Cardiovascula

r System
Pathophysiol
ogic
Concepts
 Thrombus

• A thrombus
– a blood clot that can develop
anywhere in the vascular system
– causing the narrowing of a vessel.
– blood flow can be occluded
(reduced or totally blocked)
 Thrombus
– develop from any injury to the vessel wall
• endothelial cell injury draws platelets and other
mediators of inflammation to the area.
• substances stimulate clotting and activation of the
coagulation cascade.
• formation can occur when blood flow through a vessel
is sluggish,
• when blood flow is irregular or erratic
–during periods of irregular heartbeat or cardiac arrest
Thrombus
 Embolus

• Embolus
– a substance that travels in the
bloodstream from a primary site to a
secondary site
– becomes trapped in the vessels at the
secondary site
– causes blood flow obstruction.
– Most emboli are blood clots
(thromboemboli)
• usually deep leg veins
 Embolus
– Other sources of emboli
• fat
– released during the break of a long bone
– produced in response to any physical trauma, and
amniotic fluid
» which may enter maternal circulation during the
intense pressure gradients generated by labor
contractions.
• Air and displaced tumor cells also may act as emboli to
obstruct flow.
 Aneurysm
• Aneurysm
– is a dilation of the arterial wall caused by a
congenital or developed weakness in the wall.
– Weakness in the wall may develop as a result of an
infection, from trauma, or, more commonly, from
lesions produced by atherosclerosis.
– Aneurysms may burst with increased pressure,
leading to massive internal hemorrhage.
 Alterations in Capillary Forces
of Filtration or Reabsorption
• Occasionally, forces favoring filtration
from the capillary into the interstitial
fluid are greater than forces favoring
reabsorption of fluid into the capillary
from the interstitial space.
• The result is net filtration.
• Net filtration across the capillary
results in interstitial edema.
 Alterations in Capillary Forces of
Filtration or Reabsorption

• The opposite occurs when forces favoring

reabsorption of fluid from the interstitial
space into the capillary are greater than
those favoring filtration.
• This results in net reabsorption, which
leads to increased plasma volume, stroke
volume, and cardiac output.
• Blood pressure may be increased
significantly.
Causes of Increased Capillary
Filtration
• Causes of increased capillary filtration include
– increased capillary pressure
• caused by high blood pressure
– increased capillary leakage
• caused by injury or inflammation.
– increase in protein concentration in the
interstitial fluid
• caused by increased capillary breakdown
• decreased lymph flow to the area would
also cause net filtration, leading to edema
and swelling of the interstitial space.
 Causes of Increased
Capillary Filtration
• Causes of increased capillary filtration
include
• decreased production or increased
loss of plasma proteins
– reduce the reabsorption of fluid
back into the capillary.
– can occur with liver disease or
loss of protein in the urine.
Causes of Increased
Capillary Reabsorption
• Causes of increased
reabsorption of fluid from the
interstitial space include
– decreased blood pressure in
the capillary
• due to a decrease in
systemic pressure or
constriction of the arteriole
or precapillary sphincter.
– Increased plasma colloid osmotic pressure
also draws fluid back into the capillary.
• Plasma colloid osmotic pressure increases
with dehydration, leading to a return of fluid
from the interstitium to the plasma, which
helps return plasma volume toward normal.
– Finally, increased interstitial fluid pressure
increases reabsorption by opposing further
accumulation of fluid.
 Stenosis
• Stenosis
– a narrowing of any vessel or opening.
– stenosis of the heart valves may
occur.
– congenital defect or an inflammatory
process (e.g., after rheumatic fever).
Results of Cardiac Valve
Stenosis
• chamber upstream of the stenosis
pumping more forcefully to expel its
blood through the narrowed orifice.
• After years of this extra work,
cardiac muscle can hypertrophy
(increase in size).
• If the chamber cannot pump
forcefully enough to overcome the
stenosis, blood flow out of the
chamber will be reduced.
Results of Cardiac Valve
Stenosis
• chamber increases its oxygen
consumption and energy demands.
• The coronary arteries supplying the
muscle may be unable to supply adequate
oxygen to meet this demand.
• blood may accumulate in the chamber
and stretch its muscle fibers.
• If this is significant or prolonged, a
decrease in muscle contractility can result.
Examples of Cardiac Valve Stenosis

