2. -Congenital Heart Disease (CHD) occurs in 1/125 live
births.
-Most common birth defect.
-Occurring during the 1st 8 weeks of fetal development.
-Majority have no known cause.
3. Factors contributing to CHD:
-85 to 90% of cases, there is no identifiable cause for the Heart defect
and generally considered to be caused by multifactorial inheritance.
-Factors are usually both genetic and environmental, where a
combination of genes from both parents, in addition to unknown
environmental factors, produce the trait or condition.
-Maternal Factors :
°Seizure disorders with intake of anti-Seizure medications.
°Intake of lithium for depression.
°Rubella-1st trimester of pregnancy.
4.
5. Fetal Circulation :
-Blood flows from the placenta.
=>IVC.
=>RA.
=>through the FO.
=>LA.
=>LV.
=>ascending aorta.
10. Pink Baby(L-R Shunt) :
-L to R shunts cause CHF and pulmonary hypertension.
-This leads to RV enlargement, RV failure.
-They are not typically cyanotic.
-Examples: ATRIAL SEPTAL DEFECT (ASD).
PATENT DUCTUS ARTERIOSUS(PDA).
VENTRICULAR SEPTAL DEFECT (VSD).
COARCTATION OF THE AORTA.
12. ASD :
-An opening in the atrial septum.
-Allows oxygenated (red) blood to pass from the left atrium, through the
opening in the septum, and mix with unoxygenated (blue) blood in right
atrium.
-Types Of ASD:
1.Ostium Primum:Opening at the lower end of septum.
2.Ostium Secondum:Opening near the centre of the septum.
3.Sinus Venosus:Opening near the junction of superior vena cava and
right atrium.
13.
14.
15. Effects :
When blood passes through ASD from LA to RA
=>a larger volume of blood than normal must be
handled by side of heart =>extra blood the passes
through PA into lungs =>Pulmonary hypertension
& pulmonary congestion.
16. Signs And Symptoms :
-Child tires easily when playing.
-Infants tires easily when feeding.
-Tachypnea, Tachycardia.
-Shortness of breath.
-Sweating.
18. Treatment:
Medical Management :
-Digoxin :Helps strengthen the heart muscles, enabling it to pump more efficiently.
-Diuretic :Relieve Pulmonary congestion.
Infection Control :
-Prophylactic antibiotics to prevent bacterial endocarditis before any procedures.
Surgical Repair:
-The patient is placed on CPB, the right atrium is then opened to allow access to
the atrial septum below.
20. VSD:
-An opening in the ventricular septum.
-Allows oxygenated blood to pass from the left ventricle, through
the opening in the septum, and then mix with unoxygenated
blood in the right ventricle.
-VSDs are the most commonly occurring type of Congenital Heart
defect.
-Occur when the partitioning process does not occur completely,
leaving an opening in the ventricular septum.
21.
22. Effects :
-When blood passes through the VSD from the left ventricle to the right
ventricle =>a larger volume of blood than normal must be handled by
the right side of the heart=>extra blood then passes through the
pulmonary artery into the lungs=>Pulmonary hypertension and pulmonary
congestion =>Pulmonary arteries becomes thickened and obstructed due to
increased pressure.
-If VSD is not repaired and lung disease being to occur=>pressure in
the right side of the heart will eventually exceed pressure in the
left=>R to L Shunt =>Cyanosis.
23. -Due to high pressure :tissue damage may eventually occur in
the right ventricle =>bacteria in the blood stream can
25. Treatment :
*Medical Management :
-Digoxin.
-Diuretics.
*Prophylactic antibiotics to prevent bacterial endocarditis.
*Surgical Repair :VSD will be closed with stitches or special patch.
28. -Charaterized by a connection between the aorta and
the pulmonary artery.
-All babies are from with a ductus arteriosus.
-As the baby takes the first breath, the blood vessels
in the lungs open up, and blood begins to flow =>the
ductus arteriosus is not needed to bypass the lungs.
-In some baby's, however, the ductus arteriosus
remains open (patent).
-The opening between the aorta and the pulmonary
artery allows oxygenated blood to pass back through
the blood vessels in the lungs.
29. Effects :
-PDA=>Oxygenated blood passes from the aorta to the
pulmonary artery & mixes with the unoxygenated blood without
goes to the lungs=>increase blood volume to the lungs
=>Pulmonary hypertension and pulmonary congestion.
Further, because blood is pumped st high pressure through the
PDA, the lining of the pulmonary artery will become irritated and
inflamed. Bacteria in the blood stream can easily
33. -Narrowing of the aorta.
-Can occurs anywhere, but is most likely to happen
in the segment just after the aortic arch.
-This Narrowing restricts the amount of blood to the
lower part of the body.
-Occurs in about 8-11% of all children with CHD.
34. Effects:
-The left ventricle has to work harder to try to move
blood through the narrowing in the aorta =>Left-
sided heart failure.
-BP is higher above the narrowing and lower below
the narrowing.
-Older children may have headaches from too much
pressure in the vessels in the head, or cramps in the
legs or abdomen from too little blood flow in that
region.
35. -The walls of the arteries may become weakened by
high pressure =>spontaneous tears=>cause a stroke
or uncontrollable bleeding.
-Increased risk of bacterial endocarditis.
36.
37. SYMPTOMATIC INFANTS:
HISTORY :
-Poor feeding, dyspnea, and poor weight gain or
signs of acute circulatory shock may develop in the
first 6 weeks of life.
PHYSICAL EXAMINATION :
-Infants with COA are pale and experience varying
degrees of respiratory distress.
-Oliguria or anuria, general circulatory shock, and
severe acidemia are common.
-Peripheral pulse may be weak.
38. -A blood pressure differential may become apparent
only after improvement of cardiac function with
administration of rapidly acting inotropic agants.
