2. INCIDENCE:
The overall incidence of congenital heart diseases is
about
8 – 10 percent per 1000 live births
Defect
VSD
PDA
ASD
Coarctation of aorta
TOF
Percentage
25 – 30 %
10 %
10 %
6%
5- 9 %
3. ETIOLOGY AND INCIDENCE
Hereditary factors
CHD affects 8 – 12 of every 1000 neonates
Associated factor for CHD include
Ø
Fetal or Maternal infection during the first trimester
(Rubella)
Ø
Chromosomal abnormality (Trisomy 21, 18, 13)
Ø
Maternal diabetes
Ø
Teratogenic effects of drugs and alcohol
Syndromes that include CHD
Ø
Ø
Ø
Ø
Marfan`s syndrome
Turner’s syndrome
William’s syndrome
Down syndrome
: Mitral value prolapse
: Aortic value stenosis, COA
: Dysplastic pulmonary value
: Triosomy
4. Congenital Heart diseases
Cyanotic heart
Acyanotic heart
Obstructive
diseases ( R to L shunt )
diseases ( L to R ) .Coarctation of aorta
Fallots tetrology
. VSD
. Vascular ring
Transposition of greater
. ASD
. Pulmonary stenosis
vessels
Tricuspid atresia
. PDA
. Aortic stenosis
5. VENRICULAR SEPTAL DEFECT (VSD)
Definition
VSD is an abnormal communication between the two ventricles
6.
7.
8. Pathophysiology
Blood is shunted from the left to the right ventricles in most of
the
cases due to the relatively high pressure of the left ventricles
chamber
If the defect is large, the amount of blood shunted in to the right
ventricles may be quite large resulting in increased workload
for both ventricles
Right ventricles increased
pulmonary
enlargement develops
right
ventricular
out
put
and
Blood increased return to the left atrium, thus increasing the
work
of the left ventricles, resulting in bi – ventricular hypertrophy
Pulmonary over circulation cause a change in the pulmonary
arterial bed, leading to increased pulmonary artery vascular
resistance
9. Clinical manifestation
Small VSD`s : usually a symptomatic; High spontaneous closure
rate during the first year of life
Large VSD`s:
CHF – tachypnea, tachycardia, excessive sweating
Frequent URI
Poor weight gain, Failure to thrive
Feeding difficulties
Murmur present
Pulmonary vascular obstructive diseases
10. Investigation
X. - ray chest - ventricular hypertrophy
Small
-Normal
Moderate VSD - shunt vascularity (pulmonary plethora)
Ventricular hypertrophy
o
Pulmonary artery increased size.
Large VSD
- Biventricular hypertrophy
Increased pulmonary trunk
Left arterial enlargement.
– Enlarged main pulmonary artery
--- Right ventricular hypertrophy
--- Peripheral pruning with apparent decrease in shunt
--- vascularity.
12. ECG
Echo and Doppler study
Cardiac cauterization study
Angiocardiography
13. Management of VSD:
Aims: 1. To achieve normal growth by controlling ccf
2. Prevention and treatment of anemia
3. Prevention and treatment of infective endocarditis
14. 1. Medical management : 1. CHF management :digoxin and diuretics(furasemide,
spironolactone) and after reduction
2. Avoid oxygen : - oxygen is a potent pulmonary
vasodilator and will increase blood flow in to the P.A
3. Increase caloric intake: fortify formula or breast milk
to make 24 to 30 caloz formula, supplemental
nasogastric feeds as needed.
4. Ineffective endocarditis prophylaxis for 6 months after
surgery.
2.Surgical treatment: Corrective surgery done in first 2 years of life
prevents progression of pulmonary hypertension
15. Surgeries: 1. Corrective surgery –patch graft – Dacron / Natural
2. Palliative surgery – Pulmonary artery banding
Complication:
C.H.F
Recurrent respiratory tract infection
Ineffective endocarditis
Failure to thrive: poor weight gain
Pulmonary
arterial
hypertension
elsenmengerisation
Aortic or tricuspid regurgitation
Right ventricular outflow tract obstruction.
and
16. ATRIAL SEPTAL DEFECT(ASD )
Definition: Atrial septal defect is an abnormal communication
between the two atria.
