HEART
THE HEART
• Normal
• Pathology
– Heart Failure: L, R
– Heart Disease
•
•
•
•
•
•
•
•

Congenital: LR shunts, RL shunts, ...
NORMAL Features
•
•
•
•
•

6000 L/day
250-300 grams
40% of all deaths (2x cancer)
Wall thickness ~ pressure
(i.e., a wall ...
TERMS
• CARDIO”MEGALY”
– DILATATION, any chamber, or all
– HYPERTROPHY, and chamber, or all
STRIATIONS

NUCLEUS

DISCS

SARCOLEMMA

SARC. RETIC.

MITOCHONDRIA

ENDOTHELIUM

FIBROBLASTS

GLYCOGEN

A.N.P.
S.A. NodeAV NodeBundle of HIS L. Bundle, R. Bundle
AX

Anterior
Lateral
Posterior
Septal

VLA

HLA
VALVES
• AV:
– TRICUSPID
– MITRAL

13 cm
11 cm

• SEMILUNAR:
– PULMONIC
– AORTIC

8 cm
6 cm
CARDIAC AGING
Chambers

Epicardial Coronary
Arteries

Increased left atrial cavity size

Tortuosity

Decreased left ventri...
CARDIAC AGING
Aorta
Dilated ascending aorta with rightward shift
Elongated (tortuous) thoracic aorta
Sinotubular junction ...
BROWN
ATROPHY, HEART

LIPOFUCSIN
Pathologic Pump Possibilities
• Primary myocardial failure
(MYOPATHY)
• Obstruction to flow (VALVE)
• Regurgitant flow (VA...
• DEFINITION
• TRIAD

CHF

– 1) TACHYCARDIA
– 2) DYSPNEA
– 3) EDEMA
• FAILURE of Frank Starling mechanism
• HUMORAL FACTOR...
HYPERTROPHY
• PRESSURE OVERLOAD (CONCENTRIC)
• VOLUME OVERLOAD (CHF)
• LVH, RVH, atrial, etc.
• 2X normal weight ischemia...
CHF: Autopsy Findings
• Cardiomegaly
• Chamber Dilatation
• Hypertrophy of myocardial fibers,
BOXCAR nuclei
Left Sided Failure
• Low output vs. congestion
• Lungs
– pulmonary congestion and edema
– heart failure cells

• Kidneys
–...
Left Heart Failure Symptoms
• Dyspnea
– on exertion
– at rest

• Orthopnea
– redistribution of peripheral edema fluid
– gr...
LEFT Heart Failure
Dyspnea

Orthopnea
PND (Paroxysmal Nocturnal
Dyspnea)
Blood tinged sputum
Cyanosis
Elevated pulmonary “...
Right Sided Heart Failure
• Etiology
– left heart failure
– cor pulmonale

• Symptoms and signs
– Liver and spleen
• passi...
RIGHT Heart Failure
FATIGUE
“Dependent” edema
JVD
Hepatomegaly (congestion)
ASCITES, PLEURAL EFFUSION
GI
Cyanosis
Increase...
HEART DISEASE
• CONGENITAL (CHD)
•
•
•
•

ISCHEMIC (IHD)
HYPERTENSIVE (HHD)
VALVULAR (VHD)
MYOPATHIC (MHD)
CONGENITAL HEART
DEFECTS

• Faulty embryogenesis (week 3-8)
• Usually MONO-morphic (i.e., SINGLE
lesion) (ASD, VSD, hypo-R...
Incidence per Million Live
Births

%

4482

42

1043

10

Pulmonary stenosis

836 

8 

Patent ductus arteriosus

781 

7 ...
GENETICS
• Gene abnormalities in only 10% of CHD
• Trisomies 21, 13, 15, 18, XO
• Mutations of genes which encode for
tran...
ENVIRONMENT
• RUBELLA
• TERATOGENS
CHD

• LR SHUNTS: all “D’s” in their names
– NO cyanosis
– Pulmonary hypertension
– SIGNIFICANT pulmonary hypertension is...
• ASD
• VSD
• ASVD
• PDA

LR

NON CYANOTIC

IRREVERSIBLE
PULMONARY
HYPERTENSION
IS THE MOST
FEARED
CONSEQUENCE
ASD
• NOT patent foramen ovale
• Usually asymptomatic until adulthood
• SECUNDUM (90%): Defective fossa
ovalis
• PRIMUM (5...
VSD
•
•
•
•
•

By far, most common CHD defect
Only 30% are isolated
Often with TETRALOGY of FALLOT
90% involve the membran...
PDA
• 90% isolated
• HARSH, machinery-like murmur
• LR, possibly RL as pulmonary
hypertension approaches systemic
pressu...
AVSD

• Associated with defective,
inadequate AV valves
• Can be partial, or COMPLETE
(ALL 4 CHAMBERS FREELY
COMMUNICATE)
RL
• Tetralogy of Fallot
• Transposition of great arteries
• Truncus arteriosus
• Total anomalous pulmonary venous
connec...
RL SHUNTS
• TETRALOGY of FALLOT most COMMON
– 1) VSD, large
– 2) OBSTRUCTION to RV flow
– 3) Aorta OVERRIDES the VSD
– 4)...
TGA (TRANSPOSITION
of GREAT ARTERIES)
• NEEDS a SHUNT for
survival, obviously
– PDA or PFO (65%),
“unstable” shunt
– VSD (...
TRUNCUS ARTERIOSIS
TRICUSPID ATRESIA
• Hypoplastic RV
• Needs a shunt, ASD, VSD, or PDA
• High mortality
Total Anomalous Pulmonary
Venous Connection (TAPVC)
• PULMONARY VEINS do NOT go into
LA, but into L. innominate v. or
coro...
OBSTRUCTIVE CHD
• COARCTATION of aorta
• Pulmonary stenosis/atresia
• Aortic stenosis/atresia
COARCTATION of AORTA
•
•
•
•
•

M>F
But XO’s frequently have it
INFANTILE FORM (proximal to PDA) (SERIOUS)
ADULT FORM (CLO...
PULMONIC
STENOSIS/ATRESIA
• If 100% atretic, hypoplastic RV with ASD
• Clinical severity ~ stenosis severity
AORTIC
STENOSIS/ATRESIA
• VALVULAR
– If severe, hypoplastic LVfatal

• SUB-valvular (subaortic)
– Aortic wall THICK BELOW...
HEART DISEASE
• CONGENITAL (CHD)

• ISCHEMIC (IHD)
• HYPERTENSIVE (HHD)
• VALVULAR (VHD)
• MYOPATHIC (MHD)
SYNDROMES of IHD
•
•
•
•

Angina Pectoris: Stable, Unstable
Myocardial Infarction (MI, AMI)
Chronic IHD CHF (CIHD)
Sudden...
IHD RISK
• Number of plaques
• Distribution of plaques
• Size, structure of plaques
ACUTE CORONARY SYNDROMES
• “The acute coronary syndromes are
frequently initiated by an
unpredictable and abrupt conversio...
EPIDEMIOLOGY
(same as atherosclerosis)

• ½ million die of IHD yearly in USA
• 1 million in 1963. Why?
– Prevention of con...
ACUTE CORONARY
SYNDROME FACTORS
• ACUTE PLAQUE CHANGE *******
• Inflammation
• Thrombus
• Vasoconstriction
******* MOST IM...
ACUTE PLAQUE CHANGE
• Rupture/Refissuring
• Erosion/Ulceration, exposing ECM
• Acute Hemorrhage

