This document discusses several types of cyanotic heart defects including tetralogy of Fallot, pulmonary valve stenosis, ventricular septal defect, overriding aorta, right ventricular hypertrophy, hypoplastic left heart syndrome, and atrial septal defect. Tetralogy of Fallot involves four abnormalities that reduce oxygenated blood flow to the body. Hypoplastic left heart syndrome occurs when the left side of the heart does not properly develop. Atrial septal defects allow oxygenated blood to flow from the left to right atria, reducing blood oxygen levels. These defects cause symptoms like cyanosis and shortness of breath and often require surgery to repair or replace the heart.

1. CYANOTIC HEART
DEFECTS

2. TETRALOGY OF FALLOT

3. FOUR HEART ABNORMALITIES
1. STENOSIS
2. RIGHT VENTRICULAR HYPERTROPHY
3. VENTRICULAR SEPTAL DEFECT
4. AORTA OVERIDES THE SEPTAL
DEFECT
TETROLOGY OF FALLOT

4. PULMONARY VALVE STENOSIS
Pulmonary valve stenosis is a narrowing of the pulmonary
valve — the valve that separates the lower right chamber
of the heart (right ventricle) from the main blood vessel
leading to the lungs (pulmonary artery).
Narrowing (constriction) of the pulmonary valve reduces
blood flow to the lungs. The narrowing might also affect
the muscle beneath the pulmonary valve. In some severe
cases, the pulmonary valve doesn't form properly
(pulmonary atresia) and causes reduced blood flow to the
lungs

5. VENTRICULAR SEPLAT DEFECT
● A ventricular septal defect is a hole (defect) in the wall (septum)
that separates the two lower chambers of the heart — the left
and right ventricles. The hole allows deoxygenated blood in the
right ventricle — blood that has circulated through the body and
is returning to the lungs to replenish its oxygen supply — to flow
into the left ventricle and mix with oxygenated blood fresh from
the lungs.
● Blood from the left ventricle also flows back to the right ventricle
in an inefficient manner. This ability for blood to flow through the
ventricular septal defect reduces the supply of oxygenated blood
to the body and eventually can weaken the heart.

6. OVERRIDING AORTA
Normally the aorta — the main artery leading out to the body
— branches off the left ventricle. In tetralogy of Fallot, the
aorta is shifted slightly to the right and lies directly above the
ventricular septal defect.
In this position the aorta receives blood from both the right
and left ventricles, mixing the oxygen-poor blood from the
right ventricle with the oxygen-rich blood from the left
ventricle.

7. RIGHT VENTRICULAR
HYPERTROPHY
When the heart's pumping action is
overworked, it causes the muscular wall of the
right ventricle to thicken. Over time this might
cause the heart to stiffen, become weak and
eventually fail.

8. CAUSES
Causes is unclear.
Assoiated with
● Chromosome 22 deletions
● DiGeorge syndrome

9. SYMPTOMS
● A bluish coloration of the skin caused by blood low in oxygen
(cyanosis)
● Shortness of breath and rapid breathing, especially during feeding
or exercise
● Loss of consciousness (fainting)
● Clubbing of fingers and toes — an abnormal, rounded shape of
the nail bed
● Poor weight gain
● Tiring easily during play or exercise
● Irritability
● Prolonged crying
● A heart murmur

10. TET SPELLS
Sometimes, babies who have tetralogy of Fallot will
suddenly develop deep blue skin, nails and lips after
crying or feeding, or when agitated.
These episodes are called tet spells and are caused by a
rapid drop in the amount of oxygen in the blood. Tet
spells are most common in young infants, around 2 to 4
months old. Toddlers or older children might instinctively
squat when they're short of breath. Squatting increases
blood flow to the lungs.

11. RISK FACTORS
● A viral illness during pregnancy, such as rubella
(German measles)
● Alcoholism during pregnancy
● Poor nutrition during pregnancy
● A mother older than age 40
● A parent who has tetralogy of Fallot
● The presence of Down syndrome or DiGeorge
syndrome

12. COMPLICATIONS
● All babies who have tetralogy of Fallot need corrective
surgery.
● Baby may also be at an increased risk of serious
complications, such as infective endocarditis — an infection
of the inner lining of the heart or heart valve caused by a
bacterial infection.
● Untreated cases of tetralogy of Fallot usually develop severe
complications over time, which might result in death or
disability by early adulthood.

13. DIAGNOSIS
● Echocardiography
● Electrocardiogram
● Chest X-ray.
● Oxygen level measurement (pulse oximetry).
● Cardiac catheterization.

