CONGENITAL HEART

DISEASE(CHD)
Zemene Tigabu
Assistant prof. of pediatrics
Outline
 Prevalence
 Etiology
 Evaluation of the patient
 Specific lesions
 Pathophysiology
 Clinical manifestations
 Diagnosis
 Management
 complications
 prognosis
 Congenital heart disease
 PREVALENCE.
 In live births (0.5–0.8%)
 In premature infants (about 2% excluding
patent ductus arteriosus [PDA])
 In stillborns (3–4%)
 In spontaneous abortuses (10–25%)
 This overall incidence does not include mitral valve
prolapse, PDA of preterm infants, and bicuspid aortic
valves (present in 1–2% of adults).
 The incidence of congenital heart disease
in the normal population is ≈0.8%
 and this incidence increases to 2–6% for a
2nd pregnancy after the birth of a child
with congenital heart disease or if a
parent is affected.
 This recurrence risk is highly dependent
Con’d
 Congenital cardiac defects have a wide spectrum of severity
in infants:
 about 2–3 in 1,000 newborn infants will be
symptomatic with heart disease in the 1st yr
of life.
 The diagnosis is established by :
 1 wk of age in 40–50% of patients
 1 mo of age in 50–60% of patients.
 With advances in both palliative and corrective surgery, the
number of children with congenital heart disease surviving
to adulthood has increased dramatically.
 Despite these advances, congenital heart disease remains
the leading cause of death in children with congenital
malformations
 Con’d
• Relative Frequency of Major Congenital Heart Lesions [*]
• LESION % OF ALL LESIONS
– Ventricular septal defect 35–30
– Atrial septal defect (secundum) 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
– d-Transposition of great arteries 3–5
– Hypoplastic left ventricle 1–3
– Hypoplastic right ventricle 1–3
– Truncus arteriosus 1–2
– Total anomalous pulmonary venous return 1–2
– Tricuspid atresia 1–2
– Single ventricle 1–2
– Double-outlet right ventricle 1–2
– Others 5–10
Con’d
 Most congenital defects are well tolerated in the
fetus because of the parallel nature of the fetal
circulation.
 Even the most severe cardiac defects (hypoplastic
left heart syndrome) can usually be well
compensated for by the fetal circulation.
 One notable exception is the case of severe
regurgitant lesions, most commonly of the tricuspid
valve. In these lesions (Ebstein anomaly ), the
parallel fetal circulation cannot compensate for the
volume load imposed on the right side of the heart.
 In utero heart failure, often with fetal pleural and
pericardial effusions, and generalized ascites
(nonimmune hydrops fetalis) may occur.
Fetal circulations
Con’d
 ETIOLOGY.
 The cause of most congenital heart
defects is unknown.
 Most cases of congenital heart disease
were thought to be;
 multifactorial -result from a combination of
genetic predisposition and environmental
stimulus.
 A small percentage of congenital heart
lesions are related to chromosomal
abnormalities
 Evaluation of the Infant or Child with
Congenital Heart Disease

 The initial evaluation for suspected congenital heart

disease involves a systematic approach with three major
components.
 First, congenital cardiac defects can be
divided into two major groups based on the
presence or absence of cyanosis, which can
be determined by physical examination
aided by pulse oximetry.
 Acyanotic
 Cyanotic
 Second, these two groups can be further
subdivided according to whether the chest
radiograph shows evidence of:
 increased
 Normal pulmonary vascular markings
 decreased
Con’d
 Finally, the electrocardiogram can be
used to determine whether :
 right
 left hypertrophy exists.
 biventricular

 The final diagnosis is then confirmed by

echocardiography, CT ,MRI or cardiac
catheterization.
I- Acyanotic Congenital Heart Disease(CHD)