NB: Plaques do NOT have t...
INFLAMMATION
• Endothelial cells release CAMs,
selectins
• T-cells release TNF, IL-6, IFN-gamma to
stimulate and activate ...
THROMBUS
• Total occlusion
• Partial
• Embolization
VASOCONSTRICTION
• Circulating adrenergic agonists, i.e., α
• Platelet release products, e.g., ADP
• Endothelially release...
Coronary Artery Pathology in Ischemic Heart Disease
Syndrome

Plaque
Stenoses Disruption

Stable angina

>75%

No

No

Uns...
ANGINA PECTORIS
•
•
•
•
•

Paroxysmal (sudden)
Recurrent
15 sec.15 min.
Reduced perfusion, but NO infarction
THREE TYPES
...
MYOCARDIAL INFARCTION
• Transmural vs. Subendocardial (inner 1/3)
• DUH! EXACT SAME risk factors as
atherosclerosis
• Most...
MYOCARDIAL RESPONSE
Feature

Time

Onset of ATP depletion

Seconds

Loss of contractility

<2 min

ATP reduced
to 50% of n...
PROGRESSION OF NECROSIS
TIMING of Gross and Microscopic Findings

GROSS

MICROSCOPIC

½–4 hr

None

Usually none; variable waviness of fibers at b...
1 day (pyknosis, “waviness”)
3-4 days (neutrophils)
7 days (macrophages)
Weeks (organization)
Months (fibrosis)
RE-PERFUSION

• Thrombolysis
• PTCA
• CABG

• Reperfusion CANNOT restore necrotic
or dead fibers, only reversibly injured
...
AMI DIAGNOSIS
• SYMPTOMS
• EKG 1) Q-waves, 2) T-wave inversion, 3)

ST-T

elevation

• DIAPHORESIS
• (10% of MIs are “SILE...
COMPLICATIONS
•
•
•
•
•
•
•
•
•

Wall motion abnormalities
Arrhythmias
Rupture (4-5 days)
Pericarditis
RV infarction
Infar...
CIHD, aka, ischemic
“cardiomyopathy”
• Progress to CHF often with no
pathologic or clinical evidence of
localized infarcti...
SUDDEN CARDIAC DEATH
• 350,000 in USA yearly from atherosclerosis
• NON-atherosclerotic sudden cardiac death includes:

– ...
AUTOPSY findings in SCD
• >75% narrowing of 1-3 vessels
• Healed infarcts 40%
• “ARRHYTHMIA” is often a very
convenient co...
HEART DISEASE
• CONGENITAL (CHD)
• ISCHEMIC (IHD)

• HYPERTENSIVE (HHD)
• VALVULAR (VHD)
• MYOPATHIC (MHD)
HHD (Left)
• DEFINITION: Hypertrophic
adaptive response of the heart,
which can progress:
– Myocardial dysfunction
– Cardi...
NEEDED for DIAGNOSIS:
• LVH (LV>2.0 and/or Heart>500 gm.)
• HTN (>140/90)
PREVALENCE:
• WHAT % of USA people have
hypertension?
PREVALENCE:
• WHAT % of USA people have
hypertension?

•

Answer:

25%
HISTOPATHOLOGY
• INCREASED FIBER (MYOCYTE)
THICKNESS

• INCREASED nuclear size with
increased “blockiness” (boxcar nucleus...
CLINICAL
• EKG in LVH
Summary of LVH Criteria
1) R-I + S-III >25 mm
2) S-V1 + R-V5 >35 mm
3) ST-T depr. in L lead
4) R-wav...
COURSE:
• NORMAL longevity, death from
other causes
• Progressive IHD
• Progressive renal damage,
hemorrhagic CVA (Which a...
HHD (Right) = COR PULMONALE
• ACUTE: Massive PE (No RVH)
• CHRONIC: COPD, CRPD,
Pulmonary artery disease, chest
wall motio...
Diseases of the Pulmonary
Parenchyma
Chronic obstructive pulmonary disease

Disorders Affecting Chest Movement
Kyphoscolio...
HEART DISEASE
• CONGENITAL (CHD)
• ISCHEMIC (IHD)
• HYPERTENSIVE (HHD)

• VALVULAR (VHD)
• MYOPATHIC (MHD)
V HD
alvular

• Opening problems:
•

Stenosis
Closing problems: Regurgitation
or Incompetence or “insufficiency” (as
oppos...
• AS

70% of all VHD

– Calcification of a deformed valve
– “Senile” calcific AS
– Rheum, Heart Dis.

• MS
–Rheumatic Hear...
AORTIC STENOSIS
2X gradient pressure
LVH (but no hypertension), ischemia
Cardiac decompensation, angina, CHF
50% die in 5 ...
MITRAL ANNULAR
CALCIFICATION
• Calcification of the
mitral “skeleton”
• Usually NO
dysfunction
• Regurgitation
usually, bu...
REGURGITATIONS

• AR

– Rheumatic
– Infectious
– Aortic dilatations
• Syphilis
• Rheumatoid Arthritis
• Marfan

• MR

–MVP...
Mitral Valve Prolapse (MVP)
• MYXOMATOUS degeneration of the
mitral valve
• Associated with connective tissue
disorders
• ...
MVP: CLINICAL FEATURES
•
•
•
•
•
•

Usually asymptomatic
Mid-systolic “click”
Holosystolic murmur if regurg. present
Occas...
RHEUMATIC Heart Disease
• Follows a group A strep infection, a
few weeks later
• DECREASE in “developed” countries
• PANCA...
ACUTE:
-Inflammation
-Aschoff bodies
-Anitschkow cells
-Pancarditis
-Vegetations on
chordae tendinae at
leaflet junction

...
CLINICAL FEATURES
•
•
•
•
•

Migratory Polyarthritis
Myocarditis
Subcutaneous nodules
Erythema marginatum
Sydenham chorea
INFECTIOUS ENDOCARDITIS
• Microbes
–

Usually

strep viridans

– Often Staph aureus in IVD users
– Enterococci

– HAČEK (n...
INFECTIOUS ENDOCARDITIS
• Acute: 50% mortality (course=days)

• SUB-acute: LOW mortality (course=weeks)
VEGETATIONS
• INFECTIVE >5mm
• NON-Infective <5mm
DIAGNOSIS=MMm, Mmmm, mmmmm
• MAJOR
•
•
•

•
•
•
•
•
•
•

Positive blood culture(s) indicating characteristic organism or p...
NON-infective VEGETATIONS
• <5 mm
• PE
• Trousseau syndrome (migratory
thrombophlebitis with malignancies)
• s/p Swan-Ganz...
•
•
•
•

Carcinoid Syndrome
Episodic skin flushing
Cramps
Nausea & Vomiting
Diarrhea

• ↑serotonin, ↑ 5HIAA in urine
• FIB...
ARTIFICIAL VALVES
• Mechanical
• Xenografts (porcine)
• 60% have complications within
10 years
HEART DISEASE
• CONGENITAL (CHD)
• ISCHEMIC (IHD)
• HYPERTENSIVE (HHD)
• VALVULAR (VHD)

• MYOPATHIC (MHD)
• PERICARDIAL D...
CARDIOMYOPATHIES
•
•
•
•
•
•
•

Inflammatory
Immunologic
Metabolic
Dystrophies
Genetic
Idiopathic
Toxic

• DILATED (DCM)
–...
Functional
Pattern
Dilated

LVEF
<40%

Mechanisms of
Heart Failure
Impairment of

contractility
(systolic
dysfunction)

Hy...
Cardiac Infections

Toxins

Metabolic

Viruses: Cox-B, esp.

