Congenital Heart Defects

7/17/2010
Learning Objectives:
Explain the pathophysiology,
manifestations, diagnosis and
Congenital Heart management of congenital cardiac
Disease anomalies.
Describe the implications of cardiac
Arthur Jones, EdD, RRT
anomalies for respiratory care.
http://rc-edconsultant.com/
Development of the heart

Parallel tubes convolute to form
chambers
Development of the Septa and valves form from
endocardial cushion
Cardiovascular System
Development of the heart Fetal circulation- anatomy

Single artery (truncus arteriosus) Includes placental circulation- low
divided by aorticopulmonary septum resistance circuit
At eight weeks, fetal circulation Foramen ovale-- window between
is complete atria
Ductus arteriosus-- vessel
connecting aorta to pulmonary
artery
Ductus venosus- bypasses liver
truncus
arteriosus
Copyright 2008 AP Jones 1

7/17/2010
Fetal circulation- anatomy Fetal circulation- physiology

High pulmonary vascular resistance
foramen Left side includes low resistance
ovale pre-ductal placental circuit
flow
Venous admixture at all shunts
ductus
arteriosus
Pre-ductal blood with highest
ductus PaO2 to upper body
venosus
placenta
Click to see a diagram of fetal circulation

http://clem.mscd.edu/~haysc/public_html/bio232/fetalcirc.jpg
Changes at Birth
Removal of placental circuit
increases left-sided resistance
Increased PaO2 lowers pulmonary Congenital Heart Disease
vascular resistance
Foramen ovale functionally
closed- resistance on left > right
Ductus closes due to increased
PaO2, etc., about 15 hours
postpartum
Etiologic Factors Medical history

maternal infections- rubella, failure to thrive
syphilis retarded growth, development
maternal metabolic dx- diabetes decreased exercise tolerance
maternal drug ingestion squatting
phenytoin (Dilantin) fainting
thalidomide
sex hormones

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Medical history Physical examination

chronic pulmonary infections small stature, underdeveloped
chronic cough color- may be cyanotic
feeding difficulties clubbing
headaches
epistaxis (nosebleeds)
'noisy breathing'
Physical examination Physical examination

color- may be cyanotic cyanosis
clubbing clubbing
heart murmurs- abnormal heart murmurs
blood flow displaced point of maximal
valve activity impulse (PMI)
precordial bulge
Click for information on the physiology of heart

murmurs
http://www.wilkes.med.ucla.edu/Physiology.htm
Physical examination Physical examination

wheezing- CHD often mistaken for wheezing- CHD often mistaken for
asthma asthma
tachypnea tachypnea
tachycardia tachycardia
blood pressure greater in arms
weak femoral pulses
epistaxis

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Diagnosis Diagnosis
Radiography Electrocardiography
chest radiograph Blood gases and/or oximetry
angiography pre, post-ductal SO2
Echocardiography- replaced SO2 in various compartments
catheterization for many defects Cardiac catheterization
diagnostic
therapeutic
FYI - Click for information on echocardiography and CHD FYI - Link to article on therapeutic cardiac catheterization and CHD
http://www.echoincontext.com/advanced/chd_01.asp http://www.americanheart.org/presenter.jhtml?identifier=3043183
Categories
Acyanotic CHD
Obstructive defects
Conduction defects
Cyanotic CHD
Acyanotic Cardiac Anomalies
Miscellaneous
Dextrocardia
Vascular rings
Acyanotic Anomaly Types Acyanotic Anomaly Types

Persistent fetal structures Obstructive defects
patent ductus arteriosus coarctation of aorta
patent foramen ovale aortic stenosis
Septal defects Conduction defects
ventricular septal defects
atrial septal defects
endocardial cushion defects

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Persistent Fetal Structures Persistent Fetal Structures

Types Normal pulmonary vascular
persistent ductus arteriosus resistance
patent foramen ovale left-to-right shunt
May persist asymptomatically, no effect on arterial blood gases
through adulthood. elevated mixed venous PO2
exacerbated by pulmonary increased LV work
hypertension (hypoxemia) LV failure
shunt may change to right-to-left CHF
with PEEP, worsening hypoxemia
Persistent Fetal Structures Patent Ductus Arteriosus

