Neonatalogy congenital heart malformation

1. COLLEGE OF HEALTH SCIENCE
DEPARTMENT OF MIDWIFERY
COURSE NEWBORN CARE
FOR POSTBASIC
Congenital Heart Disease
1
03-01-2023 BY SHambel N.

2. Learning objectives
At end of this topic student able to
• Know what is congenital heart diseases
• Describe types of congenital heart diseases according to levels of
arterial oxygen saturation
• Understand the cyanotic and acyanotic heart diseases ?
• Understand the defect classified under teratology Fallot
• Know the clinical presentation and management of cyanotic and
acyanotic heart diseases

3. Congenital heart disease
Congenital heart disease (CHD) is a defect or problem with the heart’s
structure that’s present at birth, such as:
A hole in the heart wall.
Issues with the blood vessels ( blood flowing too slowly, to the wrong place or
in the wrong direction).
Problems with the heart valves.
Some cases of CHD are simple and may not cause any symptoms, but
others can be life-threatening and require treatment.
Heart defects may be detected early (before a baby is born or shortly
afterward).

4. congenital heart disease…
• It is the most common type of birth defect, affecting 8 to 9 per 1,000
live births.
• There are two main groups of CHD according to levels of arterial
oxygen saturation
• cyanotic congenital heart disease
• acyanotic congenital heart disease.

5. Cyanotic heart disease
It’s sometimes called critical congenital heart disease
It is congenital heart defect (CHD) that occurs due to deoxygenated
blood bypassing the lungs and entering the systemic circulation, or a
mixture of oxygenated and unoxygenated blood entering the systemic
circulation
When a baby is born with cyanotic CHD, their skin has a bluish tint, called
cyanosis.
It involves heart defects that reduce the amount of oxygen delivered to the
rest of the body.
Babies born with cyanotic congenital heart disease usually have low levels
of oxygen and need surgery

6. Cyanotic heart disease
Types Cyanotic heart disease
Left heart obstructive lesions
Right heart obstructive lesions
Mixing lesions
• Left heart obstructive lesions : This type of defect reduces blood flow
between the heart and the rest of the body (systemic blood flow).
• Examples include hypoplastic left heart syndrome (when the heart is too small
on the left side) and interrupted aortic arch (aorta is incomplete).
• Interrupted aortic arch (IAA) is a rare condition in which baby’s aorta — the
large blood vessel coming from their heart that carries blood to the rest of their
body — is incomplete or interrupted

7. Cyanotic heart disease…
Right heart obstructive lesions:
• This type of defect reduces blood flow between the heart and lungs (pulmonary
blood flow).
• Examples include tetralogy of Fallot (a group of four defects), pulmonary atresia ,
overriding aorta and tricuspid atresia (valves don’t develop correctly).
Mixing lesions:
• With this type of defect, the body mixes systemic and pulmonary blood flow.
• Example
• transposition of the great arteries when the two main arteries leaving the heart are reversed.
• Another is truncus arteriosus, when the heart has only one main artery, instead of two, to carry
blood to the body.

8. Hypoplastic left heart syndrome (HLHS
Hypoplastic left heart syndrome (HLHS) is a complex and rare heart
condition that’s present at birth (congenital).
accounts for about 2% to 3% of all congenital heart diseases
 one side of your baby’s heart didn’t develop correctly.
In babies born with hypoplastic left heart syndrome, some structures in the
left side of their heart didn’t develop completely, including:
Left ventricle: is lower left heart’s chamber, which pumps oxygen-rich blood to
aorta.
Aorta: largest blood vessel. It takes blood from heart to the rest of body.
Mitral and aortic valves: These flaps of tissue open and close to control how blood
moves through your heart.
 aortic valve allows blood to pass from left ventricle to aorta.
 mitral valve controls blood flow from top heart chamber (atrium) to lower heart chamber
(ventricle)

9. Hypoplastic left heart syndrome (HLHS..
Hypoplastic left heart syndrome vs. normal heart
• In normal heart the right side moves oxygen-poor blood to lungs to
get oxygen. The left side sends oxygen-rich blood to body.
• In a baby with hypoplastic left heart syndrome, the heart’s left side is
too small to pump enough blood to the rest of the body.
• Their right ventricle or right lower chamber of their heart then takes
over to pump blood to both their lungs.