Alcohol

Hyperthroidism

Chlamydia

Cobalt

Hypothyroidism

R...
DILATED cardiomyopathy
•
•
•
•
•
•

Chamber thickness (not just LVH)
Adults
Progressively declining LVEF
LVEF ~ prognosis
...
DCM

Path:
4 chamber dilatation
Hypertrophy, also 4 chambers
Interstitial Fibrosis
Arrhythmogenic Right Ventricular Cardiomyopathy
(Arrhythmogenic Right Ventricular Dysplasia)

This is an uncommon dilated ...
HYPERTROPHIC cardiomyopathy
• Also called IHSS, (Idiopathic Hypertrophic
Subaortic Stenosis)
– GENETIC defects involving:
...
RESTRICTIVE cardiomyopathy
•
•
•
•

(idiopathic)
↓ ventricular compliance
Chiefly affects DIASTOLE
NORMAL chamber size and...
MYOCARDITIS
• INFLAMMATION of MYOCARDIUM
• Chiefly microbial

–COXACKIE A & B, CMV, HIV
–
–
–
–
–

Trypanosoma cruzi (Chag...
LYMPHOCYTIC INFILTRATES are the USUAL pattern of ALL myocarditis, but
eosinophils, giant cells, and even trypanosomes can ...
OTHER Myocarditides
•
•
•
•

Adriamycin
Cyclophosphamide
Catecholamines (Pheochromocytomas)
Amyloid, systemic or primary c...
PERICARDIUM
• Normally 30-50 ml clear serous fluid
– Visceral (epicardium)
– Parietal (fibrous pericardium)
– PERICARDIAL ...
PERICARDITIS
• SEROUS: Rheum. Fever (RF), SLE,
scleroderma, tumors, uremia

