Cardiac Pathology

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I.

I. ISISCHCHEMEMIC HIC HEAEART DRT DISISEAEASESE

Ischemic heart disease (IHD) is the generic designation for a group

Ischemic heart disease (IHD) is the generic designation for a group of closely related syndromesof closely related syndromes resulting from

resulting from myocardiamyocardial l ischemiaischemia—an imbalance between the supply (perfusion) and demand—an imbalance between the supply (perfusion) and demand of the heart

of the heart for oxygenated blood. Ischemia comprises not only insufficiency of oxygen, but for oxygenated blood. Ischemia comprises not only insufficiency of oxygen, but alsoalso reduced availability of nutrient substrates and inadequate removal of

reduced availability of nutrient substrates and inadequate removal of metabolitesmetabolites

•

• Reduction or absence of blood supply to Reduction or absence of blood supply to the heartthe heart •

• 80-90% of all heart dse mortality80-90% of all heart dse mortality •

• CAUSESCAUSES

o

o Atherosclerosis (90-95%)Atherosclerosis (90-95%) o

o Vasospasm- d/t vascular phenomenaVasospasm- d/t vascular phenomena o

o Thrombosis- Virchow’s Triad Thrombosis- Virchow’s Triad (hypercoagulab(hypercoagulability, endothelial damage, hemodynamicility, endothelial damage, hemodynamic

changes: stasis and

changes: stasis and turbulence)turbulence)

o

o Stenosis- d/t thrombosis or thickening of vesselsStenosis- d/t thrombosis or thickening of vessels o

o Inflammatory arteritisInflammatory arteritis SYNDROMES SYNDROMES ANGINA PECTORIS ANGINA PECTORIS (reversible) (reversible) -- SSuuddddeen cn chheesst pt paaiinn MYOCARDIAL MYOCARDIAL INFARCTION INFARCTION CHRONIC CHRONIC ISCHEMIC ISCHEMIC HEART HEART DISEASE DISEASE SUDDEN SUDDEN CARDIAC CARDIAC DEATH DEATH Stable/typical Stable/typical angina angina Prinzmetal/var Prinzmetal/var iant angina iant angina Unstable/cresce Unstable/cresce ndo angina ndo angina

--permanent permanent myocardial myocardial damage d/t damage d/t total total occlusion of occlusion of bld supply bld supply heart dies-> heart dies-> coagulative coagulative necrosis necrosis

--

due todue to irreversible irreversible myocardial myocardial ischemia ischemia

-- popoststininfafarcrctitioo n n decompensa decompensa tion (CHF) tion (CHF) ** CHF-CHF- end ptend pt of all cardiac of all cardiac dse dse MORPHOLOG MORPHOLOG Y: Y: Heart usually Heart usually enlarged and enlarged and heavy, heavy, secondary to secondary to left left ventricular ventricular hypertrophy hypertrophy and dilation and dilation..   _Invariably_Invariably there is there is moderate to moderate to severe severe stenosing stenosing atheroscleros atheroscleros is of the is of the coronary coronary arteries and arteries and sometimes sometimes total total occlusion. occlusion.  _Discrete,_Discrete, gray-white gray-white scars of healed scars of healed infarcts are infarcts are usually usually

--

unexpecteunexpecte d death d death from from cardiac cardiac causes causes after or after or without the without the onset of onset of symptoms symptoms -- ccoonnggeenniittaall or acquired or acquired

--

fatalfatal arrhythmia arrhythmia (most (most common common cause) cause)

--

( ( e.g.,e.g., asystole, asystole, ventricular ventricular fibrillation) fibrillation)

--

chest painchest pain during during exertion exertion _do_do more work more work increase increase demand demand the most the most common form common form   appearsappears to be to be caused caused by the by the reduction reduction of of coronary coronary perfusion perfusion to a to a critical critical level by level by chronic chronic stenosing stenosing coronary coronary atheroscl atheroscl erosis; erosis;   is usuallyis usually relieved relieved by rest by rest (thereby (thereby decreasin decreasin g g demand) demand) or or nitroglyce nitroglyce rin, a rin, a strong strong

-- chchesest pt paiain dn d/t/t vasospasm vasospasm (dec in vessel (dec in vessel caliber) caliber)   anan uncommon uncommon pattern of pattern of episodic episodic angina that angina that occurs at occurs at rest and is rest and is due to due to coronary coronary artery artery spasm. spasm. 

