CONGENITAL HEART DISEASE

INTRODUCTION
The incidence of congenital heart disease in children is generally believed to be 4-10 % of 1000
live birth and is the major cause of death in the first year. The etiology of the most congenital heart
disease is not known. However several factor are associated with a higher then normal incidence
of the disease. This include prenatal factors eg. Maternal rubella during pregnancy, maternal
alcoholism, maternal age over 40 years, and maternal type-1 diabetes.

DEFINITION:
The term congenital heart diseases include several different type of defects, all of which singly or
in combination produce certain effects on the body.

These include:
• Inadequate cardiac out put into the systemic circulation.
• Inadequate cardiac out put into the pulmonary circulation.
• Pulmonary hypertension due to increased vascular resistance in the lungs.
• Systolic or diastolic overloading of the heart chambers.
• Lack of adequate tissue oxygenation if unoxygenated blood is shunted into systemic
circulation.

ETIOLOGY:
The etiology of the most congenital heart defects is not known. However several factors are
associated , these include:
• Prenatal factors such as:
– Maternal rubella during pregnancy.
– Maternal alcoholism
– Maternal age over 40 years
– Maternal type-1 diabetes
– Hereditary-inherent through the genes.
– Teratogenic effect of radiation and medicine .eg. alcohol, phenytoin.

ASSESSMENT:
• The assessment is based on the health history of the mother during pregnancy and of the
child (if older then a neonate).
• HISTORY OF MOTHER AND INFANT
• PHYSICAL EXAMINATION:
• The nurse play an important role in early diagnosis by observing all newborn infants
carefully:
• Neonatal vital signs-persistent bradycardia < 120/mint or tachycardia > 60/mint, dyspnea.
• Skin color – cyanosis and pallor.
• Weight gain and loss.
• Feeding behavior.
• Restless, lethargic.
DIAGNOSTIC PROCEDURES :
• Two types of diagnostic procedures:
• Invasive diagnostic procedures
• Non-invasive diagnostic procedures

NON-INVASIVE PROCEDURES:
• Chest radiography.
• Electrocardiography
• Echocardiography
• Ultrasonography
• Fluoroscopy

INVASIVE PROCEDURES:
• Laboratory studies
• Angiocardiography
• Cardiac catheterization

TYPES OF CONGENITAL HEART DISEASE:

• 1. Acyanotic heart disease
• 2. cyanotic heart disease

• Acyanotic heart disease: acyanotic heart disease is in which the infant has no cyanosis
because there is no mixing of unoxygenated blood in to systemic circulation.

• cyanotic heart disease: in which the infant shows varying degree of cyanosis because
unoxygenated blood enters the systemic circulation.

Acyanotic heart disease:

– COARCTATION OF THE AORTA.
– PATENT DUCTUS ARTERIOSUS.
– ATRIAL SEPTAL DEFECT.
– VENTRICULAR SEPTAL DEFECT.
– PULMONIC STENOSIS
– AORTIC STENOSIS

COARCTATION OF THE AORTA:

• is a localized malformation that result in a narrowing of the lumen of the vessel.

CLINICAL MANIFESTATIONS:
– High blood pressure and bounding pulse
– Signs of congestive heart failure in infants
– Dizziness
– Headache
– Fainting
– Epistaxis resulting from hypertension
– Risk of rupture of aorta , aortic aneurysm etc.

DIAGNOSIS:
• Radiography may show left ventricular hypertrophy and dilatation of the aorta.
MANAGEMENT:
– Non-surgical management: balloon angioplasty as a primary intervention for coarctation of
aorta(COA) , but risk for aneurysm formation.
– Surgical management : surgical repair consist removing the narrowed portion of the aorta
with anastmosis of the ends.
– Surgery is postponed until the preferred age 3-6 years, if the infant condition permits.
 PATENT DUCTUS ARTERIOSUS (PDA)
DEFINITION:
Failure of the fetal ductus arteriosus (artery connecting the aorta and pulmonary artery) to close
within the first week of life. The continued potency of this vessel allows blood to flow from the
higher pressure aorta to the lower pressure pulmonary artery causing a left to right shunt.