• FIBRINOUS: MI (Dressler), uremia, radiation,...
TUMORS

• 90% benign “mesenchymal”, i.e., stromal

– MYXOMAS (LEFT ATRIUM MOST
COMMON)
–
–
–
–

FIBROMAS
LIPOMAS
FIBROELAS...
MYXOMA
Cardiac effects of NON-cardiac tumors
• Direct Consequences of Tumor
– Pericardial and myocardial metastases
– Large vesse...
CARDIAC TRANSPLANT
PATHOLOGY
• Most patients are on
immunosuppressives
• 5 year survival >60%
CARDIAC TRANSPLANT
PATHOLOGY
Minarcik robbins 2013_ch12-heart
Minarcik robbins 2013_ch12-heart
Minarcik robbins 2013_ch12-heart
Minarcik robbins 2013_ch12-heart
Minarcik robbins 2013_ch12-heart
Minarcik robbins 2013_ch12-heart
Minarcik robbins 2013_ch12-heart
Minarcik robbins 2013_ch12-heart
Minarcik robbins 2013_ch12-heart
Minarcik robbins 2013_ch12-heart
Minarcik robbins 2013_ch12-heart
Minarcik robbins 2013_ch12-heart
Minarcik robbins 2013_ch12-heart
Minarcik robbins 2013_ch12-heart
Minarcik robbins 2013_ch12-heart
Minarcik robbins 2013_ch12-heart
Minarcik robbins 2013_ch12-heart
Minarcik robbins 2013_ch12-heart
Minarcik robbins 2013_ch12-heart
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  • Just as we said the Blood Vessel chapter was 1) atherosclerosis and 2) everything else
    We can now say the heart chapter is 1) ischemic heart disease and 2) everything else
  • This is the chapter outline, fairly logical
  • Remember 1.5 cm is considered to be the AVERAGE LV wall thickness, RV is 1/3 that, and atria are ½ RV.
  • This is a section from “Shotgun Histology”, in other words, the terms on the left describe the entire myocardium. Atrial natriuretic peptide (ANP), atrial natriuretic factor (ANF), atrial natriuretic hormone (ANH), or atriopeptin, is a powerful vasodilator, and a protein (polypeptide) hormone secreted by heart muscle cells. It is involved in the homeostatic control of body water, sodium, potassium and fat (adipose tissue). It is released by muscle cells in the upper chambers (atria) of the heart (atrial myocytes), in response to high blood pressure. ANP acts to reduce the water, sodium and adipose loads on the circulatory system, thereby reducing blood pressure.
  • The specialized myocytes of the heart’s conduction system, Purkinje fibers, running sub-endocardially, have this unique appearance.
    I do not recall any pathologist ever pinpointing an EKG abnormaility to a specific histopathologic abnormality of a Purkinje fiber.
  • Whichever artery winds up supplying the posterior interventricular septum is said to be “DOMINANT”.
    A thrombosis of WHICH coronary artery would usually result in sudden death? Ans: MAIN left coronary artery.
  • The myocardial perfusion is a good test of coronary artery and myocardial function.
  • R&gt;L
  • These features are seen so commonly in autopsies of elderly people no matter what they died from. Also keep in mind that most people who do not die ACUTELY, die in cardiac failure. Also keep in mind that even though atherosclerosis is NOT considered normal aging, many of the atherosclerotic changes cannot be completely separated from “normal” aging, because it is so common.
  • One very key philosophical question is whether atherosclerosis is part of aging or not. We can leave that for the philosophers.
  • The pigment which accumulates with age is called lipofucsin, and caused the heart to appear “browner” than normal. This is called “brown” atrophy of the heart. Lipofucsin is another typical example of a golden brown, slightly refractile, INtrinsic pigment, which looks like hemosiderin, melanin, or bile, but accumulates, as a rule, on opposite poles of the myocyte nucleus. It is also called, appropriately, AGING pigmernt.
    It appears to be the product of the oxidation of unsaturated fatty acids.
  • This is the same analogy as the “straw” we talked about in the last chapter on blood vessels. You can classify cardiac diseases functionally into these 5 “pump” categories, like we had only 2 categories with the blood vessels described as straws or conduits.
  •  ANP acts to reduce the water, sodium and adipose loads on the circulatory system, thereby reducing blood pressure
  • Very FEW hearts of elderly people at autopsy weigh the normal 250-300 gm. Atherosclerotic or CHF hearts weigh twice as much, hypertensive hearts weight three times as much, and cardiomyopathic hearts often weigh more.
  • LVH, how do you know this is probably NOT a cardiomyopathy? Ans: Thickening limited to LV
  • A good general diagram.
  • Note that not only is the FIBER thick, but so are the nuclei. Note squaring off of the nuclei, so called “BOXCAR” effect.
  • Can you understand why all of these findings can be related to LEFT sided heart failure? Ans: YES, primarily PULMONARY.
  • Can you understand why all of these findings can be related to RIGHT sided heart failure? Ans: YES, primarily STSTEMIC.
  • Does this look like it covers all bases? Ans: YES
    You can always logically remember heart diseases as being in one of these 5 categories.
  • Do the NAMES of these congenital heart conditions adequately describe the pathology? Ans: YES
    Why have I highlighted the “D”s and the “T”s? Ans: D = LR shunt, T= RL shunt (cyanosis, or “blue” babies).
    # kinds of Congenital heart diseases
    LR shunts
    RL shunts
    Stenoses of Aorta or Pulmonary Artery
  • LEFT to RIGHT SHUNTS, NON-cyanotic
  • All the RL congenital shunts are CYANOTIC, and have T’s in their names.
    CYANOSIS means UN-oxygenated blood is BYPASSING the lungs.
  • CLASSICAL “TETROLOGY” of FALLOT:
    1) VSD, large
    2) OBSTRUCTION to RV flow
    3) Aorta OVERRIDES the VSD
    4) RVH, including subpulmonic (causing stenosis to Pulmonary Artery)
  • In TRUNCUS ARTERIOSUS, the  embryological structure known as the truncus arteriosus fails to properly divide into the pulmonary trunk and aorta
  • This is our THIRD category of congenital heart diseases after L—R and RL shunts.
    In this THIRD type, there is NO LR or RL shunting.
  • Can ATRESIA be thought of, anatomically, as being SEVERE stenosis? Ans: YES
  • Would you expect LVH in all cases? YES
  • If you would like to think of this as a spectrum, be my guest.
  • CHRONIC plaque PLUS acute thrombosis = Acute coronary syndromes.
  • Back to the inflammation saga?
  • IHD “spectrum”
    It is NOT unusual for the stenosis of stable angina to be MORE than the stenosis of an acute coronary syndrome on which there is plaque disruption!
  • What happens to myocardium when arteries are suddenly occluded, much of which is gotten from animal research.
    Note that the above changes are way earlier than gross or microscopic changes!
  • Why does the necrosis spread from the endocardium to the pericardium (i.e., epicardium)? Ans: Because the subendocardium is the LEAST well perfused by the subepicardial arteries. The “furthest away” theory, BOTH furthest away from the artery and furthest away from the base.
    The APEX of the myocardium is like the FOOT of a human being, it is the most likely to receive the brunt of ischemic and infarctive phenomena, no matter WHERE along the course of the artery the disease occurs. The apex therefore the most common site of wall motion abnormalities, logically.
  • NOTE: In ischemia, NO gross or microscopic findings are seen, visible changes are seen only with INFARCTION. You cannot see ISCHEMIA!!!
    When might myocardial rupture occur? Why?
  • Coagulative necrosis is PALE early. Or purple.
    Yellow when macrophages chew up the dead tissue.
    Sometimes red and soft again with organization or neovascularation.
    White and firm with fibrosis.
  • In “reperfusion”, not only are you oxygenating dead myocardial cells, but you are oxygenating the often destructive inflammatory process too.
  • C-reactive protein (CRP) is a protein found in the blood, made by the liver, the levels of which rise in response to inflammation (i.e. C-reactive protein is an acute phase reactant). Its physiological role is to bind to phosphocholine expressed on the surface of dead or dying cells (and some types of bacteria) in order to activate the complement system.
  • All logical, I hope?
  • Even though myopathies are regarded as separate from IHD, you can have an ischemic myocardiopathy.
  • With the exception of the last two* items, all are easily demonstrated by autopsy.
  • Why might there NOT be an easily demonstrated thrombus or myocardial changes?
  • A chamber wall is only as thick as it has to be, i.e., more pressure  more thickness.
    Please remember LVH does NOT CAUSE the hypertension, it is the adaptive REACTION to it!
    CHF causes HYPERTENSION, and HYPERTENSION causes CHF!
  • LV should be no more than 1.5 cm. LV should be no more than 1.5 cm. LV should be no more than 1.5 cm.
    LV should be no more than 1.5 cm. LV should be no more than 1.5 cm. LV should be no more than 1.5 cm.
    LV should be no more than 1.5 cm. LV should be no more than 1.5 cm. LV should be no more than 1.5 cm.
  • *Answer: owing to left atrial enlargement (i.e., dilatation, i.e. “stretching”)
  • Is this LV primarily hypertrophic, or dilated? What is an EDV?
  • Answer: lenticulostriate in basal ganglia most susceptible to hypertensive CVA
    HYPERTENSION causes CHF, CHF causes HYPERTENSION, HYPERTENSION causes CHF, CHF causes HYPERTENSION, HYPERTENSION causes CHF, CHF causes HYPERTENSION, HYPERTENSION causes CHF, CHF causes HYPERTENSION, HYPERTENSION causes CHF
  • As the alveoli EXPAND in COPD, the arterioles NARROW!
    In which type of cor pulmonale, might there be NO RVH, acute (such as massive PE or “saddle” embolism”), or chronic?
  • A reasonably logical way of looking at COR PULMONALE, or RIGHT HEART FAILURE
  • Why do BOTH stenosis and regurgitation cause hypertrophy of the chamber proximal to the valve?
  • If you have 4 valves and 2 possibilities of each valve (stenosis or regurgitation), then you have 8 possibilities, but these 2/8 cover 70% of all.
    So that mean, practically, clinically, R&gt;L and Stenosis&gt;Regurgitation
    Would a stenosis be more likely than a regurg to be chronic? Ans: YES
  • LVH is almost a reflex knee jerk conclusion to AS, but in this case there may be NO systemic hypertension.
    The LV extra work is at the aortic level, not the arteriolar level!
  • Idiopathic
  • Define:
    Stenosis?
    Regurgitation?
    Insufficiency?
    Incompetence?
    Prolapse?
  • “Myxomatous” degeneration? Describe it.
  • Granuloma:Giant Cell::Aschoff Body:Anitschkow Cell
  • A GREAT classical Sydenham chorea (St. Vitus “Dance”) can be seen at www.youtube.com/watch?v=RnxqqW_nH0k
  • The great BRIDGE between infectious and autoimmune diseases!
    Also note this is a PAN-carditis, i.e., :
    1) Endocardium
    2) Myocardiom
    3) Pericardium
  • What is a haček?
  • What is difference between “ACUTE” and “SUBACUTE” bacterial endocarditis? Ans: RATE and SEVERITY. The histology may be identical.
  • Vegetations: 1) rheumatoid = small, at chordae tendinae junction, 2) infectious = big (&gt;5 mm), 3) lupus (Libman-Saks) = BOTH sides
    4) NBTE = non-bacterial thrombotic endocarditis (&lt;5 mm)
  • Another diagram which shows “quantification” of a diagnosis
  • Splinter hemorrhages, Janeway lesions (palms, soles), Osler’s “nodes” (raised), Roth’s spots (eye).
    Do you think that for every skin lesion you see there may be 10 visceral lesions which you do NOT see? Ans: Yes, I believe so!
  • Which valves would you expect to see Swan-Ganz vegetations? Ans: RIGHT HEART
  • Most carcinoids are from the GI tract, but only 10% of carcinoids produce “carcinoid syndrome”.
    Why are carcinoids selectively nasty to the RIGHT heart intima? ANS: Lungs break down serotonin.
    NOTE WELL: The CCPP for carcinoids!
  • Note the six ETIOLOGIC classes on the LEFT,
    And 3 FUNCTIONAL classes on the RIGHT
  • A “restrictive” cardiomyopathy is a wall which is NOT thickened or dilated necessarily, but RIGID in diastolic relaxation, i.e., loss of “compliance”. EF=1/EDV
  • LVEF = (EDV-ESV)/EDV, strictly a PERCENTAGE
    LVEF usually differentiateds the HYPERTROPHIC from the DILATED cardiomyopathies.
    EF=1/EDV
  • I hope as we run down the list all these etiologic explanations seem logical?
  • DILATED may be a misleading classification, because these hearts are HYPERTROPHIC also.
  • A genetic disease
  • How can you have decreased SV, decreased diastolic filling, but HIGH LVEF?
  • When you think RESTRICTIVE, think ↓↓↓ COMPLIANCE
  • MYOCARDIOPATHY/MYOCARDITIS spectrum!
  • The “bread and butter” pericarditis is classically and most often described in uremia or pericardial infections.
    What is the exudate? Ans: Fibrin
    “Bread and butter” pericarditis = fibrinous pericarditis.
  • True neoplasm?
  • HVG
  • Minarcik robbins 2013_ch12-heart

