Congenital heart disease 2013

Congenital heart disease
(Formulation of the problem)
Antonio Souto
acasouto@bol.com.br
Médico coordenador
Unidade de Medicina Intensiva Pediátrica
Unidade de Medicina Intensiva Neonatal
Hospital Padre Albino
Professor de Pediatria nível II
Faculdades Integradas Padre Albino
Catanduva / SP

Dr. Antonio Souto acasouto@terra.com.br 2013
UTI Pediátrica & Neonatal Hospital Padre Albino
Congenital heart diseases are a dynamic group of
anomalies that originate in fetal life and change
considerably during postnatal development.
Routine neonatal examination fails to
detect more than half of babies with
heart disease; examination at 6 weeks
misses one third.

Early recognition, urgent identification and timely
referral to a pediatric cardiologist and timely
intervention has great implications in prognosis,
is the key in reducing mortality
and morbidity.

Congenital heart disease in the
newborn requiring
early intervention ????
Life threatening heart diseases may not have obvious
evidence early after birth, the diagnosis is difficult
sometimes and always a great concern to pediatricians.
High index of suspicion is essential
to decision making.

Clinical Presentation of CHD in the Neonate
• Fetal Diagnosis
• Cyanosis
• CHF/Shock/Circulatory Collapse
• Arrhythmia
• Asymptomatic Heart Murmur

Formulation of the problem
?
a great concern to
pediatricians

Classification of CHD (clinical point of view)
1.Life-threatening CHD
-Cardiovascular collapse is likely and compromised if not
treated early
Transposition of the great arteries (TGA), critical
pulmonary and aortic valvular stenosis/atresia,
hypoplastic left heart syndrome (HLHS),
obstructed total anomalous pulmonary venous
return (TAPVR).

Classification of CHD (clinical point of view)
2. Clinically significant CHD
-Cardiac malformations that have effects on heart function but where
the collapse is unlikely to be need early intervention.
Ventricular septal defect (VSD), complete atrioventricular
septal defect (AVSD), atrial septal defect (ASD) and
tetralogy of Fallot (TOF) with good pulmonary artery
anatomy.
3. Clinically non-significant CHD
-No functional and clinical significance.
Small VSD, atrial septal defect (ASD), mild pulmonary stenosis (PS).

• Total Anomalous Pulmonary Veins
• Tetrology of Fallot
• Tricuspid Atresia
• Transposition
• Truncus Arteriosus
55 ““TT’’ss””
Most common cyanotic lesions of the newborn

Cardiac malformations - 10% of infant mortality
Most common lethal diagnosis:
Left ventricular outflow tract
obstruction
•Hypoplastic left heart syndrome
•Coarctation of aorta
•Aortic stenosis

How do we improve the detection of babies
with congenital heart disease ?
•Understanding normal changes at birth
•Simple way to think about congenital heart disease
•Systematic examination of the cardiovascular system
•Simple additional screening
•Robust care pathways

Pulse Oximetry
•easy to use, harmless when done correctly
•accuracy of 2% in the range of 70 to 100%
•consider cyanotic when Sat <94% at 24 hours of age
•should be obtained prior to discharge from nursery
= Policy at Sanford Children’s
Measure sat in foot
If <95%, gets evaluation

FLUID DYNAMICS
The function of the human heart is that of a mechanical
pump that receives the low pressure blood from the venous
system and ejects it with higher pressure into the arterial
system.