10. Hypoplastic left heart syndrome (HLHS)…

11. Hypoplastic left heart syndrome (HLHS)…

12. Hypoplastic left heart syndrome (HLHS)…
Hypoplastic left heart syndrome symptoms may include:
Cyanosis, or a grayish (in dark-skinned people) or bluish (in light-skinned
people) discoloration of skin, lips and nails.
Difficulty breathing
Difficulty feeding
Lack of energy (lethargy)
Rapid heartbeat.
Sweaty, clammy or cool skin
Weak pulse

13. cause
• No exact known cause
• Genetic. Babies with mutations (changes) in specific genes may have
a higher risk of hypoplastic left heart syndrome.
• Turner syndrome or trisomy 18

14. Diagnosis
Ultrasound.
Fetal echocardiogram (ultrasound) to evaluate baby’s heart before
birth.
Chest X-ray: This shows the size and shape of baby’s heart and
lungs.
Echocardiogram: This ultrasound shows internal heart structures.
Electrocardiogram (EKG): This measures electrical changes during
a heartbeat.
Pulse oximetry screening: This tells how much oxygen is in baby’s
bloodstream

15. Management
• prostaglandin -keeps their ductus arteriosus open and functioning.
• surgeries to direct blood flow to their lungs and body
• Hypoplastic left heart syndrome(HLHS) surgery stages are the
Norwood, Glenn and Fontan procedures.
• Reconstruct underdeveloped aorta to provide blood flow to their body.
• Create a connection between the upper chambers of their heart. This
drains oxygen-rich blood from their lungs to supply their body.

16. Complications with the treatment
• issues with their right ventricle not working right.
• An aortic valve leak.
• Abnormal heart rhythms.
• Blood clots
• Infection
• Trouble eating
• seizures
• Kidney problems
• Cardiac arrest

17. Tetralogy of Fallot
• Tetralogy of is a birth defect that affects normal blood flow through
the heart.
• It happens when a baby’s heart does not formed correctly as the baby
grows and develops in the mother’s womb during pregnancy.
• Tetralogy of Fallot is made up of the following four defects of the
heart and its blood vessels:
• A hole in the wall between the two lower chambers―or ventricles―of the
heart--ventricular septal defect.
• A narrowing of the pulmonary valve --pulmonary stenosis.
• Overriding of the aorta, in which the aorta lies directly above the ventricular
septal defect. Normally, the aorta is connected to the left ventricle, and
oxygenated blood flows out of the ventricle into the aorta---overriding aorta
• The muscular wall of the lower right chamber of the heart (right ventricle) is
thicker than normal. This also is called ventricular hypertrophy

18. Tetralogy of Fallot…

19. Tetralogy of Fallot…
• may need surgery or other procedures soon after birth, this birth defect is
considered a critical congenital heart defect.
• Infants with tetralogy of Fallot can have a bluish-looking skin color―called
cyanosis―because their blood doesn’t carry enough oxygen.
• Infants with tetralogy of Fallot have problems including:
• A higher risk of getting an infection of the layers of the heart, called endocarditis.
• A higher risk of having irregular heart rhythms, called arrhythmia.
• Dizziness, fainting, or seizures, because of the low oxygen levels in their blood.
• Delayed growth and development

20. Causes and Risk Factors
• The causes of heart defects (such as tetralogy of Fallot) among most
babies are unknown.
• genes or chromosomes.
• genes and environment or
• what the mother eats or drinks or the medicines she uses.

21. Diagnosis
Physical examination.
• The patient is cyanotic with a systolic ejection murmur along the left sternal border.
• Loud murmurs are associated with more flow across the right ventricular outflow
tract and milder degrees of desaturation.
• newborn screening using pulse oximetry
Chest radiograph.
• The chest radiograph film reveals a small, often “bootshaped” heart, with
decreased pulmonary vascular markings.
Electrocardiography
• The ECG may be normal or may demonstrate right ventricle hypertrophy
(RVH).
Echocardiography. Usually diagnostic, with an overriding aorta, ventricular
septal defect (VSD), and small right ventricular outflow tract.

22. Treatments
• surgery soon after the baby is born
• widen or replace the pulmonary valve and enlarge the passage to the
pulmonary artery.
• place a patch over the ventricular septal defect to close the hole
between the two lower chambers of the heart. These actions will
improve blood flow to the lungs and the rest of the body.
• Tetralogy of Fallot is a lifelong medical condition. Therefore it needs
regular follow-up visits with a cardiologist to monitor their progress
and check for other health conditions that might develop as they get
older.