Increased pulmonary vascular Second most common anomaly in
resistance term infants
right-to-left shunt Etiologic factors
hypoxemia, refractory to neonatal asphyxia, hypoxemia
supplemental O2 maternal viral infections, e.g., rubella
low socioeconomic status- nutrition
Note- patent ductus is necessary for
survival in patients with ductal-
dependent anomalies
Click to see persistent fetal circulation
http://www.kumc.edu/instruction/medicine/pedcard/cardiology/pedcardio/pfcdiagram.gif
Patent Ductus Arteriosus Patent Ductus Arteriosus

Complications Manifestations
excessive workload on left ventricle persistent murmur
pulmonary artery disease decreased lung compliance ==>
(Eisenmenger's) increased work of breathing
chronic pulmonary infections cardiomegaly
diagnosed by echocardiogram
Click for more information and pictures of

PDA
http://www.pted.org/?id=patentductus1

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Patent Ductus Arteriosus Septal Defects

Management Normal pulmonary vascular
Medical resistance (PVR)
ibuprofen (Advil) to close ductus left-to-right shunt
indomethacin to close ductus no effect on arterial blood gases
intubate and ventilate with PEEP elevated mixed venous PO2
to improve oxygenation
increased LV work
Surgical
LV failure
ligation (sometimes done in NICU)
CHF
division- requires thoracotomy
FYI - Link to article on PDA closure
http://content.nejm.org/cgi/content/full/343/10/674
Septal Defects Septal Defects

Normal PVR Increased pulmonary vascular
left-to-right shunt resistance
increased LV work right-to-left shunt
excessive pulmonary blood flow hypoxemia, refractory to
causes chronic pulmonary supplemental O2
infections
causes remodeling of pulmonary
vasculature (Eisenmenger's
complex)
Click to see evolution of Eisenmenger's complex
http://img.medscape.com/slide/migrated/editorial/cmecircle/2006/6423/images/landzberg/24.jpg
Septal Defects Small VSD

Small VSD (less than diameter of Manifestations
aortic valve) may be asymptomatic
left-to-right shunt if VSD < 50% only clinical sign may be murmur
aortic diameter other data normal
RV & LV pressures normal
May close spontaneously
Click to hear VSD murmur

http://www.wilkes.med.ucla.edu/Systolic.htm

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Small VSD- Left-to-Right Shunt Large VSD

VSD diameter > aortic valve
Hemodynamics
excessive PA flow ==> vascular
remodeling ==> increased PVR ==>
right-to-left shunt (hypoxemia)
Prolonged left-to-right shunt that
causes remodeling of pulmonary
vessels necessitates a heart & lung
transplant
VSD- Right-to-Left Shunt Large VSD

venous admixture to left ventricle Manifestations
Murmur
CHF
Cyanosis with pulmonary
hypertension
LV hypertrophy
Click for more information and pictures of VSD

http://www.pted.org/?id=ventricularseptal1
Large VSD Large VSD

Diagnosis Management
Echocardiography Palliation, to reduce pulmonary
Heart catheterization blood flow
Angiography PA banding
subambient FIO2- causes pulmonary
vasoconstriction
Correction- Gortex patch closure

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Pulmonary Artery Banding Atrial Septal Defect

Palliative procedure to reduce Categories- based on position of
pulmonary blood flow the defect on atrial wall
PA ostium primum
bands
ostium secundum
sinus venosus
Click to see Flowatch PA band

http://ejcts.ctsnetjournals.org/cgi/content-nw/full/23/3/317/FIG1
Click to see Flowatch in place
http://ejcts.ctsnetjournals.org/cgi/content-nw/full/23/3/317/FIG2
Atrial Septal Defect Atrial Septal Defect