Diagnosis
Applied clinical logic
Heart and circulation
Perfect harmony between structure and function
Logical thought
Gross morphology / physiologic derangements
Clinical manifestation
Accurate observation + Correct inferences

Logical thought
Gross morphology / physiologic derangements
-Right side
Start proximally (vena cavae) and
end distally (pulmonary arteries)
-Left side
Start proximally (pulmonary veins) and
end distally (Aorta)
-What is the level of shunt?
Acyanotic (left to right)
Cyanotic (right to left)
Atrial, ventricular, great artery

General Approach to CHD Patient
1. Define cardiovascular pathology
2. Predict pathophysiology
3. Determine hemodynamic goals
4. Anticipate emergency treatments

Rosen: “any neonate
in shock that does not
respond to fluids
or pressors has
LV outflow obstruction
until proven otherwise”

Evaluation for and treatment of
presumptive sepsis should be
undertaken simultaneously with
evaluation for cardiac and pulmonary
disease.

What Are The Odds?
• Congenital Heart Disease
8/1000 live births
• “Critical” CHD
3/1000 live births
• In the USA:
~ 32,000 children born/year with CHD
~ 11,000/year with “Critical” CHD

Cardiac malformations - 10% of infant mortality
Most common lethal diagnosis:
Left ventricular outflow tract obstruction
•Hypoplastic left heart syndrome
•Coarctation of aorta
•Aortic stenosis

Left Ventricular Outflow Tract Obstruction
Major source of neonatal M&M from CHD
•Accounts for ~ 12% of congenital cardiac disease in
infancy
•~ 75% discharged from hospital w/o diagnosis
•~ 65% - normal newborn screen examination
•6% died before diagnosis
•96% symptoms by 3 wks of life

Relative
Frequency of
Lesions %
• Ventricular septal defect 25-30
• Atrial septal defect 6-8
• Patent ductus arteriosus 6-8
• Coarctation of aorta* 5-7
• Tetralogy of Fallot 5-7
• Pulmonary valve stenosis 5-7
• Aortic valve stenosis * 4-7
• Transposition of great arteries 3-5
• Hypoplastic left ventricle * 1-3
• Hypoplastic right ventricle 1-3
• Truncus arteriosus 1-2
• Total anomalous pulm venous return 1-2
• Tricuspid atresia 1-2
• Double-outlet right ventricle 1-2
• Others 5-10

•The neonatal myocardium has fewer myofibrils in a
disordered pattern, making the myocardium stiffer.
•The neonatal heart follows the Frank e Starling
relationship but with a limited increase in stroke
volume for a given increase in ventricular filling
volume.
•The neonatal myocardium is dependent
on heart rate to increase cardiac output.

•Near peak of Starling curve
•Stroke volume relatively fixed
•C.O. relatively heart rate dependent

60%

Ductus Arteriosus
•Shunt between the descending aorta to the left
pulmonary artery
•Open because low PaO2 and
circulating prostaglandins (PGE2)
•Ductus closes within the first
days (24/48 h) of life in the
term infant
•Permanent closure due to fibrosis
takes 4-6 weeks

Ductus Arteriosus
When patent ductus arteriosus (PDA) is
opened widely, many serious
malformations may not be noticed easily in
the early life.
Most of anomalies compatible with six months of
intrauterine life permit live offspring at term (Fetal
circulation)

Ductal-dependent Heart Disease ?
Inadequate systemic oxgenation /
pulmonary blood flow due to heart disease
• Inadequate pulmonary blood flow
• Inadequate systemic delivery of oxygenated blood
• Inadequate mixing

Right sided obstruction

Left sided obstruction

Inadequate Mixing
Survival Depends Upon
Mixing Between
Systemic and Pulmonary
Circuits

Ductus Arteriosus
•Congenital heart disease in which either pulmonary or systemic
blood flow is dependent on shunting through the ductus arteriosus.
•Postnatally closure of the ductus arteriosus would be fatal, progress
as severe acidosis/shock/cyanosis.
•Prostaglandin E1 (PGE1 or Alprosdatil™)
allow stabilization.
•PGE1 must be started immediately after
delivery.