 Usually thereUsually there is an is an elevated ST elevated ST segment on segment on the the electrocardi electrocardi ogram ogram (ECG), (ECG), indicative of indicative of transmural transmural ischemia. ischemia. 

 the anginalthe anginal attacks are attacks are unrelated to unrelated to physical physical activity, activity, heart rate, heart rate, or blood or blood pressure. pressure. -- cchheesst t ppaaiinn even at even at rest rest -- BBV V aarree partially partially occluded occluded (preinfarct (preinfarct ))   Refers to aRefers to a pattern of pattern of pain that pain that occurs with occurs with progressively progressively increasing increasing frequency frequency , is, is precipitated precipitated with with progressively progressively less effort, less effort, often occurs often occurs at rest, and at rest, and tends to be of tends to be of more more prolonged prolonged duration. duration.

Subject: Pathology

Topic: Cardiac Pathology

Lecturer: Dr. Cruz

Date of Lecture: September 26, 2011

Transcriptionist:

ᄏᄋᄐᄏᄋᄐ

& The Soloist

Pages: 11

SS YY 22 00 11 11 --22 00 11 22

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for some for some superficial, superficial, patchy, fibrous patchy, fibrous thickenings, thickenings, although mural although mural thrombi may thrombi may be present. be present. The major The major microscopic microscopic findings findings include include myocardial myocardial hypertrophy, hypertrophy, diffuse diffuse subendocardi subendocardi al al vacuolization vacuolization , and scars of , and scars of previously previously healed healed infarcts. infarcts. NOTE:

NOTE: In more than 90% of cases, the cause of myocardial ischemia is reduction in coronary blood flowIn more than 90% of cases, the cause of myocardial ischemia is reduction in coronary blood flow due to atherosclerotic coronary arterial obstruction.

due to atherosclerotic coronary arterial obstruction. Thus, IHD is often termedThus, IHD is often termed coronary artery diseasecoronary artery disease (CAD

(CAD) ) or or coroncoronary ary hearheart t disdiseaseease. . In In mosmost t cascases, there is es, there is a a lonlong g perperiod (decaiod (decadesdes) ) of of silsilentent, , sloslowlywly progressive, coronary atherosclerosis before these disorders become manifest. Thus,

progressive, coronary atherosclerosis before these disorders become manifest. Thus, the syndromes of the syndromes of IHD are only the late manifestations of coronary atherosclerosis that probably began during childhood or IHD are only the late manifestations of coronary atherosclerosis that probably began during childhood or adolescence

adolescence •

• Acute myocardial infarction, unstable angina, and sudden Acute myocardial infarction, unstable angina, and sudden cardiac death are sometimes referred tocardiac death are sometimes referred to as

as acute acute coroncoronary ary syndrosyndromes.mes. Ac Acute ute corcoronaonary ry synsyndrodromes mes arare e frefrequequentlntly y iniinitiatiated ted by by anan unp

unpredredictictablable e anand d ababruprupt t conconverversiosion n of of a a stastable ble athatheroerosclscleroerotic tic plaplaque que to to an an unsunstabtable le and and po

potententiatially lly liflife-te-threhreatateniening ng athatheroerothrthrombombotiotic c lelesiosion n thrthrougough h supsuperferficiicial al eroerosiosion, n, ulculceraeratiotion,n, fissuring, rupture, or deep

fissuring, rupture, or deep hemorrhaghemorrhage, usually with e, usually with superimposed thrombosissuperimposed thrombosis PATHOGENESIS:

PATHOGENESIS:

The dominant influence in the causation of the IHD syndromes is diminished coronary perfusion The dominant influence in the causation of the IHD syndromes is diminished coronary perfusion relative to myocardial demand, owing largely to a complex and dynamic interaction among fixed relative to myocardial demand, owing largely to a complex and dynamic interaction among fixed atherosclerotic narrowing of the epicardial coronary arteries, intraluminal thrombosis overlying a atherosclerotic narrowing of the epicardial coronary arteries, intraluminal thrombosis overlying a disrupted atherosclerotic plaque, platelet aggregation, and

disrupted atherosclerotic plaque, platelet aggregation, and vasospasmvasospasm

In summary, Acute coronary syndromes—angina, acute MI, and sudden death—share a common In summary, Acute coronary syndromes—angina, acute MI, and sudden death—share a common pathophysiologic basis in coronary atherosclerotic plaque disruption and associated intraluminal pathophysiologic basis in coronary atherosclerotic plaque disruption and associated intraluminal platelet-fibrin thrombus formation. The critical consequence is downstream myocardial ischemia. platelet-fibrin thrombus formation. The critical consequence is downstream myocardial ischemia.