Manifestations Manifestations
murmur murmur
may be otherwise asymptomatic may be otherwise asymptomatic
for 20-30 years for 20-30 years
normal PVR ==> left-to-right shunt normal PVR ==> left-to-right shunt
==> elevated RA and RV PO2 ==> elevated RA and RV PO2
first sign may be right ventricular
failure
may follow pathophysiology of
Click to hear murmur with ASD VSD
http://www.wilkes.med.ucla.edu/Systolic.htm
Atrial Septal Defect Atrial Septal Defect

Diagnosis Diagnosis
ECG - Right axis deviation ECG - Right axis deviation
Echocardiography- detected with Echocardiography- detected with
bubble test bubble test
Heart catheter- elevated RA, RV Heart catheter- high RA, RV SO2
SO2 Treatment- closure in catheterization
lab.
Click to see ASD closure

http://www.rch.org.au/cardiology/health-info.cfm?doc_id=3490

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Atrial Septal Defect Endocardial Cushion Defect

left-to-right shunt Pathogenesis- incomplete
development of ECD
Associated with trisomy 21
(Down's syndrome)
Defects- permutations of:
ASD
VSD
Cleft mitral, tricuspid valve
leaflets
Click for more information and pictures of ASD
http://www.pted.org/?id=atrialseptal1
Endocardial Cushion Defect Endocardial Cushion Defect

Hemodynamics-- depend on Manifestations
specific defects May be asymptomatic
ASD- L to R shunt May develop severe CHF &
VSD- L to R shunt ==> left pulmonary edema
ventricular Diagnosis
hypertrophy ECG- left axis deviation
Mitral regurgitation ==> left atrial Heart catheter- increased SaO2
hypertrophy in RA & RV
Increased PA flow ==> vascular Echocardiography
remodeling & increased PVR
Endocardial Cushion Defect Endocardial Cushion Defect

Complete AV canal Management
palliative PA banding
cleft AV heart failure managment
ASD leaflets diuretics
VSD digitalis
surgical correction
septal defect closure- Dacron patch
valvuloplasty- technically difficult
Click for more information and pictures of AV canal

http://www.pted.org/?id=atrioventricularcomplete1

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Aortic stenosis
Narrowed aortic outflow tract
Hemodynamics- increased
Obstructive Anomalies resistance to LV outflow ==>
increased LV work ==> hypertrophy
==> LV failure
Aortic stenosis Coarctation of the Aorta

Manifestations Narrowing of portion of aorta
Ejection systolic murmur Hemodynamics
Left ventricular hypertrophy aortic obstruction
CHF, sudden death (severe) severity dependent on degree of
Management narrowing
Valvotomy, balloon valvuloplasty Associated with chromosomal
Valve replacement abnormality- Turner's syndrome
Click for more information and pictures of aortic stenosis

http://www.pted.org/?id=aorticstenosis1
Coarctation of the Aorta Coarctation of the Aorta

Manifestations Manifestations
reduced pulses, blood pressure in left ventricular hypertrophy
lower extremities CHF, pulmonary edema
headaches neonates- lower body cyanosis
epistaxis pre-ductal coarctation
leg cramps in presence of PDA
Click for more information and pictures of coarctation

http://www.pted.org/?id=coarctation1

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Coarctation of the Aorta Coarctation of the Aorta

Pre-ductal- coarctation proximal to  Management
ductus arteriosus avoid heavy exercise
Coarctation balloon dilatation with stent
resection- may require graft
venous
admixture
PDA
FYI - Click to see surgical repair of coarctation (9 min)

http://www.youtube.com/watch?v=AGohu9fqKHg
Conduction defect
Wolff-Parkinson-White syndrome
Impulse aberrantly conducted
through bundle of Kent Cyanotic Anomalies
Manifestations
PR interval < 0.12s
paroxysmal atrial tachycardia
(PAT)
Treatment
Medical- antidysrhythmics
Electrophysiology- ablation
Cyanotic Anomalies Cyanotic Anomalies

Categories: Requirements for arterial
increased pulmonary blood flow desaturation
decreased pulmonary flow Communication between systemic &
pulmonary circulation
abnormal vessels
septal defects
PVR > SVR
Desaturation due to intracardiac
shunt is unresponsive to increased
FiO2

