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A Review of
Congenital Heart Disease
February 11, 2008
Presentation
• Symptomatic infants with congenital heart
disease will have one or more of the following
three main presentations:
– Cyanosis
– Heart Failure
– Shock
• Infants with ductal dependent lesions usually
present in the first week of life and are often
critically ill
Presentation
• Infants with heart failure have nonspecific
symptoms and signs, which are frequently very
different from those of older children and adults.
• Symptoms can include poor feeding, weight loss,
FTT, palpitations, apnea, chest pain, syncope, and
fever.
• Signs can include murmurs, hypertension,
dysrhythmias, respiratory distress or failure,
cyanosis, heart failure, and shock.
Presentation: Vitals
• Hypertension
– Can be a manifestation of underlying cardiovascular
diseases including coarctation and aortic stenosis
• Hypotension
– Can be due to poor cardiac output from sepsis or
shock
• A difference in blood pressure and/or pulses
between the extremeties suggests coarctation of
the aorta or an interruption of the aortic arch
Presentation
• Although a significant murmur implies the
presence of cardiac disease, the absence of a
murmur does not rule out underlying heart
disease
• Some heart defects (for example transposition
of the great arteries) may not have a murmur
initially or may have only a nondescript
murmur (e.g. single ventricle)
Physical Exam
• Abnormal heart sounds are a clue that
underlying heart disease is present
• A wide, fixed, split S2 is characteristic of an
atrial septal defect
• With pulmonary atresia or truncus arteriosus,
S2 is single
Physical Exam
• After the first day of life, a systolic ejection
click is always abnormal and is found with
dilation of the aorta or pulmonic trunk
• The murmur of a patent ductus arteriosus is a
continuous, even murmur “machine-like,” best
heard in the left scapular area
Presentation
• Hepatosplenomegaly can occur with right-sided
heart failure
• Palpation of the pulses can reveal bounding
pulses with a wide pulse pressure indicative of a
patent ductus arteriosus or aortic insufficiency, or
the weak thready pulses occurring with low
cardiac output
• Ashen, blue, grey, cold, clubbed, edematous and
clammy extremities suggest a cyanotic lesion
Presentation
• Because specific cardiac defects are associated
with chromosomal abnormalities, abnormal
facies, or skin lesions can be a clue to
underlying congenital heart disease
Heart Failure
• The usual age at which a large left-to-right
shunt will become symptomatic is about six
weeks
Heart Failure
• At birth there is a marked initial decrease in
the pulmonary vascular resistance followed by
a continued slow drop over the next 2-6
weeks of life, with more blood being shunted
from left to right
• Infants with these defects are asymptomatic
at birth, and symptoms appear and gradually
worsen over the next few weeks
Heart Failure
• The classic heart failure triad in infancy is
tachypnea, tachycardia, and hepatomegaly
• Pallor and diaphoresis are additional findings
in infants
• Rales may be heard on auscultation, but the
absence of rales does not rule out heart
failure
Ductal-Dependent
Congenital Heart Defects
• Many patients with congenital heart defects
depend on a patent ductus arteriosus for
blood flow
PDA
• When the ductus arteriosus closes, patients
with ductal-dependent lesions develop
symptoms of acute heart failure and
cardiogenic shock with circulatory collapse
• Closure of the ductus usually occurs during
the first week of life
PDA
• Functional closure occurs in the first 10-14 hours
of life, although anatomic closure can be delayed
for 2-3 weeks because of hypoxia, acidosis, and
prematurity
• The classic presentation of patients with ductaldependent lesions is an infant, asymptomatic at
birth and in the first few hours or days of life,
who develops shock/circulatory collapse toward
the end of the first week of life or in the first few
weeks of life
Who gets Prostin?
• For symptomatic patients with ductaldependent congenital heart defects, a PGE1
infusion will keep the ductus arteriosus open
until definitive therapy can be undertaken via
interventional cardiac catheterization or
surgery
Prostin
• PGE1 is given as an infusion, using the mimimum
effective dose because side effects are dose
dependent and include:
–
–
–
–
–
Apnea
Hypotension
Bradycardia
Seizures
Tremors
• Because of the side effects, patients are usually
intubated prior to beginning PGE1
PDA in preemies
• PDA is the most common cardiovascular abnormality in
preterm infants
• It occurs in up to 60% of infants born at less than 28 weeks
gestation
• A significant L  R shunt through a PDA increases the
morbidity and mortality of these infants.
