria: migratory polyarthritis, carditis, subcutaneous nodules, erythema marginatum, Sydenham's cholera (St. Vitus "Dance")

Minor Criteria: fever, arthralgia, Hx of sore throat, inc anti-steptolysin O, inc acute phase reactants: ESR, CR

Central zone of degenerating, hyper-eosinophilic extracellular matrix infiltrated by lymphocytes, plasma cells, and plump activated macrophages

plump activated macrophages ; pathognomonic for RF

Diffuse inflammation and Aschoff bodies may be found in any of the 3 heart layers

irregular thickenings, usually in the LA; subendocardial fibrosis

Verruccal necrotic vegetations on lines of closure, Aschoff bodies, Anitschkow monocytes, Caterpillar cells, Mac Callum plaques

Latent period (20-30 years) before symptoms appear; Multi-valvular

Fish mouth deformity; commissural fusion of cusps -> stenosis/regurgitation; deformity or "buttonhole" stenosis (typically affecting MV) and regurgitaion

Leaflet thickening, commissural fusion and shortening, and thickening and fusion of the chordae tendinae; fibrosis & subendocardial fibrosis

large and bulky lesions on the valve cusps that can extend onto the chordae

small, warty vegetations along the lines of closure of the valve leaflets

small, bland vegetations usually attached at the line of closure

big and small, up and down (both sides of valve leaflets) vegetations

Most common form of valvular disease in industrialized countries; 20-40 ages, females; MV are enlarged, hooded or floppy; developmental anomaly of connective tissue from a dense collagen to a myxoid tissue

Myxomatous degeneration; where one or more mitral valve leaflets are “floppy” and prolapse into the LA during systole (because of hooding of valve)

Interchondrial ballooning (hooding) of the mitral leaflets, prolapse into the atrium; leaflets are enlarged, redundant, thick and rubbery; tendinous cords: elongated, thinned, or even ruptured and the annulus is dilated

Mid-systolic click (snapping and tensing of everted cusp)

Characteristically produced by non infectious inflammatory diseases; Scanty Inflammation; May or may not cause pericardial serous effusion

Causes of Serious Pericarditis

Composed of serous fluid variably admixed with fibrous exudate

Reflects an active infection caused by microbial invasion; Frank infection leads to systemic symptoms: Spiking fevers and rigors; Organization by scarring is the usual outcome

Exudate composed of blood mixed with fibrinous or suppurative effusion

Most commonly caused by the spread of malignant neoplasm to the pericardial space; In persons with bleeding diathesis; Often follows cardiac surgery

Pericardial involvement occurs by direct spread from TB foci within the tracheobronchial nodes; Common antecedent of fibrocalcific, chronic constrictive pericarditis; Epitheloid granuloma with Langhan’s giant cell surrounded by PMN inflammation

Obliteration of the pericardial sac with adhesion to parietal pericardium that strains cardiac function;
S/S: Sytolic retraction of the rib cage and diaphragm, pulsus paradoxus

organization merely produces plaque-like fibrous thickenings

Obliteration of the pericardial sac with encasement of the heart in a dense, fibrous or fibrocalcific scar that limits diastolic expansion and cardiac output

Extreme cases can resemble plaster mold (concretio cordis); dense enclosing scar, cardiac hypertrophy and dilation cannot occur; S/S: distant or muffled heart sounds, elevated jugular venous pulse, peripheral edema

most common primary cardiac tumor in adults; benign neoplasm from primitive multipotent mesenchymal cells

GNAS1

PRKAR1A

Region of the fossa ovalis (favored site of origin); sessile or pedunculated masses; vary from globular hard masses, mottled with hemorrhage to soft, transluscent, papillary, or villous lesions having gelatinous appearance

most common primary cardiac tumor in children; commonly discovered in the first years of life; most arise from obstruction of a valvular orifice or ventricular chamber

large rounded, or polygonal cells with cytoplasmic glycogen vacuoles

Vacuoles are separated by strands of cytoplasm running from the plasma membrane to the more or less centrally located nucleus

TSC1 and TSC 2 are absent = myocyte growth

often regress spontaneously, they may be considered as hamartomas rather than true neoplasms

inhibits mTOR

Localized, well circumscribed, benign tumors, mature fat cells, occurs in subendocardium, subepicardium, or myocardiuml; most often located in left ventricle, right atrium, or atrial septum

Nonneoplastic deposition of fat in the atrial septum

Lesions include white and brown adipose tissue and small interspersed areas of myocardium

Curious, usually incidental, sea-anemone-like lesions; resembles Lambl excresences that represents remotely organized thrombus on the aortic valves of older individuals