• Acyanotic congenital heart lesions can be classified

according to the predominant physiologic load that they
place on the heart.
1) lesions that cause a volume load
A) left-to-right shunt lesions.
• ASD
• VSD
• PDA
• AVSD
• PAPVR
• Aorticopulmonary Window Defect
• Ruptured Sinus of Valsalva Aneurysm
• Coronary- Arteriovenous Fistula (Coronary-Cameral
Fistula)
Con’d
B) Regurgitant lesions
 PR=common
 MVP
 MR
 TR
 AR
C) some of the cardiomyopathies
Con’d
2) lesions that cause a pressure load (The
Obstructive Lesions)
– most commonly secondary to:
• ventricular outflow obstruction
– Pulmonic valve stenosis
– aortic valve stenosis
• narrowing of one of the great vessels
– coarctation of the aorta
– Rarely
• MS
• TS
 The chest radiograph and
electrocardiogram are useful tools for
differentiating between these major classes
of volume and pressure overload lesions.
1.1 Atrial Septal Defect(ASD)

 can occur in any portion of the atrial septum

 4 types
 secundum(OS)- the most common type
 primum(OP)-ECD
 sinus venosus(SV)
 Coronary sinus defect
 Less commonly, the atrial septum may be nearly
absent, with the creation of a functional single
atrium.
 An isolated valve-incompetent patent foramen ovale
(PFO) is a common echocardiographic finding during
infancy. It is usually of no hemodynamic significance
and is not considered as ASD
Con’d…
 PATHOPHYSIOLOGY.
– left-to-right shunting increased pul. blood
flow
 At atrial level in Os and SV
 Both at atrial and ventricular level in OP
– The degree of left-to-right shunting is
dependent on:
 the size of the defect
 the relative compliance of the right and left ventricles
 and the relative vascular resistance in the pulmonary and
systemic circulations.
– The paucity of symptoms in infants with ASDs is
related to the structure of the right ventricle in
early life when its muscular wall is thick and less
compliant, thus limiting the left-to-right shunt.
Con’d
 results in:
 enlargement of the right atrium and
ventricle
 dilatation of the pulmonary artery.
 The left atrium may be enlarged
 the left ventricle and aorta normal in size.
 Despite the large pulmonary blood flow, pulmonary arterial
pressure is usually normal because of the absence of a high-
pressure communication between the pulmonary and
systemic circulations.
 Pulmonary vascular resistance remains low throughout
childhood, although it may begin to increase in adulthood
and may eventually result in reversal of the shunt and
clinical cyanosis.
Con’d
Con’d
 CLINICAL MANIFESTATIONS.
 A child with an ostium secundum ASD
is most often asymptomatic.
 Even an extremely large secundum
ASD rarely produces clinically evident
heart failure in childhood.
 In younger children, subtle failure to
thrive may be present.
 in older children, varying degrees of
exercise intolerance may be noted.
Con’d
 The physical findings.
 mild left precordial bulge.
 A right ventricular systolic lift is generally
palpable at the left sternal border.
 A loud 1st heart sound and sometimes a
pulmonic ejection click can be heard.
 In most patients, the 2nd heart sound is
characteristically widely split and fixed.
 A systolic ejection murmur is best heard at
the left middle and upper sternal border.
 A short, rumbling mid-diastolic murmur at
the tricuspid valve is often audible at the
lower left sternal border.
 Holosystolic murmur at the apex in OP(ECD)
Con’d
 DIAGNOSIS.
 Clinical
 Chest x-ray
 ECG
 ECHO
 Catheterization
 COMPLICATION
 Pulmonary hypertension -eisnmenger
syndrome
 arrhythmia
 tricuspid or mitral insufficiency
 heart failure
Con’d
 TREATMENT.
 Surgical or transcatheter device closure is
advised for :
 all symptomatic patients
 asymptomatic patients with a Qp : Qs ratio of at
least 2 : 1.
 The timing for elective closure is usually after the
1st yr and before entry into school.
 Closure carried out at open heart surgery is
associated with a mortality rate of <1%..
 In patients with small secundum ASDs and
minimal left-to-right shunts, the consensus is that
closure is not required.
Con’d
 PROGNOSIS
 ASDs detected in term infants may
close spontaneously.
 Secundum ASDs are well tolerated
during childhood.
 The results after surgical or device
closure in children with moderate to
large shunts are excellent.
1.2 Ventricular Septal Defect(VSD)

 VSD is the most common cardiac malformation .

