Congenital heart disease II

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

UTI Pediátrica & Neonatal


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

Dr. Antonio Souto

acasouto@terra.com.br

2013



• Ventricular septal defect
Relative
Frequency of • Atrial septal defect
• Patent ductus arteriosus
Lesions %
•
•
•
•
•
•
•
•
•
•
•
•
Dr. Antonio Souto

25-30
6-8
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
Truncus arteriosus
Total anomalous pulm venous return
Tricuspid atresia
Double-outlet right ventricle
Others

1-3
1-2
1-2
1-2
1-2
5-10
2013



Left Ventricular Outflow Tract Obstruction
Major source of neonatal M&M from CHD

•10% of infant mortality
•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
Dr. Antonio Souto


2013



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.
Dr. Antonio Souto


2013



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.

Dr. Antonio Souto


2013



Formulation of the problem

?
a great concern to
pediatricians

Dr. Antonio Souto


2013



Clinical Presentation of CHD in the Neonate
•
•
•
•
•

Fetal Diagnosis
Cyanosis
CHF/Shock/Circulatory Collapse
Arrhythmia
Asymptomatic Heart Murmur

Dr. Antonio Souto


2013



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.
Dr. Antonio Souto


2013



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).

Dr. Antonio Souto


2013



Cardiac malformations - 10% of infant mortality
Most common lethal diagnosis:

Left ventricular outflow tract
obstruction
•Hypoplastic left heart syndrome
•Coarctation of aorta
•Aortic stenosis

Dr. Antonio Souto


2013



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

Dr. Antonio Souto


2013



Typically, 2 g/dL of reduced hemoglobin
5g/dL of reduced Hb
clinical cyanosis

75%
65%

35%

Dr. Antonio Souto

25%


2013



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

Dr. Antonio Souto


2013



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).

Dr. Antonio Souto


2013



5 “T’s”
Most common cyanotic lesions of the newborn

•
•
•
•
•

Total Anomalous Pulmonary Veins
Tetrology of Fallot
Tricuspid Atresia
Transposition
Truncus Arteriosus

Dr. Antonio Souto


2013



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).

Dr. Antonio Souto


2013


Dr. Antonio Souto



2013



•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.

Dr. Antonio Souto


2013



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

Dr. Antonio Souto


2013



60%

Dr. Antonio Souto


2013



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
Dr. Antonio Souto


2013



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)

Dr. Antonio Souto


2013



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

Dr. Antonio Souto


2013



Right sided obstruction

Dr. Antonio Souto


2013



Left sided obstruction

Dr. Antonio Souto


2013



Inadequate Mixing
Survival Depends Upon
Mixing Between
Systemic and Pulmonary
Circuits

Dr. Antonio Souto


2013



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.
Dr. Antonio Souto


2013



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

Dr. Antonio Souto


2013



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.
Dr. Antonio Souto


2013



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.

Dr. Antonio Souto


2013



FLUID DYNAMICS

Dr. Antonio Souto


2013



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
Dr. Antonio Souto


2013



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

Dr. Antonio Souto


2013



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?

Dr. Antonio Souto


2013



• 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%)
Dr. Antonio Souto

CYANOTIC
Tetralogy of Fallot (5%)
Transposition of the great
arteries (5%)
Atrioventricular septal defect –
complete (2%)
Other complex – 20%


2013



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.
Dr. Antonio Souto


2013



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

Dr. Antonio Souto


2013



Maternal Risk Factors
•
•
•
•
•

Diabetes mellitus
PKU
Hyperthyroidism
Lupus, collagen vascular disease
Rubella, CMV, Coxsackie, Parvovirus

Dr. Antonio Souto


2013



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

Dr. Antonio Souto


2013



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.
Dr. Antonio Souto


2013



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 ?
Dr. Antonio Souto


2013



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

Dr. Antonio Souto


2013



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

Dr. Antonio Souto


2013



Cyanosis
• Pulmonary X cardiac problems ?

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

Dr. Antonio Souto


2013



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

Dr. Antonio Souto


2013



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
Dr. Antonio Souto


2013



HYPEROXIA TEST
GIVE 100% O2
ASSES PO2
PO2>200

PO2<150

NO CCHD

CCHD

PASS

FAIL
150-200
?CCHD WITH PBF OR PPHN

Dr. Antonio Souto


2013



What information do we require?

– 4 extremity BP’s
– H&P
• Murmurs
• Organomegaly
• Pulses
• ECG
• Labs, CXR findings, saturations

Dr. Antonio Souto


2013



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

Dr. Antonio Souto


2013



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

Dr. Antonio Souto


2013



Murmurs
•
•
•
•
•

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

Dr. Antonio Souto


2013



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

Dr. Antonio Souto


2013



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.
Dr. Antonio Souto


2013



Diagnosis
Chest x ray

Dr. Antonio Souto


2013



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.

Dr. Antonio Souto


2013



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
Dr. Antonio Souto


2013



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.

Dr. Antonio Souto


2013



Diagnostic ladder

Dr. Antonio Souto


2013



•Clinical evaluation with CXR and Hyperoxia test
excludes CHD in most cases.

•Echocardiography recommended
in all doubtful cases.
•

% exames negativos (normais)

Dr. Antonio Souto


2013



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


2013



Hypercyanotic spells
Cyanotic heart diseases

• Tetralogy of Fallot
• Pulmonary atresia
• Transposition of great arteries
• Tricuspid atresia

Dr. Antonio Souto


2013



• 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

Dr. Antonio Souto


2013



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

Dr. Antonio Souto


2013



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

Dr. Antonio Souto


2013



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

Dr. Antonio Souto


2013



• 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.

Dr. Antonio Souto


2013



Keep in your mind

Dr. Antonio Souto


2013



•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)

Dr. Antonio Souto


2013



Thanks a
lot!!!

Dr. Antonio Souto


2013

Congenital heart disease II

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