17. Pathophysiology:
Blood flows from the higher pressure left atrium
across the ASD in to the lower pressure right atrium.
Increased blood return to the right heart leads to right
ventricular volume over load.
Right ventricular dilatation
Increased pulmonary blood flow leads to elevated
pulmonary artery pressure.
18.
19. Clinical manifestation: 1. Usually a symptomatic
2. CHF
3. Frequent upper respiratory tract infection
4. Poor weight gain
5. Decreased exercise tolerance.
Diagnostic evaluation: X-Ray Right atrial and ventricular enlargementenlargement of pulmonary artery
E.C.G
Auscultation:
soft systolic ejection murmur heard
best at the left upper sternal border.
Cardiac caterization
20. Management: 1.Medical management
a).Monitor and reassess
b).Treatment with anticongestive therapy (digoxin and
lasix) may be necessary. if signs of CHF are
present
c).Infective endocarditis prophylaxis for 6 months
after
surgery or atrial occlusion devise is used.
2. Cardiac catherisation for placement of an atrial
occlusion device for ostium secundam defects.
3.Surgical intervention:
a) Primary repair suture closure of the ASD.
b) Patch repair of the ASD.
22. Patent ductus arteriosus (PDA)
Definition
This defect, which normally occurs during fetal life, short circuits the
normal pulmonary vascular system and allows blood to mix between the
pulmonary artery and the aorta. Prior to birth, there is an open passageway
between the two blood vessels, which closes soon after birth. When it does
not close, some blood returns to the lungs. Patent ductus arteriosus is
often seen in premature infants.
23. Pathophysiology:
During fetal life, the ductus arteriosus allows blood to by pass the
pulmonary circulation and flow directly in to the systemic
circulation.
After birth, the ductus arteriosus is no longer needed. Functional
closure usually occurs within 48 hrs after birth.
When the ductus arteriosus fails to close blood from the aorta
( high pressure) flows in to the lower pressure PA.
Resulting in pulmonary over circulation
Increased pulmonary blood flow leads to a volume- loaded LV.
24.
25. Clinical manifestation:
1. Growth retardation
2. External dyspnea
3. CCF
4. Pericardial pain
5. Cough
6. Dyspnoea
7. Tachypnoea.
8. Dyspnoea
9. Tacycardia
10. Hepato splenomegali
11. Machinery murmur. It is harsh and may be
localized to
second left intercostals space or
transmitted to left
clavicle to lower down (ie) left
sternal border. It is accomplished by a thrill .
26. Diagnostic evaluation : Chest X-Ray- cardiomegaly
ECG
ECHO
Cardiac catherization; raised pressure in right ventricles and
pulmonary artery.
Management: 1.In the symptomatic premature neonate; Indomethacin. Given IV.
2.Medical management:
a) Monitor growth and development
b) Reassures for spontaneous PDA closure
c) Increase caloric intake as needed for normal weight gain
d) Diuretics: furusemide (lasix), spironolactone (Aldactone).
e) Ineffective endocarditis prophylaxis for 6 months after
surgery.
27. 3.Cardiac catherization:
a) For small PDAs coil occlusion
b) For large PDAs closure device may be used.
4.Surgical management through PDA ligation.
Complication:
1. CHF, pulmonary oedema
2. Infective endocarditis
3. Pulmonary hypertension
4. Recurrent pneumonia.
29. PATHOPHYSIOLOGY
The blood normally returns from the systemic circulation to the
systemic circulation to the right atrium and right ventricles
The outflow of blood from the right ventricles is resisted by the
pulmonary stenosis so that the blood flows through the
ventricular septal defect in to the aorta
There is right to left shunt. Hypertrophy of the right ventricles
occurs as a result of the pressure exerted against the pulmonary
stenosis.
Because, the blood from the right ventricles is unoxygenated,
cyanosis results.
30. Clinical manifestation: 1. Cyanotic episodes: cyanotic spells may occur while
crying and after feeding. After cyanotic spells, there
may be limpness, fatigue and fainting.
2. Dyspnoea
3. Delayed physical growth and development
4. Pansystolic murmur may be heard at the middle to
lower
sternal borders
5. Cyanosis- may be seen mucous membrane of the
lips,
mouth and pharynx and in fingernails and
toe- nails.
