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CONGENITAL HEART DEFECTS
Pathophysiology, Hemodynamics
and Clinical Recognition
Mary Fran Hazinski, RN, MSN, FAAN,
FAHA, FERC
Compensated
Senior Science
Editor for Emergency
Cardiovascular Care Programs,
American Heart Association
Professor, Vanderbilt University School of
Nursing
Nashville, TN
CHD: For Additional Reading
Please
refer to detailed WORD handout
provided on CF conference website
Hazinski MF, Nieves J, Fosse G, Rummell M
and others: Cardiovascular Problems, In
Hazinski MF (Ed), Nursing Care of the
Critically Ill Child, ed 3, Saint Louis, 2013,
Mosby, an imprint of Elsevier
If you don’t understand the
child’s CHD
Ask
cardiologist to draw a
picture
Ask surgeon to draw a picture
Keep references at hand to
create pictures
Objectives
Provide
framework and overview of the
major classifications of defects and their
hemodynamic consequences
– Describe perinatal circulatory changes
– Describe factors increasing and decreasing
pulmonary vascular resistance
– Provide overview of hemodynamic principles
Highlight
defects
most common congenital heart
Congenital Heart Defects
CONGENITAL HEART DISEASE
DEFINITION:
Defect in heart or
great vessels present from birth
INCIDENCE: 8-10/1,000 live births
(0.8-1.0% of all life births)
Most multifactorial in origin
 May
be part of genetic
disease or syndrome
(if so, look for other
associated problems)
 May be isolated defect
 Often of multi-factorial
inheritance with
genetic predisposition
See Fosse, G. Etiologies of Congenital Heart Disease; Non-Inherited and
Genetic Factors, In Cardiovascular Problems chapter, In Hazinski, MF
Nursing Care of Critically Ill Child ed 3, Saint Louis, 2013, Mosby.
Fetal Circulation—Web figure
Fetal circulation and perinatal
circulatory changes
Fetus: High pulmonary
vascular resistance
PERINATAL CHANGES
Placental
to pulmonary oxygenation
Pulmonary blood flow increases,
pulmonary vascular resistance falls
Fetal shunts close
Systemic vascular resistance rises
Postnatal Circulation—© Ross Labs
Reactive pulmonary
vasoconstriction
Postnatal Circulation: Pulmonary
vascular resistance falls
PULMONARY VASOCONSTRICTORS
4 H’S
(Alveolar)
Hypoxia
Hydrogen ion (acidosis)
Hyperdistension of alveoli
Hypothermia
PULMONARY VASODILATORS
6 A’S
Alveolar
oxygenation
Alkalosis
Appropriate
tidal volume and temperature
Analgesia
Avoidance
of stimulation
ALSO:
Administered nitric oxide and other
vasodilators
CHF: Causes in Neonatal Period
CHD
causing L heart/aortic obstruction
Large shunt causing increased PBF
Any large shunting lesions
Pulmonary venous obstruction
Cor pulmonale
Uncommon causes
Hemodynamic Principles
“Happy heart” with normal
pressure, flow and resistances
Pressure
This
= Flow X Resistance
copyrighted figure
removed at request of speaker
Effect of Increased Resistance
Hemodynamic Principles—cont’d
For
Blood
Right to left shunt
CONGENITAL HEART DEFECTS
Classification
flow to remain unchanged,
driving pressure must increase
This copyrighted cartoon
removed at request of the
speaker
Resistance
to flow through
pulmonary outflow tract must
be greater than that through
aortic outflow tract
This copyrighted figure
removed at request of speaker
will flow in path of least
resistance
Acyanotic
defects producing increased
pulmonary blood flow or pulmonary
obstruction
Defects producing hypoxemia
Defects resulting in left heart or aortic
obstruction
CONGENITAL HEART DEFECTS
Classification
Atrial Septal Defect—U
Kansas fig
Acyanotic
defects producing increased
pulmonary blood flow or pulmonary
obstruction
Defects producing hypoxemia
Defects resulting in left heart or aortic
obstruction
Atrial septal defects--types
Ventricular Septal Defect—U
Kansas
fig
From: Kambam J, ed. Cardiac Anesthesia for Infants and Children. St
Louis, Mosby, 1994
High pulmonary blood flow
under high pressure = CHF
Netter—CIBA illustration --copyrighted
Ventricular Septal Defect with
pulmonary vascular disease
© MF Hazinski—do
not reproduce
without permission
Patent ductus arteriosis ©U Kansas
Atrioventricular Septal Defect
U of Kansas figure
AVSD—Complete Canal
Figure © Hazinski, Nursing Care of Critically Ill Child, ed 3
AVSD—Complete Canal Repair
Figure © Hazinski, Nursing Care of Critically Ill Child, ed 3
Double-Outlet RV --wth SubAo
VSD, no PS—VSD type
Figure © Hazinski, Nursing Care of Critically Ill Child, ed 3
SubPulm VSD without PS—TGA
type
Double-Outlet RV --wth SubAo
VSD and PS—Tetralogy type
Figure © Hazinski, Nursing Care of Critically Ill Child, ed 3
Pulmonary Valve Stenosis
U Kansas figure
Figure © Hazinski, Nursing Care of Critically Ill Child, ed 3
Pulmonary valve stenosis
angiogram—©MF Hazinski
Pulmonary valvuloplasty—cath
From: Kambam J, ed. Cardiac Anesthesia for Infants and Children. St
Louis, Mosby, 1994
CONGENITAL HEART DEFECTS
Classification
Pulmonary valvotomy
Acyanotic
defects producing increased
pulmonary blood flow or pulmonary
obstruction
Defects producing hypoxemia
Defects resulting in left heart or aortic
obstruction
HYPOXEMIA of Cardiac Origin
PaO2 <
80 mmHg when patient breathing
room air at sea level
Central cyanosis (vs peripheral)
Generally does not respond to O2
CHD: TGA, tetralogy, pulmonary atresia,
truncus arteriosus, tricuspid atresia, single
ventricle, TAPVC
Newborn Hyperoxia Test
 Obtain
baseline PaO2 from RIGHT radial artery
 Administer 100% oxygen for 10-15 minutes
 Obtain “post oxygenation” arterial sample from
RIGHT radial artery
– PaO2 <50mm Hg = probable cyanotic CHD,
administer prostaglandins
– PaO2 50-250 mm Hg = possible cyanotic CHD
(consider prostaglandins)
– PaO2 >250 mm Hg= cyanotic CHD unlikely
Cyanotic Heart Disease
Hypoxemia
PaO2)
(low
Arterial oxygen
content may be near
normal (depends on
[Hbg])
 DO NOT ALLOW AIR
TO ENTER ANY IV
CATHETER


