CYANOTIC CONGENITAL HEART DISEASES

1. Ventricular Septal Defect

2. Atrial Septal Defect
3. Patent Ductus Arteriosus
CYANOTIC HEART DISEASES
CASE 1: Ventricular Septal Defect **In cyanotic heart diseases, hyperaerated lungs are
1 month old boy; Failure to thrive; CHF usually seen.
PE: Systolic murmur-low pericardium 1) Normal blood flow
Holosystic murmur –extends 2nd heart sound
a) Coarctation of
Soft low pitch early diastolic murmur – apical area
the Aorta
CASE 4
CXR FINDINGS:
• 14 day old
• Cardiomegaly
infant
• Inc pulmo bld flow
• Dyspnea
• LAE
• Poor feeding
• Displacement of L
• Tachycardia
mainstem
• Peripheral
bronchus
cyanosis
• R aortic arch
o Moderate to marked cardiomegaly
• CHF (infants)
o Pulmo venous congestion
CASE 2: Atrial Septal Defect (common in children) o Post stenotic dil. of the
Systolic ejection murmur in descending aorta
pulmonic region
Parasternal diastolic 2) Increased blood flow
murmur a) Transposition of Great Arteries
CASE 5
Mild cardio w/ RAE &
• Infants of DM
RVE
• Cyanosis at
Mild increase Pulmo bld
flow birth
• Acidosis
• CHF
• No murmur
• CXR FINDINGS:
CASE 3: Patent Ductus arteriosus (common in adults) o
2y/o Hyperinflated
Failure to thrive CHF lungs
Machinery like murmur in the o Low set HD
pulmonic area o Narrowed based
inc pulmo bld flow o heart
Cardiomegaly o Egg shaped on side
Aorta prominent
o Thymic atrophy
Prox asc aorta in arch of the
o Cardiomegaly
aorta
Focal aortic fil
LAE & LVE b) Total Anomalous
Pulmonary Venous
Return
• N cardiac conf
PATENT DUCTUS ARTERIOSUS • Pulmo
venous/edema
• Thymic atrophy
• Low set HD
• Occ’l PE
• Hemodiaphragm
• Snowman’s sign

3) Decreased blood flow

a) Tetralogy of Fallot
CASE 7
• 6 mos old
• Cyanosis
• Dyspnea – 1st sign

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 • Loud ejection ACYANOTIC
murmur- L sternal
angle Increase PBF

• CXR FINDINGS:
o Boot RVH LVH / CVH
shaped
heart Atrial Septal Defect Ventricular Septal Defect
o Concave MPA
o Dec hilar and L to R Shunt w/ PHPN Patent Ductus Arteriosus
central pulmo
vessels PAPVR AVSD
o
Hyperexpanded hyperlucent lungs
o Thymic atrophy
CYANOTIC
o Rt aortic arch
Dec PBF
b) Tricuspid Valve Atresia
Case 8
• Dyspnea RVH LVH LVH / CVH

• Jugular pulsations
• Loud murmur along the L sternal border VSD w/ PS Tricuspid Atresia TA w/ Hypoplastic
PAs
• Mild cardiomegaly
Tetralogy of Fallot Pulmonary Atresia
• Dec pulmo bld flow Hypolastic RV TGA w/ PS
• Hyperlucent lungs
• Concave MPA Ebstein’s Anomaly
Single Ventricle w/
• Rt aortic arch PS
• RAE and RVE Eisenmenger

CYANOTIC

Increase PBF

RVH LVH / CVH

TAPVR TGA w/ VSD

Hypoplastic Single Ventricle

c) Ebstein's Anomaly
Syndrome
CXR findings:
Truncus Arteriousus
• Marked cardio
• RAE w/ globular TGA
PVOD
cardiac silhouette
• Dec pulmo bld
Bidirectional Shunt
flow
• Boxed heart
CARDIAC TUMORS
• Any benign/malignant neoplasm arising primarily
from the myocardium or w/in a cardiac chamber
• Approximately 70% are benign and 30% are
malignant
• Metastatic tumors are not classified as cardiac
tumors
BENIGN MALIGNANT
Myxoma Angisarcoma
Lipoma Rhabdomyosarcoma
Papillary Fibroadenoma Mesothelioma
Hemangioma Fibrosarcoma
Mesothelioma Lymphoma
Fibroma Osteosarcoma