PATHOPHYSIOLOGY:
At birth the resistance in pulmonary and systemic circulation is almost identical.
As the systemic pressure exceeds the pulmonary pressure.
↓
Blood begins to shunt from aorta to the pulmonary artery.
↓
The additional blood recirculates and returned to the left atrium and left ventricular.
↓
Due to the effect of altered circulation increased workload on the left side of the heart.
 ↓
Pulmonary edema and congestion.
↓
Increased right ventricular pressure and hypertrophy.

DIAGNOSTIC EVALUTATION:
• Radiological examination reveals left ventricular and left atrial enlargement and a dilated
ascending aorta and pulmonary artery
• ECG shows left ventricular hypertrophy and possible right ventricular hypertrophy.
CLINICAL MANIFESTATIONS:
• Clinical manifestations depends upon size of the ductus and the amount of shunting.
• Dyspnea on exertion
• Easy fatigability
• Presence of murmur
• Physical underdevelopment
• Increased respiratory infections
• Heart rate over 150/mint
• Bounding pulse.
MANAGEMENT:
• Supportive medical management:
• Supportive management include fluid restriction with diuretics and digitalization for
congestive heart failure.
• Pharmacologic management:
• Pharmacological closure of ducts has been achieved in low birth weight infants with
indomethacin which inhibit prostaglandin synthesis.
• Indomethacin may also reduce the amount of albuminbound serum bilirubin and therefore
may contribute to the development of hyperbilirubenemia.
• Also danger of transient renal dysfunction and hyponatremia when indomethacin is used.
Surgical treatment:
• Surgical treatment is ligation of the ductus.
• Surgery can be done at any time, although is preferably done at 6 months of age.
 ATRIAL SEPTAL DEFECT:

DEFINITION:

Abnormal opening between the right and left atria, allowing blood flow from higher pressure left
atrium to flow into the lower pressure right atrium.

TYPES:
• OSTIUM PREMIUM: Opening at lower end of septum may be associated with mitral valve
abnormality
• OSTIUM SECUNDUM: Opening near centre of the septum.
• SINUS VENOSUS DEFECT: Opening near junction of superior venacava and right atrium,
may be associated with partial anomalous pulmonary venous connection.
 PATHOPHYSIOLOGY:
• Because of left atrial pressure slightly exceeds right atrial pressure
• ↓
• Blood flow from the left to the right atrium
• ↓
• Increased flow from the left side of oxygenated blood to right side.
• ↓
• This volume is well tolerated by the right ventricle.
• ↓
• Although there is right atrial and ventricular enlargement.

CLINICAL MANIFESTATIONS:
• Patient may asymptomatic.
• Some times may develop congestive heart failure
• Murmur
• Atrial dysrhythmias
• Pulmonary vascular obstructive disease and emboli formation.

MANAGEMENT:
– Surgery on the effected children before they enter school even if no symptoms are present.
– In small defects closure is done by stitching and pulling it closed.
– If surgery is not done during childhood, it leads to pulmonary hypertension, atrial
dysarrythmias, and cardiac failure.
 VENTRICULAR SEPTAL DEFECT:
• Ventricular septal defect an abnormal opening between the right and left ventricles.
• This may vary in size from a small pin hole to absence of the septum in resulting in a
common ventricle.
• Many VSD (20%-60%) are thought to be closed spontaneously. Spontaneous closure is
most likely to occur during the first year of life in children having small or moderate defect.

PATHOPHYSIOLOGY:
• Because of the higher pressure with in the left ventricle and systemic circulation offers
resistance than pulmonary circulation.

Blood shifts through the defect into pulmonary circulation.

• Increased pulmonary vascular resistance , pressure increased in the right ventricle.