Prostaglandin E 1
•Always given as continous IV infusion.
•Start at 0.05-0.1µg/kg/min, can be reduced to 0.005 -
0.01µg/kg/min once duct is opened
•Efficacy ↓ with ↑ age, less effective after 2 weeks of
life, not effective after 4 weeks
•Continous cardiorespiratory monitoring

Ductus Arteriosus
•Before anatomic closure of the ductus arteriosus and
foramen ovale, certain stresses can cause the newborn
to revert to fetal circulation
•Increased pulmonary vascular reactivity, raised PVR
(Pulmonary Hypertension) and right-to-left shunting at the
PFO and PDA, the clinical result is cyanosis.
Hypothermia, hypercarbia, acidosis,
hypoxia and sepsis can all cause a
reversion to fetal circulation.

Cyanosis
Chronically adapted to the hypoxia in the uterine life,
newborn infants are able to tolerate some degree of
cyanosis than older infants or children

Typically, 2 g/dL of reduced hemoglobin
5g/dL of reduced Hb clinical cyanosis
35%
65%
25%
75%

Cyanosis
Category of cyanotic CHD
decreased pulmonary flow with right to left shunting
lesions (PA, TA with shunting at the atrial or ventricular
level)
poor mixing lesions (transposition physiology)
right to left shunt with intra cardiac mixing lesions
(TAPVR, single ventriclular physiology, truncus
arteriosus).

Formulation of the problem
Basic questions
1.Is the patient acyanotic or cyanotic?
2.How is body/pulmonary arterial blood flow ?
3. Does the malformation originate in the left or right
side of the heart?
4. Which is the dominant ventricule?
5. Is pulmonary hypertension present or not?

• Commonly divided into acyanotic and cyanotic
• 9 common conditions
ACYANOTIC
LEFT RIGHT SHUNTS
Ventricular septal defect (30%)
Patent ductus arteriosus (12%)
Atrial septal defect (7%)
OUTFLOW
OBSTRUCTION
Pulmonary stenosis (7%)
Aortic stenosis (5%)
Coarctation of the aorta (5%)
CYANOTIC
Tetralogy of Fallot (5%)
Transposition of the great
arteries (5%)
Atrioventricular septal defect –
complete (2%)
Other complex – 20%

Clinical manifestations
-The clinical sign in the neonate may be vague
For pediatricians:
-identify the newborn “not doing well”
•Persistent central cyanosis, unexplained acidosis, tachypnea without
lung problems, etc.
•Assessment of saturation monitoring, status of perfusion (blood gas
analysis) and pulses/blood pressures in all extremities.

Maternal Risk Factors
• Congenital heart disease
• Cardiac teratogen exposure
– Lithium
– Amphetamines
– Alcohol
– Anticonvulsants: phenytoin, valproic acid,
carbamazepine, and trimethadione
– Isotretinoin

Maternal Risk Factors
• Diabetes mellitus
• PKU
• Hyperthyroidism
• Lupus, collagen vascular disease
• Rubella, CMV, Coxsackie, Parvovirus

Fetal Risk Factors
• Trisomies, Turner’s syndrome, abnormal karyotype
• Congenital malformations: duodenal atresia, TEF,
omphalocele, diaphragmatic hernia, renal dysgenesis,
and hydrocephalus
• Fetal arrhythmias
• IUGR
• Nonimmune hydrops
• ?2 vessel cord

Dyspnea
• Lung or heart problems?
• Large shunt lesions:dyspnea, tachypnea, feeding
difficulty, irritability and distress.
• Ventilator weaning can be difficult in premature infants
with large left to right cardiac shunts.
Cyanosis with markedly reduced pulmonary
blood flow usually leads to "quiet tachypnea”,
without significant respiratory distress.

Sign of poor perfusion
• Ductus dependent systemic circulatory ?
• Progressive dyspnea, cold, clammy mottled skin, which
indicates poor perfusion and acidosis, shock, oliguria
• Cardiovascular collapse at the time of ductal closure
•Shock in newborn ?