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 Teratoma Thymoma
Granular cell tumor Neurogenic Sarcoma
Neurofibroma Leiomyosarcoma
Lympahngioma Liposarcoma
Rhabdomyoma Synovial sarcoma

• LIPOMATOUS INFILTRATION OF INTERATRIAL

• SEPTUM

Epicardium, the sheath of tissue that covers the heart

• Myocardium, the muscles of the heart wall
• Endocardium, the membrane that lines the cavities
of the heart
NOTES:
Benign Tumors Fat – bright on T1 and T2
Myxoma (most common) CSF – white on T2, black
• Morphology: on TI
o Usually PEDUNCULATED w/ short, broad-
based attachment Malignant Tumors
o Characteristically polypoid, project into Sarcoma
cardiac chamber and about 5+6 cm in • Malignant tumor w/ wide
diameter variety of types,
o Gelatinous/ mucoid texture and covered w/ originating from
endothelium mesenchyme
o Arises from endocardium as small, uniform • Subtype include,
cells in myxomatous stroma angisarcoma,
o Rarely metastasizes rhabdomyosarcoma,
• Location fibrosarcoma,
o LA 75% - most common location osteosarcoma,
o RA 20% neutrogenic sarcoma,
o Atrial myxomas usually arises from atrial leiomyosarcoma,
septum liposarcoma and
o Ventricles: <10% synovial sarcoma
o Multicentric: 5% • MC: RA & in mid-adulthood
o Valve: rare • Operation indicated to obtain accurate dx
o Myxoma of left atrium o Prognosis is poor, as most px have distant
metastases at presentation
o Adjuvant therapy may have some roles

NOTES:
Most common location in
both Sarcoma and
Lymphoma: RA

Lipoma Papillary fibroelastomas

• Well-Encapsulated Tumors, Usually Found
Incidentally
• Consist Of Mature Fat Cells
• Most commonly occur in ATRIAL SEPTUM as
part of lipomatous hyperthropy of the
interatrial septum
• Incidental tumors should probably be resected
• PERICARDIAL LIPOMA
• Would seem to be approx. equivalent to Lipoma
in prevalence
• Over 90% occur on cardiac valves, making them
the commonest neoplasm of the valves
• Many are asymptomatic, and Sx if present are
usually related to embolic events
• There appears to be slight predilection for left-
sided valves
• On MRI- it appears as a HYPO-INTENSE mobile
mass

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Rhabdomyoma METASTASES FROM AN ABDOMINAL ISLET CELL TUMOR
• Yellow-gray tumor that occur invariably in the
ventricles commonly in multiple locations
o Altered myocytes, often not discrete from
surrounding normal myocardium
• Associated w/ TUBEROUS SCLEROSIS
• MC: 1o cardiac tumor in children
o Cause cardiac failure from obstruction of
conduction pathways and ventricular
tachycardia
• Over 90% present before age 15, usually in the
first few days of life
• Tumors maybe unresectable at surgery
• About 50% of survivors will eventually develop
tuberous sclerosis

Primary Cardiac Lymphomas

• Are exceedingly rare, are typically of the non-
Hodgkin B-cell type, and are confined to the NON-SMALL CELL BRONCHOGENIC CARCINOMA
heart or pericardium (tumors in lungs extending to the left atrium)
• They usually occur in immunocompromised px
but are not restricted solely to this group
• Presentation is w/ rapidly worsening heart
failure, obstructive Sx or arrhythmias
• This prognosis is invariably poor, although there
have been reported remissions w/
chemotherapy
• They most commonly involve the RT side of the
heart, in particular the RA, w/ frequent
involvement of more than one chamber and
invasion of the pericardium
• At MRI: they are ISOTENSE on t1-weighted
images and heterogeneously HYPERINTENSE on
T2-weighted images; they demonstrate
heterogeneous enhancement after
administration of gadolinium contrast material,
w/ areas of low enhancement in the center of
the lesion compared to the periphery
• Secondary malignancies involving the heart are
20-40times more frequent than primary cardiac
neoplasms
• In autopsy studies, Px w/ known malignant
neoplasms will have cardiac metastatic
involvement in 10-20% of cases
• Involvement in many cases may go unnoticed,
but object manifestations are most commonly DIRECT VENOUS EXTENSION OF A LEFT SIDED
due to pericardial effusions and the associated RENAL CELL TUMOR
impairment of the RT cardiac filling that result.
• Presenting Sx may include shortness of breath,
chest wall pain, and peripheral edema. As w/
other cardiac malignancies, arrhythmias may
also be a feature.