Muscle hypertrophy

• Right ventricle is unable to manage increased workload; and in response to right ventricle
right atrium also enlarge.
 DIAGNOSIS:
• CARDIAC CATETERIZATION: findings include the increased pressure in right ventricle and
possibly an obstruction in pulmonary artery.
• RADIOGRAPHIC EXAMINATION: radiographic examination may reveal enlargement of
both ventricles and the atrium.
• ECG: shows the left and right ventricular hypertrophy

CLINICAL MANIFESTATIONS:
• The size of the defect and the pulmonary circulation determines the clinical manifestations:
• Most frequently seen defects are small in size and these defects are asymptomatic.
• Medium size during infancy may produce:
– Dyspnea
– Tachypenia
– Slow physical development
– Pulmonary infections
• With large defect:
– Episodes of cardiac failure
– Hypertrophy of the right side of the heart
• Evident with cardiac enlargement.

MANAGEMENT:
• If the defect is small and asymptomatic, spontaneous closure may present before 1-2 years
of age.
• If defect is enlarge medical management for heart failure.
• Surgical care require open heart surgery.
• As with atrial septal defect symptomatic defect are closed with purse-string suture.
• Antibiotic prophylaxis is given for ant dental procedure and other.
 PULMONARY STENOSIS

DEFINITION:
• Pulmonic stenosis is obstruction and narrowing at the entrance of to the pulmonary artery.
Resistance to blood flow cause right ventricular hypertrophy and decreased pulmonary blood flow.

PULMONARY ATRASIA:
• Is the extreme form of pulmonary stenosis and no blood flow.

PATHOPYSIOLOGY:
• Due to pulmonary stenosis resistance to the blood flow.
↓
Right ventricular hypertrophy
↓
If right ventricular failure develop
↓
Right atrial pressure increases
↓
Reopening of the foramen ovale and shunting of unoxygenated blood into the left atrium and
systemic cyanosis

DIAGNOSIS:
• Radiography shows the enlargement of the heart with hypertrophy of right ventricle and
atrium.
• Murmur also analyzed.
 CLINICAL MANIFESTATIONS:
• Depends upon the pulmonic stenosis:
• In mild stenosis no symptom except murmur
• In sever cases cyanosis and cardiac failure may present
• Poor exercise tolerance
• Exertinal dyspnea
MANAGEMENT:
• Children having mild and moderate pulmonary stenosis may need no treatment.
• Children having more sever defect require surgical pulmonary valvotomy if obstruction at
valvular level.
• The leaflets are separated with incision.

AORTIC STENOSIS:

DEFINITION:

• Narrowing or stricture of the aortic valve, causing resistance to blood flow in the left
ventricle, decreased cardiac output , left ventricular hypertrophy and pulmonary congestion.

PATHOPHYSIOLOGY:
Due to aortic stenosis
↓
Resistance to ejection of blood from left ventricle.
↓
Extra workload in the left ventricle cause hypertrophy
↓
 Left ventricular failure
↓
Left atrial pressure increased
↓
Increased pressure in pulmonic vein
↓
Pulmonary vascular congestion

CLINICAL MANIFESTATIONS:
• Fatigue
• Dizziness
• Fainting
• Pulmonary edema
• Sever stenosis can result in sudden death
• Most prominent physical manifestations is the presence of systolic ejection murmur,
accompanied bye a thrill over the aortic area.
MANAGEMENT:
– Valvotomy is done to carried out with surgical interventions.
– Leaflets of the valve are seprated by incision.

BIBLIOGRAPHY:
1. Wong’s, “pediatric nursing” edition 6th
Published by Mosby, Pp 936-948.
2. Dorthy R.marlow , “textbook of pediatric
nursing” edition sixth,
published by saunders, Pp
466-491.
3. piyush gupta, ‘Essential pediatric nursing’
Second edition,Pp283-288.
4. 4.www.google.com