Congenital Heart Defect
Congenital Heart Defect
Congenital Heart Defect
Definition: alteration in the circulation of the blood due to defects in the heart that happens
during early embryonic life; one of the leading causes of death in the premature
and small- for- gestational age babies.
Predisposing/Precipitating Factors
Alcoholism and poor maternal nutrition
Virus (especially the rubella virus)
Teratogenic drugs
Radiation exposure in the first trimester
Advance maternal age (above 40 years) and chromosomal aberrations
Presence of congenital anomalies
Parents and siblings with heart defects
Causes:
TOF occur during fetal growth, when the baby’s heart is developing. While
factors such as poor maternal nutrition, viral illness or genetic disorders might
increase the risk of conditions, in most cases the cause of TOF is UNKNOWN.
1. Ventricular Septal Defects
VSD is a hole defects in the wall (septum) that separates the two-lower chamber
of the heart – the left and right ventricles.
The hole allows deoxygenated blood in the right ventricle – blood that has
circulated through the body and is returning to the lung to replenish its oxygen
supply – to flow into the left ventricle and mix with oxygenated blood fresh from
the lungs.
2. Pulmonary Valve Stenosis
Is a narrowing of the pulmonary valve – the valve that separates the lower right
chamber of the heart (right ventricles) from the main blood vessels leading to the lungs
(pulmonary artery).
Narrowing of the pulmonary valve reduces the valve flow to the lungs. And may affect
the muscles beneath the pulmonary valve.
3. Overriding of the Aorta
Normally the aorta – the main artery leading out to the body – branches off the left
ventricles. In TOF, the aorta is shifted slightly to the right and lies directly above the
ventricular septal defect.
In this position the aorta receives from 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 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.
TRUNCUS ARTERIOSUS(TA)
Is a rare type of the heart disease in which a single blood vessel (Truncus arteriosus)
comes out of the right and left ventricles, instead of the normal 2 vessels (pulmonary
artery and aorta). And it is present at birth
Causes: UNKNOWN
Diagnostic test:
Chest x – ray
Echocardiogram
Cardiac catheterization
Surgical management:
1. Complete intracardiac repair, preferred treatment in early years
2. Pulmonary banding
3. Homograft conduit
TRICUSPID ATRESIA
Is characterized by absence for or complete closure of the tricuspid valve and therefore
no connection between the RA and the RV.
Causes:
This condition occurs during fetal development. Some genetic factors, such as Down
syndrome. But the cause of congenital heart disease is UNKWON
Signs and symptoms:
Cyanosis – within the first day of life
Severe hypoxic spells
Diagnostic Test:
Chest x – ray
Echocardiogram
Cardiac catheterization
Surgical management:
1. PALLIATIVE
BLALOCK TAUSSIG SHUNT – anastomosis between the subclavian artery to the
pulmonary artery
WATERSTON SHUNT – anastomosis between the ascending aorta and the right
pulmonary artery
2. COMPLETE SURGERY
FONTAN PROCEDURE – complete corrections involves: 1. Placement of a tubular
conduit with a valve between the right atrium and main pulmonary artery. 2. Closure of
the atrial septal defect; and 3. Ligation just above the pulmonary valve of the main
pulmonary artery.
Causes: UNKNOWN
Diagnostic test:
Electrocardiogram
Chest x – ray
Echocardiogram
Cardiac catheterization
Medical management:
Prostaglandin (PGE1) – is initiated to promote oxygenated blood flow from the
pulmonary artery to the aorta via RETROGRADE
Surgical management:
1. PALLIATIVE PROCEDURE
RASHKIND PROCEDURE (the preferred treatment in hypoxic infants)
Surgical creation of arterial septal defect through a surgical incision or balloon
arterial septostomy
2. CORRECTIVE PROCEDURE
MUSTARD PROCEDURE – surgical procedure to direct blood flow: simultaneous closure
of the ventricular septal defects and relief of left ventricular outflow obstruction; done
in child when is old enough
Clinical manifestation:
Infant with small VSD is likely to be asymptomatic, while infant with a moderate
to large defect will demonstrate signs of CHF.