 Defects may occur in any portion of the ventricular
septum.
 3 types
 membranous - commonest
 Supracristal- less common
 Muscular
 single or
 multiple (Swiss cheese septum).
 Con’d
 PATHOPHYSIOLOGY
 Left-to-right shunt increased
pul. blood flow.
 Restrictive VSDs(usually <0.5 cm2)
 right ventricular pressure is normal
 the size of the defect limits the magnitude of
the shunt
 Nonrestrictive VSDs (usually >1.0 cm2)
 right and left ventricular pressure is equalized
 the direction of shunting and shunt magnitude
are determined by the ratio of pulmonary to
systemic vascular resistance
 Con’d
 The magnitude of intracardiac shunts is usually described by
the Qp : Qs ratio.
 If the left-to-right shunt is small (Qp : Qs
<1.75 : 1),
 the cardiac chambers are not appreciably enlarged and
the pulmonary vascular bed is probably normal.
 If the shunt is large (Qp : Qs >2 : 1),
 left atrial and ventricular volume overload occurs
 right ventricular volume overload and pulmonary arterial
hypertension.
 The main pulmonary artery, left atrium, and left ventricle
are enlarged.
 When the ratio of pulmonary to systemic
resistance approaches 1 : 1,
 the shunt becomes bidirectional
 the signs of heart failure abate, and the patient becomes
cyanotic (Eisenmenger physiology)
Con’d
Con’d
 CLINICAL MANIFESTATIONS
 The clinical findings of patients with a
VSD vary according to the size of the
defect and pulmonary blood flow and
pressure.
 Small VSDs with trivial left-to-right shunts and
normal pulmonary arterial pressure are the most
common.
 These patients are asymptomatic
 Characteristically, a loud, harsh, or blowing
holosystolic murmur is present and heard best
over the lower left sternal border
 it is frequently accompanied by a thrill.
Con’d
• Large VSDs
– dyspnea
– feeding difficulties
– poor growth
– profuse perspiration
– recurrent pulmonary infections
– cardiac failure in early infancy

Physical finding
– Prominence of the left precordium is common
– palpable parasternal lift
– laterally displaced apical impulse
– apical thrust, and a systolic thrill
– The holosystolic murmur of a large VSD is generally
less harsh than that of a small VSD and more blowing .
– Early systolic murmur at pulmonic area
– a mid-diastolic, low-pitched rumble at the apex(shunt
ratio >2:1)
– The pulmonic component of the 2nd heart sound may
be increased
Con’d
 DIAGNOSIS.
 Clinical
 Chest x-ray
 ECG
 ECHO
 catheterization
Con’d
 TREATMENT
 medical management
 has two aims:
 to control heart failure and
 prevent the development of pulmonary vascular
disease.
 Surgical management
 Indications for surgical closure :
 patients at any age with large defects in whom
clinical symptoms and failure to thrive cannot be
controlled medically
 infants between 6 and 12 mo of age with large
defects associated with pulmonary hypertension,
even if the symptoms are controlled by medication
 patients older than 24 mo with a Qp : Qs ratio
greater than 2 : 1. -- Patients with supracristal
VSD of any size.
--Severe pulmonary vascular disease is a
contraindication to closure of a VSD.
Con’d
 COMPLICATIONS
 Pulmonary hypertensions
 Infective endocarditis
 Heart failure
 Recurrent lung infections
 PS
 AR
 Con’d
 PROGNOSIS.
 The natural course of a VSD depends to a large
degree on the size of the defect.
 A significant number (30–50%) of small defects
close spontaneously, most frequently during the
1st 2 yr of life.
 Small muscular VSDs are more likely to close (up
to 80%) than membranous VSDs are (up to 35%).
 The vast majority of defects that close do so
before the age of 4 yr, although spontaneous
closure has been reported in adults
 It is less common for moderate or large VSDs to
close spontaneously (up to 8% may close
completely)
 The results of primary surgical repair are
excellent
 1.3 Patent Ductus Arteriosus(PDA)

