Disorders of Cardiac Function1

DISORDERS OF THE PERICARDIUM

Disease
Definition
Pericardial Effusion
Accumulation of fluid in pericardial
cavity

Characteristics
Major threat is compression of
the heart chambers

Pathogenesis
-Small p. effusions may be
asymptomatic
-Large, slow p. effusions may
have no sx if pericardium can
stretch and avoid heart
compression
-Sudden accumulation
increases intracardiac pressure
(may limit venous return to
heart)

Manifestations
Cardiac Tamponade
: slow or rapid heart
compression
d/t fluid, pus, blood
accumulation in pericardial
sac

- capillary permeability
resulting in fibrinous exudates
in pericardial space (heal/scar
tissue deposition, adhesion
between serous pericardium
layers)

Viral Infection (most common)

Rheumatic fever,
postpericardiotomy syndrome,
post traumatic pericarditis,
metabolic disorders assocd
with connective tissue disease

TRIAD:
Chest Pain
Pericardial friction rub
ECG changes

Chronic Pericarditis
with Effusion

Increase in inflamry exudates

beyond the acute period

Associated with rheumatic

fever, congenital heart lesions,
hypertensive heart disease
Caused by systemic diseases
and metabolic disturbances

Accumulated fluid may

compress adjacent cardiac
structures impair cardiac
filling

Constrictive
Pericarditis

Fibrous scar tissue between

layers or serous pericardium
which contracts and interferes
with diastolic filling

PERICARDITIS
Acute Pericarditis

Signs and Symptoms

Seriousness of C. Tamponade
: intercardiac pressure,
ventricular diastolic filling,
CO, SV
Severity of C.T
- rapid accumn results in
central venous pressure,
JVD, venous return to heart,
CO despite HR, Systolic
BP, circry shock

Pulsus Paradoxus
: 10 mm Hg fall in systolic BP
during normal breathing
: LV is compressed from within
(interventricular septum) and
from without (pericardial fluid)
decrease in LV filling , SV
output, S BP

Inflammation of the pericardium

CORONARY HEART DISEASE

Disease
Definition
CORONARY HEART
Heart disease d/t impaired blood flow
DISEASE

Characteristics

Fixed CO and Cardiac

Reserve

Pathogenesis
-Atherosclerosis (m. common)
-Atheroslerotic Lesions
1. Stable Plaque obstructs
blood flow;
2. Unstable Plaque ruptures;
cause platelet adhesion &
thrombus formn
-Determinants of Plaque
Rupture
1. Size of Lipid Rich core
2. Inflamn with plaque rupture
3. Lack of smooth muscles;
impaired healing; plaque
stabilization

Manifestations
Types of Thrombi
1. White, platelet containing unstable angina
2. Red, fibrin containing
- dev from vessel occlusion in
MI

Abrupt, sharp Pain in

precordial area
: worse with breathing,
cough, swallow, position
changes d/t changes in venous
return and cardiac filling
: outer layer of parietal
pericardium below 5th & 6th ICS
inflamn of surr pleura

Ascites
Pedal edema, dyspnea on
exertion, fatigue
JVD
Signs and Symptoms

Kussmaul sign
: inspiratory JVD d/t inability
of RA to accommodate
increase in venous return

Disorders of Cardiac Function2

CHRONIC ISCHEMIC
HEART DISEASE
Stable Angina

Syndromes resulting from myocardial

ischemia (imbalance between blood
supply and demand for oxygenated
blood)
*Angina pectoris symptomatic
paroxysmal chest pain/pressure
sensation assocd with transient
myocardial ischemia

Silent Myocardial
Ischemia

Occurs in the absence of pain

Variant/Vasospastic
Angina

- Prinzmetal angina
- Caused by spasms of the coronary
arteries

ACUTE CORONARY
SYNDROMES

Determinants of ACS
1. ECG changes
T wave: altered Myocardial repolarn
ST segment ischemia Myo
injury

Unstable Angina

Clinical syndrome of myocardial

ischemia ranging between stable
angina and MI

Acute Myocardial
Infarction

Heart Attack
Ischemic death of myocardial tissue

-Fixed coronary obstruction

produces disparity in coronary
blood flow and metabolic
demands of myocardium
- Precipitated by situations that
increase work demands on the
heart
- Provoked by exertion or
emotional stress
Affects asymptomatic people
with CHD; had MI before; also
with angina
Occurs during rest of minimal
exercise; nocturnal (12 am 8
am)