Cyanosis
•Central cyanosis
•noted in the trunk, tongue, mucous membranes
•due to reduced oxygen saturation
•Peripheral cyanosis
•noted in the hands and feet, around mouth
•due to reduced local blood flow

central peripheral
CAUSE ARTERIAL BLOOD
DESATURATION OR
ABNORMAL Hb
CUTANEOUS
VASOCONSTRICTION
DUE TO LOW CO
CONDITIONS Seen in R-L shunt,
impaired pulmonary
function, abnormal Hb
exposure to cold air or
water and abnormally
greater extraction ofO2
from normally saturated
blood
SITES conjunctiva,palate,tongue,
inner side of lips& cheeks
limited to
ears,nose,cheeks outer
side of lips hands
feet&digits
certainly central if
associated with clubbing
and polycythemia,
clubbing is absent
probably central if it
deepens on effort

Cyanosis
• Pulmonary X cardiac problems ?
• Persistent hypoxia refractory to 100%
oxygen supply would indicate cyanotic CHD
rather than pulmonary problems.
• Hyperoxia test

Hyperoxia test
arterial blood gas analysis while 100% oxygen
• PaO2 > 220 mm Hg would suggest respiratory disease
• PaO2 100‒220 mm Hg would require evaluation for
cyanotic CHD
• PaO2 < 100 mm Hg would suggest cyanotic CHD
• PaO2 < 40‒50 mm Hg would be likely to have a poor
mixing disease such as TGA

HYPEROXIA TEST
GIVE 100% O2
ASSES PO2
PO2>200 PO2<150
NO CCHD CCHD
PASS FAIL
150-200
?CCHD WITH PBF OR PPHN

Additional screeningAdditional screening
•• PulsePulse oximetryoximetry
–– PostPost ductalductal
saturationssaturations
–– < 95% warning< 95% warning
signsign
–– PULSOX studyPULSOX study
DUCTDUCT
RR
handhand
L Hand andL Hand and
both feetboth feet

What information do we require?
– 4 extremity BP’s
– H & P
• Murmurs
• Organomegaly
• Pulses
• ECG
• Labs, CXR findings, saturations

The “Noncardiac” Cardiac Exam
• Vital signs, growth percentiles
• UE/LE blood pressure & pulse oximetry
• Color - cyanosis, pallor, mottling
• Lungs - work of breathing, rate, equality, crackles
• Abdomen - hepatomegaly, situs
• Extremities - pulses, capillary refill time
• Dysmorphic features, other organ system abnormalities

Initial evaluation of child’s heart
•Listen to heart first when/if infant quiet
•First concentrate on S1 and especially S2
•Louder than normal?
•Split normally?
•Systolic murmur:
•Diastolic murmur?
•Widely radiating murmur?
•Palpate liver
•BP in arm and leg
•Tongue - cyanosis

Murmurs
• Loudness graded 1-6. Presence of thrill > 4
• Timing – systolic/diastolic
• Duration – ejection/mid/pansystolic
• Site where loudest
• Radiation

Grading of murmurs
• Grade 1: only a cardiologist can hear
• Grade 2: murmur softer than S1/S2
• Grade 3: murmur louder than S1/S2
• Grade 4: thrill palpable
• Grade 5: murmur audible with stethoscope partially
off chest
• Grade 6: murmur audible with stethoscope
completely off chest

Diagnosis
Chest x ray
• Usually performed to rule out pulmonary disease as
well as to evaluate pulmonary vascular marking and
cardiomegaly.
• Some CHD has characteristic features
• Most of the serious CHD have no specific findings
except vague cardiomegaly, change of pulmonary
vascular marking and subtle finding of pulmonary
venous congestion.

Diagnosis
Electrocardiography
EKG has been considered a useful tool in the diagnosis of
CHD,especially if echocardiogram is not easily available.
Ventricular maturation and associated ECG changes
• The fetal heart is right-side dominant
• Right axis deviation and R wave dominance in lead V1 and S wave
dominance in lead V6.
• At 3 e 6 months the classical left ventricular dominance pattern of
adulthood is established as ventricular hypertrophy occurs in
response to increased systemic vascular resistance.