14. TREATMENT
● Intracardiac repair Surgery -
○ 1st year
○ Septal defect closed
○ RV outflow tract enlarged
○ RV returns to normal thickness
○ So only oxygen rich blood flow into the
aorta
● Temporary surgery

15. HYPOPLASTIC LEFT
HEART SYNDROME

16. ● Hypoplastic left heart syndrome (HLHS) is a birth
defect that affects normal blood flow through the
heart.
● As the baby develops during pregnancy, the left
side of the heart does not form correctly.
● A baby with this defect needs surgery or other
procedures soon after birth.

17. Hypoplastic left heart syndrome affects a number of structures on
the left side of the heart that do not fully develop, for example:
● The left ventricle is underdeveloped and too small.
● The mitral valves is not formed or is very small.
● The aortic valve is not formed or is very small.
● The ascending portion of the aorta is underdeveloped or is too
small.
● Often, babies with hypoplastic left heart syndrome also have
an atrial septal defect, which is a hole between the left and
right upper chambers (atria) of the heart.

18. In babies with hypoplastic left heart syndrome, the left side
of the heart cannot pump oxygen-rich blood to the body
properly. During the first few days of life for a baby with
hypoplastic left heart syndrome, the oxygen-rich blood
bypasses the poorly functioning left side of the heart through
the patent ductus arteriosus and the patent foramen ovale.
The right side of the heart then pumps blood to both the
lungs and the rest of the body. However, among babies with
hypoplastic left heart syndrome, when these openings close,
it becomes hard for oxygen-rich blood to get to the rest of
the body.

19. CAUSES
Causes is unknown
Theory;
Primary defect
⬇
Reduces blood flow through LV & outflow tract
during fetal development
⬇
Causing abnormal growth

20. SYMPTOMS
Babies born with hypoplastic left heart syndrome usually
are seriously ill soon after birth. Hypoplastic left heart
syndrome symptoms include:
● Grayish-blue skin color (cyanosis)
● Rapid, difficult breathing
● Poor feeding
● Cold hands and feet
● Being unusually drowsy or inactive

21. In a baby with hypoplastic left heart syndrome, if the
natural connections between the heart's left and right sides
(foramen ovale and ductus arteriosus) are allowed to close,
he or she may go into shock and may die. Signs of shock
include:
● Cool, clammy skin that may be pale or gray
● A weak and rapid pulse
● Abnormal breathing that may be either slow and shallow
or very rapid
● Dilated pupils
● Lackluster eyes that seem to stare

22. COMPLICATIONS
Without surgery, hypoplastic left heart syndrome is fatal, usually
within the first few weeks of life.
With treatment, many babies survive, although most will have
complications later in life. Some of the complications may include:
● Tiring easily when participating in sports or other exercise
● Heart rhythm abnormalities (arrhythmias)
● Fluid buildup in the lungs, abdomen, legs and feet (edema)
● Formation of blood clots that may lead to a pulmonary embolism
or stroke
● Developmental problems related to the brain and nervous system
● Need for additional heart surgery or transplantation

23. ATRIAL SEPTAL DEFECT
An atrial septal defect (ASD) allows freshly
oxygenated blood to flow from the left upper
chamber of the heart (left atrium) into the
right upper chamber of the heart (right
atrium). There, it mixes with deoxygenated
blood and is pumped to the lungs, even
though it's already refreshed with oxygen.

24. If the atrial septal defect is large, this extra
blood volume can overfill the lungs and
overwork the right side of the heart. If not
treated, the right side of the heart
eventually enlarges and weakens. If this
process continues, the blood pressure in
your lungs may increase as well, leading to
pulmonary hypertension.

25. SYMPTOMS
Many babies born with atrial septal defects don't have associated signs or
symptoms. In adults, signs or symptoms may begin around age 30, but
in some cases signs and symptoms may not occur until decades later
Atrial septal defect signs and symptoms may include:
● Shortness of breath, especially when exercising
● Fatigue
● Swelling of legs, feet or abdomen
● Heart palpitations or skipped beats
● Stroke
● Heart murmur, a whooshing sound that can be heard through a
stethoscope

26. RISK FACTORS
● Rubella infection.
● Drug, tobacco or alcohol use, or
exposure to certain substances.
● Diabetes or lupus.
● Obesity
● Phenylketonuria

27. COMPLICATIONS
Larger defects can cause serious problems,
including:
■ Right-sided heart failure
■ Heart rhythm abnormalities (arrhythmias)
■ Increased risk of a stroke
■ Shortened life span
■ Pulmonary hypertension.
■ Eisenmenger syndrome

28. DIAGNOSIS
● Before birth ~ Prenatal ultrasound
● After birth ~ Echocardiography

29. TREATMENT
● Medication
○ Prostaglandin/ alprostadil-helps dilate
the blood vessels and keeps the
ductus arteriosus open
● Atrial septostomy
● Surgery
○ Fix heart defect
○ Heart transplant

30. THANK YOU
AMMU BALAN