• Cyclooxygenase inhibitors such as indomethacin or
ibuprofen are used to induce closure of the PDA in preterm
infants
Cyanotic Infants
• The “five Ts” are common etiologies of
cyanotic congenital heart disease:
–
–
–
–
–
Tetralogy of Fallot
Transposition of the great arteries
Truncus arteriosus
Tricuspid valve abnormalities
Total anomalous pulmonary venous return
• The letter “S” is added to include severe or
“critical” pulmonic stenosis
Cyanotic lesions
• The first priority is to determine whether the
etiology of the cyanosis is cardiac or noncardiac
• The hyperoxia test or oxygen challenge test can
make this clear.
• The patient is placed on 100% oxygen:
– If pulmonary disease is present, the PaO2 should rise
by 30 mmHg or to >150 mmHg, and the pulse ox
should rise by at least 10%
– There will be negligible or minimal improvement if the
cyanosis is due to CHD
Cyanotic CHD with Decreased
Pulmonary Blood Flow
• Cyanotic CHD with decreased pulmonary
blood flow include:
– Severe tetralogy of Fallot
– Pulmonary atresia
– Tricuspid atresia
– Severe Ebstein anomaly
– HRHS
– Critical or severe pulmonic stenosis
Cyanotic CHD with Decreased
Pulmonary Blood Flow
• These lesions usually are ductal dependent
• These patients will present critically ill and
cyanotic (and sometimes with heart failure
and shock) in the first few hours or days of life
when the ductus arteriosus closes
• The management of such infants involves
supporting the ABCs and administering PGE1
Tetralogy of Fallot
• Tetralogy of Fallot is the most common
cyanotic congenital heart defect after infancy,
accounting for 5%-10% of all congenital heart
disease
• It consists of:
– VSD
– Obstruction of the RV outflow tract
– Overriding aorta
– RVH
Tetralogy of Fallot
Tetralogy of Fallot
• Patients with TOF usually present as older infants
with paroxysmal hypercyanotic attacks (tet spells)
• However, TOF can present in neonates, critically
ill and severely hypoxic with extreme pulmonic
stenosis
• These infants are dependent on a PDA for
pulmonary blood flow, and prostin is necessary
Tetralogy of Fallot
• Conversely are the “pink tets” who have mild
right ventricular outflow tract obstruction and
present with heart failure from the large LR
shunt and have little or no cyanosis
• Older infants and children can present with a
tet spell, cyanosis, murmur, exercise
intolerance, dyspnea on exertion, clubbing,
poor growth, or failure to thrive
Tetralogy of Fallot
• Classic physical findings include:
– Holosystolic VSD murmur at the left 3rd intercostal
space
– A systolic diamond-shaped pulmonic stenosis
murmur at the left 2nd intercostal space
– An abnormal second heart sound split with a soft
P2
Tetralogy of Fallot
• Chest radiograph findings are:
– cardiomegaly (especially RV)
– Decreased pulmonary vascularity
– The characteristic “boot-shaped” heart created by
a concavity in the left heart border usually
occupied by the pulmonary artery
“Boot
Shaped”
Tetralogy of Fallot
• “Bootshaped”
Tetralogy of Fallot
Tetralogy of Fallot
• Tet spells occur especially during the first 2
years of life
• They can be precipitated by any physical
activity or can occur spontaneously and can
last a few minutes or for hours
• Since there is a fixed right ventricular outflow
tract obstruction, increased right-to-left
shunting occurs
Tetralogy of Fallot
• Factors presdisposing to a tet spell include:
– Dehydration
– Anemia
– Acidosis
– decreased systemic vascular resistance
– increased catecholamine levels
Tetralogy of Fallot
• Treatment:
– Calming the child
– Supplemental oxygen
– Morphine calms/sedates the child, depresses
respiration, decreases SVR, and decreases RVOT
obstruction
– Volume infusion can increase the RV preload and
correct systemic hypotension