The infant with large VSD is usually tachypneic, diaphoretic, fatigues, and is
underweight for age
If a VSD is detected in an older child for first time, it is because the defect is small
and the child has had no symptoms.
Decrease cardiac output is present
Diagnosis:
Diagnosis of VSD is often suspected when a loud holosystolic murmur is heard. The
intensity of the murmur can reflect the size of the defect.
X – ray
Echocardiogram
Cardiac catheterization – is rarely indicated
Treatment:
The infant with a small VSD usually requires no treatment because 75 -80% of these
defects may close in the first two year of life
Many of the child with hemodynamically insignificant VSD is never require surgery
Surgical management:
Surgical correction: CLOSURE OF VSD
Direct closure or with cardiopulmonary bypass by suture or patch usually by 3 –
4 years
Corrective surgery may be done earlier (before 2 years of age) in the presence of
pulmonary arterial hypertension to prevent irreversible pulmonary bed changes
Clinical manifestation: Signs and symptoms of decreased cardiac out-put may be present
Decreased peripheral pulses
Exercise intolerance
Feeding difficulties
Hypotension
Irritability, restlessness, lethargy
Oliguria
Pale, cool extremities
Tachycardia
Treatment:
Preoperative interventions is only indicated in the infant child with large ASD that result
in congested heart failure.
Surgical repair is generally performed in the preschool age period.
Diuretics are given to control the symptoms of CHF
In the term newborn the ductus begins to close within 12 hours and should be
completely closed by 2 – 3 weeks. A ductus remains open, in a full – term baby, after
several weeks of life is termed a patent ductus arteriosus
Management:
Indomethacin (Indocin) a prostaglandin inhibitor, may be administered to close s
patent ductus in a premature infant and some newborn
The defects may be closed during cardiac catheterization, or the defect may require
surgical management
PULMONIC STENOSIS
Is narrowing at the entrance to the pulmonary artery
Resistance to blood flow causes right ventricular hypertrophy and decreased pulmonary
blood flow; the right ventricle may be hypoplastic
Pulmonary atresia is the extreme form of pulmonary stenosis in that there is total fusion
of the commissures and no blood flows to the lungs
Signs and Symptoms:
The infant with mild to moderate PS is asymptomatic, and generally a murmur is
discovered on routine examination. Growth is generally normal for infants and children
with PS, and symptoms are usually present only in those with severe PS include dyspnea
upon exertion and fatigue. Cyanosis is common with severe PS but is not usually seen
with the milder forms.
Management:
Dilatation of the narrowed valve may be done during cardiac catheterization
Surgical management
a. In infants: Transventricular (closed) valvotomy procedure
b. In children: Pulmonary valvotomy with cardiopulmonary bypass
DIGOXIN (LANOXIN)
o Is the drug of choice for children because of rapid onset and peak of action of drugs –
less toxicity
Signs of Digitalis Toxicity:
o Anorexia
o Vomiting
o Diarrhea
o Nausea – for children who can express the subjective feeling of nausea
o Arrythmias
o Bradycardia
o Xanthopsia – yellow green halos (for older children with color recognition and the ability
to verbalize)
Check cardiac rate before drug administration and withhold if cardiac rate is less than
o 100 – infant
o 90 – toddler
o 80 – in preschoolers
o 60- school age and less than 60 in adolescents (adults)
Nursing Care for Children with Congenital Heart Disorders
1. Maintain respiration /oxygenation
a. Proper positioning: first independent nursing action on admission of a child in
distress:
Acyanotic child: with severe dyspnea: ORTHOPNIC POSITION; use infant seat as
needed
Cyanotic child: lateral position, knee chest, squatting for preschool or older children
b. Administer oxygen as ordered
c. Suction, prn
d. Monitor ABG
2. Promote rest
a. Modify activity as indicated
b. Provide minimal handling; organize care
c. Anticipate needs to prevent excessive crying
d. Give small and frequent feedings instead of large, infrequent meals
e. Use soft, premie, nipple or give NGT feeding as indicated/ordered