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Cyanotic Anomalies Cyanotic Anomalies

Conditions with low pulmonary flow Conditions with high pulmonary flow
Tetralogy of Fallot Transposition of great vessels
Pulmonary atresia Persistent truncus arteriosus
Tricuspid atresia Total anomalous pulmonary venous
Bicuspid atresia, AKA hypoplastic return
left ventricle
Tetralogy of Fallot Tetralogy of Fallot

Defects (tetra = four)
Pulmonary stenosis
Ventricular septal defect (VSD)
Overriding aorta-- aorta straddles
Aorta
both ventricles Stenotic PV
over VSD
Right ventricular hypertrophy RVH VSD
right-to-
left shunt

Hemodynamics Hemodynamics
Pulmonary stenosis VSD- channel for shunt
Determines PA resistance to flow Will be left-to-right with low
Regulates resistance to right pulmonary resistance
ventricular flow Usually large
Determines right to left shunt
Leads to RV hypertrophy
Degree of stenosis determines
urgency of surgical intervention

7/17/2010

Hemodynamics Spectrum from "pink tets" to
Overriding aorta emergent cases in neonatal stage
Carries outflow from both May not appear until closure of
ventricles PDA, then pulmonary blood flow
Contributes to severity of shunt declines
RV hypertrophy
Chronic elevated flow resistance
Very large VSD- equalizes
pressures in LV and RV
Click for more information and pictures of TOF
http://www.pted.org/?id=tetralogyfallot1

Manifestations Chest xray- 'boot-shaped' heart
cyanosis- "tet spells" with exertion ECG-- right axis deviation
squatting to relieve exertional Echocardiography- usually
spells definitive
clubbing Catheterization
growth retardation
systolic ejection murmur
Click to hear pulmonary stenosis murmur Click to see 'boot-shaped' heart on xray
http://www.wilkes.med.ucla.edu/Systolic.htm http://www.bcm.edu/radiology/cases/pediatric/text/3a-desc.htm

Management of tet spells Palliation- arterial to pulmonary
fetal positioning artery shunts
morphine bypass stenotic pulmonary valve
oxygen- an exception for increase pulmonary blood flow
supplemental O2
Total correction
bicarbonate
Excision of PV obstruction
propanolol
Patch closure of VSD
vasoconstrictors

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Tricuspid atresia Tricuspid atresia

Defects
Atretic tricuspid valve- does not
open, so blocks blood flow from ASD
atrium to ventricle Atretic
valve VSD
Diminutive (small) RV
Diminutiv
VSD & ASD e
RV

Blood flow Blood flow
Vena cava to Vena cava to
RA to ASD to RA to ASD to
LA to LV to LA to LV to
RV (via VSD) RV (via VSD) to
PA
Click for more information and pictures of tricuspid atresia

http://www.pted.org/?id=tricuspidatresia1

Signs Diagnosis
early cyanosis (from birth) ECG- left axis deviation
worsening, death on closure of Echocardiography
ductus arteriosus diminutive right ventricle
growth retardation absent tricuspid echoes
squatting Catheterization-- catheter will not
clubbing enter RV

7/17/2010

Palliative procedures- to increase Management
pulmonary blood flow Corrective- Fontan
Maintain PDA high risk, high failure rate
subambient FIO2 bypass RV by directing blood
alprostadil from RA to PA
stent placement pulmonary blood flow becomes
Waterston shunt-- aorta to RPA dependent on passive venous
return.
Blalock-Taussig (BT) shunt- from
subclavian artery to PA
Click to see pictures of the Fontan procedure
http://www.pted.org/?id=fontan1
Bicuspid atresia- hypoplastic LV Hypoplastic LV