2. Serum Markers
Creatine kinase intracellular
enz in muscle cells; above
normal 4-8 hrs after Myo
injury; declines within 2-3 days
Primary progression of
variant or stable angina
Secondary d/t non-CH
condition
Area of infarction is det by
affected coronary artery and
blood flow distribution

Pain as constricting,
squeezing, suffocating;
precordial or substernal;
radiate to left shoulder, jaw,
arm

Less pain d/t shorter

episodes; less myocardial
tissue involved; defects in
pain threshold; sensory
denervation

Result from hyperactive

sympathetic NS; defect in
handling Calcium in vascular
smooth muscle; disturbed
release/prod of endotheliumderived relaxing factors;
imbalance with contracting
factors

Arrhythmias with severe pain

Myoglobin-02 carrying ptn;

elevated within 1 hr after myo
cell death; peak 4-8 hrs

Troponin Complex part of

actin filament; regulates
Calcium mediated actinmyosin contractile process
Pain occurs at rest/min
exertion; severe/frank pain of
new onset; pattern is severe,
prolonged, frequent

Results from atherosclerotic

plaque disruption; platelet
aggregation; 2 hemostasis
Loss of function in ischemic
area (minutes)
Necrosis (20-40 min)
Pathologic changes:
1. Transmural Infarct/ ST
segment elevn AMI
- full thickness of ventricular
wall; obstrn of single artery
2. Subsendocardial Infarct/
non-ST segment elevation
AMI
- inner 1/3-1/2 of vent wall;

Pain-someone sitting on
chest
; substernal; radiates to left
arm, neck, jaw; prolonged
Reperfusion reestablish
blood flow thru thrombolytic
therapy
Stunned myocardium
recovering area

Epigastric distress, n/v

- r/t severe pain & vagal stim

- mistaken for indigestion

Tachycardia, anxiety,
restlessness, impending
doom
Moist, pale, cool skin
Hypotension and shock

Disorders of Cardiac Function3

severely narrowed but patent
arteries
MYOCARDIAL DISEASES
Disease
MYOCARDITIS

CARDIOMYOPATHY

Definition
Inflammation of the heart
muscle and conduction
system w/o evidence of MI

Dilated
Cardiomyopathy

Group of disorders that affect

heart muscle
Progressive cardiac
hypertrophy and dilatation
and impaired pumping ability
of one/both ventricles

Hypertrophic
Cardiomyopathy

Excessive ventricular
hypertrophy

Idiopathic hypertrophic
subaortic stenosis

Asymmetric septal
hypertrophy

Symptomatic hypertrophic
cardiomyopathy
Restrictive Cardiomyopathy

Peripartum Cardiomayopathy

Restricted ventricular filling

d/t excessive rigidity of
ventricular walls, although
contractile elements remain
normal
LV dysfunction developing in
the last month before
delivery to 5 mos postpartum

Characteristics
Frequent in people with AIDS

Pathogenesis
Necrosis
d/t direct invasion of toxins by
systemic pathogen or
destruction of cardiac tissue by
immc mechanisms by
infectious agent molecular
mimicry provides continuous
stimulus

Manifestations
Vary from absence of sx to
profound heart failure or
sudden death

Signs and Symptoms

Acute Symptomatic
Myocarditis
-flu like: malaise, low grade
fever, tachycardia
- hx of upper respi tract or GI
infection

Ventricles are more dilated

than atria
Wall thinning with dilatation,
even if hypertrophy occurs
Normal cardiac valves

Inherited as autosomal
dominant/recessive, or X linked
pattern
High alcohol consumption

Profound LV ejection fraction

(SV:EDV is 40%)
Worsening of HF, mural
thrombi devt ventricular
arrhythmias
Mural thrombi source of
thromboemboli

Impaired ventricular filling

Increased wall stiffness

Gradual elevation of LV
diastolic pressure

Dyspnea on exertion,
paroxysmal nocturnal
dyspnea, orthopnea
Weakness, fatigue, ascites,
peripheral edema
Enlarged apical beat with S3
or S4 --- regurgitation of AV
valves
Atrial fibrillation cause of
death in those with
arrhythmias
Dyspnea