Stable angina

Stable angina results from increases in myocardial oxygen demand that outstrip the ability of results from increases in myocardial oxygen demand that outstrip the ability of markedly stenosed coronary arteries to increase oxygen delivery but

markedly stenosed coronary arteries to increase oxygen delivery but is not is not usually associated withusually associated with plaque disruption.

plaque disruption. Unstable anginaUnstable angina derives from a sudden change in plaque morphology, whichderives from a sudden change in plaque morphology, which induces partially occlusive platelet aggregati

induces partially occlusive platelet aggregation or on or mural thrombus, and vasoconstriction leading tomural thrombus, and vasoconstriction leading to severe but transient reductions in coronary blood flow. In some cases, distal microinfarcts occur severe but transient reductions in coronary blood flow. In some cases, distal microinfarcts occur seco

secondarndary y to to throthromboemmboemboli. In boli. In MI, acute MI, acute plaqplaque ue chanchange ge induinduces ces totatotal l throthrombotimbotic c occluocclusion.sion. Finally, sudden cardiac death frequently involves a coronary lesion in which disrupted plaque and Finally, sudden cardiac death frequently involves a coronary lesion in which disrupted plaque and often partial thrombus and possibly embolus have

often partial thrombus and possibly embolus have led to led to regional myocardiregional myocardial ischemia that inducesal ischemia that induces a fatal

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* Fibrin strand- stabilizes platelet

aggregates thus contributing to atheroma

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II.

II. MYOMYOCARCARDIAL DIAL INFAINFARCTRCTIONION

•

• produced most often by produced most often by atherosclerosis leaatherosclerosis leading to thrombosisding to thrombosis

•

• ischemic coagulative necrosis of myocardial fibers with loss ischemic coagulative necrosis of myocardial fibers with loss of normal contractile (vascularof normal contractile (vascular

portion)

portion) and conductive (suband conductive (subendocardium) responseendocardium) responses of affected myocardiums of affected myocardium

•

• microscopic examination of an infarct can be seen 3hours post microscopic examination of an infarct can be seen 3hours post infarction and some of theinfarction and some of the

infarcted parts are mostly evident 6hours post infarction infarcted parts are mostly evident 6hours post infarction

•

• EpidemiologyEpidemiology

o

o age peak : 55-64yrs (males); 80’s (females)- may be d/t age peak : 55-64yrs (males); 80’s (females)- may be d/t estrogenestrogen

o

o sex sex : : 3:1, 3:1, M:FM:F

o

o risk factors: risk factors: HPN, hypercholesterolemia, cigarette smokingHPN, hypercholesterolemia, cigarette smoking,, diabetes melittus,diabetes melittus,

sedentary lifestyle and oral contraceptive use sedentary lifestyle and oral contraceptive use

PATHOGENESIS: PATHOGENESIS:

In the typical case of MI, the

In the typical case of MI, the following sequence of events can be proposed:following sequence of events can be proposed: •

• The initial event is a sudden change in the morphology of an The initial event is a sudden change in the morphology of an atheromatous plaquatheromatous plaquee, that is, disruption, that is, disruption —manifest as intraplaque hemorrhag

—manifest as intraplaque hemorrhage, erosion or e, erosion or ulceration, or rupture or fissuring.ulceration, or rupture or fissuring. •

• ExpoExposed to ssed to subenubendothedothelial lial collacollagen agen and necnd necrotic protic plaqulaque contee contents,nts, pla platelettelets s undeundergo rgo adheadhesionsion,, aggregati

aggregation, activation, and on, activation, and release of potent release of potent aggregatoraggregatorss including thromboxane Aincluding thromboxane A22, serotonin, and, serotonin, and

platelet factors 3 and 4. platelet factors 3 and 4. •

• Vasospasm Vasospasm is is stimulated stimulated by by platelet platelet aggregation aggregation and and the the release release of of mediators.mediators. •

• Other mOther mediators ediators activate activate the the extrinsic extrinsic pathway pathway of coagof coagulation, ulation, adding adding to the to the bulk bulk of thof the throme thrombus.bus. •

• Frequently within minutes, the thrombus evolves to completely occlude the lumen of the coronary Frequently within minutes, the thrombus evolves to completely occlude the lumen of the coronary vessel.

vessel.

•

• PathologyPathology

o

o common location: left ventricle (because this is the active portion of common location: left ventricle (because this is the active portion of the heart)the heart)

o

o 2 forms:2 forms:



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--Most myocardial infarcts are

Most myocardial infarcts are transmuraltransmural, in which the ischemic necrosis, in which the ischemic necrosis involves the full or nearly fullinvolves the full or nearly full thi

thicknckness ess of of the the venventrictriculaular r walwalll in in the distrthe distribuibutiotion n of of a a sinsingle gle corcoronaonary ry artarteryery. . ThiThis s patpattertern n of of infarction is usually associated with coronary atherosclerosis, acute plaque change, and superimposed infarction is usually associated with coronary atherosclerosis, acute plaque change, and superimposed thrombosis

thrombosis 

 _{Subendocardial}_{Subendocardial}_{- inner 1/3 of}_{- inner 1/3 of the heart, mistaken for epigastric pain or heartburn}_{the heart, mistaken for epigastric pain or heartburn} 

 constitutes an area of ischemic necrosis limited to the innerconstitutes an area of ischemic necrosis limited to the inner one third or at most one half of theone third or at most one half of the ventricular wall

ventricular wall; under some circumstances, it may extend laterally beyond the perfusion ; under some circumstances, it may extend laterally beyond the perfusion territory of territory of a single coronary artery.

a single coronary artery. 

