Transcript INTERACTIVE CASE 4 CARDIAC B

Section 3D-4
Sabalvaro, D.K., Salac, C.N., Salazar, J., Salazar, R., Salcedo, V.E., Saldana, E., Sales, M.S.A., Salonga,
C.A., San Diego, P., San Pedro, R.
INTERACTIVE CASE 4
CARDIAC B
1
OBJECTIVES
• To present a case of a 2 year old “blue baby”
• To give the clinical impression and differential diagnosis
of the case based on the history and physical
examination
• To present the Chest X ray of the patient
• To discuss the pathophysiology and symptomatology of
Tetralogy of Fallot
• To discuss other diagnostic procedures that may be
useful in TOF
• To discuss the management and prognosis of a patient
with TOF
2
OBJECTIVES
• To present a case of a 2 year old “blue baby”
• To give the clinical impression and differential diagnosis
of the case based on the history and physical
examination
• To present the Chest X ray of the patient
• To discuss the pathophysiology and symptomatology of
Tetralogy of Fallot
• To discuss other diagnostic procedures that may be
useful in TOF
• To discuss the management and prognosis of a patient
with TOF
3
GENERAL DATA
 B.B.
 3 year old male
 “blue baby”
 Chief complaint: progressive cyanosis
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HISTORY OF PRESENT ILLNESS
 Born “ blue baby”
 Unspecified heart disease
1 week PTA
More bluish lips
(+) easy fatigability
(+) seen to sit down after a
short walk
Few hours PTA
While feeding, patient
developed bluish hue of the
face and extremities
ADMISSION
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REVIEW OF SYSTEMS
 Poor appetite, poor weight gain
 No skin lesions; cyanotic since birth
 Shortness of breath on exertion
 “Bluish” episodes while feeding
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PAST MEDICAL HISTORY
 Born a “blue baby”
 Poor birth weight, retarded growth
 Occasional visit to ER due to cyanosis when
crying
 (-) Asthma/allergies
 (-) Previous surgeries
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FAMILY HISTORY
 (-) Heart disease
 (-) Diabetes
 (-) Asthma/allergies
 (-) PTB
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PERTINENT PHYSICAL EXAMINATION
 Conscious, agitated
 BP 90/60, HR 90 bpm, RR 30’s, small for age
 Warm moist skin, no dermatoses
 Bluish lip and buccal ,mucosa
 Heart:
(+) thrill along left sternal border
(+) harsh systolic murmur
Single S2
 Extremities: bluish nail beds, (+) clubbing
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MISSING DATA
 Obstetrical History: Illnesses of mother
during gestation
 Episodes of “tet” spells - marked increase in
cyanosis followed by syncope
 Squatting (?)
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OBJECTIVES
• To present a case of a 2 year old “blue baby”
• To give the clinical impression and differential diagnosis
of the case based on the history and physical
examination
• To present the Chest X ray of the patient
• To discuss the pathophysiology and symptomatology of
Tetralogy of Fallot
• To discuss other diagnostic procedures that may be
useful in TOF
• To discuss the management and prognosis of a patient
with TOF
11
SALIENT FEATURES
 3 y/o Male
 Cyanotic since birth
 Difficulty in feeding, poor birth weight,
retarded growth
 (+) dyspnea and easy fatigability
 (+) clubbing, cyanosis of the lips, buccal
mucosa and nail beds
 Heart: (+) thrill along left sternal border
(+) harsh systolic murmur
single S2
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CLINICAL IMPRESSION
Congenital Cyanotic Heart Disease,
probably Tetralogy of Fallot
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DIFFERENTIAL DIAGNOSIS
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Transposition
of Great
Vessels
Total
Anomalous
Pulmonary
Venous
Return
Tetralogy
of Fallot
Cyanosis
Ebstein’s
Anomaly
Truncus
Arteriosus
Hypoplastic
Left Heart
Syndrome
Tricuspid
Atresia
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Congenital Anomaly
Transposition of
Great Vessels
Total Anomalous
Pulmonary Venous
Return
Pathology
Signs and
Symptoms
Chest Radiograph
Two major arteries
leaving the heart are
connected to the wrong
ventricles or lower
chambers of the heart.
Blue or cyanotic
shortly after birth. The
blueness doesn't go
away even if the baby is
given extra oxygen.