Copyrighted cartoon
removed at request
of the speaker
Tetralogy of Fallot—U Kansas fig
Tetralogy of Fallot--surgery
Palliative
shunt to increase
pulmonary blood flow
Correction
Tetralogy of Fallot
Surgical Correction
Truncus Arteriosus--I
U Kansas figure
Figure © Hazinski, Nursing Care of Critically Ill Child, ed 3
Truncus arteriosus repair—
Modified Rastelli
Figure © Hazinski, Nursing Care of Critically Ill Child, ed 3
Pulmonary atresia with VSD
U Kansas figure
Pulmonary atresia with intact
ventricular septum—©U Kansas
Pulmonary atresia with intact
ventricular septum--Chest x-ray
© Mary Fran Hazinski
Pulmonary atresia with IVS
RV sinuisoids
Tricuspid atresia with VSD
U Kansas figure
In Kambam J, ed. Cardiac Anesthesia for Infants and Children. St Louis,
Mosby, 1994
Bidirectional Glenn (SVC-PAs)
© Nichols D (Ed), Critical heart disease in infants and children,
Edition 3, Saint Louis, 2008 Mosby.
Fontan—original procedure
Fontan—extracardiac Fontan
Transposition of the Great
Arteries—U Kansas figure
Sidebotham D, McKee A,
Gillham M, and others.
Cardiothoracic Critical Care.
Philadelphia, Butterworth
Heinemann/Elsevier, 2007
Transposition—parallel
circulations
Transposition—Arterial switch
(Jatene procedure)

From Kambam J.
Transposition of the great
arteries. In Kambam J, ed..,
Cardiac Anesthesia for
Infants and Children. St
Louis, Mosby, 1994
From Kambam J Transposition of the great arteries In Kambam J ed
CONGENITAL HEART DEFECTS
Classification
Acyanotic
defects producing increased
pulmonary blood flow or pulmonary
obstruction
Defects producing hypoxemia
Defects resulting in left heart or aortic
obstruction
Coarctation of the Aorta
U of Kansas figure
angio
© MF Hazinski
Aortic Stenosis
Copyrighted cartoon removed at request of the speaker
Hypoplastic Left Heart Syndrome
© MFHazinski art
Hypoplastic Left Heart Syndrome
If too much pulmonary blood flow
 CHF
 Poor
systemic
perfusion
 High SaO2 (>80%)
© Nichols D (Ed), Critical heart disease in infants and children,
Edition 3, Saint Louis, 2008 Mosby.
Norwood Procedure—his art
from NEJM
PULMONARY VASOCONSTRICTORS
4 H’S
(Alveolar)
Hypoxia
Hydrogen ion (acidosis)
Hyperdistension of alveoli
Hypothermia
Hypoplastic Left Heart Syndrome
If too little pulmonary blood flow
 Severe
hypoxemia
 Adequate systemic
perfusion
 Low SaO2 (<70%)
© Nichols D (Ed), Critical heart disease in infants and children,
Edition 3, Saint Louis, 2008 Mosby.
PULMONARY VASODILATORS
6 A’S
Alveolar
Alkalosis
Appropriate
framework and overview of the
major classifications of defects and their
hemodynamic consequences
– Describe perinatal circulatory changes
– Describe factors increasing and decreasing
pulmonary vascular resistance
– Provide overview of hemodynamic principles
Highlight
defects
most common congenital heart
Avoidance
Allen HD, Driscoll DJ, Feltes TF, Shaddy RE, eds. Moss and Adams’ Heart Disease in
Infants, Children, and Adolescents, Including the Fetus and Young Adult, ed 7.
Philadelphia, Lippincott Williams & Wilkins, 2008 (see specific defects and Cardiac Intensive
Care chapters in this excellent text).