    1. 1. HEART
    2. 2. THE HEART • Normal • Pathology – Heart Failure: L, R – Heart Disease • • • • • • • • Congenital: LR shunts, RL shunts, Obstructive Ischemic: Angina, Infarction, Chronic Ischemia, Sudden Death Hypertensive: Left sided, Right sided Valvular: AS, MVP, Rheumatic, Infective, Non-Infective, Carcinoid, Artificial Valves Cardiomyopathy: Dilated, Hypertrophic, Restrictive, Myocarditis, Other Pericardium: Effusions, Pericarditis Tumors: Primary, Effects of Other Primaries Transplants
    3. 3. NORMAL Features • • • • • 6000 L/day 250-300 grams 40% of all deaths (2x cancer) Wall thickness ~ pressure (i.e., a wall is only as thick as it has to be) – LV=1.5 cm – RV= 0.5 cm – Atria =.2 cm • Systole/Diastole • Starling’s Law
    4. 4. TERMS • CARDIO”MEGALY” – DILATATION, any chamber, or all – HYPERTROPHY, and chamber, or all
    5. 5. STRIATIONS NUCLEUS DISCS SARCOLEMMA SARC. RETIC. MITOCHONDRIA ENDOTHELIUM FIBROBLASTS GLYCOGEN A.N.P.
    6. 6. S.A. NodeAV NodeBundle of HIS L. Bundle, R. Bundle
    7. 7. AX Anterior Lateral Posterior Septal VLA HLA
    8. 8. VALVES • AV: – TRICUSPID – MITRAL 13 cm 11 cm • SEMILUNAR: – PULMONIC – AORTIC 8 cm 6 cm
    9. 9. CARDIAC AGING Chambers Epicardial Coronary Arteries Increased left atrial cavity size Tortuosity Decreased left ventricular cavity size Increased cross-sectional luminal area Sigmoid-shaped ventricular septum Calcific deposits Atherosclerotic plaque Myocardium Valves Aortic valve calcific deposits Mitral valve annular calcific deposits Fibrous thickening of leaflets Buckling of mitral leaflets toward the left atrium Increased mass Increased subepicardial fat Brown atrophy Lipofuscin deposition Basophilic degeneration (glyc.) Amyloid deposits
    10. 10. CARDIAC AGING Aorta Dilated ascending aorta with rightward shift Elongated (tortuous) thoracic aorta Sinotubular junction calcific deposits Elastic fragmentation and collagen accumulation Atherosclerotic plaque
    11. 11. BROWN ATROPHY, HEART LIPOFUCSIN
    12. 12. Pathologic Pump Possibilities • Primary myocardial failure (MYOPATHY) • Obstruction to flow (VALVE) • Regurgitant flow (VALVE) • Conduction disorders (CONDUCTION SYSTEM) • Failure to contain blood (WALL INTEGRITY)
    13. 13. • DEFINITION • TRIAD CHF – 1) TACHYCARDIA – 2) DYSPNEA – 3) EDEMA • FAILURE of Frank Starling mechanism • HUMORAL FACTORS – Catecholamines (nor-epinephrine) – ReninAngiotensionAldosterone – Atrial Natriuretic Polypeptide (ANP) • HYPERTROPHY and DILATATION
    14. 14. HYPERTROPHY • PRESSURE OVERLOAD (CONCENTRIC) • VOLUME OVERLOAD (CHF) • LVH, RVH, atrial, etc. • 2X normal weight ischemia • 3X normal weight HTN • >3X normal weightMYOPATHY, aortic regurgitation
    15. 15. CHF: Autopsy Findings • Cardiomegaly • Chamber Dilatation • Hypertrophy of myocardial fibers, BOXCAR nuclei
    16. 16. Left Sided Failure • Low output vs. congestion • Lungs – pulmonary congestion and edema – heart failure cells • Kidneys – pre-renal azotemia – salt and fluid retention • renin-aldosterone activation • natriuretic peptides • Brain: Irritability, decreased attention, stuporcoma
    17. 17. Left Heart Failure Symptoms • Dyspnea – on exertion – at rest • Orthopnea – redistribution of peripheral edema fluid – graded by number of pillows needed • Paroxysmal Nocturnal Dyspnea (PND)
    18. 18. LEFT Heart Failure Dyspnea Orthopnea PND (Paroxysmal Nocturnal Dyspnea) Blood tinged sputum Cyanosis Elevated pulmonary “WEDGE” pressure (PCWP) (nl = 2-15 mm Hg)
    19. 19. Right Sided Heart Failure • Etiology – left heart failure – cor pulmonale • Symptoms and signs – Liver and spleen • passive congestion (nutmeg liver) • congestive spleenomegaly • ascites – Kidneys – Pleura/Pericardium • pleural and pericardial effusions • transudates – Peripheral tissues
    20. 20. RIGHT Heart Failure FATIGUE “Dependent” edema JVD Hepatomegaly (congestion) ASCITES, PLEURAL EFFUSION GI Cyanosis Increased peripheral venous pressure (CVP) (nl = 2-6 mm Hg)
    21. 21. HEART DISEASE • CONGENITAL (CHD) • • • • ISCHEMIC (IHD) HYPERTENSIVE (HHD) VALVULAR (VHD) MYOPATHIC (MHD)
    22. 22. CONGENITAL HEART DEFECTS • Faulty embryogenesis (week 3-8) • Usually MONO-morphic (i.e., SINGLE lesion) (ASD, VSD, hypo-RV, hypo-LV) • May not be evident until adult life (Coarctation, ASD) • Overall incidence 1% of USA births • INCREASED simple early detection via non invasive methods, e.g., US, MRI, CT, etc.
    23. 23. Incidence per Million Live Births % 4482 42 1043 10 Pulmonary stenosis 836  8  Patent ductus arteriosus 781  7  Tetralogy of Fallot 577  5  Coarctation of aorta 492  5  Atrioventricular septal defect Aortic stenosis 396  4  388  4  Transposition of great arteries Truncus arteriosus Total anomalous pulmonary venous connection Tricuspid atresia 388  4  136  1  120  1  Malformation Ventricular septal defect Atrial septal defect
    24. 24. GENETICS • Gene abnormalities in only 10% of CHD • Trisomies 21, 13, 15, 18, XO • Mutations of genes which encode for transcription factorsTBX5ASD,VSD  NKX2.5ASD • Region of chromosome 22 important in heart development, 22q11.2 deletionconotruncus, branchial arch, face
    25. 25. ENVIRONMENT • RUBELLA • TERATOGENS
    26. 26. CHD • LR SHUNTS: all “D’s” in their names – NO cyanosis – Pulmonary hypertension – SIGNIFICANT pulmonary hypertension is IRREVERSIBLE • RL SHUNTS: all “T’s” in their names – CYANOSIS (i,.e., “blue” babies) – VENOUS EMBOLI become SYSTEMIC “paradoxical” • OBSTRUCTIONS: aorta or pulomnary artery
    27. 27. • ASD • VSD • ASVD • PDA LR NON CYANOTIC IRREVERSIBLE PULMONARY HYPERTENSION IS THE MOST FEARED CONSEQUENCE
    28. 28. ASD • NOT patent foramen ovale • Usually asymptomatic until adulthood • SECUNDUM (90%): Defective fossa ovalis • PRIMUM (5%): Next to AV valves, mitral cleft • SINUS VENOSUS (5%): Next to SVC with anomalous pulmonary veins draining to SVC or RA
    29. 29. VSD • • • • • By far, most common CHD defect Only 30% are isolated Often with TETRALOGY of FALLOT 90% involve the membranous septum If muscular septum is involved, likely to have multiple holes • SMALL ones often close spontaneously • LARGE ones progress to pulmonary hypertension
    30. 30. PDA • 90% isolated • HARSH, machinery-like murmur • LR, possibly RL as pulmonary hypertension approaches systemic pressure • Closing the defect may be life saving • Keeping it open may be life saving (Prostaglandin E1). Why? Ans: TGA, TA, TAPVC
