2. INTRODUCTION
Congenital heart disease, or a congenital heart defect, is a heart abnormality
present at birth. The problem can affect:
• the heart walls
• the heart valves
• the blood vessels
There are numerous types of congenital heart defects. They can range from
simple conditions that don’t cause symptoms to complex problems that cause
severe, life-threatening symptoms.
3. ETIOLOGY
Exact cause of CHD is unknown about 90% of cases.
Heredity & consanguineous marriage.
Genetic disorders & chromosomal aberrations ( Trisomy -21,Turner’s
Syndrome)
Fetal & Maternal teratogenic infection ( Rubella)
Teratogenic drug (Thalidomide) intake
Alcohol intake by mother
Maternal IDDM
Fetal Hypoxia
Birth Asphyxia
4. CLASSIFICATION OF CHD
CHD can be grouped into three categories
A) ACYANOTIC CHD :- There is increases pulmonary blood flow due to left to right shunt .It
includes
Atrial Septal Defect (ASD)
Ventral Septal Defect (VSD)
Patent Ductus Arteriosus (PDA)
Atrio –Ventricular Canal(AVC)
B)CYNOTIC CHD:- There is diminished pulmonary blood flow due to Right to left shunt. It
include
Tetralogy of fallot
Transposition of great arteries
Truncus arteries
5. CONTINU……
Hypo plastic left heart syndrome
Tricuspid atresia
Total anomalous pulmonary venous return
Eisemenger syndrome or complex
C)OBSTRUCTIVE LESIONS
Coaructaion of aorta
Aortic Valve Stenosis
Pulmonary Value Stenosis
Congenital Mitral Stenosis
6. ATRIAL SEPTAL DEFECT (ASD)
An atrial septal defect (ASD) is a hole in the wall (septum) between the two upper
chambers of heart (atria). The condition is present at birth (congenital).
Small defects might be found by chance and never cause a problem. Some small atrial
septal defects close during infancy or early childhood.
It is an Abnormal opening between the RIGHT & LEFT atria resulting LEFT to RIGHT
shunting of blood.
9% cases of CHDs
7. TYPES OF ASD
There are three common types of atrial septal defect (ASD):
Ostium Secundum - located in the center or middle of the atrial septum (most
common type)
Ostium Primum - located near the lower portion of the atrial septum, may be associated
with defects in the mitral and tricuspid valve (second most common type)
Sinus Venosus- located near the top of the atrial septum and frequently associated
with abnormal connection of the right pulmonary vein(s) to the right atrium instead to
the left atrium (least common type)
9. CLINICAL MANIFESTRATION
Ostium Secundum & Sinus Venous ASDs are usually
asymptomatic
Recurrent of chest infection
Dyspnea on exertion
Easy fatigability
Bulging of the chest
Poor weight gain
Cardiac enlargement
CCF
10. DIAGNOSIS
Auscultation of heart sound
Ostium Secundum ASD ,Soft Systolic flow murmur heard at
the left upper sternal broader.
Chest X-ray
ECG
Echocardiogram
Color Flow Mapping
Cardiac catheterization
11. MANAGMENT
Surgical closure of the defect in early childhood to prevent further complication .
Antibiotic prophylaxis
Repair the defect is done by surgical closure or pericardial patch repair by open heart
surgery.
If the defect is larger ,a knitted Dacron Patch is used to occlude the aperture.
SURGICAL CLOSURE:-
1.Cardiopulmonary bypass
2. Purse string closure
3. knitted Dacron Patch
12. VENTRICULAR SEPTAL DEFECT (VSD)
A ventricular septal defect (VSD), a hole in the heart, is a
common heart defect that's present at birth (congenital). The
hole (defect) occurs in the wall (septum) that separates the
heart's lower chambers (ventricles) and allows blood to pass
from the left to the right side of the heart. The oxygen-rich blood
then gets pumped back to the lungs instead of out to the body,
causing the heart to work harder.
13. CONTINU…
It can be small or large .Large VSD can be restrictive or non- restrictive type.
a.) Restrictive VSD small defects that allow little or no blood to flow from the left side of
the heart to the right side of the heart.
b.) Non-restrictive defects are large defects that allow a significant amount of blood to
flow from the left side to the right of the heart.
It can be found approximately 25% of all CHD .
Most common type of acynotic Congenital heart diseases with LEFT TO RIGHT shunt.
15. CLINICAL MANIFESTRIATION
The most common symptoms include:
• Shortness of breath.
• Fast breathing.
• Hard breathing.
• Paleness.
• Failure to gain weight.
• Fast heart rate.
• Sweating while feeding.
• Frequent respiratory infections.
16. DIAGNOSTIC EVALUATION
History of illness
Physical examination
Auscultation of harsh systolic murmur
Chest x-ray
ECG
Eco cardiogram with Doppler study & color flow of mapping.
17. MANAGMENT
In small VSD ,usually no medical management required.
Spontaneous closure of VSD occurs in 30 to 50% with small defects.
In large VSD ,initial management of problems like CCF,Endocardiatis,should be done
with appropriate treatment.
Surgery is done as one stage or two stages operation.
One stage operation with patch closure of VSD open heart method.
Two stage approach is done with first stages to band the pulmonary artery to
restrict pulmonary blood flow by closured heart method.