-The S2 is sing and loud ;a loud S3 gallope is
usually present.
-No heart Murmur is present in 50% of sick Infants.
40. Management :
MEDICAL :
-Intensive angi congestive measures with short
acting inotropic agants (Eg:Dopamine,
dobutamine), Diuretics, and oxygen should be
started.
-Balloon angioplasty can be a useful procedure for
sick Infants in whom a standard surgical
management carries a high risk.
41. SURGICAL :
-Indications and timing.
-If CHF or circulatory shock develops early in life,
surgery should be performed on a urgent basis.
PROCEDURES :
-Resection and end-to-end Anastomosis consist of
resecting the coarctation segment and anastamosing
the proximal and distal aorta.
-Subclavian flap aortoplasty consists of dividing the
distal subclavian artery and inserting a flap of the
proximal portion of this vessel between the two sides
42. of the longitudinally split aorta through out the
coarctation segment.
-With patch aortoplasty, the aorta is opened
longitudinally through the contraction segment and
extending to the left subclavian artery, and the fibrous
shelf and any existing membrane are excised. And
eliptic woven dacron patch is inserted to expand the
diameter of the lumen.
50. Effects :
-If the right ventricle obstruction is severe, or if the
pressure in the lung is high=>a large amount of
oxygen - poor (blue) blood passes through the VSD,
mixes with the oxygen - rich (red) blood in the left
ventricle, and is pumped to the body =>cyanosis.
-The more blood that goes through the VSD, the
less blood that goes through the pulmonary artery to
the lungs=>decrease oxygenated blood to the left
side of the heart.
51. -Soon, nearly all the blood in the left ventricle is
oxygen poor(blue). This is an emergency situation,
as the body will not have enough oxygen to meet its
needs.
52. Clinical Manifestation
History :
-A heart Murmur is audible at birth.
-Most patients are symptomatic with cyanosis at
birth or shortly thereafter. Dyspnea on exertion or
hypoxic spells develop later, even in mildly cyanotic
Infants.
-Immediately after birth, severe cyanosis is seen in
patients with TOF And pulmonary atresia.
53. Physical Examination :
-varying degrees of cyanosis, Tachypnea, and
clubbing (in order Infants and childrens) are present.
- Are RV tap along the left sternal border and a
systolic thrill at the upper and Mid-left sternal
borders are commonly present(50%).
-An eject click that originates in the aorta may be
audible.
55. Signs and symptoms :
-Infants with a cyanotic TOF gradually become
cyanotic.
-Patients who are already cyanotic become more
cyanotic as a result of the worsening condition of
the infundibular stenosis and polycythemia.
-Polycythemia develops secondary to cyanosis.
-Hypoxic spells may develops in Infants.
-Growth retardation may be present if cyanosis is
severe
56. Management:
MEDICAL :
-Educate parents to recognize the spell and know
what to do.
-Oral propranolol therapy, 0.5-1.5 mg/kg every six
hours, is occasionally used to prevent hypoxic spell
while waiting for an optimal time for a corrective
surgery.
-Balloon dialatation of the RV outflow tract and
pulmonary valve, it is not widely practiced.
57. -A relative iron defeciency state should be detected
and treated.
58. SURGICAL :
-Palliative Shunt procedures.
-Classic Blalock - Taussig Shunt,Anastomosed
between the subclavian artery and the ipsilateral PA,
is usually performed for Infants older than 3 months
because the Shunt is often thrombosed in younger
Infants with smaller arteries.
61. -The aorta is connected to the right ventricle, and the
pulmonary artery is connected to the left ventricle.
-Oxygen poor(blue) blood returns to the right atrium
from the body =>passes through the right atrium and
ventricle, =>into the misconnected aorta back to the
body.
-Oxygen rich(red) blood returns to the left atrium
from the lungs=>passes through the left atrium and
ventricle, =>into the pulmonary artery and back to
the lungs.
62. (+) Other heart defects are often associated with
TGA
-Atrial or ventricular septal defect may be
necessary in order for the infant with TGA to
survive, allow mixing of blood - providing a least
smaller amount of oxygen to the body.
63. CLINICAL MANIFESTATIONS:
HISTORY :
History of cyanosis from birth is always present.
-Sign of congestive heart failur(CHF) with dyspnea
and feeding difficulties develop during the new
born period.
64. Physical Examination :
-Moderate to sever cyanosis is present.
-athe S2 is single and loud:If CHF superveins,
hepatomegaly and dyspnea developed.
66. Natural History :
Without surgical intervention, death occurs in 90% of
patients before they reach 6 months of age.
67. Management :
Medical:
-Arterial blood gases and pH should be obtained and
metabolic acidosis should be corrected.
-PGE1 infusion should be started to improve arterial
oxygen saturation bt reopening the ductus.
-Oxygen administration.
-A oxygen balloon atrial septotomy (rashkind
procedure) may be performed to increase the mixing
by opening the atrial septum.
68. Surgical :
-An arterial switch procedure - performed in first
week of life.
-Transecting the great arteries and anastomosing
the main pulmonanary artery to the proximal aorta
and anastomosing the ascending aorta to the
proximal pulmonary artery.
-The coronary arteries are switched from proximal
aorta to the proximal pulmonary artery to create a
new aorta.
69. +Intra atrial baffle repairs-An Intra atrial baffle is
created to divert the venous blood to the mitral valve
and pulmonary venous blood to the tricuspid valve
using patients atrial septum or a prosthetic valve.
70. +Rastelli Procedure:Operative choice in patients
with TGA, VSD,and severe pulmonic stenosis.
-It involves closure of VSD with baffle=>LV blood
directed through VSD into aorta.
-Pulmonic valve is then closed,and a conduit is
placed from RV to PA.