6. Clubbing of the fingers
7.
Paroxysmal dyspneic attacks (anoxia, “ blue “
spells)
occur during the first 24 months of life and
last for a few minutes to hours .
31. Diagnosis: 1. Blood studies.
2. X-Ray chest
3. ECG- right ventricular hypertrophy.
4. Echo- evidence of the aortic override, thick anterior
right ventricular wall and large aorta.
Medical and Nursing management :
Palliative and corrective surgery for tetrology of fallot
is being done in infants and children of all ages.
32. Transposition of the great arteries
This congenital heart defect, the positions of the pulmonary
artery and the aorta are reversed, thus:
o The aorta originates from the right ventricle, so most of the
blood returning to the heart from the body is pumped back out
without first going to the lungs.
o The pulmonary artery originates from the left ventricle, so that
most of the blood returning from the lungs goes back to the
lungs again
33.
34. Pathophysiology : In this anomaly the aorta has its origin in the right ventricles and
pulmonary artery has its origins in the left ventricles.
Hence, the aorta carries unoxygenated blood to the systemic
circulation and the pulmonary circuit carries oxygenated blood
back to the lungs.
The pulmonary venous return is to the left atrium and the
systemic veins returns to the right atrium.
There is two separate circulatory systemic exist, one pulmonary
and one systemic. An infant can survive with this malformation
initially only if an associated with defect or PDA is present
There co-existing lesions provide a means for mixing venous and
35. Clinical manifestation : 1. Cyanosis from neonatal period and polycythemia
2. Congestive cardiac failure
3. Hypercapnoea due to low arterial oxygen
4. Delayed growth and development
5. Metabolic acidosis
6. Clubbing of the finger and toes.
Diagnostic evaluation : 1. Physical examination – if defect is there murmur can be heard
2. X-ray- cardiomegaly and increased pulmonary vasculature
3. Fluoroscopy- egg shaped” cardiac contour can be identified
4. Echo- Right ventricular hypertrophy
5. ECG
6. Angiocardiography
Cardiac catherization
36. Treatment: Procedure used for the treatment of transposition of
the great vessels are palliative and corrective.
37. Coarctation of the aorta
Aortic coarctation is a narrowing of part of the aorta (the
major artery leading out of the heart). It is a type of birth
defect. Coarctation means narrowing. It accounts for 8 -10%
of CHD and is 2 to 5 time more common in male.
40. Causes
The aorta carries blood from the heart to the vessels that supply
the body with blood and nutrients. If part of the aorta is
narrowed, it is hard for blood to pass through the artery.
Aortic coarctation is more common in Turner syndrome.
Coarctation of the aorta may be seen with other congenital heart
defects, such as:
Bicuspid aortic valve
Defects in which only one ventricle is present
Ventricular septal defect
41. Clinical manifestation
Asymptomatical until the PDA begin to close
After PDA closure:Sever CHF
Tachypnea
Acidosis
Prograsive circulatory shock
Absent femoral and pedal pulses
42. Chest pain
Cold feet or legs
Dizziness or fainting
Decreased ability to exercise
Failure to thrive
Leg cramps with exercise
Nosebleed
Poor growth
Pounding headache
43. Diagnostic evaluation
1. Physical examination –The pulse in the groin (femoral) area
or feet will be weaker than the pulse in the arms or neck
(carotid). Sometimes, the femoral pulse may not be felt at all and
murmur sound can be heard ,
2. X-ray- cardiomegaly
3. ECG
4. Echo- Right ventricular hypertrophy
5. Angiocardiography
6. Cardiac catherization
7. Heart CT may be needed in older children
8. MRI or MR angiography of the chest may be needed in older
children
44. Management
1. Medical Menagement
Resuscitation and stabilization with Prostaglandin E1 infusion
Intubation and ventilation as needed
Infective endocarditis prophylaxis
Anticongestive theraphy( digixin and lasix)
Assessment of renal ,hepatic,and nurologic function.
2. Ballon angioplasty may be indicated for infants who are a high surgical
risk.
3. Surgical intervention: usually performed as soon as the diagnosis is made
Subclavian flap repair
End to end anastomosis
Dacron patch repair
4. hypertention management is needed for the older children
46. Pulmonary stenosis
Pulmonary valve stenosis is a heart valve disorder that
involves the pulmonary valve.