Obstructed LV outflow

Ventricular pressure (eject

blood into aorta) limits CO

Chest pain, fatigue, syncope

Myofibril disarray branch

off at right angles to an
adjacent fiber with which they
connect
abn ventricular movt :
uncoordinated contraction and
impaired relaxation
Disproportionate involvement
of ventricular septum
intermittent ventricular
outflow obstruction
Most common cause of death
among young people

Risk Factors:
- advanced maternal age
- African-American race
- multifetal pregnancy
- preeclampsia

Pulsus alternans pulse

regularly alternates between
weaker and stronger volume

Familial origin; Mutation in 1/10

genes encoding ptn in cardiac
sarcomeres

Causes:
- myocarditis, abn imm R to
pregnancy/ its hemodynamic
stresses, prolonged inhibition
of contractions in premature

Dyspnea on exertion,
paroxysmal nocturnal
dyspnea, Orthopnea,
peripheral edema, ascites,
fatigue, weakness
Dyspnea on exertion,
paroxysmal nocturnal
dyspnea, Orthopnea,
Weakness, fatigue, ascites,
peripheral edema

Advanced: All signs of HF

except cardiomegaly
Pulsus alternans
(s/sx
- same as dilated
myocardiomyopathy; are

Disorders of Cardiac Function4

ENDOCARDIAL DISEASES
Disease
Infective Endocarditis
Bacterial Endocarditis

Definition
Colonization/invasion of heart
valves and the mural
aendocardium by a microbial
agent

- gestational hpn

labor

Characteristics
bulky, friable vegetations
and destruction of cardiac
tissues

Pathogenesis
Contributing Factors:
1. damaged endorcardial
surface
2. portal of entry of organism
Env conducive to bacterial
growth valvular disease,
prosthetic heart valves,
congenital heart defects
IV drug abuse; Infected
intravascular or urinary tract
catheters

Vegetative Lesions
collection of infectious
organisms, cellular debris
enmeshed in fibrin strands of
clotted blood
bacteremia that initiate
immune responses

Vegetative Lesions cause

valve regurgitation, abscesses
of valve ring with heart block,
valve perforation
- its loose organization
permits organisms and
fragments to form emboli
going thru the bloodstream

Acute
Subacute
Rheumatic Heart Disease

Manifestations

Tricuspid lesions

Acute, immune mediated,

multisystem inflammatory
disease following group A (
hemolytic) streptococcal
(GAS) throat infection

Devt of chronic valvular

disorders produce
permanent cardiac
dysfunction; fatal heart failure

Risk of recurrence is high

after each subsequent
exposure
Untreated GAS phayngitis
rheumatic fever

common in pregnancy, thus

may be ignored)

Signs and Symptoms

Fever
Signs of Systemic Infection
Change in Heart murmur
Embolic distribution of veg
lesions
Small petecchial hemorrhages
emboli in skin, nail beds, and
mucus mem
Splinter hemorrhages (dark red
lines)
Systemic Emboli cough
dyspnea, arthalgia/arthritis,
diarrhea, abdominal/flank pain

Peripheral embolization
metastatic infections and
abscess formation (brains and
kidneys)
Right sided endocarditis
(tricuspid valve) septic
emboli to lung infarction
and lung abscesses

Staphylococcus auerus
Streptococcus viridans

Rheumatic fever 5 - 15 y/o

VALVULAR HEART DISEASE

Enlarged apical beat with S3

or S4 --- regurgitation of AV
valves

Rheumatic Fever
1. Acute stage strep
infection, conn tissue involvt
of heart, blood vessels, joints,
subQ tissues
- Aschoff body: localized
area of tissue necrosis surrd
by imm cells
2. Recurrent Phase
extended cardiac effects
3. Chronic Phase
permanent deformity of heart
valves, may lead to mitral
valve stenosis
- appears 10 yrs after initial
attack