Defects Signs
Atretic bicuspid valve early cyanosis
Diminutive LV shock
VSD & ASD AS Atretic worsening, death with DA
D valve closure
Diminutiv
VS e
D LV
Hypoplastic LV Hypoplastic LV
Echocardiogram Maintain PDA
diminutive left ventricle Surgical management
absent bicuspid echo Norwood- multiple stage procedure
Fontan
Blalock-Taussig (BT) shunt
Click to see hypoplastic LV

http://www.pted.org/?id=hypoplasticleft1

7/17/2010
Persistent truncus arteriosus Persistent truncus arteriosus

Defects Hemodynamics
Single artery for LV & RV truncus carries blood to PA &
VSD aorta
flow is dependent upon resistance
to flow at each side
increased SVR ==> increased
pulmonary flow
increased PVR ==> increased
systemic flow

Decreased PVR ==> excessive Manifestations
pulmonary blood flow ==> Cyanosis
high output LV failure (CHF) CHF
pulmonary vascular dx
Increased PVR ==> reduced in
pulmonary blood flow ==>
hypoxemia
Click for more information and pictures of truncus

arteriosus
http://www.pted.org/?id=truncusarteriosus1

Diagnosis Management
CXR- cardiomegaly Heart failure
ECG- combined hypertrophy digoxin
Echocardiogram diuretics
visualize vessel origins Palliative- reduce PA flow
one semilunar valve PA banding
Catheterization- equal LV & RV subambient FIO2
pressures
Click to see chest xray of patient with truncus arteriosus

http://www.childrenshospital.org/cfapps/mml/index.cfm?CAT=media&MEDIA_ID=1341

7/17/2010
Persistent truncus arteriosus Transposition of great arteries (TGA)

Corrective surgry Defects
Aorta arises from RV
main trunk moved to left
Pulmonary artery arises from LV
creation of outflow tube from RV to ASD and/or VSD, PDA (increase chance for
PAs survival)
closure of VSD to periphery
to lungs
from from
periphery lungs
aort pulmonary
a RV LV
artery
TGA TGA
Hemodynamics Signs
Separate circulations diabetic mother- high risk
Pulmonary venous blood to LA early cyanosis
to LV through PA to lung CHF
Systemic venous return to RA
to RV to aorta to system
Without septal defect, life
impossible
With VSD, there is mixing
Click for more information and pictures of TGA
http://www.pted.org/?id=transpositiond1
TGA TGA
Diagnosis Management
CXR-- cardiomegaly Palliative
Echocardiogram- visualize vessels maintain PDA
Catheterization- catheter enters balloon septostomy
aorta from RV
Click for more information and pictures of TGA

http://www.pted.org/?id=transpositiond1

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TGA
Management
Corrective
Mustard-- baffle in atria
Jatene (switch)- vessels switched to Therapeutics
correct ventricles
Maintaining a PDA Subambient O2 Therapy

Indication- ductal dependent goals
cardiac anomaly; e.g.: increase pulmonary vascular
transposition of great arteries resistance to reduce pulmonary
tricuspid atresia blood flow
large VSD
mitral atresia
endocardial cushion defect
Methods
persistent truncus arteriosus
stent
prevent closure of ductus
alprostadil (Prostin) arteriosus
subambient O2 transposition of great arteries
tricuspid atresia
mitral atresia
Subambient O2 Therapy Post-surgical Considerations

methods
bleed-in nitrogen to ventilator Procedure Response
circuit Action
obtain premixed subambient DA closure Increase CL Decrease

mixture in cylinder PIP
PA band
titrate FIO2 to SaO2 80-85%
problem- some O2 analyzers may A-PA shunts Decrease CL Increase
not measure subambient FO2 PIP
DA patency

7/17/2010
Special issues Summary & Review

Transplant organs difficult to obtain. Development of the cardiovascular
Patients' hearts can outgrow. system
synthetic structures, like valves. endocardial cushion
Oxygen therapy can kill patients with truncus arteriosus
ductal dependent anomalies. fetal circulation with shunts
changes at birth
Summary & Review Summary & Review

Congenital heart disease Acyanotic defects
etiologic factors types
historical manifestations persistent fetal structures
physical manifestations ventricular septal defects
diagnostic procedures atrial septal defects
categories endocardial cushion defects
acyanotic complications
cyanotic remodeling of pulmonary vessels
obstructive left ventricular failure
conduction defects