***MC 1o neoplasm is BROCHOGENIC CARCINOMA

followed by lymphomas, leukemia and carcinomas of
the breast and esophagus
***MC site of involvement is the pericardium w/ or
w/o invasion of the underlying myocardium
***In approximately 1/3 of Px w/ cardiac involvement,
death will be directly attributable to the metastases as Thrombus
a result of pericardial tamponade, CHF, or coronary
• MC mimic of cardiac neoplasm
artery invasion

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• Most likely to be located posteriorly in the LA,
commonly in the presence of atrial fibrillation, or in
severely dysfunctional left ventricles. It can also be
found in the right side of the heart
• ACUTE THROMBUS will appear bright on both
T1 and T2-weighted images
• SUBACUTE THROMBUS will appear bright on
T1-weighted images with low signal intensity
areas on T2-weighted images due 2 the
paramagnetic effects of methemoglobin and
shortening of the T2 relaxation times.
• CHRONIC ORGANIZED THROMBUS will have Foreign body
low signal intensity on both T1 and T2- BULLET IN INTRAVENTRICULAR SEPTUM
weighted images due to depleted water with or
without calcification of the thrombus (similar to
adjacent parenchyma)
• Gadolinium contrast material is also useful for
differentiating thrombus from tumors, as the
former should not enhance.

REVIEW OF HEART ANATOMY

1. Atheromatous Aorta
- Calcific in periphery
***EXTRACARDIAC MASSES can mimic cardiac tumors.
Pericardial and bronchogenic cysts, intrathoracic
neoplasms and rarely GI hernias may compress the
heart to mimic a cardiac mass.

Pericardial cysts
• Are congenital in origin and are usually found at
the right cardiophrenic angle, although they may
occur anywhere in the mediastinum.
• They are unilocular and contain water-based fluid
w/o internal septa
NOTES: Pericardial cyst – dark on TI,
bright on T2 2.
• They usually demonstrate the MRI character of Dilated
simple fluid and do not enhance after contrast
material administration
• They occasionally may contain relatively protein-
ascending aorta
aqueous fluid and thus may have high signal
intensity on both T1 and T2 weighted images

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3. Pseudoaneurysm
- does not go into the area of the aorta; does not
enhance

4. Aneurysm of ascending aorta

- reaches > 5; emergency operation should be DIlated aortic arch w/ type A dissection
done

2) TYPE B = the tear is located just past the left

subclavian artery (the blood vessel beneath
the collarbone that supplies the left arm)

***Type B dissection w/ prior Type a repair:

5. Aortic dissections
STANDFORD CLASSIFICATION:
1) TYPE A = the tear begins in the ascending
aorta and progresses throughout the vessel,
extending as far as the arteries in the leg
***Dissection extends into subclavian artery
Type A Dissection