Spiking fever, chills

Low grade fever, gradual onset
Anorexia, malaise, lethargy
Rheumatic fever strep
infection
: sore throat, headache, fever,
abdl pain, N/V, swollen glands
at angle of jaw
Acute Rheumatic Carditis
- peri/myocarditis are self
limited, acute stage
- Endocardium
- valves (mitral and aortic)
Veg lesions on
leafletsfibrous scar tissue
contracts shortened
chordiae tendinae
- heart murmur, cardiomegaly,
friction rub, congestive HF

Polyarthritis of large joints,

migratory
Erythema marginatum
lesions maplike, macular
areas seen on trunk and inner
aspects of upper arm and
thigh
Subcutaneous Nodules
hard, painless, freely
movable, overlie extensor
muscles of wrist, elbow, ankle,
knee joints
Chorea Sydenham
chorea; St. Vitus dance
- spontaneous, rapid,
purposeless jerky movements

Disorders of Cardiac Function5

Hemodynamic Derangements
Mechanical Disruptions that occur with Valvular HD:
1. Narrowing of valve opening so it does not open properly
2. Distortion so it does not close properly
Stenosis narrowing of the valve orifice; failure of leaflets to open normally increases resistance to blood flow thru the valve, converting normally smooth laminar flow to a less efficient turbulent flow
increases volume and work of chamber emptying thru narrowed valve
Incompetent or Regurgitant Valve permits backward flow to occur when valve should be closed
Backflow to LV during diastole when aortic valve is affected; back to LA during systole when mitral valve is affected
Heart Murmurs result from turbulent blood flow thru diseased valve
MITRAL VALVE DISORDERS
Mitral Valve - controls flow bet LA and LV - subjected to high pressure from LV; chordiae tendinae prevent eversion of valve leaflets into LA
Disease
Definition
Characteristics
Pathogenesis
Manifestations
Mitral Valve Stenosis
Incomplete opening caused by Occurs as a result of
Resistance to flow valve
CO d/t impaired ventricular
fibrous replacement of valvular rheumatic fever
LA becomes dilated,
filling
tissue along with stiffness and
LA pressure
LA enlargement, atrial
fusion of valvular apparatus
Pulmonary congestion occurs
arrhythmias, mural thrombi
when LA pressure is
transmitted to pulmonary
venous system
Mitral valve regurgitation

Incomplete closure of the

mitral valve
LV Stroke volume
Forward SV thru aorta
Regurgitant SV- back to LA
in systole

RHD is assocd with rigid,

thickened valve does not
open/close completely

Result from rupture of chordiae

tendinae or papillary muscles,
papillary muscle dysfunction,
stretched valve structures d/t
dilated LV or valve orifice

Asymptomatic when increased

vol work is tolerated

Increase in LV-EDV Total

SV, with restored forward flow
into aorta

Characteristic Features:

Unloading of LV into LA:

preload and N/ afterload

Signs and Symptoms

Pulmonary congestion
Nocturnal paroxysmal
dyspnea, Orthopnea
Palpitations, chest pains,
weakness, fatigue
Premature atrial heart beats,
paroxysmal atrial tachycardia,
atrial fibrillation d/t LA
enlargement

Mural thrombi d/t fibrillation

and distention
Arterial emboli leads to stroke
Murmur during diastole low
pitch, rumbling, heard at apex

Predisposes to atrial valve

regurgitation

- enlarged LV, hyperdynamic

LV impulse, pansystolic heart
murmur

Accommodation of regurgitant
volume at lower filling
pressure:
Gradual in LA size

Mitral Valve Prolapse

Floppy mitral valve syndrome

Myxedematous (mucinous)
degeneration of mitral valve
leaflets enlarged and floppy
leaflets prolapsed and
ballon back into LA during
systole

Impaired LV func,
forward/aortic SV, LA
pressure
Arise from disorders of
myocardium that causes abn
movement of ventricular wall
or papillary muscle pressure
on mitral valve

Traction of prolapsing valve

leaflets
Abn ANS function

Pain d/t ischemia; prolonged,

ill defined, not with exercise
or exertion
Anxiety, palpitations,
arrhythmias

Auscultation varies: silent,

mid systolic clicks, late
systolic murmur

Disorders of Cardiac Function6

AORTIC VALVE DISORDERS
Aortic Valve has no chordae tendineae; thicker cusps than mitral valve
Orifices of main coronary arteries located behind the valve, at right angles to direction of blood flow
Disease
Definition
Characteristics
Aortic Valve Stenosis
Increased resistance to
Most common causes are
ejection of blood from LV into
rheumatic fever and congenital
aorta
valve malformations