• Any cardiac valve

• Mitral stenosis
– narrowing of the valve between the left atrium
and left ventricle.
• Aortic stenosis
– narrowing of the valve between the left
ventricle and the aorta.
• Tricuspid stenosis
– narrowing of the valve between the right
atrium and the right ventricle.
• Pulmonary stenosis
– narrowing of the valve between the right
ventricle and the pulmonary artery.
Valve Incompetence

– An incompetent valve is one that

does not close completely, allowing
blood to move in both directions
through that valve when the heart
contracts (valve regurgitation).
– Any of the cardiac valves may be
incompetent.
– Each chamber may hypertrophy.
Cardiac Shunts
• a shunt is a connection between the
pulmonary vascular system and the
systemic vascular system.
– During fetal life, shunts between the right
and left sides of the heart and between the
aorta and pulmonary artery are normal.
– The direction blood flows through a shunt
is determined by resistance to flow in each
direction.
Right-to-Left Shunt
• the flow of blood from the right side of the
heart to the left, or from the pulmonary artery
to the systemic circulation.
• After birth, right heart and pulmonary artery
blood is poorly oxygenated. Therefore, a right-
to-left shunt delivers poorly oxygenated blood
to the systemic circulation.
• called a cyanotic shunt because delivery of
poorly oxygenated blood to the systemic
circulation causes cyanosis (bluish tinge to
the skin).
Left-to-Right Shunt
• the flow of blood from the left side of
the heart to the right side, or from the
aorta to the pulmonary circulation.
• Left heart blood is well oxygenated.
• Blood going to the lungs from the left
side of the heart recirculates to the
left atrium and left ventricle.
• Because the blood is well-
oxygenated, this shunt is acyanotic.
Two Types of Cardiac Defects
• Congenital
– Anatomic>abnormal function
• Acquired
– Disease process
• Infection
• Autoimmune response
• Environmental factors
• Familial tendencies
 Causes of CHD
• Chromosomal/genetic = 10%-12%
• Maternal or environmental = 1%-2%
– Maternal drug use
• Fetal alcohol syndrome—50% have CHD
– Maternal illness
• Rubella in 1st 7 wks of pregnancy→50%
risk of defects including PDA and
pulmonary branch stenosis
• CMV, toxoplasmosis, other viral
illnesses>> cardiac defects
• IDMs = 10% risk of CHD (VSD,
cardiomyopathy, TGA most common)
• Multifactorial = 85%
CHD
• Incidence: 5-8 per 1000 live births
– About 2-3 of these are symptomatic in first year
of life
– Major cause of death in first year of life (after
prematurity)
– Most common anomaly is VSD
– 28% of kids with CHD have another recognized
anomaly (trisomy 21, 13, 18, +++ )
Older Classifications of CHD
• Acyanotic
– May become cyanotic
• Cyanotic
– May be pink
– May develop CHF
Newer Classification of
CHD
• Hemodynamic characteristics
– Increased pulmonary blood
flow
– Decreased pulmonary blood
flow
– Obstruction of blood flow out
of the heart
– Mixed blood flow
Increased Pulmonary
Blood Flow Defects
• Abnormal connection between
two sides of heart
– Either the septum or the great
vessels
• Increased blood volume on
right side of heart
• Increased pulmonary blood flow
• Decreased systemic blood flow
Increased Pulmonary
Blood Flow Defects
• Atrial septal defect
• Ventricular septal defect
• Patent ductus arteriosus
ATRIAL SEPTAL DEFECT (ASD)
• Abnormal opening between the atria
• an abnormal opening between the left and
right atria.
• foramen ovale fails to close after birth, or
when another opening between the left
and right atria is present due to improper
closure of the wall between the two atria
during gestation.
• PATHOPHYSIOLOGY
– Blood flows from left to the
right
– Increase flow of oxygenated
blood nto the right side of the
heart
ASD
• CLINICAL MANIFESTATIONS
– Asymptomatic
– May develop congestive heart
failure
– Murmur
– Atrial dysrhythmias
VENTRICULAR SEPTAL
DEFECT (VSD)
• Abnormal opening between
right and left ventricle
• the most common cardiac
congenital defect
• May close spontaneously : 1st
year of life
VSD
PATHOPHYSIOLOGY
• Blood flow from higher pressure
left ventricle to low pressure
right ventricle
• Increased blood volume is
pumped into the lungs
• Right ventricular hypertrophy
• CLINICAL MANIFESTATIONS
– Murmur
– Congestive heart failure is
common
PATENT DUCTUS
ARTERIOSUS
• Failure of the fetal ductus
arteriosus (artery connecting
aorta and pulmonary artery) to
close within the first weeks of
life
PATHOPHYSIOLOGY
• Blood flow from high pressure
aorta to lower pressure pulmonary
artery
• Increased workload on the left side
of the heart
• Hypertrophy
PDA
• CLINICAL
MANIFESTATIOn
– Aymptomatic
– Signs of congestive heart
failure
– Machinery-like murmur
– Widened pulse pressure
Obstructive Defects
• Coarctation of the aorta
• Aortic stenosis
• Pulmonic stenosis
COARCTATION OF THE AORTA
(COA)
• Localized narrowing near the
insertion of the ductus
arteriosus,
• increased pressure proximal to
the defect
COA
CLINICAL MANIFESTATION