Acyanotic defects Obstructive defects
management types
palliation with PA bands aortic stenosis
total correction coarctation of the aorta
manifestations
management
limit exercise
surgical repair

7/17/2010

Conduction defect- WPW syndrome Cyanotic defects
abnormal conduction pathway types- high, vs. low pulmonary
ECG- decreased P-R interval blood flow
management ductal dependence
medications for PAT manifestations
ablation of bundle of Kent cyanosis
tetralogy spells
ventricular failure

Cyanotic defects Subambient oxygen therapy
tetralogy of Fallot Postoperative expectations
tricuspid atresia Issues in congenital heart disease
hypoplastic left ventricle (mitral
atresia)
Persistent truncus arteriosus
Transposition of great arteries
References
Barnhart SL, Cervinske, MP. Perinatal and
Pediatric Respiratory Care 2003. WB Saunders
Company, Phila.
Levin DL, Morriss FC. Essentials of Pediatric
Intensive Care (volume one) 1997. Churchill-
Livingston, NY.
END
Johnson KB, Oski FA. Oski's Essential Pediatrics
1997. Lippincott-Raven, Phila.

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7/17/2010

Development of the heart

Development of the heart Fetal circulation- anatomy

Copyright 2008 AP Jones 1

Fetal circulation- anatomy Fetal circulation- physiology

Click to see a diagram of fetal circulation

Etiologic Factors Medical history

Copyright 2008 AP Jones 2

Medical history Physical examination

Physical examination Physical examination

Click for information on the physiology of heart

Physical examination Physical examination

Copyright 2008 AP Jones 3

Acyanotic Anomaly Types Acyanotic Anomaly Types

Copyright 2008 AP Jones 4

Persistent Fetal Structures Persistent Fetal Structures

Persistent Fetal Structures Patent Ductus Arteriosus

Patent Ductus Arteriosus Patent Ductus Arteriosus

Click for more information and pictures of

Copyright 2008 AP Jones 5

Patent Ductus Arteriosus Septal Defects

Septal Defects Septal Defects

Septal Defects Small VSD

Click to hear VSD murmur

Copyright 2008 AP Jones 6

Small VSD- Left-to-Right Shunt Large VSD

VSD- Right-to-Left Shunt Large VSD

Click for more information and pictures of VSD

Large VSD Large VSD

Copyright 2008 AP Jones 7

Pulmonary Artery Banding Atrial Septal Defect

Click to see Flowatch PA band

Atrial Septal Defect Atrial Septal Defect

Atrial Septal Defect Atrial Septal Defect

Click to see ASD closure

Copyright 2008 AP Jones 8

Atrial Septal Defect Endocardial Cushion Defect

Endocardial Cushion Defect Endocardial Cushion Defect

Endocardial Cushion Defect Endocardial Cushion Defect

Click for more information and pictures of AV canal

Copyright 2008 AP Jones 9

Aortic stenosis Coarctation of the Aorta

Click for more information and pictures of aortic stenosis

Coarctation of the Aorta Coarctation of the Aorta

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Copyright 2008 AP Jones 10

Coarctation of the Aorta Coarctation of the Aorta

FYI - Click to see surgical repair of coarctation (9 min)

Cyanotic Anomalies Cyanotic Anomalies

Copyright 2008 AP Jones 11

Cyanotic Anomalies Cyanotic Anomalies

Tetralogy of Fallot Tetralogy of Fallot

Tetralogy of Fallot Tetralogy of Fallot

Copyright 2008 AP Jones 12

Tetralogy of Fallot Tetralogy of Fallot

Tetralogy of Fallot Tetralogy of Fallot

Tetralogy of Fallot Tetralogy of Fallot

Copyright 2008 AP Jones 13

Tricuspid atresia Tricuspid atresia

Tricuspid atresia Tricuspid atresia

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Tricuspid atresia Tricuspid atresia

Copyright 2008 AP Jones 14

Tricuspid atresia Tricuspid atresia