Shortness of breath
Poor feeding
Clubbing of the
fingers or toes
 Increased vascularity
Cardiomegaly
Cardiac silhouette:
“egg on its side”; “apple
on a stem”
 Narrow vascular
pedicle
None of the
pulmonary veins are
connected to the left
atrium but instead all
drain by various routes
into the right atrium.
Cyanosis and
respiratory distress
 Cyanosis made worse
on feeding, especially
with the infracardiac
type caused by the
compression of the
common pulmonary
vein by the food-filled
esophagus.
(-) Cardiac murmur
 Increased pulmonary
vascular markings
which, if untreated,
progress to cardiac
failure.
 A 'snowman' sign or
figure of 8 configuration
in the supracardiac
type but rarely before 4
months of age.
Type1: supracardiac
Type2: intracardiac
Type3: infracardiac
Reference: http://www.bcm.edu/radiology/cases/pediatric/text/2i1A.htm
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Signs and
Symptoms
Congenital Anomaly
Pathology
Truncus Arteriosus
 Only one artery connects
to the heart instead of two.
Large VSD over which a
large, single great vessel
(truncus) arises. This single
great vessel carries blood
both to the body and to
lungs.
Blood from both
ventricles mixes together
as it all exits through the
single valve exiting from
the heart.
Bluish cast to their skin,
especially around the nose
and mouth.
Breathlessness
Rapid breathing
Excessive sweating
Restlessness
 Increased vascularity
Pulmonary venous
edema
Cardiomegaly
Left atrium and
either/both ventricles
prominence
Concave main pulmonary
artery segment
Large aortic shadow
Right aortic arch in 35%
Complete lack of
formation of the tricuspid
valve with absence of direct
connection between the
right atrium and right
ventricle.
Congestive heart failure
Growth retardation
Poor skin coloration
(pallor to frank cyanosis)
Inability to complete a
feeding session
Frequent pauses during
feeding, and/or frank
anorexia
Respiratory difficulties
reported as nasal flaring or
muscle retractions, digital
clubbing
Cardiomegaly with a
prominent right heart
border reflecting RA
enlargement
Diminished pulmonary
vascular markings
A right aortic arch in 38% of cases
Tricuspid Atresia
Chest Radiograph
References: http://emedicine.medscape.com/article/158359-diagnosis and http://www.bcm.edu/radiology/cases/pediatric/text/2h-desc.htm
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Congenital Anomaly
Hypoplastic Left
Heart Syndrome
Ebstein’s Anomaly
Pathology
Signs and
Symptoms
Chest Radiograph
Marked hypoplasia of the
left ventricle and ascending
aorta
atretic, hypoplastic or
stenotic aortic and mitral
valves
Intact ventricular septum
PDA supplies blood to
the systemic circulation.
(+) ASD or patent
foramen ovale
Coexisting coarctation of
the aorta
Respiratory symptoms
and profound systemic
cyanosis at birth
As the ductus arteriosus
begins to close normally
over the first 24-48 hours
of life, symptoms of
cyanosis, tachypnea,
respiratory distress, pallor,
lethargy, metabolic
acidosis, and oliguria
develop.
No intervention to
reopen the ductus
arteriosus  death
Chest radiographic
findings are not specific for
hypoplastic left heart
syndrome.
Cardiomegaly and
increased pulmonary
venovascular markings are
typically present.
Marked pulmonary
edema may be noted in
infants with obstructed
pulmonary venous return.
Apical displacement of
the septal and posterior
tricuspid valve leaflets 
atrialization of the right
ventricle with a variable
degree of malformation
and displacement of the
anterior leaflet.
Fatigue
Dyspnea
Palpitations
Clubbing
Precordial asymmetry
Ankle edema
Ascites
(Symptoms of right heart
failure)
Normal findings
Cardiomegaly
Small aortic root and
main pulmonary artery
shadow
Decreased pulmonary
vasculature
Large right atrium
Reference: http://emedicine.medscape.com/article/154447-diagnosis
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Congenital Anomaly
Tetralogy of Fallot
Pathology
Maldevelopment of RV
infundibulum
Pulmonary artery
stenosis
VSD
Deviation of the aortic
origin to the right
RVH
The degree of right
ventricular outflow tract
obstruction accounts for
the deleterious
hemodynamics of TOF.