– Propranolol is given as a negative inotropic on the
RVOT
Tricuspid Valve Abnormalities
• Tricuspid valve abnormalities include:
– Tricuspid valve stenosis
– Tricuspid valve displacement (Ebstein anomaly)
– HRHS
Ebstein Anomaly
• An Ebstein anomaly is the downward
displacement of an abnormal TV into the RV
• May be due to maternal use of lithium
• Part of the TV—the anterior cusp—has some
attachment to the valve ring, and the other
valve leaflets are attached to part of the RV wall
Ebstein Anomaly
Cyanotic Congenital Heart Disease
with Increased Pulmonary Blood Flow
• Includes:
– Transposition of the great arteries
– Truncus arteriosus
– Total anomalous pulmonary venous return
Transposition of the Great Arteries
• Most frequent diagnosis in critically ill
neonates with cardiac disease
• Pulmonary artery arises from the LV and the
aorta from the RV
Transposition of the Great Arteries
Transposition of the Great Arteries
• Some mixing of blood is needed for survival
through a:
– Patent foramen ovale,
– PDA, or
– VSD
Transposition of the Great Arteries
• “egg on a string”
appearance
– Narrow
mediastinum
and small
thymus
– Narrow cardiac
silhouette
Truncus Arteriosus
• A single arterial trunk overrides a VSD and
receives mixed arterial and venous blood from
the RV and LV
• This one arterial trunk provides blood flow to
the systemic, pulmonary, and coronary
circulations
• The one “truncal” valve is an abnormally
formed semilunar valve
Truncus Arteriosus
• First few hours and days of life:
– Pulmonary vascular resistance is highpulmonary
blood flow is normal
• Later during first month of life:
– Pulmonary blood flow increases with the
postnatal decrease in pulmonary vascular
resistanceheart failure
Truncus Arteriosus
• Cyanosis can be mild because of the increased
pulmonary blood flow
• Eventually Eisenmenger syndrome occurs if
untreated
Truncus Arteriosus
Truncus Arteriosus
Total Anomalous
Pulmonary Venous Return
• The pulmonary veins return to and enter a
structure other than the left atrium
• Can be partial:
– Some oxygenated blood enters the LA, and some
enters another anomalous structure
– acyanotic
Total Anomalous
Pulmonary Venous Return
• Can be total:
– None of the pulmonary veins enter the LA
– Cyanotic: complete mixing of both systemic and
pulmonary venous return either at or before the
RA
– The mixed blood in the RA either is ejected into
the RV or through an ASD or PFO into the LA
Total Anomalous
Pulmonary Venous Return
• When the anomalous pulmonary veins enter
the brachiocephalic vein and the persistent
left superior vena cava, there is a “snowman”
or “figure 8” appearance created by a large
supracardiac shadow along with the normal
cardiac shadow
Total Anomalous
Pulmonary Venous Return
• “Snowman”
or “Figure 8”
Eisenmenger Syndrome
• When a large LR shunt eventually causes
increased pulmonary blood flow and volume
overload to the lungs, resulting in pulmonary
vascular disease/pulmonary hypertension
• This causes a reversal to a RL shunt and
cyanosis, often by teenage years or as a young
adult
Eisenmenger Syndrome
• Symptoms:
– Cyanosis
– Exertional dyspnea
– Fatigue
– Hemoptysis
– palpitations
Eisenmenger Syndrome
• Physical exam reveals a loud P2 from
pulmonary HTN
• The CXR will reveal decreased pulmonary
vascularity
• EKG shows RVH
• Therapy involves pulmonary vasodilator
therapy
References
• Mace, S. Broken Hearts: Infants With Acyanotic
Congenital Heart Disease. CDEM. 2007; 21(11): 29.
• Mace, S. Broken Hearts: Infants With Cyanotic
Congenital Heart Disease. CDEM. 2007; 21(11):
12-23.
• Multimedia Library. Childrens Hospital Boston.
http://www.childrenshospital.org/
• Beerman, L; et al. Cardiology. Atlas of Pediatric
Physical Diagnosis. Fifth Edition. 2007; 127-160.