Diagnosis
Echocardiography
Echocardiogram is the most valuable method in the
diagnosis of CHD.
• Identification of cardiac anatomy
• Assessment of systolic ventricular function
• Measurement of chamber dimensions and wall thickness
• Assess the pressure gradients across the stenotic or regurgitation flow
through the valves
• Assess abnormal cardiac physiology
• Flow in the descending aorta
• Estimation of pulmonary arterial pressure
• Defining the direction of flow when valve regurgitation and shunt exist

Diagnosis
Cardiac Catheterization
• The diagnostic frequency of cardiac catheterization is
relatively decreasing especially in the neonate.
• It is still the key in defining certain anatomic variants
difficult to be delineated by echocardiography alone
• Therapeutic catheterizations are considered as one of
the life savingmodalities in some fields.

Diagnostic ladder

•Clinical evaluation with CXR and Hyperoxia test
excludes CHD in most cases.
•Echocardiography recommended in all doubtful
cases.
•Prior stabilization and a monitored transport to
tertiary center ensures a optimal pre-operative
state.
•Early intervention with very encouraging results
is realistic for most forms of critical CHD in
newborns

Consultation: may be more cost-
effective! 95% sens/spec for
discriminating CHD from innocent
murmur

Hypercyanotic spells
Cyanotic heart diseases
• Tetralogy of Fallot
• Pulmonary atresia
• Transposition of great arteries
• Tricuspid atresia

• Sudden severe episodes of intense cyanosis caused by
reduction of pulmonary flow
• The level of cyanosis and onset of cyanotic spell is
determined the SVR & level of PS component

Clinical Presentation
• peak incidence age: 3 to 6 months
• often in the morning, can be precipitated by crying,
feeding or defecation
• severe cyanosis, hyperpnoea, metabolic acidosis
• in severe cases, may lead to syncope, seizure, stroke or
death
• there is a reduced intensity of systolic murmur during spell

Management
• treat this as a medical emergency
• knee-chest/squatting position:
- place the baby on the mother’s shoulder with the knees tucked up
underneath.
- this provides a calming effect, reduces systemic venous return
and increases systemic vascular resistance
• administer 100% oxygen
• give IV/IM/SC morphine 0.1 – 0.2 mg/kg to reduce
distress and hyperpnoea

Management
• IV Propranolol 0.05 – 0.1 mg/kg
• IV Esmolol 0.5 mg/kg slow bolus over 1 min,
followed by 0.05 mg/kg/min for 4 mins.
• volume expander, crystalloid, 20 ml/kg rapid IV push to
increase preload
• give IV sodium bicarbonate 1 mEq/kg to correct metabolic
acidosis
• heavy sedation, intubation and mechanical ventilation

• a single episode of hypercyanotic spell is an
indication for early surgical referral
(either total repair or Blalock Taussig shunt)
• oral propranolol 0.2 – 1 mg/kg/dose 8 to 12 hourly
should be started soon after stabilization while
waiting for surgical intervention.

Thomas-Blalock-Taussig Shunt
Vivien Thomas, Partners of the Heart, 1998 and
Something the Lord Made - Best Made-for-TV Movie, 2004
Helen Taussig
Alfred Blalock
Vivien Thomas

November 29, 1944
Thomas-Blalock-Tuassig

Keep in your mind

•Routine neonatal examination fails to detect more than
half of babies with heart disease
•High index of suspicion is essential to decision making
•“not doing well”
•Any neonate in shock that does not respond to fluids or
pressors has LV outflow obstruction until proven otherwise
•If you think you have a ductal dependent lesion
PGE1 must be started immediately
(don’t be afraid of prostin)

Thanks a
lot!!!

Congenital heart disease 2013

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