Signs and
Symptoms
Poor feeding, cyanosis
during feeding, fussiness,
tachypnea, and agitation
Dyspnea on exertion
Squatting
Low birthweight
Growth retardation
Delayed development
and puberty
Chest Radiograph
Coeur en sabot
secondary to uplifting of
the cardiac apex from RVH
and the absence of a
normal main pulmonary
artery segment
Decreased pulmonary
vascularity
RA enlargement
Right-sided aortic arch
(20-25% of patients) with
indentation of leftwardpositioned
tracheobronchial shadow
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OBJECTIVES
• To present a case of a 2 year old “blue baby”
• To give the clinical impression and differential diagnosis
of the case based on the history and physical
examination
• To present the Chest X ray of the patient
• To discuss the pathophysiology and symptomatology of
Tetralogy of Fallot
• To discuss other diagnostic procedures that may be
useful in TOF
• To discuss the management and prognosis of a patient
with TOF
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1
2
3
4
5
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NORMAL CHEST X RAY OF A 3-YEAR OLD BABY
Trachea
Bronchial
bifurcation
Cardiophrenic
angle
Right
atrium
NORMAL CHEST X RAY OF A 3-YEAR OLD BABY
Costophrenic
angle
CARDIO-THORACIC RATIO and DIAPHRAGMS
A/B 0.65
A
B
NORMAL CHEST X RAY
LEGEND:
1 - Trachea
2 - Ascending aorta
3 - Brachiocephalic vessels
4 - Pulmonary artery
5 - L ventricle
6 - Retrosternal space
7 - R hemidiaphragm
8 - L hemidiaphragm
NORMAL CHEST X RAY: LATERAL VIEW
PA VIEW
LATERAL VIEW
PATIENT’S CHEST X RAY
PA VIEW
CT RATIO = 0.66
Decreased pulmonary
vasculature
A
B
PATIENT’S CHEST X RAY
Cardiac apex
displaced upward
“coer en sabot”
Lifted up
Apex
PATIENT’S CHEST X RAY
PATIENT’S CHEST X RAY
R Aortic
Arch
Concave MPA
RPA
Right ventricular hypertrophy
PATIENT’S LATERAL VIEW
OBJECTIVES
• To present a case of a 2 year old “blue baby”
• To give the clinical impression and differential diagnosis
of the case based on the history and physical
examination
• To present the Chest X ray of the patient
• To discuss the pathophysiology and symptomatology of
Tetralogy of Fallot
• To discuss other diagnostic procedures that may be
useful in TOF
• To discuss the management and prognosis of a patient
with TOF
30
Tetralogy of Fallot
4 Features:
1. Ventricular Septal
Defect
2. Obstruction of the
right ventricular
outflow tract
3. Overriding aorta
4. Right Ventricular
hypertrophy
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Symptomatology
 Each child can present with different symptoms which depend upon
the severity of the obstruction and stenosis as seen in the
pathophysiology of the disease.
 Blue color of the skin, lips and nail beds (central and peripheral
cyanosis) which occurs with crying or feeding.
 Some babies do not have noticeable cyanosis, but may instead be
very irritable or lethargic due to a decreasing amount of oxygen
available in the bloodstream due to admixing of oxygenated and less
oxygenated blood.
 Some children may have pale or ashen color and may have cool
clammy skin.
 Dyspnea and syncope
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Physical Exam Findings
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RV predominance on palpation
May have a bulging left hemithorax
Systolic thrill at the lower left sternal border
Aortic ejection click
Single S2 - Pulmonic valve closure not heard
Systolic ejection murmur - varies in intensity inversely with the
degree of RVOT obstruction
 More cyanotic patients have greater obstruction and a softer
murmur.
 An acyanotic patient with TOF has a long, loud, systolic murmur
with a thrill along the RVOT.
Cyanosis and clubbing - variable
Squatting position- compensatory
Scoliosis - common
Retinal engorgement
Hemoptysis
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Pathophysiology
 Result from anterosuperior displacement of the
infundibular septum
 Severity of obstruction to RV outflow determines the
direction of blood flow.
o If subpulmonary stenosis is mild- shunt, may be left-to-right w/o cyanosis.
o ↑ in severity of obstruction = ↑ in resistance to RV outflow, right-to-left
shunting predominates along with cyanosis
 ↑ subpulmonic stenosis, pulmonary arteries are smaller
and hypoplastic and the diameter of the aorta becomes
progressively larger.
 RVH - compensatory
 As the child grows, the pulmonic orifice does not expand
proportionally to the enlarging heart, making the
obstruction worse.