 Th The e subsubendendocaocardirdial al zonzone e is is nornormalmally ly thethe lealeast st welwell-pl-perfuerfused sed regregion ion of of myomyocarcardiudium m anandd therefore is most vulnerable to any reduction in coronary flow

therefore is most vulnerable to any reduction in coronary flow..

•

• Distribution of arterial involvement and resultant infarctionDistribution of arterial involvement and resultant infarction

o

o LAD/Left anterior descending coronary arteryLAD/Left anterior descending coronary artery (40% to 50%): infarct involves(40% to 50%): infarct involves

anterior wall of left ventricle near apex; anterior portion

anterior wall of left ventricle near apex; anterior portion of ventricular septum; apexof ventricular septum; apex circumferentially

circumferentially

o

o RAD: Right Anterior Descending (30-40%)RAD: Right Anterior Descending (30-40%) - post. wall of LV, post 1/3 of IVS- post. wall of LV, post 1/3 of IVS o

o LCA: Left Circumflex ArteryLCA: Left Circumflex Artery (15-20%) - lateral wall of LV except at apex(15-20%) - lateral wall of LV except at apex

•

• Location, size, morphology depends onLocation, size, morphology depends on

o

o location, severity and rate of occlusionlocation, severity and rate of occlusion o

o size of vascular bed perfused by occluded vesselsize of vascular bed perfused by occluded vessel o

o duration of occlusionduration of occlusion o

o metabolic/oxygen demands of myocardium at rmetabolic/oxygen demands of myocardium at riskisk o

o extent of collateral blood vesselsextent of collateral blood vessels o

o presence, spresence, site, and ite, and severity of severity of coronary coronary spasmspasm o

o alterations in BP, heart rate, cardiac rhythmalterations in BP, heart rate, cardiac rhythm

•

• CollateralizationCollateralization

o

o May be d/t diligent/ aerobic excerciseMay be d/t diligent/ aerobic excercise

o

o Is the growth of aIs the growth of a blood vesselblood vessel or several blood vessels that serve the same endor several blood vessels that serve the same end

organ or vascular bed as

organ or vascular bed as another blood vessel that cannot adequately supply thatanother blood vessel that cannot adequately supply that end organ

end organ or vascular bed sufficiently. (Wikipedia)or vascular bed sufficiently. (Wikipedia)

•

• ComplicationsComplications

o

o cardiac arrhythmias - 75-95% of complicated cases (most common)cardiac arrhythmias - 75-95% of complicated cases (most common) o

o LV congestive heart failure - 60%LV congestive heart failure - 60% o

o cardiogenic shock - 10-15%cardiogenic shock - 10-15% o

o rupture of free wall (cardiarupture of free wall (cardiac tamponade), septuc tamponade), septum or papillary muscle (murmm or papillary muscle (murmurs)- urs)- 1-

1-5% 5%

o

o thromboembolism- 15-40%thromboembolism- 15-40% o

o OTHERS: OTHERS: pericarditispericarditis, ventricular aneurys, ventricular aneurysms (portion of the heart wall is nms (portion of the heart wall is not rupturedot ruptured

but weakened) but weakened)

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Infarcted part of the heart is the pale area

on the circle (d/t loss

on the circle (d/t loss of bld supply): Dx-

of bld supply):

Dx-Old MI

Old MI

Enlarged coronary artery

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(7)

Coronary Artery

Atheromatous plaque

Tigering Ischemia

Thrombus

aneurysm

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III.

III. HYPERHYPERTENSIVE HETENSIVE HEART DISEAART DISEASESE •

• Response of the heart to Response of the heart to increased demands induceincreased demands induced byd by systemic or pulmonarysystemic or pulmonary

hypertension hypertension

•

• CausesCauses

o

o 95%- d/t essential HPN, idiopathic, or 95%- d/t essential HPN, idiopathic, or benignbenign o

o 5%- d/t secondary HPN5%- d/t secondary HPN o

o EndocrineEndocrine o

o Structural defectStructural defect

•

• Systemic HHDSystemic HHD

o

o Minimal Criteria for Dx of Minimal Criteria for Dx of HHD:HHD: 

 _{LVH in the absence of other}_{LVH in the absence of other cardiovascul}_{cardiovascular pathology}_{ar pathology} 

 _{history of hypertension}_{history of hypertension}

Cardiac enzymes r/t MI

• •

CPK (Creatine

Phosphokinase)

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o

o Pathologic features:Pathologic features: 