 Functional closure of the ductus normally occurs soon after

birth
 The aortic end of the ductus is just distal to the origin of the
left subclavian artery, and the ductus enters the pulmonary
artery at its bifurcation.
 Female patients with PDA outnumber males 2 : 1.
 PDA is also associated with maternal rubella infection during
early pregnancy.
 It is a common problem in premature infants, where it can
cause severe hemodynamic derangements and several major
sequelae
 Con’d
• Pathology
– the wall of the ductus is deficient in both the
mucoid endothelial layer and the muscular
media in term infants
• PATHOPHYSIOLOGY.
• Left-to-right shunt increased pul. blood flow.
– The extent of the shunt depends on
• the size of the ductus
• the ratio of pulmonary to systemic vascular resistance.
– In extreme cases, 70% of the left ventricular
output may be shunted through the ductus to
the pulmonary circulation.
– If the PDA is small, pressure within the
pulmonary artery, the right ventricle, and the
right atrium is normal.
Con’d
Con’d
 CLINICAL MANIFESTATIONS.
 A small patent ductus does not usually
have any symptoms.
 A large PDA will result in:
 heart failure.
 Retardation of physical growth may be a major
manifestation in infants with large shunts
 Con’d
 physical signs in large PDA
– wide pulse pressure( runnoff blood)
– bounding peripheral arterial pulses.
– The apical impulse is prominent and, with cardiac
enlargement, is heaving.
– A thrill, maximal in the 2nd left interspace
– The classic continuous murmur is localized to the
2nd left intercostal space or radiate down the left
sternal border , to the left clavicle or toward the
apex
– The murmur radiates along the pulmonary arteries and is
often well heard over the left back.
– When pulmonary vascular resistance is increased,
the diastolic component of the murmur may be
less prominent or absent.
– a low-pitched mitral mid-diastolic murmur may be
audible at the apex
– Splitting of S2
Con’d
 DIAGNOSIS.
 Clinical
 Chest x-ray
 ECG
 ECHO
 catheterization
Con’d
 TREATMENT.
 Medical
 Surgical
 Irrespective of age, patients with PDA require
surgical or catheter closure.
 preferably before 1 yr of age
Con’d
 COMPLICATIONS
 Heart failure
 Pulmonary hypertension (Eisenmenger
syndrome)
 Infective endarteritis.
 Pulmonary or systemic emboli may occur
 Rare complications include
 aneurysmal dilatation of the pulmonary artery or the
ductus
 calcification of the ductus
 noninfective thrombosis of the ductus with
embolization
 paradoxical emboli
Con’d
 PROGNOSIS
 Spontaneous closure of the ductus
after infancy is extremely rare.
 The chronic left ventricular volume
load is less well tolerated with aging.
2. The Obstructive Lesions