– High BP and bounding pulses in

arms
– Weak or absent femoral pulses
– Cool lower extremities
– Low BP in the lower extremities
– Dizziness
– Headache
– Fainting
– Epistaxis
Aortic Stenosis
 Aortic Stenosis

• a narrowing in the opening of the valve between

the left ventricle and the aorta.
• follows rheumatic fever or is a congenital
malformation.
• ventricle must pump more forcefully to expel
blood through the narrow orifice.
• This causes ventricular hypertrophy and
eventually reduces compliance. As blood backs
up in the ventricle, atrial pressure increases and
blood backs up into the pulmonary system and
the right side of the heart.
 Clinical Manifestations

• Clinical manifestations may be absent or severe,

depending on the level of stenosis.
• Pulmonary congestion, with signs of dyspnea and
pulmonary hypertension, may occur if blood backs up
into the pulmonary vascular system.
• Dizziness and fatigue may occur due to decreased
cardiac output and decreased stroke volume. Heart
rate may be elevated via sympathetic stimulation.
• Diagnostic Tools
– A systolic heart murmur may
be heard as blood rushes
through the narrow orifice.
– Echocardiography may be
used to diagnose abnormal
valve structure and motion.
• Complications
– Left ventricular hypertrophy may
develop, leading to congestive
heart failure.
• Treatment
– Treatment for congestive heart
failure may be required.
Pulmonic Stenosis
 Pulmonic Stenosis
• a narrowing of the opening between the
right ventricle and the pulmonary valve.
• most commonly occurs due to a congenital
defect.
• With a narrow orifice, the right ventricle
must pump more forcefully to expel blood.
This can lead to right ventricular
hypertrophy, backing up into the right
atrium and causing dilation of the vena
cava and blood accumulation in the
systemic veins.
• Blood flow into the lungs and
left side of the heart will be
reduced if the stenosis is
severe, leading to a decrease
in blood pressure. Right heart
failure may develop.
 Clinical
Manifestations
• may be absent or severe depending
on the level of stenosis.
• Decreased pulmonary flow causes
poor oxygenation of the blood and
feelings of weakness and fatigue.
• Venous distention and swelling of the
ankles and feet are common.
• Diagnostic Tools
– Echocardiography may be used to diagnose
abnormal valve structure and motion.
• Complications
– Right heart hypertrophy and subsequent right
heart failure may occur.
• Treatment
– Treatment for heart failure may be required.
– Valve replacement or surgical correction of
the stenosis may be attempted.
Decreased Pulmonary
Blood Flow Defects
• Tetralogy of Fallot
• Tricuspid atresia
Tetralogy of Fallot
• The classic form includes
four defects
– Ventricular septal defect
– Pulmonic stenosis
– Overriding of the aorta
– Right ventricular
hypertrophy
Tetralogy of Fallot
Tetralogy of Fallot
• CLINICAL MANIFESTATIONS
– Infants
• Cyanotic at birth
–Episodes of cyanosis
and hypoxia called Blue
or tet spells
»Crying or after feeding
Tricuspid Atresia
Tricuspid Atresia