 _{concentric hypertrophy}_{concentric hypertrophy without dilatation(compensated}_{without dilatation(compensated))} 

 _{dilatation and thinning of}_{dilatation and thinning of wall (marks decompensation)}_{wall (marks decompensation)}

•

• Pulmonary HHD Pulmonary HHD (Cor (Cor Pulmonale)Pulmonale)

o

o right ventricular enlargement secondary to pulmonary HPNright ventricular enlargement secondary to pulmonary HPN o

o not a primary heart condition but d/t a pulmonary dsenot a primary heart condition but d/t a pulmonary dse o

o Acute vs ChronicAcute vs Chronic

o

o Acute cor pulmonale Acute cor pulmonale can follow massive pulmonary embolism.can follow massive pulmonary embolism. o

o Chronic cor pulmonaleChronic cor pulmonale usually implies right ventricular hypertrophy (and dilation)usually implies right ventricular hypertrophy (and dilation)

secondary to prolonged pressure overload caused by obstruction of the pulmonary secondary to prolonged pressure overload caused by obstruction of the pulmonary arteries or arterioles or compression or obliteration of septal capillaries (e.g., owing arteries or arterioles or compression or obliteration of septal capillaries (e.g., owing to primary pulmonary hypertension or emphysema).

to primary pulmonary hypertension or emphysema).

Morphology. Morphology.

•

• InInacute cor pulmonaleacute cor pulmonale, there is, there is marked dilation of marked dilation of the right ventricle the right ventricle without hypertropwithout hypertrophyhy . On cross-. On cross-section, the normal crescent shape of the right ventricle is transformed to a dilated ovoid. In chronic cor section, the normal crescent shape of the right ventricle is transformed to a dilated ovoid. In chronic cor pulmonale, the right ventricular wall thickens, sometimes up to 1.0 cm or more, and may even come to pulmonale, the right ventricular wall thickens, sometimes up to 1.0 cm or more, and may even come to approximate that of the left ventricle. More subtle stages of right ventricular hypertrophy may be observed approximate that of the left ventricle. More subtle stages of right ventricular hypertrophy may be observed as thickening of the muscle bundles

as thickening of the muscle bundles in the outflow tract, immediately below the pulmonary valve, or of thein the outflow tract, immediately below the pulmonary valve, or of the moderator band, the muscle bundle that connects the ventricular septum to the anterior right ventricular moderator band, the muscle bundle that connects the ventricular septum to the anterior right ventricular papillary muscle. Sometimes there is secondary compression of the left ventricular chamber or tricuspid papillary muscle. Sometimes there is secondary compression of the left ventricular chamber or tricuspid regurgitation with fibrous thickening of this valve.

regurgitation with fibrous thickening of this valve.

o o CausesCauses   _Obstructive_Obstructive emphysema emphysema 

 _{Chronic bronchitis}_{Chronic bronchitis}   _{Pneumoconiosis}_{Pneumoconiosis}   _Fibrosis_Fibrosis   _Carcinoma_Carcinoma   _{Bronchiectasis}_{Bronchiectasis} 

 _{Cystic lung}_{Cystic lung} 

 _{Chronic pneumonia}_{Chronic pneumonia} 

 _{Pleural effusion}_{Pleural effusion} o

o PathogenesisPathogenesis 

 _{RV enla}_{RV enlargement d}_{rgement due to pu}_{ue to pulmonary hyp}_{lmonary hypertension}_{ertension caused b}_{caused by lung}_{y lung disorders}_disorders

•

• COPD, DIP, atelectasis, cystic fibrosisCOPD, DIP, atelectasis, cystic fibrosis •

• Pulmonary embolism (acute CP), Primary Pulmonary Pulmonary embolism (acute CP), Primary Pulmonary HPNHPN •

• Kyphoscoliosis (structural defect)Kyphoscoliosis (structural defect)

•

• PickwickiaPickwickian habitus (aka: On habitus (aka: Obesity Hypoventilatibesity Hypoventilation Syndrome; on Syndrome; is ais a

condition in which severely overweight people

condition in which severely overweight people fail to breathe rapidlyfail to breathe rapidly enough or deeply enough

enough or deeply enough, resulting in low blood, resulting in low blood oxygenoxygenlevels andlevels and high blood

high blood carbon dioxidecarbon dioxide (CO2) levels. ~~ Wikipedia)(CO2) levels. ~~ Wikipedia)

•

• metabolic acidosis, hypoxiametabolic acidosis, hypoxia

Concentric Hypertrophy

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IV.