2.1 Pulmonic Stenosis

 4 types
 Valvular stenosis
 Isolated infundibular stenosis
 supravalvular pulmonary stenosis
 branch pulmonary artery stenosis
Con’d
 PATHOPHYSIOLOGY.
 right ventricular outflow obstruction
increased systolic pressure and wall stress
hypertrophy of the right ventricle
 The severity of these abnormalities depends
on the size of the restricted valve opening.
 When severe pulmonic stenosis occurs in a
neonate, markedly decreased right
ventricular compliance may lead to cyanosis
due to right-to-left shunting through a patent
foramen ovale, a condition termed critical
pulmonic stenosis
Con’d
 CLINICAL MANIFESTATIONS
– Patients with mild or moderate stenosis
usually do not have any symptoms.
 Mild -right ventricle to pulmonary artery gradient of ≤30
mm Hg.
 Moderate - right ventricle to pulmonary artery pressure
gradient in the 30–60 mm Hg range.
– If the stenosis is severe
 right ventricle to pulmonary artery pressure gradient >60
mm Hg range
 signs of right ventricular failure
 exercise intolerance may be present.
 In a neonate or young infant with critical pulmonic
stenosis, signs of right ventricular failure may be more
prominent, and cyanosis is often present because of
shunting at the foramen ovale.
Con’d
 The pulmonary component of the 2nd
sound is usually inaudible.
 A loud, long, and harsh systolic
ejection murmur, usually accompanied
by a thrill, is maximally audible in the
pulmonic area and may radiate over
the entire precordium, to both lung
fields, into the neck, and to the back.
 The peak of the murmur occurs later in
systole as valve opening becomes
more restricted.
Con’d
• DIAGNOSIS
– The electrocardiogram shows
• gross right ventricular hypertrophy, frequently
accompanied by a tall, spiked P wave.
– Radiographic studies
• cardiac enlargement with prominence of the right
ventricle and right atrium.
• Prominence of the main pulmonary artery segment may
be seen due to poststenotic dilatation
• Intrapulmonary vascularity is decreased.
– echocardiogram shows
• severe deformity of the pulmonary valve and right
ventricular hypertrophy.
– catheterization
Con’d
 TREATMENT
 Surgical
 COMPLICATIONS
 Heart failure
 Infective endocarditis
 is a risk but is not common in childhood.
 PROGNOSIS
 In untreated severe stenosis, the
course may abruptly worsen with the
development of right ventricular
dysfunction and cardiac failure.
Con’d
2.2 Aortic Stenosis
 Valvular stenosis
 Subvalvular (subaortic) stenosis
 Supravalvular aortic stenosis
 PATHOPHYSIOLOGY.
obstruction to Left ventricular outflow
increased Left
ventricular systolic pressure
left ventricular wall hypertrophy
Con’d
• CLINICAL MANIFESTATIONS.
– Symptoms in patients with aortic stenosis depend on
the severity of the obstruction.
• In mild stenosis
– Normal pulse and apical impulse
– Normal to enlarged heart size
– Systolic ejection murmur
• Severe aortic stenosis (critical aortic stenosis).
– Heart failure, cardiomegaly, and pulmonary edema
are severe.
– the pulses are weak in all extremities, and the skin
may be pale or grayish.
– Urine output may be diminished.
– Rarely, fatigue, angina, dizziness, or syncope may
develop in an older child
– Sudden death has been reported with aortic stenosis
Con’d
 The physical findings are dependent on the degree
of obstruction to left ventricular outflow.
 If the stenosis is severe
 the 1st heart sound may be diminished
 2nd sound may be split paradoxically (becoming
wider in expiration).
 A 4th heart sound may be audible
 The typical murmur is audible maximally at the
right upper sternal border and radiates to the
neck and the left midsternal border.
Con’d
 DIAGNOSIS
 Clinical
 Chest x-ray
 ECG
 ECHO
 Left heart catheterization
 Graded exercise testing
Con’d
 TREATMENT.
 Balloon valvuloplasty
 Surgery
 COMPLICATION
 Heart failure
 Infective endocarditis
 Sudden death
 arrhythmia
Con’d
 PROGNOSIS
 Neonates with critical aortic stenosis
may have severe heart failure and
deteriorate rapidly to a low-output
shock state.
 Those who survive may develop signs
of left ventricular diastolic muscle
dysfunction (restrictive
cardiomyopathy) later in life
Con’d
2.3 Coarctation of the Aorta
 Occur at any site from the arch of aorta to iliac
bifurcation
 98% juxta ductal