23. Acyanotic congenital heart disease
• With this type of heart defect, blood contains enough oxygen, but it’s
pumped throughout the body abnormally.
• These defects don’t interfere with the amount of oxygen or blood
delivered to the rest of the body.
• Acyanotic congenital heart disease involves a defect that causes blood
to pump through the body abnormally. For example:
• Obstruction (‘narrowing’)
• Pulmonary stenosis 8%
• Coarctation of the aorta (6%)
• Aortic stenosis (5%)

24. Acyanotic congenital heart disease..
• Shunts (‘holes’), left‐to‐right shunting
• SD (ventricular septal defect) 32% •
• PDA (patent ductus arteriosus) 12%
• ASD (atrial septal defect) 6%
• AVSD (atrioventricular septal
• Pump failure
• Supraventricular tachycardia (SVT)
• Cardiomyopathy

25. Acyanotic congenital heart disease..
Aortic stenosis:
The aorta is the main artery that carries blood away from your heart to the rest of the
body.
The aortic valve sits between the heart and aorta.
It opens and closes with each heart cycle to let blood out of the heart and keep blood
from leaking back into the heart.
When the aortic valve becomes narrowed and doesn’t open well, it’s called aortic
stenosis.
Pulmonary stenosis:
The pulmonary artery carries oxygen-poor blood from the right side of the
heart to the lungs to be oxygenated.
The pulmonary valve sits between the right side of the heart and the
pulmonary artery. When the pulmonary valve becomes narrowed and
doesn’t open well, it's called pulmonary stenosis.

26. Acyanotic congenital heart disease..
Atrial septal defect:
is a hole in the wall that separates the heart’s two upper chambers, called the
atria.
Most ASDs don't close on their own.
Tiny ASDs can often be left alone, but larger ASDs may require a procedure or
heart surgery to close them.
• Most of the patients with ASD usually remain asymptomatic. The
following signs can be elicited during the examination of
the cardiovascular system.
• Systolic ejection murmur
• Chest X-ray shows cardiomegaly with prominent pulmonary vasculature

27. Acyanotic congenital heart disease..
Atrioventricular septal defect:
An atrioventricular septal defect is a hole in the center of the heart, in the wall
that separates the upper chambers from the lower chambers.
 Most people with atrioventricular septal defect have abnormalities of some of
their heart valves (the mitral and tricuspid valves) as well
The defect was previously also called an atrioventricular canal defect or an
endocardial cushion defect

28. Acyanotic congenital heart disease..
Ventricular septal defect:
is like an atrial septal defect, but it’s a hole in the wall that separates the
heart’s two lower chambers (the ventricles).
VSD is the most common congenital heart defect.
Many of these holes can close on their own, but some may require a
procedure or a surgery to close them.

29. Acyanotic congenital heart disease..

30. Causes of acyanotic heart disease
• Abnormal chromosomes
• Genetics
• Illnesses in the mother during pregnancy, such as diabetes,
phenylketonuria (a blood disorder), drug use or viral infection

31. Clinical manifestation
• Breathlessness.
• Dizziness.
• Fatigue (extreme tiredness).
• Syncope (fainting).

32. Diagnosis
Physical examination--a systolic murmur resulting from
atrioventricular (AV) valve insufficiency may be heard.
Chest X-ray: takes pictures inside the chest to reveal any structural
abnormalities.
Electrocardiogram: (EKG or ECG) measures the electrical activity of
the heart.
Echocardiogram: (echo) uses ultrasound to create images of the
heart’s valves and chambers.
Heart catheterization: Right heart catheterization can tell how well
heart is pumping.

33. Treatment
Acyanotic heart disease sometimes corrects itself during childhood.
Catheter procedure to place a plug into the defect.
Medications to help the heart work more efficiently or to control
blood pressure.
Surgery to close the defect.

34. Cyanotic vs Acyanotic Congenital Heart Defects
Cyanotic Acyanotic Congenital
Blood moves from right side to
the left side of the heart.
Blood moves from left side to the right
side of the heart.
The blood moving to the left side
is deoxygenated.
The blood moving to the right side is
oxygenated.
Cyanosis is present Cyanosis is absent.