IV. CONGECONGENITAL HEARNITAL HEART DISEASEST DISEASES

•

• Structural abnormality of heart present at birthStructural abnormality of heart present at birth

•

• May be COMPATIBLE vs. INCOMPATIBLE with life (depends on the presence of May be COMPATIBLE vs. INCOMPATIBLE with life (depends on the presence of life-savinlife-savingg

shunts) shunts)

•

• Incidence/ etiology:Incidence/ etiology: Affects 0.3to 1% of all Affects 0.3to 1% of all livebirths/1000; 9births/1000livebirths/1000; 9births/1000

•

• FrequenciesFrequencies

o

o VSD- 33% (most common)VSD- 33% (most common)

o o ASD- 5%ASD- 5% o o PDA- 10%PDA- 10% o o TGA- 5% TGA- 5% o o PS- 10%PS- 10% o o A-V SD- 4%A-V SD- 4% o o TOF- 9% TOF- 9% o o T ARTERIOSUS- 1% T ARTERIOSUS- 1% o o AS- 8%AS- 8% o o TA- 1% TA- 1% o o CoA- 5%CoA- 5% o o TAPVC- 1 TAPVC- 1 •

• Clinical effects of congenital heart diseaseClinical effects of congenital heart disease

o

o CyanosisCyanosis o

o ClubbingClubbing

o

o PolycythemiaPolycythemia- compensatory - compensatory mech d/tmech d/t

poor oxygenation poor oxygenation

o

o pulmonary vascular disease- d/t too pulmonary vascular disease- d/t too muchmuch

bld in the pulmonary vascular bed bld in the pulmonary vascular bed

o

o hypertrophic changeshypertrophic changes o

o stunted growthstunted growth

o

o paradoxical embolization- venous embolusparadoxical embolization- venous embolus

becomes a systemic embolism, shunt vessel becomes a systemic embolism, shunt vessel should be present

should be present

o

o infectious endocarditisinfectious endocarditis

Saddle Pulmonary Embolism

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Classification of Congenital Heart Diseases by RUBIN Classification of Congenital Heart Diseases by RUBIN

IINNIITTIIAAL L LLEEFFT T TTO O RRIIGGHHT T SSHHUUNNTTSS RRIIGGHHT T TTO O LLEEFFT T SSHHUUNNTTSS NNO O SSHHUUNNTTSS V VSSDD AASSDD PPDDAA TTOOFF TTGGAA TruncusTruncus Arteriosus Arteriosus TATA Total Anomalous Total Anomalous Pulmonary Venous Pulmonary Venous Connection Connection Coarctation of Coarctation of aorta aorta Pulmonary Pulmonary stenosis stenosis -- mostmost common common congenital congenital heart defect heart defect,, 30% isolated 30% isolated - frequently - frequently associated with associated with other other anomalies anomalies - defect is - defect is significant if significant if greater than greater than 1.0cm in 1.0cm in diameter diameter - Life- saving - Life- saving defect: TOF defect: TOF Types: Types: a. a.MembranouMembranou s- close to s- close to AV bundle AV bundle of HIS of HIS 90% 90% b.

b.MuscMusc ular ular --below below pulmonary pulmonary valve; valve; muscular muscular septum septum c.

c. InfInfracracrisristaltal d.

d. SuprSupracristacristalal ROGER'S ROGER'S DISEASE DISEASE

-- mumuscsculularar,, less than less than 0.5cm in 0.5cm in diameter) diameter) - most - most common in common in ADULTS ADULTS --communicati communicati on between on between atria because atria because of abnormal of abnormal opening opening - most - most common common CHD in adults CHD in adults to be to be clinically clinically apparent apparent Major Major Types: Types: a. a. OstOstiumium

Secundum Secundum - 90% of - 90% of all ASD all ASD --deficiency deficiency or or fenestratio fenestratio n of n of septum septum primum, primum, deficiency deficiency of septum of septum secundum, secundum, or both or both - involves - involves the valve the valve or limbus or limbus of the of the fossa fossa ovalis ovalis b. Ostium b. Ostium --aortopulmona aortopulmona ry channel ry channel remains remains open: open: • • 85-90%85-90% isolated isolated • • normallynormally open for open for 1-2month 1-2month after birth after birth (closes at (closes at the end of the end of 2mos) 2mos) • • prematuriprematuri ty ty -delayed delayed closure closure especially especially if large if large • • distresseddistressed with low with low O2 O2 tension-delayed delayed closure closure - Life-saving - Life-saving defect: TOGV defect: TOGV and and Pulmonary Pulmonary atresia atresia - cyanosis of - cyanosis of the lower the lower extremities extremities since after since after subclavian subclavian artery artery - Mngt: - Mngt: surgical surgical - results - results embryologicall embryologicall y from y from antero-superior superior displacement displacement of the of the infundibular infundibular septum septum -- mostmost common form common form of cyanotic of cyanotic congenital congenital heart disease heart disease 4 4 Features:Features: 1. 1.VSDVSD (membrano (membrano us, size of us, size of the aortic the aortic lumen) life lumen) life saving saving 2. 2.OBSTRUCTIOBSTRUCTI ON TO THE ON TO THE RIGHT RIGHT VENTRICUL VENTRICUL AR AR OUTFLOW OUTFLOW TRACT TRACT (Subpulmon (Subpulmon ary ary stenosis) stenosis) 3. 3. AOAORTARTA OVERRIDES OVERRIDES THE VSD THE VSD 4 . 4 . R VR VHH * remember: * remember: PROV PROV P PDADA - AKA: - AKA: Ventriculoarte Ventriculoarte rial rial discordance discordance - aorta arises - aorta arises from the right from the right ventricle and ventricle and the the pulmonary pulmonary artery artery emanates emanates from the left from the left ventricle ventricle - embryologic - embryologic defect is defect is abnormal abnormal formation of formation of the truncal the truncal and and aortopulmona aortopulmona ry septa ry septa --incompatible incompatible with life in the with life in the absence of absence of shunts: shunts: a. a.VSDVSD (35%) (35%) -stable stable shunt shunt b.