    31. 31. AVSD • Associated with defective, inadequate AV valves • Can be partial, or COMPLETE (ALL 4 CHAMBERS FREELY COMMUNICATE)
    32. 32. RL • Tetralogy of Fallot • Transposition of great arteries • Truncus arteriosus • Total anomalous pulmonary venous connection • Tricuspid atresia
    33. 33. RL SHUNTS • TETRALOGY of FALLOT most COMMON – 1) VSD, large – 2) OBSTRUCTION to RV flow – 3) Aorta OVERRIDES the VSD – 4) RVH – SURVIVAL DEPENDS on SEVERITY of SUBPULMONIC STENOSIS – Can be a “PINK” tetrology if pulmonic obstruction is small, but the greater the obstruction, the greater is the RL shunt
    34. 34. TGA (TRANSPOSITION of GREAT ARTERIES) • NEEDS a SHUNT for survival, obviously – PDA or PFO (65%), “unstable” shunt – VSD (35%), “stable” shunt – RV>LV in thickness – Fatal in first few months – Surgical “switching”
    35. 35. TRUNCUS ARTERIOSIS
    36. 36. TRICUSPID ATRESIA • Hypoplastic RV • Needs a shunt, ASD, VSD, or PDA • High mortality
    37. 37. Total Anomalous Pulmonary Venous Connection (TAPVC) • PULMONARY VEINS do NOT go into LA, but into L. innominate v. or coronary sinus • Needs a PFO or a VSD • HYPOPLASTIC LA
    38. 38. OBSTRUCTIVE CHD • COARCTATION of aorta • Pulmonary stenosis/atresia • Aortic stenosis/atresia
    39. 39. COARCTATION of AORTA • • • • • M>F But XO’s frequently have it INFANTILE FORM (proximal to PDA) (SERIOUS) ADULT FORM (CLOSED DUCTUS, i.e., NO PDA) Bicuspid aortic valve 50% of the time
    40. 40. PULMONIC STENOSIS/ATRESIA • If 100% atretic, hypoplastic RV with ASD • Clinical severity ~ stenosis severity
    41. 41. AORTIC STENOSIS/ATRESIA • VALVULAR – If severe, hypoplastic LVfatal • SUB-valvular (subaortic) – Aortic wall THICK BELOW cusps • SUPRA-valvular – Aortic wall THICK ABOVE cusps in ascending aorta
    42. 42. HEART DISEASE • CONGENITAL (CHD) • ISCHEMIC (IHD) • HYPERTENSIVE (HHD) • VALVULAR (VHD) • MYOPATHIC (MHD)
    43. 43. SYNDROMES of IHD • • • • Angina Pectoris: Stable, Unstable Myocardial Infarction (MI, AMI) Chronic IHD CHF (CIHD) Sudden Cardiac Death (SCD) • “Acute” Coronary Syndromes: – UNSTABLE ANGINA – AMI – SCD (Sudden Cardiac Death)
    44. 44. IHD RISK • Number of plaques • Distribution of plaques • Size, structure of plaques
    45. 45. ACUTE CORONARY SYNDROMES • “The acute coronary syndromes are frequently initiated by an unpredictable and abrupt conversion of a stable atherosclerotic plaque to an unstable and potentially lifethreatening atherothrombotic lesion through superficial erosion, ulceration, fissuring, rupture, or deep hemorrhage, usually with superimposed thrombosis.”
    46. 46. EPIDEMIOLOGY (same as atherosclerosis) • ½ million die of IHD yearly in USA • 1 million in 1963. Why? – Prevention of control controllable risk factors – Earlier, better diagnostic methods – PTCA, CABG, arrythmia control • 90% of IHD patients have ATHEROSCLEROSIS (no surprise here)
    47. 47. ACUTE CORONARY SYNDROME FACTORS • ACUTE PLAQUE CHANGE ******* • Inflammation • Thrombus • Vasoconstriction ******* MOST IMPORTANT
    48. 48. ACUTE PLAQUE CHANGE • Rupture/Refissuring • Erosion/Ulceration, exposing ECM • Acute Hemorrhage NB: Plaques do NOT have to be severely stenotic to cause acute changes, i.e., 50% of AMI results from thromboses of plaques showing LESS THAN 50% stenosis
    49. 49. INFLAMMATION • Endothelial cells release CAMs, selectins • T-cells release TNF, IL-6, IFN-gamma to stimulate and activate endothelial cells and macrophages • CRP (α-2 globulin) predicts the probability of damage in angina patients
    50. 50. THROMBUS • Total occlusion • Partial • Embolization
    51. 51. VASOCONSTRICTION • Circulating adrenergic agonists, i.e., α • Platelet release products, e.g., ADP • Endothelially released factors, such as endothelin
    52. 52. Coronary Artery Pathology in Ischemic Heart Disease Syndrome Plaque Stenoses Disruption Stable angina >75% No No Unstable angina Variable Frequent Nonocclusive, often with thromboemboli Transmural myocardial infarction Variable Frequent Occlusive Subendocardial myocardial infarction Variable Variable Widely variable, may be absent, partial/complete, or lysed Sudden death Usually severe Frequent Often small platelet aggregates or thrombi and/or thromboemboli Plaque-Associated Thrombus
    53. 53. ANGINA PECTORIS • • • • • Paroxysmal (sudden) Recurrent 15 sec.15 min. Reduced perfusion, but NO infarction THREE TYPES – STABLE: relieved by rest or nitro – PRINZMETAL: SPASM is main feature, responds to nitro, S-T elevation. Often younger with people not much atherosclerotic narrowing. – UNSTABLE (crescendo, PRE-infarction, Q-wave angina): perhaps some thrombosis, perhaps some non transmural necrosis, perhaps some embolization, but DISRUPTION of PLAQUE is universally agreed upon
    54. 54. MYOCARDIAL INFARCTION • Transmural vs. Subendocardial (inner 1/3) • DUH! EXACT SAME risk factors as atherosclerosis • Most are TRANSMURAL, and MOST are caused by coronary artery occlusion • In the 10% of transmural MIs NOT associated with atherosclerosis: – Vasospasm – Emboli, e.g., mural thrombus – UNexplained
    55. 55. MYOCARDIAL RESPONSE Feature Time Onset of ATP depletion Seconds Loss of contractility <2 min ATP reduced to 50% of normal   10 min to 10% of normal   40 min Irreversible cell injury 20–40 min Microvascular injury >1 hr
    56. 56. PROGRESSION OF NECROSIS
    57. 57. TIMING of Gross and Microscopic Findings GROSS MICROSCOPIC ½–4 hr None Usually none; variable waviness of fibers at border 4–12 hr Occasionally dark mottling Beginning coagulation necrosis; edema; hemorrhage   12–24 hr Dark mottling Ongoing coagulation necrosis; pyknosis of nuclei; myocyte hypereosinophilia; marginal contraction band necrosis; beginning neutrophilic infiltrate   1–3 days Mottling with yellow-tan infarct center Coagulation necrosis, with loss of nuclei and striations; interstitial infiltrate of neutrophils   3–7 days Hyperemic border; central yellow-tan softening Beginning disintegration of dead myofibers, with dying neutrophils; early phagocytosis of dead cells by macrophages at infarct border   7–10 days Maximally yellow-tan and soft, with depressed redtan margins Well-developed phagocytosis of dead cells; early formation of fibrovascular granulation tissue at margins   10–14 days Red-gray depressed infarct borders Well-established granulation tissue with new blood vessels and collagen deposition   2–8 wk Gray-white scar, progressive from border toward core of infarct Increased collagen deposition, with decreased cellularity   >2 mo Scarring complete Dense collagenous scar
