Congenital heart disease

Neonatal Heart Diseases

A neonatal heart disease refers to the defect in heart or great vessels, or persistence of a fetal
structure after birth.

Incidence:

Congenital heart defects occur in an estimated 1% of all pregnancies and one in every 170 live
births.

Etiology:

Fetal exposure to drugs like phenytoin, lithium, warfarin and alcohol.

Maternal viral viral infections such as rubella and coxsackie
Maternal metabolic disorders such as phenylketonuria, diabetes mellitus and
hypercalcemia
Maternal complications of pregnancy- increased age and antepartal bleeding
Genetic factors
Chromosomal abnormalities- turner syndrome, marfan syndrome, cri-du chat syndrome

Classifications:

Defects are classified on the basis of pathophysiology and hemodynamics. These include:

 Increased pulmonary blood flow

 Decreased pulmonary blood flow
 Obstructed systemic blood flow
 Mixed defects in which infant survival is dependent on the mixing of systemic and
pulmonary blood.

Clinical manifestations:

Pathophysiology Clinical manifestations Types of defects

Increased pulmonary blood  Tachypnea  PDA

flow  Tachycardia  ASD
 Murmur  VSD
 Congestive heart  AV canal defect
failure  Truncus arteriosus
 Weight gain  Total anomalous

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  Diaphoresis venous return
 Periorbital edema
 Frequent respiratory
infections
Decreased pulmonary blood  Cyanosis  Pulmonic stenosis
flow  Hypercyanotic  TOF
episodes  Pulmonary atresia
 Poor weight gain  Tricuspid atresia
 Polycythemia  TGA

Obstructed systemic blood  Diminished pulses  Coarctation of aorta

flow  Poor color  Aortic stenosis
 Decreased urine output  Hypoplastic left heart
 Congestive heart syndrome
failure with pulmonary  Mitral stenosis
edema  Interrupted aortic arch

Mixed defects  Cyanosis  TGA

 Poor weight gain  Total anomalous
 Pulmonary congestion pulmonary venous
 Congestive heart connection
failure  Truncus arteriosus
 Double outlet right
ventricle

S.n Type of defect Clinical manifestation/ Clinical therapy

o diagnostic procedures

DEFECTS THAT INCREASES

PULMONARY BLOOD FLOW:

1. Patent Ductus Arteriosus: a Clinical features:  Surgical ligation of PDA

congenital defect caused by  Tachypnea  Intravenous indomethacin
persistent fetal circulation.  Tachycardia helps closure of ductus
When pulmonary circulation is  Bounding pulses arteriosus.
established and systemic vascular  Widened pulse  Transcatheter closure by
resistance increases at birth, pressure obstructive device is
pressures in the aortabecome  Hypotension attempted in children over

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 greater than pulmonary arteries,  Intercostals 18 months of age.
leading to shunting of blood from retractions
aorta to the pulmonary arteries,  Hepatomegaly
increasing circulation to pulmonary  Growth failure
system.

Diagnosis:
 Chest X ray
 ECG
 Echocardiogram

2. Atrial septal defect: C/M:

The opening in the atrial septum  CHF  Surgery to close or patch
permits left to right shunting of  Easy tiring the ASD is performed.
blood.  Poor growth  Secundum ASD may be
closed by a transcatheter
Diagnosis: device (septal occluder)
 Echocardiogram during cardiac
 Chest Xray catheterization.
 ECG
3. Ventricular septal defect: C/M:  Surgical patching of VSD
An opening in the ventricular  Systolic murmur is during infancy is
septum causes increased pulmonary rd
auscultated at 3 or 4 th
performed.
blood flow. left intercostal space at  Closure of VSD by
sterna border. transcatheter device during
cardiac catheterization.
Diagnosis:
 Echocardiogram
 Chest Xray
 ECG
4. Atrioventricular canal defect C/M:  Palliative pulmonary artery
It refers to a combination of defects  Tachypnea banding to reduce blood
in atrial and ventricular septa and  Tachycardia flow to lungs and CHF.
portions of tricuspid and mitral  Poor growth  Oxygen may be required
valves.  Recurrent respiratory until surgery
infections  Patches are placed over
 Repeated respiratory septal defects and valve
tissue is used to form

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 failure functioning valves.

Diagnosis:
 Chest Xray
 ECG
 Echocardiogram
 Cardiac catheterization
DEFECTS THAT DECREASES
PULMONARY BLOOD FLOW:

5. Pulmonic stenosis: C/M:

It is narrowing of the valve, valve  Dyspnea  Dilation by balloon
area or great artery above the valve,  Fatigue on exertion valvuloplasty during
obstructing the blood flow into  Heart failure cardiac catheterization.
pulmonary artery thus increasing  Chest pain  Surgical valvotomy when
preload and results in ventricular VSD is present
hypertrophy. Diagnosis:  Surgical resection may be
 Chest Xray needed for narrowing
above the valve area.
 ECG
 Echo
 Cardiac catheterization

6. Tetralogy of fallot (TOF): Clinical features:  Place the child in a knee

It consists of four defects-  Hypoxic episodes chest position
 Pulmonic stenosis  Cyanotic  Provide oxygen
 Right ventricular  Systolic murmur  Administer morphine and
hypertrophy  Polycythemia propranolol intravenously.
 VSD  Metabolic acidosis  Monitor for metabolic
 Overriding of aorta acidosis
 Poor growth
 Palliative shunt procedure
 Clubbing
(Blalock-Taussig) may be
 Exercise intolerance
performed

Diagnosis:
 Chest Xray
 ECG
 Echocardiogram
 Blood tests-elevated

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 hematocrit, Hb levels
7. Pulmonary or tricuspid atresia:  PGE1 is given immediately
It is the absence of communication Clinical features: to maintain a PDA.
between the right ventricle and  Cyanosis at birth  Digoxin and diuretics are
pulmonary artery,  Tachypnea also used.
 CHF  Balloon atrial septostomy
 Pulmonary edema is performed to increase
 Hepatomegaly atrial opening.