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BeBAKEY CLASSIFICATION:
1) TYPE I – involves the ascending aorta, aortic
arch and descending aorta (similar to Type A of
Standford)
2) TYPE II – confined to the ascending aorta
(similar to Type A of Standford)
3) TYPE III – confined to the descending aorta
distal to the left subclavian artery (similar to
Type B of Standford)
a) Type IIIa = refers to dissections that
originate distal to the left subclavian artery
but extend both proximally and distally,
most above the diaphragm
b) Type IIIb = refers to dissection that
originate distal to the left subclavian
artery, extend only distally and may extend
below the diaphragm.
2 common congenital anomaly:
1. Diaphragmatic hernia
• Types: Morgagni – anterior defect
Bochdalek – posterior defect
• A Bochdalek hernia involves an opening
on the left side of the diaphragm. The
stomach and intestines usually move up
into the chest cavity; A Morgagni hernia
involves an opening on the right side of
the diaphragm. The liver and intestines
usually move up into the chest cavity.
• Common in the left side
• Shifting of mediastinum to contralateral
side
• Gastric bubbles
2. Congenital Cystic Adenomatoid Malformation
(CCAM)
• Multiples areas of lucencies
• Gastric bubbles
• Intact hemidiaphragm
• No shifting of medial structures
• In Ultrasound: peristalsis is seen in
diaphragmatic hernia
Congenital Cystic Adenomatoid Malformation
(CCAM)

PEDIATRIC NORMAL CHEST – PA VIEW

NEWBORN CXR W/ DIFFUSE INFILTRATES
1. Wet lung disease
Case: FT NB delivery via
CS
BOB during the
1st 4 hrs of life
• Aka. TRANSIENT
TACHYPNEA of the
newborn, retention
of the fetal lung
fluid/ transient
NOTES: respiratory distress
Chest X-ray findings in pedia: of the newborn
• Heart is slightly elevated • Mild to moderate
• Normal cardio-thoracic ratio in respiratory distress
pedia: 0.55 sometime in the 1st 4hrs of life
(In adults: 0.5; if > than this, • Conditions that predispose the disease:
enlarged) a) CS
• Gastric bubble just below b) Precipitous delivery
hemidiaphragm c) Very small, hypotonic or sedated infants

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 • ROENTGENOGRAPHIC FINDINGS:
a) Mild to moderate overaeration Pulmonary Interstitial Pneumonia
b) Symmetric parahilar patches or streaks
c) Occasionally mild cardiomegaly / pleural
effusions
d) RT lung maybe more opacicied than the
LFT
e) The chest film returns to normal by 48-72
hrs of age
• Transient tachypnea of the newborn: Usually
has non homogeneous pulmonary opacities, a
normal lung volume and may have pleural fluid
• Transient tachypnea of the newborn/Wet Lung
(TTN): Supine chest radiograph of the newborn Pulmonary Interstitial
demonstrating mild cardiomegaly and bilateral Pneumonia
reticulonodular densities that radiate from the with
hila. There is atelectasis in the upper lobes. PNEUMOMEDIASTINUM

NOTES: In utero, lungs contain amniotic fluid.

Via NSD: thoracic cage is being compressed so
the remaining fluid in the lungs will be
squeezed out, then will be filled with air.
Baby now is able to breathe (inhale).
Via CS: fluid in the lungs will remain there until
this would be removed

History
• Premature rupture of the membranes(PROM)
2. Preterm baby del via during labor
NSD DOB • Shortly after birth tachypnea, retratctions, and
• Small lung volume cyanosis maybe evident
• A finely granular • Infants are often afebriel and occasionally are
(ground glass) hypothermic
• Air bronchograms • Supine chest radiograph at the 5hrs of lives
extending peripherally demonstrate diffuse bilateral granular
infiltrates
• SURFACTANT
DEFICIENCY 3. Neonatal Pneumonia
DISEASE/HYALINE • Group B strep
MEMBRANE DISEASE
pneumonia –
OF NEWBORN/IDOPATHIC RESPIRATORY
nonhomogenous
DISTRESS SYNDROME
pulmonary opacities
• MMC: respiratory
• May have pleural fluid
distress in newborn
• Lung volume is normal
infants
• It occurs in premature • COMMON
infants and also infant of PATHOGEN:
DM mothers a) Stap aureus
• Deficiency of pulmonary b) E.coli
surfactant superimposed • The infants are often afebrile & occasionally
on structural immaturity hypothermic
of the lungs • Short after birth tachypnea, retractions and
• Usually has a uniform cyanosis may be evident
distribution of • Radiographic findings:
pulmonary opacities, o Symmetric pulmonary opacities
never has pleural effusions, has a decreased o Hyperaeration are seen
lung volume o Pleural effusion may be seen
• Complications: o Group B streptococcal pneumonia,
1. Pulmonary interstitial pneumonia or pulmo reticulogranular densities
interstitial emphysema (PIE) o Consolidation occurs, it usuall is
- interstitial infiltrates multilobular
- low set hemidiaphragm
- lungs are hyperaerated
2. Pneumomediastinum