    58. 58. 1 day (pyknosis, “waviness”) 3-4 days (neutrophils) 7 days (macrophages) Weeks (organization) Months (fibrosis)
    59. 59. RE-PERFUSION • Thrombolysis • PTCA • CABG • Reperfusion CANNOT restore necrotic or dead fibers, only reversibly injured ones, and prevent further necrosis. • REPERFUSION “INJURY” – Free radicals – Interleukins
    60. 60. AMI DIAGNOSIS • SYMPTOMS • EKG 1) Q-waves, 2) T-wave inversion, 3) ST-T elevation • DIAPHORESIS • (10% of MIs are “SILENT” with Qwaves) • CKMB gold standard enzyme • Troponin-I, Troponin-T better • CRP predicts risk of AMI in angina patients
    61. 61. COMPLICATIONS • • • • • • • • • Wall motion abnormalities Arrhythmias Rupture (4-5 days) Pericarditis RV infarction Infarct extension Mural thrombus Ventricular aneurysm Papillary muscle dysfunction (regurgitation) • CHF
    62. 62. CIHD, aka, ischemic “cardiomyopathy” • Progress to CHF often with no pathologic or clinical evidence of localized infarction – Extensive atherosclerosis – No infarct – Hypertrophy & Dilatation present
    63. 63. SUDDEN CARDIAC DEATH • 350,000 in USA yearly from atherosclerosis • NON-atherosclerotic sudden cardiac death includes: – Congenital coronary artery disease – Aortic stenosis – MVP, i.e., mitral valve prolapse – Myocarditis – Cardiomyopathy (sudden death in young athletes) – Pulmonary hypertension – *Conduction defects – *HTN, hypertrophy of UNKNOWN etiology
    64. 64. AUTOPSY findings in SCD • >75% narrowing of 1-3 vessels • Healed infarcts 40% • “ARRHYTHMIA” is often a very convenient conclusion when no anatomic findings are present, i.e., “wastebasket” diagnosis
    65. 65. HEART DISEASE • CONGENITAL (CHD) • ISCHEMIC (IHD) • HYPERTENSIVE (HHD) • VALVULAR (VHD) • MYOPATHIC (MHD)
    66. 66. HHD (Left) • DEFINITION: Hypertrophic adaptive response of the heart, which can progress: – Myocardial dysfunction – Cardiac dilatation – CHF – Sudden death
    67. 67. NEEDED for DIAGNOSIS: • LVH (LV>2.0 and/or Heart>500 gm.) • HTN (>140/90)
    68. 68. PREVALENCE: • WHAT % of USA people have hypertension?
    69. 69. PREVALENCE: • WHAT % of USA people have hypertension? • Answer: 25%
    70. 70. HISTOPATHOLOGY • INCREASED FIBER (MYOCYTE) THICKNESS • INCREASED nuclear size with increased “blockiness” (boxcar nucleus)
    71. 71. CLINICAL • EKG in LVH Summary of LVH Criteria 1) R-I + S-III >25 mm 2) S-V1 + R-V5 >35 mm 3) ST-T depr. in L lead 4) R-wave in L lead >11 mm 5) LAE + other criteria Positive Criteria: 1=possible 2=probable 3=definite ATRIAL FIBRILLATION Why?* CHF, cardiac dilatation, pulmonary venous congestion and dilatation
    72. 72. COURSE: • NORMAL longevity, death from other causes • Progressive IHD • Progressive renal damage, hemorrhagic CVA (Which arteries?) • CHF
    73. 73. HHD (Right) = COR PULMONALE • ACUTE: Massive PE (No RVH) • CHRONIC: COPD, CRPD, Pulmonary artery disease, chest wall motion impairment (RVH)
    74. 74. Diseases of the Pulmonary Parenchyma Chronic obstructive pulmonary disease Disorders Affecting Chest Movement Kyphoscoliosis Marked obesity (pickwickian syndrome) Neuromuscular diseases Diffuse pulmonary interstitial fibrosis Pneumoconioses Cystic fibrosis Bronchiectasis Disorders Inducing Pulmonary Arterial Diseases of the Pulmonary Vessels Constriction Metabolic acidosis Recurrent pulmonary thromboembolism Hypoxemia Primary pulmonary hypertension Chronic altitude sickness Extensive pulmonary arteritis (e.g., Wegener  Obstruction to major airways Idiopathic alveolar hypoventilation granulomatosis) Drug-, toxin-, or radiation-induced vascular  obstruction Extensive pulmonary tumor microembolism
    75. 75. HEART DISEASE • CONGENITAL (CHD) • ISCHEMIC (IHD) • HYPERTENSIVE (HHD) • VALVULAR (VHD) • MYOPATHIC (MHD)
    76. 76. V HD alvular • Opening problems: • Stenosis Closing problems: Regurgitation or Incompetence or “insufficiency” (as opposed to coronary “insufficiency”)
    77. 77. • AS 70% of all VHD – Calcification of a deformed valve – “Senile” calcific AS – Rheum, Heart Dis. • MS –Rheumatic Heart Disease
    78. 78. AORTIC STENOSIS 2X gradient pressure LVH (but no hypertension), ischemia Cardiac decompensation, angina, CHF 50% die in 5 years if angina present 50% die in 2 years if CHF present
    79. 79. MITRAL ANNULAR CALCIFICATION • Calcification of the mitral “skeleton” • Usually NO dysfunction • Regurgitation usually, but Stenosis possible • F>>M
    80. 80. REGURGITATIONS • AR – Rheumatic – Infectious – Aortic dilatations • Syphilis • Rheumatoid Arthritis • Marfan • MR –MVP – – – – Infectious Fen-Phen Papillary muscles, chordae tendinae Calcification of mitral ring (annulus)
    81. 81. Mitral Valve Prolapse (MVP) • MYXOMATOUS degeneration of the mitral valve • Associated with connective tissue disorders • “Floppy” valve • 3% incidence, F>>M • Easily seen on echocardiogram
    82. 82. MVP: CLINICAL FEATURES • • • • • • Usually asymptomatic Mid-systolic “click” Holosystolic murmur if regurg. present Occasional chest pain, dyspnea 97% NO untoward effects 3% Infective endocarditis, mitral insufficiency, arrythmias, sudden death
    83. 83. RHEUMATIC Heart Disease • Follows a group A strep infection, a few weeks later • DECREASE in “developed” countries • PANCARDITIS: 1) Endocarditis, 2) Myocarditis, 3) Pericarditis
    84. 84. ACUTE: -Inflammation -Aschoff bodies -Anitschkow cells -Pancarditis -Vegetations on chordae tendinae at leaflet junction CHRONIC: THICKENED VALVES COMMISURAL FUSION THICK, SHORT, CHORDAE TENDINAE
    85. 85. CLINICAL FEATURES • • • • • Migratory Polyarthritis Myocarditis Subcutaneous nodules Erythema marginatum Sydenham chorea
    86. 86. INFECTIOUS ENDOCARDITIS • Microbes – Usually strep viridans – Often Staph aureus in IVD users – Enterococci – HAČEK (normal oral flora, gram - , in children) • • • • • Hemophilus influenzae Actinobacillus Cardiobacterium Eikenella Kingella – Fungi, rickettsiae, chlamydia
    87. 87. INFECTIOUS ENDOCARDITIS • Acute: 50% mortality (course=days) • SUB-acute: LOW mortality (course=weeks)
    88. 88. VEGETATIONS • INFECTIVE >5mm • NON-Infective <5mm