Pathogenesis
 In utero arch hypoplasia

Rt heart output passes through the ductus

 Con’d

Clinical Manifestation
 Hypertension → mechanical obstruction
→ renal
 Differential cyanosis → pale upper extr.
→ cyanosed lower extr.
 Classic signs
- Disparity in pulse & BP
- Radio-femoral delay
- Systolic M at LMSB & inter-scapular area
-systolic or continuous murmurs may be heard
over the left and right sides of the chest laterally and
posteriorly.
- a palpable thrill can occasionally be
appreciated in the intercostal spaces on the
Con’d

Diagnosis
- Clinical
- CXR - cardiomegaly & pulm. congestion
- Notching of ribs
- ECG
- Echocardiography
Prognosis – Untreated cases succumb by 20-40 years
Complications - CVA(cerebrovascular accident)
- I/E
- Aneurysms
Treatment
- Medical - IV PGE1 in neonatal age
- Surgery
Con’d

Pulmonary Vascular Disease (Eisenmenger syndrome)

- Occur in shunt lesions
VSD - mainly
ASD
PDA
- Reversal of shunt due to pulm. HTN
→ Cyanosis
II. Cyanotic Congenital
Heart Disease

1.Cyanotic lesions with decreased

pulmonary blood flow
2.Cyanotic CHD With increased
pulmonary blood flow
1.
Con’d
Cyanotic lesions with decreased pulmonary
blood flow
1.1 Tetralogy of Fallot
Consists: 1. Rt ventricular outflow obst.
2. Ventricular septal defect
3. Dextroposition of the aorta
4. Right ventricular hypertrophy
Cyanotic CHD…
Pathophysiology
- Outflow obstruction to right ventricle
- Hypertrophy of subpulmonic muscle
- Normal or small pulmonary valve annulus
- Rarely pulmonary atresia
- VSD - Non-restrictive, located just below
aortic valve
- Aortic arch is right side in 20%
- Right ventricular output shunts to the aorta
 When obstruction to right ventricular outflow is mild to

moderate and a balanced shunt is present across the VSD, the

patient may not be visibly cyanotic (acyanotic or “pink”
tetralogy of Fallot). When obstruction is severe, cyanosis will
be present from birth and worsen when the ductus begins to
close.
Cyanotic CHD…
Clinical Manifestation
- Rarely pink TOF - in the absence of
obstruction
- Cyanosis
- Clubbing
- Squatting position in walking children
- Paroxysmal hypercyanotic attacks
 occur during 1st 2 years
- Systolic ejection M at pulmonic area
- Delayed growth & development
- Single 2nd heart sound
Cyanotic CHD…
Diagnosis
CXR - Narrow base & uplifted apex
- A boot or wooden shoe
- decreased pulm. vascularity
- Right side aortic arch in 20%
ECG
Echocardiography
Complication
- Cerebral thrombosis - in < 2 years
- Brain abscess
- Infective endocarditis
- Polycythemia
- CHF in pink TOF
Cyanotic CHD…
Treatment
Severe outflow obstruction
- Medical Px - PGE1 infusion
- Prevent dehydration
- Partial exchange transfusion
-iron supplementation
- Oral propranolol for tet spells
- Surgery - Blalock Taussig
- Total correction
 Cyanotic CHD…
1.2 Pulmonary Atresia
- With VSD - Extreme form of TOF
- Without VSD - No egress of blood from Rt vent.
- Shunt through foramen ovale to Lt
atrium

Left ventricle

systemic
circulation
Aorta
pulmonic
circulation(Pda)
- Hypoplastic right ventricle)
Cyanotic CHD…
Clinical Manifestation
- Cyanosis at birth
- Respiratory distress
- Single 2nd heart sound
- No murmur
Diagnosis
- CXR
- ECG
- Echocardiography
Treatment - PGE1
- Surgery
Cyanotic CHD…
1.3 Tricuspid atresia
- No outlet from Right atrium to right vent.
- Systemic venous return