b.PDA ANDPDA AND PATENT PATENT FORAMEN FORAMEN OVALE OVALE (65%)-unstable unstable - RHV and - RHV and thin-walled thin-walled left ventricle left ventricle --development development al failure of al failure of separation of separation of the the embryologic embryologic arteriosus arteriosus into the aorta into the aorta and and pulmonary pulmonary artery artery resulting in a resulting in a single artery single artery receiving receiving blood from blood from both both ventricles ventricles - usually - usually accompanied accompanied by VSD by VSD - complete - complete occlusion of occlusion of the tricuspid the tricuspid valve orifice valve orifice - results - results from from unequal unequal distribution distribution of the of the normal AV normal AV canal canal - associated - associated with right with right ventricular ventricular hypoplasia hypoplasia - right to left - right to left shunt shunt maintained maintained thru ASD/ thru ASD/ patent patent foramen foramen ovale with ovale with VSD so VSD so communicat communicat ion between ion between Left and Left and great artery great artery from the from the hypoplastic hypoplastic right right ventricle ventricle - results - results embryologically embryologically when the common when the common pulmonary vein fails pulmonary vein fails to develop or to develop or becomes atretic becomes atretic causing primitive causing primitive systemic venous systemic venous channels from the channels from the lungs to remain lungs to remain patent patent - 50% cases drains - 50% cases drains into the innominate/ into the innominate/ brachiocephalic vein brachiocephalic vein (L) or the coronary (L) or the coronary sinus sinus - associated with - associated with PDA or patent PDA or patent foramen ovale foramen ovale - constriction, - constriction, narrowing of narrowing of aorta aorta - more common - more common in in MALESMALES 2 Types: 2 Types: a.

a. PrPre-e-duductctalal (Infantile (Infantile type) type) - tubular - tubular hypoplasia of hypoplasia of the aortic the aortic arch arch - patency of - patency of PDA PDA necessary for necessary for life life - RVH in - RVH in utero utero - early CHF - early CHF - cyanosis of - cyanosis of the lower the lower half of the half of the body body b.

b. PoPostst-d-ducuctatall (adult type) (adult type) --asymptomati asymptomati c in early c in early stage stage --hypertension hypertension in the upper in the upper extremities extremities but with but with weak pulses weak pulses and lower BP and lower BP in in extremities extremities - a dynamic - a dynamic or fixed or fixed obstruction obstruction to flow from to flow from the

the rightright ventricle ventricle of of the heart to the heart to the

the pulmonpulmon ary artery ary artery.. - usually d/t - usually d/t isolated isolated valvular valvular obstruction, obstruction, but may be but may be d/t d/t subvalvular subvalvular or or supravalvul supravalvul ar ar obstruction. obstruction. - It may - It may occur in occur in association association with more with more complicate complicate d d congenital congenital heart heart disorders. disorders. (Wikipedia) (Wikipedia) Primum Primum Defect Defect - 5% of all - 5% of all ASD ASD - occurs - occurs low low and and adjacent adjacent to valves, to valves, anteroinfe anteroinfe rior rior to the to the fossa fossa ovalis ovalis c. Sinus c. Sinus Venosus Venosus Defect Defect - 5% of all - 5% of all ASD ASD - located - located high high in the in the septum, septum, posterior posterior to to fossa fossa ovalis ovalis near the near the entrance entrance of of the SVC the SVC LUTEMBACHE LUTEMBACHE R'S R'S SYNDROME SYNDROME - small ASD - small ASD associated associated with mitral with mitral stenosis stenosis closure, closure, INDOMETHACI INDOMETHACI N (PG N (PG inhibitor) inhibitor) R Rightight Ventricular Ventricular Hypertrophy Hypertrophy O