    89. 89. DIAGNOSIS=MMm, Mmmm, mmmmm • MAJOR • • • • • • • • • • Positive blood culture(s) indicating characteristic organism or persistence of unusual organism Echocardiographic findings, including valve-related or implant-related mass or abscess, or partial separation of artificial valve New valvular regurgitation minor Predisposing heart lesion or intravenous drug use Fever Vascular lesions, including arterial petechiae, subungual/splinter hemorrhages, emboli, septic infarcts, mycotic aneurysm, intracranial hemorrhage, Janeway lesions Immunologic phenomena, including glomerulonephritis, Osler nodes, Roth spots, rheumatoid factor Microbiologic evidence, including single culture showing uncharacteristic organism Echocardiographic findings consistent with but not diagnostic of endocarditis, including new valvular regurgitation, pericarditis
    90. 90. NON-infective VEGETATIONS • <5 mm • PE • Trousseau syndrome (migratory thrombophlebitis with malignancies) • s/p Swan-Ganz • Libman-Saks with SLE (both sides of valve)
    91. 91. • • • • Carcinoid Syndrome Episodic skin flushing Cramps Nausea & Vomiting Diarrhea • ↑serotonin, ↑ 5HIAA in urine • FIBROUS INTIMAL THICKENING – RV, Tricuspid valve, Pulmonic valve (all RIGHT side) – Similar to what Fen-Phen does on the LEFT side
    92. 92. ARTIFICIAL VALVES • Mechanical • Xenografts (porcine) • 60% have complications within 10 years
    93. 93. HEART DISEASE • CONGENITAL (CHD) • ISCHEMIC (IHD) • HYPERTENSIVE (HHD) • VALVULAR (VHD) • MYOPATHIC (MHD) • PERICARDIAL DISEASE
    94. 94. CARDIOMYOPATHIES • • • • • • • Inflammatory Immunologic Metabolic Dystrophies Genetic Idiopathic Toxic • DILATED (DCM) – SY-stolic dysfunction • HYPERTROPHIC (HCM) – DIA-stolic dysfunction • RESTRICTIVE (RCM) – DIA-stolic dysfunction
    95. 95. Functional Pattern Dilated LVEF <40% Mechanisms of Heart Failure Impairment of contractility (systolic dysfunction) Hypertrop hic 50– Impairment of 80% compliance (diastolic dysfunction) Restrictive 45– 90% Impairment of compliance (diastolic dysfunction) Causes Indirect Myocardial Dysfunction (Not Cardiomyopathy) Idiopathic; alcohol; peripartum; genetic; myocarditis; hemochromatosis; chronic anemia; doxorubicin (Adriamycin); sarcoidosis Ischemic heart disease; valvular heart disease; hypertensive heart disease; congenital heart disease Genetic; Friedreich ataxia; storage diseases; infants of diabetic mothers Hypertensive heart disease; aortic stenosis Idiopathic; amyloidosis; radiation-induced fibrosis Pericardial constriction
    96. 96. Cardiac Infections Toxins Metabolic Viruses: Cox-B, esp. Alcohol Hyperthroidism Chlamydia Cobalt Hypothyroidism Rickettsia Catecholamines (stress) Hyperkalemia Bacteria Carbon monoxide Hypokalemia Fungi Lithium Protozoa Hydrocarbons Nutritional deficiency (protein, thiamine, other avitaminoses) Arsenic Neuromuscular Disease Friedreich ataxia (frataxin) Muscular dystrophy Congenital atrophies Immunologic Myocarditis (several forms) Post-transplant rejection Hemochromatosis Cyclophosphamide Infiltrative Doxorubicin (Adriamycin) and daunorubicin Leukemia Carcinomatosis Storage Disorders and Sarcoidosis Other Depositions Radiation-induced Hunter-Hurler syndrome (Dermatan-Heparan) Glycogen storage disease Fabry disease (glyco-lipid) Amyloidosis fibrosis
    97. 97. DILATED cardiomyopathy • • • • • • Chamber thickness (not just LVH) Adults Progressively declining LVEF LVEF ~ prognosis 50% die in 2 years 3 Main causes – Myocarditis – ETOH – Adriamycin
    98. 98. DCM Path: 4 chamber dilatation Hypertrophy, also 4 chambers Interstitial Fibrosis
    99. 99. Arrhythmogenic Right Ventricular Cardiomyopathy (Arrhythmogenic Right Ventricular Dysplasia) This is an uncommon dilated cardiomyopathy predominantly RIGHT ventricle. So is NAXOS syndrome. Wooly Hair Palmoplantar keratoderma
    100. 100. HYPERTROPHIC cardiomyopathy • Also called IHSS, (Idiopathic Hypertrophic Subaortic Stenosis) – GENETIC defects involving: • Beta-myosin heavy chain • Troponin T • Alpha-tropomyosin • Myosin binding protein C – PATHOLOGY: Massive hypertrophy, Asymmetric septum, DISARRAY of myocytes, INTERSTITIAL fibrosis – CLINICAL: filling ↓chamber volume, ↓SV, ↓ diastolic
    101. 101. RESTRICTIVE cardiomyopathy • • • • (idiopathic) ↓ ventricular compliance Chiefly affects DIASTOLE NORMAL chamber size and wall thickness • THREE similar diseases affecting predominantly the SUBENDOCARDIAL area: – Endomyocardial Fibrosis (African children) – Loeffler Endomyocarditis (eosinophilic leukemia) – Endocardial Fibroelastosis (infants)
    102. 102. MYOCARDITIS • INFLAMMATION of MYOCARDIUM • Chiefly microbial –COXACKIE A & B, CMV, HIV – – – – – Trypanosoma cruzi (Chagas dis.), 80% Trichinosis Toxoplasmosis Lyme disease (5%) Diphtheria • IMMUNE: Post-viral, rheumatic, SLE, drug hypersensitivityalpha-methyl dopa, sulfas
    103. 103. LYMPHOCYTIC INFILTRATES are the USUAL pattern of ALL myocarditis, but eosinophils, giant cells, and even trypanosomes can be seen occasionally
    104. 104. OTHER Myocarditides • • • • Adriamycin Cyclophosphamide Catecholamines (Pheochromocytomas) Amyloid, systemic or primary cardiac – Congo red stain: green birefringence with polarization • Amyloid, aging – Congo red stain: green birefringence with polarization • Hemochromatosis (Prussian Blue) • BOTH HYPER-, HYPO- -thyroidism
    105. 105. PERICARDIUM • Normally 30-50 ml clear serous fluid – Visceral (epicardium) – Parietal (fibrous pericardium) – PERICARDIAL EFFUSIONS TAMPONADE • Ruptured MI • Traumatic perforation • Ruptured aortic dissection
    106. 106. PERICARDITIS • SEROUS: Rheum. Fever (RF), SLE, scleroderma, tumors, uremia • FIBRINOUS: MI (Dressler), uremia, radiation, RF, SLE, s/p open heart surgery • • • • PURULENT: infective, bacterial HEMORRHAGIC: Malignancy, TB CASEOUS: TB CHRONIC: (ADHESIVE, CONSTRICTIVE)
    107. 107. TUMORS • 90% benign “mesenchymal”, i.e., stromal – MYXOMAS (LEFT ATRIUM MOST COMMON) – – – – FIBROMAS LIPOMAS FIBROELASTOMAS (valvular, usually papillary) RHABDOMYOMA (Most common cardiac tumor in children) • 10% SARCOMAS
    108. 108. MYXOMA
    109. 109. Cardiac effects of NON-cardiac tumors • Direct Consequences of Tumor – Pericardial and myocardial metastases – Large vessel obstruction – Pulmonary tumor emboli • Indirect Consequences of Tumor (Complications of Circulating Mediators) – – – – Nonbacterial thrombotic endocarditis (Trousseau) (NBTE) Carcinoid heart disease Pheochromocytoma-associated heart disease Myeloma-associated amyloidosis • Effects of Tumor Therapy – Chemotherapy – Radiation therapy
    110. 110. CARDIAC TRANSPLANT PATHOLOGY • Most patients are on immunosuppressives • 5 year survival >60%
    111. 111. CARDIAC TRANSPLANT PATHOLOGY

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