Rt atrium

Lt atrium

Left ventricle

systemic Pulmonic
(VSD, PDA)
Cyanotic CHD…
Clinical Manifestation
- Cyanosis at birth
- Polycythemia
- Easily fatiquability
- Exertional dyspnea
Diagnosis
- EXR -Pulm. Under circulation
- ECG -Lt axis deviation & Lt vent.
hpertrophy
- Echocardiography
Cyanotic CHD…

Treatment
- PGE1
- Surgery - Aortico - pulmonary Shunt
- Bidirectional Glenn shunt
- Modified Fontan operation
Cyanotic CHD…
1.4 Ebstein Anomaly of the tricuspid valve
- Down ward displacement of the tricuspid valve
- Right ventricle with two parts
- atrialized
- normal ventricular myocardium
- Abnormal tricuspid valve
- Huge Rt atrium
- Tricuspid regurgitation
- Compromised Rt ventricular function
Cyanotic CHD…
Clinical Manifestations
- Easily fatigability
- Cyanosis
- Dysrhythmia
- Rt to Lt shunt through foramen ovale
- Holosystolic M at tricuspid area
- Heart failure
Diagnosis
- CXR - box shaped heart
- ECG - Right BBB
- Superior axis deviation
Treatment
- PGE1
- Surgery
 Cyanotic CHD…
2.Cyanotic CHD With increased pulmonary
blood flow
2.1 Transposition of GA
a. D -TGA (uncorrected)
- Systemic venous return to Rt atrium
Normal
- Pulmonary venous return to Lt atrium
- Aorta arises from Right ventricle
- Pulm. artery arises from Lt vent.
Pathology
Cyanotic CHD…

*Systemic & Pulmonary Circulations Consists of

two parallel circuits
*Survival is with associated - patent foramen
ovale or
- VSD or
- PDA
Clinical Manifestations
- Tachypnea & cyanosis at birth
- Rarely congestive heart failure
 Cyanotic CHD…
b. L. TGA (corrected transposition)
 Systemic VR to normally positioned Rt atrium

Through bicuspid (Mitral) valve

Right sided left ventricle

Pulmo. artery  pulm. venous return

Normally positioned Lt atrium

Through tricuspid valve

Left sided Right ventricle  Aorta
Cyanotic CHD…
 Discordant atrio-ventricular relation
(ventricular inversion)
 Transposition of great arteries
Clinical Manifestation
Depends on associated malformation
Cyanotic CHD…
Diagnosis
- Clinical
- CXR - Cardiomegaly
- Narrow mediastinum (egg on string)
- Increased pulmonary blood flow
- ECG
- Echocardiography
Treatment
- PGE1 - emergency
- Surgery
Cyanotic CHD…
2.2 Truncus arteriosus
- Single arterial trunk for both pulm. &
systemic circ.
- 4 types depending the origin of pulmonary
artery
Clinical Manifestation
- Cyanosis
- CHF at 2-3rd
month
- Systolic ejection m
Treatment - surgery
Cyanotic CHD…
2.3 Total Anomalous Pulm. Venous return
- Pulm. drainage into systemic vein
Cyanotic CHD…
2.4 Single Ventricle
- No interventricular septum
Cyanotic CHD…
2.5 Hypoplastic Left Heart Syndrome
- Under development of Lt Side of the
heart
- Atretic aortic or mitral orifice
- Non functional Lt ventricle
- Hypoplasia of ascending aorta
 Right ventricle supplies both pulm. &
systemic circulation
Cyanotic CHD…
2.6 Persistent fetal circulation
Cyanotic CHD…
2.6 Dextroposition of the heart
2.7 Dextrocardia