• The deformity consists of a

complete lack of formation of the
tricuspid valve with absence of
direct connection between the
right atrium and right ventricle.
• prevalence of 0.3-3.7% in
patients with congenital heart
disease
 Clinical
Manifestation
• detected in infancy because of
presenting cyanosis, congestive
heart failure, and growth
retardation.
• history of poor skin coloration
inability to complete a feeding
session, frequent pauses during
feeding, and/or frank anorexia.
Clinical Manifestation
• As a result, the infant
demonstrates poor growth
patterns.
• Respiratory difficulties are
often reported as nasal
flaring or muscle retractions
Mixed Defects
• Transposition of great
vessels
• Total anomalous pulmonary
venous connection
• Hypoplastic heart syndrome
– Right
– Left
Transposition of
Great Vessels
• The pumonary artery
arised from the left
ventricle and the aorta
exits from the right
ventricle
Transposition of Great Vessels
• CLINICAL MANIFESTATION
– Depend on the type and size of
associated defects
– Minimal communication
• Severely cyanotic and depressed at
birth
– Large septal defects
• Less cyanotic but may have
symptoms of CHF
 Totally Anomalous Pulmonary
Venous Connection
• Rare Defect.
• Pulmonary veins fail to join L atrium.
• Clinical manifestations: Usually
cyanotic early on. Condition rapidly
deteriorates as pulmonaryblood flow
increases and causes CHF.
Totally Anomalous Pulmonary
Venous Connection
 Hypoplastic Left Heart
• L side of heart is underdeveloped.
• L ventricle is small and aortic atresia.
• Most blood flows across patent foramen
ovale to R atrium..to R ventricle and out the
pulmonary artery.
• Descending aorta receives blood from the
PDA to supply the systemic circulation. PDA
closure >>rapid deterioration and CHF.
Hypoplastic Left
Heart
 Clinical Manifestations
• As the ductus arteriosus begins to close
normally over the first 24-48 hours of life
– Symptoms of cyanosis, tachypnea,
respiratory distress, pallor, lethargy,
metabolic acidosis, and oliguria develop.
– Without intervention to reopen the ductus
arteriosus, death rapidly ensues.
Clinical Manifestations
• With a right-to-left shunt,
cyanosis, fatigue, and
weakness occur. Knee-to-chest
or squatting behavior may be
observed. Clubbing of the digits
may develop.
Clinical Manifestations
• With a left-to-right shunt,
pulmonary congestion and
dyspnea may occur. Left heart
failure may develop.
 Treatment
• Some defects may be small, require no treatment, or close
spontaneously.
• Surgical correction of the defect is often required.
• Treatment for congestive heart failure may be necessary.
• Prostaglandin E is administered to maintain patency of the
ductus arteriosus in preductal coarctation.
• Administration of the prostaglandin inhibitor indomethacin will
initiate closure of the ductus in patent ductuctus arteriosus.