Castaneda Ar, Jonas RA, Mayer JE, and Hanley FL: Cardiac surgery of the Neonate and
Infant, Philadelphia, 1994, WB Saunders.

Chang AC, Hanley FL, Wernovsky G, Wessel DL, eds. Pediatric Cardiac Intensive Care.
Baltimore, Williams & Wilkins, 2008.

Hazinski MF (Ed), Cardiovascular disorders (with additional authors), In Hazinski MF (Ed),
Nursing Care of the critically ill child, edition 3, Saint Louis 2013, Mosby.

Hazinski MF: Cardiovascular disorders, In Hazinski MF (Ed), Manual of Pediatric Critical
Care, Saint Louis, 1999, Mosby Year Book.

Wilson W, Taubert KA, Gewitz M, and others. Prevention of Infective Endocarditis
Guidelines
From the American Heart Association: A Guideline From the American Heart Association
Rheumatic Fever, Endocarditis, and Kawasaki Disease Committee, Council on Cardiovascular
Disease in the Young, and the Council on Clinical Cardiology, Council on Cardiovascular
Surgery and Anesthesia, and the Quality of Care and Outcomes Research Interdisciplinary
Working Group. Circulation 2007;116:1736-1754.
of stimulation
ALSO:
Administered nitric oxide and other vasodilators
References




Hazinski MF, Nieves J, Fosse G, Rummell M and others. Cardiovascular
problems, In Hazinski MF (Ed), Nursing Care of the Critically Ill Child, ed 3,
Saint Louis, Mosby, In Press.
Nichols DG, Cameron DE, Greeley WJ, Lappe DG, Ungerleider RM and Wetzel
RC: Critical heart disease in infants and children, Edition 3, Saint Louis, 2008,
Mosby Year-Book.
The Society of Thoracic Surgeons. STS Congenital Heart Surgery Data
Summary, 2006-2010 Procedures, All Patients. Available at:
http://sts.org/sites/default/files/documents/STSCONGAllPatientsSummary_Fall2010.pdf
Accessed 03/28/11.
Wernovsky G, Chang AC, Wessel DL, Ravishankar C. Cardiac intensive care. In
Allen HD, Driscoll DJ, Feltes TF, Shaddy RE eds. Moss and Adams’ Heart
Disease in Infants, Children, and Adolescents, Including the Fetus and Young
Adult, ed 7. Philadelphia, Lippincott Williams & Wilkins, 2008.
OXYGEN DELIVERY
References--additional

tidal volume and temperature
Analgesia
Objectives
Provide
oxygenation
Arterial
Oxygen Content:
([Hgb]X sat’n X1.34 ml/Gm)+0.003 X PaO2
Oxygen
Delivery:
O2 Content X C.O./C.I X 10
CYANOTIC CHD
Potential Complications
Polycythemia,
thromboembolic
events
Hypercyanotic episodes
Coagulopathies
Brain abscesses
HYPERCYANOTIC
EPISODES: Characteristics
Hyperpnea
Irritability
Prolonged
crying
Increasing cyanosis
Profound hypoxemia, acidosis
Ultimate loss of consciousness
HYPERCYANOTIC
EPISODES: Treatment
Place
infant in knee-chest position
Administer oxygen
Administer morphine sulfate
Consider sodium bicarbonate or
propranolol
Urgent surgical intervention
NORWOOD STAGE I
FOR HLHS: Goals
Establish
permanent, unobstructed
communication between right
ventricle and aorta/systemic
circulation
Limit pulmonary blood flow
Provide adequate intraatrial
communication
PROSTAGLANDIN E1
Preparation of Infusion
0.3
mg/100 cc =300 mcg/100
cc (3 mcg/cc)
1.0 ml/kg/hr= 0.05 mcg/kg/min
Titrate to effect (improved
pulmonary or systemic blood
flow)
NORWOOD STAGE II
FOR HLHS: Goals
Separation
of pulmonary and
systemic circulations
Assurance of unimpeded
pulmonary venous return and
pulmonary and systemic blood
flow
I