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4. FT del via NSVD, • A pneumopericardium may be difficult to
thickly meconium distinguish from a pneumomediastinum as
• Supine chest both may have a “continuous diaphragm sign.”
radiograph from In a pneumopericardium, air will never dissect
day one of live above the level of the main pulmonary artery,
which is the superior extent
demonstrates
bilateral, irregular
coarse infiltrates

Meconium Aspiration
Syndrome
• Usually has nodular patchy non homogenous
densities and may have pleural effusion and an
increased lung volume
Pneumopericardium
• Supine chest radiograph from day one of life • Supine chest radiograph shows air outlining the
demonstrates bilateral, irregular coarse heart, but not extending above the great
infiltrates vessels
• Pneumopericardium, air will never dissect
• Similar to neonatal pneumonia (except there is above the level of the main pulmonary artery,
premature rupture of membranes in neonatal which is the superior extent of the pericardium.
pneumonia) In a pneumomediastinum, air can dissect all
the way up the mediastinim into the neck
• Preterm chest x-ray: Ground glass
appearance with air bronchogram

Trauma Chest Infections

Pneumothorax Croup
• A pneumothorax is difficult to visualize on a • History: 9 year old male
supine film. The most common place to see presents with croupy
one is in the medial basal and basilar portion of cough and respiratory
the lung. The best film to obtain to diagnose a distress
pneumothorax is a decubitus film with the • Symmetrical subglottic
suspicious side up narrowing on the AP view
• Pencil-shaped
configuration of the
subglottic edema
• “Steeple sign”
• An acute infection of the
lower air passages
• Children below age of 3s
exceptions
• Parainfluenza – most
common pathogen

Pneumomediastinum (PMS)

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Epiglottitis
• History: A 2 year old boy is brought to the
emergency department at 2:30 am
• Complaining of throat pain, not drinking,
drooling, difficulty swallowing, coughing,
naseal congestion, and fever
• The throat pain, difficulty drinking, and drooling
started in the afternoon and have worsened
over the night. He has had the coughing and
nasal congestion for 3 days now
• The black arrow points to the tip of the Atelectasis or 20 to Hilar lymphadenopathies
epiglottis and the white arrow points to the pre- • Manifests with fever
epiglottic space (vallecula) • Chest x-ray: segmented opacities in right
middle lobe

• The
epiglottis should normally be thin or triangular
in appearance. In this view, it appears to be Cystic Fibrosis
rounded and somewhat thumb-like, which is • Hx: 11 y/o male being evaluated for a surgical
similar to the classic radiographic description procedure
of epiglottitis
• Chest x-ray: hyperlucent with multiple
infiltrates
Tuberculosis Nodules in hilum
• History: 4 year old female exposed to active
tuberculosis by a sibling

Upper, middle, and lower lungs show thickened

interlobular septa and brochiectasis and
perhaps some hilar lymphadenopathy

Empyema
TB • Hx: 7 year old male
with fever and
nonproductive
cough

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 • Right upper lobe consolidation and a large right
pleural effusion
• Chest x-ray: complete opacified right lung

Pulmonary Abscess
• An enhanced chest CT exam shows a large
right pleural effusion with an enhancing rim
with associated consolidation and atelectasis of
the lung
• Enhancement of pleura

Pulmonary abscess
• Hx: an 8 year old male with fever, cough, and
bad breath
• Multi-cavitary lesions

Pleural Effusion (Lateral Decubitus)

*show a cavitary lesion with an air fluid

level in the right lower lobe

Subpulmonic Effusion
- hemidiaphragm elevated when comparted to left

7 x 7.5 x 8 cm intrapulmonary
fluid collection in the right lower
lobe with thick nodular walls and
air fluid level
Round Pneumonia

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Lymphoma
- Bilateral nodular opacities, smooth border,
mediastinal pathology

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