Overriding of verriding of the aorta the aorta V VSDSD - severity of - severity of obstruction to obstruction to RV outflow RV outflow determines determines direction of direction of blood flow blood flow "pink "pink tetralogy"- mild tetralogy"- mild pulmonary pulmonary stenosis (baby stenosis (baby is not cyanotic is not cyanotic but dusky but dusky looking) looking) - Mngt: - Mngt: surgery STAT surgery STAT --development development of collateral of collateral vessels--"rib vessels--"rib notching" 2/t notching" 2/t enlarged enlarged internal internal mammary mammary and and intercostal intercostal arteries arteries * Eisenmenge

* Eisenmenge r syndrome (or Eisenmr syndrome (or Eisenm enger's reacenger's reac tion) tion) - is defined as the - is defined as the procesproces s in which as in which a left-to-right shuntleft-to-right shunt caused by acaused by a congenital heart defectcongenital heart defect causes increased flowcauses increased flow through the pulmonary

through the pulmonary vasculaturevasculature, causing, causing pulmonary hypertensionpulmonary hypertension, which in turn, causes , which in turn, causes increased pressures in the right side of the heart and reversal of increased pressures in the right side of the heart and reversal of the shuntthe shunt into a

into a right-to-left shuntright-to-left shunt. (Wikipedia). (Wikipedia) ** Atrioventricular Septal DefectAtrioventricular Septal Defect

-- abnormal development of embryologic AV canalabnormal development of embryologic AV canal -- failurfailure of e of fusion fusion of sof superiuperior aor and ind inferionferior enr endocarddocardialial

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Primum Primum Defect Defect - 5% of all - 5% of all ASD ASD - occurs - occurs low low and and adjacent adjacent to valves, to valves, anteroinfe anteroinfe rior rior to the to the fossa fossa ovalis ovalis c. Sinus c. Sinus Venosus Venosus Defect Defect - 5% of all - 5% of all ASD ASD - located - located high high in the in the septum, septum, posterior posterior to to fossa fossa ovalis ovalis near the near the entrance entrance of of the SVC the SVC LUTEMBACHE LUTEMBACHE R'S R'S SYNDROME SYNDROME - small ASD - small ASD associated associated with mitral with mitral stenosis stenosis closure, closure, INDOMETHACI INDOMETHACI N (PG N (PG inhibitor) inhibitor) R Rightight Ventricular Ventricular Hypertrophy Hypertrophy O

Overriding of verriding of the aorta the aorta V VSDSD - severity of - severity of obstruction to obstruction to RV outflow RV outflow determines determines direction of direction of blood flow blood flow "pink "pink tetralogy"- mild tetralogy"- mild pulmonary pulmonary stenosis (baby stenosis (baby is not cyanotic is not cyanotic but dusky but dusky looking) looking) - Mngt: - Mngt: surgery STAT surgery STAT --development development of collateral of collateral vessels--"rib vessels--"rib notching" 2/t notching" 2/t enlarged enlarged internal internal mammary mammary and and intercostal intercostal arteries arteries * Eisenmenge

* Eisenmenge r syndrome (or Eisenmr syndrome (or Eisenm enger's reacenger's reac tion) tion) - is defined as the - is defined as the procesproces s in which as in which a left-to-right shuntleft-to-right shunt caused by acaused by a congenital heart defectcongenital heart defect causes increased flowcauses increased flow through the pulmonary

through the pulmonary vasculaturevasculature, causing, causing pulmonary hypertensionpulmonary hypertension, which in turn, causes , which in turn, causes increased pressures in the right side of the heart and reversal of increased pressures in the right side of the heart and reversal of the shuntthe shunt into a

into a right-to-left shuntright-to-left shunt. (Wikipedia). (Wikipedia) ** Atrioventricular Septal DefectAtrioventricular Septal Defect

-- abnormal development of embryologic AV canalabnormal development of embryologic AV canal -- failurfailure of e of fusion fusion of sof superiuperior aor and ind inferionferior enr endocarddocardialial

-- cushions with incomplete closure cushions with incomplete closure of AV septum of AV septum and inadequand inadequate formation of sepate formation of septal tricuspid and tal tricuspid and anterior anterior mitral leafletmitral leaflet

Please read and review Netter’s atlas plus Robbin’s and Cotran Patho

Please read and review Netter’s atlas plus Robbin’s and Cotran Patho book…..book….. ~~Happy Studying! ^_^~~Happy Studying! ^_^

ASD- Secundum ASD- Secundum tt ee VSD- Muscular VSD- Muscular tt ee

Transposition of the Great Transposition of the Great Arteries Arteries NOTICE TO THE NOTICE TO THE PUBLIC: PUBLIC:

Guys kindly read your Guys kindly read your books, I’ve tried to books, I’ve tried to put some infos put some infos coming from the book coming from the book but its not enough ..I but its not enough ..I think..

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