Aortic Valve Regurgitation

Incompetent aortic valve

allows backflow to LV during
diastole

Caused by Rheumatic fever

and prosthetic valve failure

Pathogenesis
resistance, work demands
on LV, vol of blood ejected
into systemic circulation

Manifestations

Aortic Valve opening

Unicuspid, bicuspid,
misshaped valve leaflets
SV, SBP, pulse pressure

Failure of valve closure

LV increases SV to include
blood from lungs and from
regurgitant valve
Chronic AV Regurgn LV vol
and pressure overload

LV vol,diastolic BP, LV size

diastolic BP, coronary blood

flow
SV for adeq forward CO;
Widened arterial blood pulse
pressure
Turbulent flow across aortic
valve

Signs and Symptoms

Soft, absent, paradoxically

split S2 sound
Harsh systolic ejection
murmur; left sternal border
Soft systolic aortic heart
murmur
LV failure s/sx:
- exertional dyspnea,
Orthopnea, paroxysmal
nocturnal dyspnea
Angina. Palpitations when
lying down
Carotid pulsations, throbbing
peripheral pulses, LV impulse
causing chest to move with
each beat
High pitched/blowing sound

HEART DISEASE IN INFANTS AND CHILDREN

Fetal Circulation
- Placenta
- Constricted pulmonary vessels fluid filled lungs; heightened hypoxic stimulus for vasoconstriction
- Umbilical Vein thru which blood enters the circulation
- Umbilical Arteries blood returns to placenta
- Ductus Venosum- allows blood from umb vein to bypass hepatic circn into inferior vena cava, RA, foramen ovale, LA, ascending aorta, head and upper extremities
-Venous blood to Right side, superior vena cava, RV, pulmonary artery, ductus Arteriosus , descending aorta
First breath of Infant
- Lung fluid replaced by air; pressure in pulmonary circ and R side of heart
- Lung expansion, pressure transmitted to pulmonary blood vessels
- Lung inflation; alveolar oxygen tension reversal of hypoxemia induced pulmonary vasoconstriction of circn RA pressure, LA pressure closure of foramen ovale and ductus arteriosus
CONGENITAL HEART DEFECTS
Shunting of blood diverting blood flow from one system to the other
- determined by the presence of abn opening bet R and L circn and degree of resistance to flow thru the opening
Right to Left Shunt results in unoxygenated blood moving from the R side into L side of the heart, ejected into systemic circulation
- cyanotic heart defect cyanosis when sufficient unoxygd blood mixes with oxygd blood in L side of the heart (more than 3 hrs; pinks up-respi defect)
Left to Right Shunt blood intended for ejection into systemic circulation is recirculated back into R side and back thru lungs increases workload on RV
- non cyanotic heart defect
Disease
Definition
Characteristics
Pathogenesis
Manifestations
Signs and Symptoms
Patent Ductus Arteriosus
Resuts from persistence of
Provides for continuous runoff
diastolic and MABP, pulse
fetal ductus beyond prenatal
of aortic blood into pulmonary
pressure

Syncope r/t cerebral

circulation (vasodilation with
fixed CO, arterial pressure)
Late signs: dyspnea, marked
fatigability, peripheral
cyanosis, low output HF

Disorders of Cardiac Function7

period

Atrial Septal Defects

artery

pulmonary venous return

and work demands
Isolated septal defect
blood flow from left to right
(more compliant RV d/t lower
pulmonary vascular resistance
vs systemic vascular
resistance)
Originates from 2 sources
1. Interventricular groove of
folded tubular heart gives
rise to muscular part of
septum
2. Endocardial Cushions
membranous portion

A hole In the atrial septum

persists d/t improper septal
formation

Ventricular Septal Defect

Opening in ventricular septum

results from imperfect
separation of ventricles during
early fetal devt

Endocardial Cushion
Defects

Endocardial Cushions
- form AV canals, upper part of
ventricular septum, lower part
of atrial septum