34
OBJECTIVES
• To present a case of a 2 year old “blue baby”
• To give the clinical impression and differential diagnosis
of the case based on the history and physical
examination
• To present the Chest X ray of the patient
• To discuss the pathophysiology and symptomatology of
Tetralogy of Fallot
• To discuss other diagnostic procedures that may be
useful in TOF
• To discuss the management and prognosis of a patient
with TOF
35
OTHER DIAGNOSTIC PROCEDURES
 Ultrasonography
 Angiography
 CT Scan
 MRI
36
Ultrasonography
Findings:
 Echocardiography is the primary imaging
method for examining a child in whom TOF is
suspected.
 Intracardiac anomalies, including pulmonary
infundibular and valvular stenosis and the
position of the aortic root overriding the
ventricular septal defect, are identified with 2dimensional echocardiography.
 The origins of the coronary arteries can also be
identified.
37
38
Ultrasonography
 Doppler ultrasonographic examination of the
pulmonary outflow tract can be used to
measure the velocity gradient in the right
ventricular outflow tract and to differentiate
severe stenosis from atresia.
 Continuity of the branch pulmonary artery
with the main pulmonary artery can be
identified, and the size of the branch
pulmonary arteries can be measured.
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Ultrasonography
 The initial placement of palliative shunts is
likely in children who have small branch
pulmonary arteries in order to allow the
pulmonary arteries to grow before corrective
surgery.
 The full length of the shunts may not be
visible; however, Doppler ultrasonography
can be used to verify shunt patency, even
when the entire length of the shunt cannot be
imaged.
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Angiography
 Gold standard in evaluating blood vessels
Findings
 Angiography is the traditional criterion
standard and best modality for the evaluation
of the pulmonary and coronary arterial
morphology, as well as the morphology of the
systemic collateral arteries.
51
Angiography
 The branch pulmonary
arteries have a
characteristic seagull
appearance.
 Pulmonary arterial
measurements for the
calculation of the McGoon
ratio and Nakata index are
critical to surgical planning.
 An aortic root injection is
used to evaluate the
position and number of
coronary arteries.
52
TOF in Angiogram
Aortic Arch
Descending
Aorta
LPA
Stump
RPA
MPA
Descending
branch of RPA
Catheter
Ascending Aorta
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OBJECTIVES
• To present a case of a 2 year old “blue baby”
• To give the clinical impression and differential diagnosis
of the case based on the history and physical
examination
• To present the chest xray of the patient
• To discuss the pathophysiology and symptomatology of
Tetralogy of Fallot
• To discuss other diagnostic procedures that may be
useful in TOF
• To discuss the management and prognosis of a patient
with TOF
56
Management
 Treatment depends on the severity of right
ventricular outflow tract obstruction.
 Immediate increase in pulmonary blood flow to
prevent hypoxia.
 Oxygenation, normal body temperature, normal
glucose level should be maintained.
 Prevention and prompt treatment of
dehydration to prevent hemoconcentration and
possible thrombotic episodes.
57
Management
 For Tet spells
 Propanolol (0.5–1 mg/kg every 6 hr)
 Morphine to reduce ventilatory drive
 Vasopressor such as epinephrine to increase blood
pressure
 Modified Blalock-Taussig shunt
 Aortopulmonary shunt
58
Prognosis
UNTREATED:
 Untreated TOF may lead to further RV
hypertrophy and dilated cardiomyopathy
(beginning on the right,then left).
 Actuarial survival for untreated tetralogy of
Fallot is approximately 75% after the first
year of life, 60% by four years, 30% by ten
years, and 5% by forty years.
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Prognosis
TREATED:
• After successful total correction, patients are generally
asymptomatic and are able to lead unrestricted lives.
– Have lower than normal exercise capacity, maximal
heart rate and cardiac output (more frequent in those
who have undergone surgery at a later age).
• Uncommon immediate postoperative problems include
right ventricular failure, transient heart block, residual
VSD with left-to-right shunting, and myocardial infarction
from interruption of an aberrant coronary artery.
• A number of children have premature ventricular beats
after repair of TOF.
• Lifetime follow-up care
60
REFERENCES
 Misra, Rakesh, et. al. A-Z of Chest Radiology, 2007.
 Novelline, Robert A. Squire’s Fundamentals of Radiology,




6th ed.
Sutton. A Textbook of Radiology and Imaging.
http://emedicine.medscape.com/article/158359diagnosis
http://www.bcm.edu/radiology/cases/pediatric/text/2i1A.
htm
http://www.bcm.edu/radiology/cases/pediatric/text/2hdesc.htm
61