 Acidosis
 Hypoxic episodes

7. Total anomalous pulmonary C/M:  Prostaglandin E1 is given

venous return:  Mild cyanosis to maintain patent ductus
The pulmonary veins empty into  Frequent respiratory arteriosus.
right atrium or veins leading to infections  Hypoxemia and CHF are
right atrium rather than into left  Precordial bulge may be treated.
atrium. The foramen ovale must palpated  Balloon atrial septostomy
remain patent for mixed blood form  Murmur may be performed to
right atrium to pass to the systemic  Gallop rhythm is heard promote better mixing of
circulation. Any obstruction of Diagnosis: blood.
pulmonary veins increases the Chest radiographs  Surgery to reconnect or
condition’s severity. baffle the pulmonary veins
ECG-hypertrophy of
to the left atrium is
rt.atrium and ventricle
performed.
Echocardiogram

DEFECTS OBSTRUCTING
SYSTEMIC BLOOD FLOW:  PGE1 is given to maintain
a patent ductus arteriosus
8. Aortic stenosis: C/M: until aortic valve can be
Narrowing of the aortic valve  Peripheral pulses may dilated
obstructs blood flow to systemic be weak  Balloon valvuloplasty is
circulation.  Syncope done done during cardiac
 Dizziness catheterization.
 Systolic heart murmur  Aortic valve replacement
 Chest pain is performed.
Diagnosis:
 Chest Xray-normal
 ECG- lt. ventricular

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 hypertrophy
 Echocardiogram

9. Coarctation of aorta(COA)
Narrowing or constriction in the C/M:  Balloon dilation
descending aorta, often near the  Asymptomatic  Surgical resection with
ductus arteriosus or left subclavian  Bounding brachial and end-to end anastomosis
artery, obstructing the systemic radial pulses
blood outflow.  Weak or absent femoral
pulses
 Weakness and pain

Diagnosis:
 Chest radiographs-
cardiomegaly,
pulmonary venous
congestion
 MRI-shows site of
coarctation
 ECG- lt.ventricular
hypertrophy
 Echocardiogram-size of
aorta, actual coarctation.

10. Hypolastic left heart syndrome: C/M:  Prostaglandin E1 is given

This is a defect with absence or  Progressive cyanosis to maintain a PDA.
stenosis of mitral or aortic valves,  Tachycardia  Supplemental oxygen is
abnormally small left ventricle,  Tachypnea avoided.
small aorta or mitral stenosis or  Retractions  Norwood procedure: it is
atresia.  Decreased peripheral performed in 1st wk of life,
pulses followed by glenn
procedure at 3-8 months,
 Systolic murmur
and fontan procedure at 18
 Poor peripheral
months-3 years of age.
perfusion
 Heart transplantation.
 Pulmonary edema
 CHF
Diagnosis:
 Chest radiograph:
cardiomegaly,

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 increased pulmonary
vascularity
 Echocardiogram- small
left ventricle

Diagnostic evaluation:

 Cardiac catheterization: it is an invasive procedure that passes a radio opaque catheter

through a large vein or artery in an arm or leg to the heart. This helps to identify-
congenital heart disease, cardiac valvular disease and coronary artery disease.
 Chest radiograph study: radiographs reveal the size and contour of the heart and
characteristics of pulmonary vascular markings.
 Echocardiogram: it identifies the heart size, structure, pattern of movement,
hemodynamics, blood flow and blood flow disturbances.
 Electrocardiogram (ECG): it detects cardiac dysrhythmia , identification of electrolyte
imbalances etc.
 Hyperoxitest: arterial blood is collected before and at least 10 minutes after giving the
child 100% oxygen. It measures differences in arterial blood gas level when an infant has
central cyanosis to help distinguish between cardiac diseases, pulmonary disease or CNS
depression.
 MRI: it provides images of heart’s myocardium, valve function, blood vessels etc.
 Laboratory test- ABG, CBC, serum digoxin levels, ASO antibody titer, ESR, C-reactive
protein, Serum lipid panel.

Nursing management:

I. Prior to surgery:
 Physiologic assessment: failure to gain weight, assessment of length and head
circumference will help to determine the full impact of the condition on growth.
 Assess the parents’ ability to cope with infant’s diagnosis, hospitalization and
allow them to express their feelings about the child’s illness.
 Encourage the parents for breastfeeding. A high caloric formula may be used in
case the infant does not gain weight.
 Instruct the parents to notify any signs of respiratory infection, fever, vomiting
and diarrhea, poor feeding etc.
 Provide all immunizations according to the recommended schedule.
 Prepare the infant for surgery

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II. Post-operative care
 Assess the child’s behavioural patterns, heart functions, respiratory functions,
weight and fluid status.
 Assess for signs of surgical complications like infection, pain, arrhythmias, and
impaired tissue perfusion.
 Monitor the vital signs and blood pressure, apical pulse, breath sounds, respiratory
efforts, signs of distress, pulse oximetry, capillary refill, extremity warmth, pedal
pulses, level of consciousness and urine output.
 Monitor the child’s temperature and inspect the surgical incision site.
 Pain management with 24 hours intravenous opioids post operatively until the
child is taking fluids. Oral analgesics once the child takes oral fluids.
 Chest physiotherapy may be performed.
 Provide diversional activities to manage with pain and frightening procedures.
 Provide reassurance to the parents by providing full information about the child’s
defect and surgery performed.
 Administer antibiotics as directed.