Second stage operation is done to patch close the VSD & remove the PA method
18. PATENT DUCTUS ARTERIOUS
Patent ductus arteriosus (PDA) is a medical condition in which the ductus
arteriosus fails to close after birth: this allows a portion of oxygenated blood
from the left heart to flow back to the lungs by flowing from the aorta, which
has a higher pressure, to the pulmonary artery.
When ductus arteriosus remains patent & open
after birth, the blood flows in the ductus from the
Aorta to the pulmonary artery due to higher
Pressure in the aorta. Common in preterm baby.
20. CLINICAL MANIFESTRATION
Symptoms
• Poor eating, which leads to poor growth.
• Sweating with crying or eating.
• Persistent fast breathing or breathlessness.
• Easy tiring.
• Rapid heart rate.
21. DIAGNOSTIC EVALUATION
History of illness & physical examination
Auscultation of heart sound revels continuous murmur(machinery murmur)
Chest X-ray
Echocardiogram with Doppler study
Color flow mapping
Cardiac Catheterization
ECG
22. MANAGMENT
Watchful waiting. In a premature baby, a PDA often closes on its own. The doctor will
monitor baby's heart to make sure the open blood vessel is closing properly. For full-term
babies, children and adults who have small PDAs that aren't causing other health
problems, monitoring might be all that's needed.
Medications. In a premature baby, nonsteroidal anti-inflammatory drugs (NSAIDs) — such
as ibuprofen (Infants' Advil, Infants' Motrin, others), available over the counter, or
indomethacin (Indocin), available by prescription — might be used to help close a PDA.
NSAIDs block the hormone like chemicals in the body that keep the PDA open. NSAIDs
won't close a PDA in full-term babies, children or adults.
23. CONTI…..
SURGICAL MANAGEMENT
PDA via a lateral thoracotomy ,a closed heart intervention is performed .
It done between the age 3 & 10 yrs. of age in asymmetric patients & in symptomatic
patients it should be done irrespective of age & in the presence of pulmonary
hypertension.
24. TETRALOGY OF FALLOT
Tetralogy of fallot is a birth defect that affects normal blood flow through the heart.it happens
when a baby’s heart does not form correctly as the baby grows & develops in the mother’s womb
during the pregnancy. It is a congenital defect ,meaning the baby is born with it.
A congenital heart condition involving four abnormalities occurring together ,including a
defective septum between the ventricles & narrowing of the pulmonary artery & accompanied by
cyanosis.
Tetralogy of fallot is made up of the 4 defects of the heart & its blood vessels.
1.VENTRICULAR SEPTAL DEFECT
2. PULMONARY STENOSIS
3. OVERRIDING OF AORTA
4. VENTRICULAR HYPERTROPHY
25. ETIOLOGY
1.) 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.
2.) Ventricular septal defect. A ventricular septal defect is a hole (defect) in the wall
(septum) that separates the two lower chambers of the heart left and right ventricles. The
hole allows deoxygenated blood in the right ventricle the 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.
26. Continue…
3.) 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.
4.) 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.
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28. CLINICAL MANIFESTRATION
Tetralogy of Fallot symptoms vary, depending on the extent of obstruction of blood flow
out of the right ventricle and into the lungs. Signs and symptoms may include:
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
Tet spells : Sudden development of deep blue skin ,nails & lips after crying ,feeding ,having
a bowel movement, or kicking his or her legs upon awakening.
29. DIAGNOSIS
Detail history of illness
Auscultation of soft or harsh systolic ejection murmur heard
Chest X-ray
Blood test
Oxygen level measurement (pulse oximetry)
Echocardiography
Cardiac catheterization & Echocardiogram helps to detect structural abnormalities.
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35. TRANSPOSITION OF GREAT ARTERIES
(TGA)
Transposition of the great arteries is a serious but rare heart defect present at birth
(congenital), in which the two main arteries leaving the heart are reversed
(transposed). The condition is also called dextro-transposition of the great arteries. A
rarer type of this condition is called levo-transposition of the great arteries.
Transposition of the great arteries changes the way blood circulates through the body,
leaving a shortage of oxygen in blood flowing from the heart to the rest of the body.
Without an adequate supply of oxygen-rich blood, the body can't function properly and
child faces serious complications or death without treatment. Transposition of the
great arteries is usually detected either prenatally or within the first hours to weeks of
life.
36. CLINICAL MANIFESTRATION
Transposition of the great arteries symptoms include:
• Blue color of the skin (cyanosis)
• Shortness of breath
• Lack of appetite
• Poor weight gain
• Metabolic acidosis
37. DIAGNOSTIC
Auscultation of heart sound
Angiocardiography
Radiology
ECG
Cardiac Cauterization
COMPLICATION
Multiorgan ischemia
Cardiomegaly(abnormal enlargement of the heart)
Growth failure
Clubbing may develop in few months
38. MANAGMENT
Medical management :- IV Prostaglandin E1 (PGE1) .It is used by slow inj. Into a vein to open
the ductus arteriosus until surgery can be carried out.
Digoxin ,diuretics & iron therapy
Sever hypoxia can be treated with balloon atrial septum.
Supportive nursing care is important.
Surgical Management:-
Depending upon the associated defects
Arterial switch operation
Restell’s Operation
Beffe’s operation