f. Provide passive, quite play
3. Keep warm
4. Prevent infection
a. Observe frequent handwashing
b. Administer ordered antibiotics
c. Give a proper diet rich in vitamins and minerals
d. Protect the child from people with infections
e. Advise on updating/completing immunization
f. Regular check temperature and prompt reporting of abnormal findings
5. Maintain nutrition and hydration
a. Give high – calorie, easily digested, non – constipating diets
b. Ensure adequate hydration:
o Underhydration or dehydration in cyanotic heart disorders can aggravate
polycythemic state and precipitate cerebral abscess/thrombosis
o Overhydration in Acyanotic heart disease can precipitate congestive heart failure
6. Administer drugs as ordered: digitalis and diuretics
a. Nursing considerations in DIGOXIN ADMINISTRATION:
o Give at regular intervals; give alone and do not mix with other drugs
o Do not give with food or milk; administer one hour or two hours after
meals/feeding
o Check cardiac rate begore administration and withhold digitalis if CR is below
100 – infant
90 – toddler
80 – in preschoolers
60- school age and less than 60 in adolescents (adults)
o Be alert for sings of digitalis toxicity:
CARDIAC ARRYTHMIAS – usually the first sign
GIT signs are common: vomiting, anorexia, and diarrhea. Nausea (highly
subjective), which is common in adults, is often not observed in young
children
o For diuretics, the single most important care is to monitor I&O and to avoid
increased oral and intravenous fluids if with CHF
7. Prepare the child/family for various diagnostic procedures and cardiac surgery as
indicated. Specific preparation depends on the specific procedures. General
preparations include:
a. Ensure that the informed consent is signed
b. Encourage verbalization of feelings and concerns
c. Answer all questions in terms that are understandable; avoid technical terms.
For child, use play, guided imaginary as indicated
d. Provide/encourage parent’s child to allow return to age appropriate activities
during, and after hospitalization
o Provide consistency in care and avoid overprotection of the child to prevent
‘emotional crippling.’
e. Instruct parents on the necessity of following up care and regular visits
8. Provide essential post – operative care to children who underwent surgical correction of
congenital heart defects
a. Ensure continuity of care: sent nursing history and care plan to ICU; endorse
essential information about the child and family to appropriate personnel
b. Monitor vital signs, I&O, CVP, and chest drainage system
c. Care of child undergoing water seal – seal chest drainage:
o Stabilize the drainage – bottle system:
1. Check the tube connection periodically
2. Avoid kinks and loops; watch for leaks of air in the drainage system, indicated
by excessive/constant bubbling in the water seal chamber;
3. Make sure there is fluctuation of the fluid level in the long glass tube, which
is indicative of effective communication between the pleural space and the
drainage bottle
4. “Milk the tubing in the direction of the drainage bottle hourly, as ordered by
the surgeon
5. Encourage deep breathing and coughing at frequent intervals; provide
adequate pain medication as indicated
d. Observe for complications:
o Respiratory: pneumothorax, atelectasis, pulmonary edema
o Congestive heart failure
o Infection: systemic fever and increased heart rate, local redness, swelling, and
drainage
o Post – pericardiotomy syndrome: fever, pleural effusion, and pericardial friction
rub
e. Provide emotional support to the child and family;
o Explain procedures, and medications, treatment, special diet. (low sodium)
o Encourage and address parent/child concerns; answer their questions
appropriately
o Encourage/allow the child to attend to his/her personal needs as he/she is able,
encourage parenteral participation in the child’s care
o Provide the child with appropriate diversion and play materials
o Understand the emotional reactions that the child may normally demonstrate:
Regressive behavior in the toilet habits (enuresis and encopresis) in
feeding thumb sucking after the oral stage), and in other learned skills
Nightmares
Increase dependency and demanding behaviors: set limits