Pulmonary Valve Stenosis

Narrow/obstructed pulmonary
outflow channel (RV to
pulmonary artery)

Tetralogy of Fallot

Consists if 4 associated
congenital HDs:
1. Ventricular Septal defect
2. Dextroposition or Shifting
to the R sorta (overriding RV)
3.Pulmonic valve Stenosis
4. RV hyperthrphy (~PV
Stenosis)

Present in 50% of Down

syndrome
Ebstein Anomaly
- displacement of tricuspid
valvular tissue into the
ventricle w/c directly attach to
RV endocardial surface/
shortened chordate tendinae

Partial AV canal defects

- 2 AV valves are complete
and separate
Ostium Primum Defect- cleft
in the mitral valve
Complete canal defect
- there is a common AV valve
orifice, with defects in both A &
V septal tissue

1 : absent/malformed
pulmonary cusps or fused at
their commissural edges
2 to Tetralogy of Fallot

Pulmonary blood flow

Workload on R heart
RV hypertrophy
Size of outlet

Pulmonary blood flow

R-L shunt causes mixing of
un/oxygd blood into
perirpheral circn
Hypercyanotic attacks (tet
spells)
~ in the morning: O2 reqt

pulmonary blood flow,

pulmonary congestion,
resistance against w/c the R
side must pump
LV failure
Changes in pulmonary
vasculature
Atrial Dilatation

Right to Left Shunt; Cyanosis

Small defect
allows a small shunt and
small increases in pulmonary
blood flow

Medium Sized Defects

larger shunt, twice as
much blood thru pulmonary
circulation

Atrial Fibrillation, Atrial

Flutter, Palpitations

Larger defect, Greater shunt,

Higher pressure in pulmonary
vascular system
In complete AV canal defects,
congestive HF, intercurrent
pulm infections appear in
infancy
L-R shunting, transatrial and
transventricular mixing of
blood
Cyanosis with progressive
shunting
Cyanosis (>2yo)
In severe stenosis,
Delayed closure of foramen
ovale
Ductus Arteriosus vital
accessor
route for perfusing lungs
Cyanosis blue babies

Acutely cyanotic, hyperpneic,

irritable, diaphoretic -- limp,
lose consciousness

Angina Pectoris
Faintness
Heart Failure

Large Defects
nonrestrictive defect; equal
pressure on both sides; blood
shinted from LA to pulm artery
under high pressures to prod
pulmonary hpn
Eisenmenger syndrome:
reversal of shunt flow from R
to L shunting

Disorders of Cardiac Function8

Pulm Valscular Resistance

(cry/defecate)
R-L shunt, Pulm blood flow
Transposition of Great
Vessels
Coarctation of the aorta

KAWASAKI DISEASE
Disease
KAWASAKI DISEASE

Aorta originates in the RV

Pulmonary Artery in the LV
Localized narrowing of the
aorta, proximal (preductal and
or coarctation of infancy) and
distal (postductal) to the ductus

Common in infants whose

mothers are diabetic, or boys
Feature of Turner Syndrome,
Disparity in Leg and Arm
pulses

Definition
Monocutaneous Lymph node
syndrome

Characteristics
Affects the skin, brain, eyes,
joints, liver, lymph nodes, heart
(aneurysm of coronary
arteries leads to acquired
HD)

Acute febrile disease of young

children characterized by
vasculitis

(Place infant in a knee-chest

/squatting position to
Systemic Vasc Resisitance)

Postductal
weak pulse in femoral,
politeal, dorsalis pedis
bounding in arms and
carotid vessels
Pathogenesis
Acute Febrile Phase

Manifestations
Abrupt onset of fever,
bilateral conjunctivitis w/o
exudates,
erythema of oral and
pharyngeal mucosa,
strawberry tongue,
dry/fissured lips
swollen hands/feet,
rash,
enlarged cervical lymph nodes

Subacute Phase

Fever breaks
Desquamation of skin of
palms and soles

Convalescent Phase

Complete resolution of
symptoms
and signs of inflamn

Vasculitis inflaman of blood

vessels

Cornonary Vasculitis
- dilated/aneurysm of coronary
arteries

Signs and Symptoms

Myocardial ischemia, overt

MI or aneurysm rupture