This valve separates the right ventricle (one of the chambers
in the heart) and the pulmonary artery. The pulmonary
artery carries oxygen-poor blood to the lungs.
Stenosis, or narrowing, occurs when the valve cannot open
wide enough. As a result, less blood flows to the lungs.
47.
48. Causes
Narrowing of the pulmonary valve is usually present at birth
(congenital). It is caused by a problem that occurs when the
unborn baby (fetus) is developing. The cause is unknown,
but genetics may play a role.
Pulmonary valve stenosis is a rare disorder.
In some cases, pulmonary valve stenosis more in families.
49. Clinical manifestation
These infants are usually found to have a murmur on a routine heart
examination.
When the valve narrowing (stenosis) is moderate to severe, the
symptoms include:
Bluish color to the skin (cyanosis) in some patients
Chest pain
Fainting
Fatigue
Poor weight gain or failure to thrive in infants with severe blockage
Shortness of breath
Sudden death
Symptoms may get worse with exercise or activity.
50. Diagnostic evaluation
Physical examination:- The health care provider may hear
a heart murmur when listening to your heart using a
stethoscope. Murmurs are blowing, whooshing, or rasping
sounds heard during a heartbeat.
Tests used to diagnose pulmonary stenosis may include:
Cardiac catheterization
Chest x-ray
ECG
Echocardiogram
MRI of the heart
51. Treatment
Sometimes, treatment may not be needed if the disorder is mild.
When there are also other heart defects, medications may be used to:
Help blood flow through the heart (prostaglandins)
Help the heart beat stronger
Prevent clots (blood thinners)
Remove excess fluid (water pills)
Treat abnormal heartbeats and rhythms
Percutaneous balloon pulmonary dilation (valvuloplasty) may be performed when
no other heart defects are present.
This procedure is done through an artery in the groin.
The doctor sends a flexible tube (catheter) with a balloon attached to the end up
to the heart. Special x-rays are used to help guide the catheter.
The balloon stretches the opening of the valve.
Some patients may need heart surgery to repair or replace the pulmonary valve.
The new valve can be made from different materials. If the valve cannot be
repaired or replaced, other procedures may be needed.
53. NURSING CARE OF THE CHILD WITH CONGENITAL HEART
DISEASES.
Nursing Assessment: Obtain a through nursing history
Discuss the care plan with the health care team (cardiologist,
cardiac surgeon, nursing care manager, social worker, nutrition
list)
Measure and record height and weight plot on a growth chart
Record vital signs and oxygen saturations.
Measure vital signs at a time when the infant / child is quit.
Choose appropriate size blood pressure cuff
Check four extremities BPxl.
Assess and record.
Skin color, pink, cyanotic, mottled
Mucous membranes; moist, dry, cyanotic
Extremities: check peripheral pulses for quality and
symmetry,
dependent edema, capillary refill, color and
temperature.
54. Assess for clubbing (cyanotic heart disease0
Assess chest
wall
for
deformities;
prominent
pericardial activity.
Assess respiratory pattern
Before disturbing the child, stand back on count
the respiratory rate.
Loosen or remove clothing to directly observe
chest
movement
Assess for signs of respiratory distress; increased
respiratory rate, granting, retraction, nasal
flaring.
Αuscultate for crackles, wheezing, congestion, and
strider.
Assess heart sounds.
Determine rate (bradycardia, tachycardia) and
rhythm( regular or irregular)
Identity murmur (type, locations, and grade)
55. Assess fluids status.
• Daily weights
• Strict intake and output (number of wet diaper,
urine
output)
Assess and record the child’s level of activity
Observe the infant while feeding, does the infant
need
frequent breaks or child asleep during
feeding,
assess for sweating, color change, or
respiratory
distress while feeding.
Observe the child at play, is play interrupted to
rest
Assess and record findings relevant to the child’s
development level, age appropriate behavior,
cognitive skill, gross and fine motor skills.
56. Summary: So
far we have discussed about congenital heart
diseases, cyanotic heart disease like fallots tetrology,
transposition of great arteries and acynotic heart
disease like VSD, ASD, PDA and Nursing care of the
child with congenital heart disease.