A NEONATAL HEART DISEASE

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

INCIDENCE

 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 complication of pregnancy – increased age and antepartal bleeding
 Genetic factors
 Chromosomal abnormalities – tuner syndrome, marfan syndrome, cri- du chat
syndrome

CLASSIFICATION

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

 Increased pulmonary blood flow

 Decreased pulmonary blood flow
 Obstructed systemic blood flow
 Mixed defect which infant survival is dependent on the mixing of systematic and
pulmonary blood

pathophisiology Clinical manifestation Type of defect

Increased pulmonary  Tachypnea  PDA
blood flow  Tachycardia  ASD
 Murmur  VSD
 Congestive heart failure  AVcanal defect
 Weight gain  Truncus arteriosus
 Diaphoresis  Total anomalous venous
 Periorbital edema return
 Frequent respiratory
infection
Decreased pulmonary  Cyanosis  Plmonic stenosis
blood flow  Hyper cyanotic episodes  TOF
 Poor weight gain  Pulmonary atresia
 Polycethemia  Tricuspid atresia
 TGA
Obstructed systemic  Diminished pulses  Coarctation of aorta
blood flow  Poor colour  Aortic stenosis
 Decreased urine output  Hypoplastic left heart
 Congestive heart failure syndrome
with pulmonary edema  Mitral stenosis
 Intrupted aortic arch
Mixed defect  Cyanosis  TGA
 Poor weight gain  Total pulmonary venous
 Pulmonary congestion connection
 Congestive heart failure  Truncus arterioses
 Double outlet right
ventricle

TYPE OF DEFECT

Defects That Increases Pulmonary Blood Flow

1. PATENT DUCTOS ARTERIOSUS: A congenital defect caused by persistent fetal

circulation is established and systemic vascular resistance increases at birth, pressures in
the aorta become greater than pulmonary arteries ,leading to shunting of blood from aorta
to the pulmonary arteries, increasing circulation to pulmonary system.

CLINICAL FEATURES

 Tachypnea
 Tachycardia
 Bounding pulses
 Widened pulse pressure Hypotension
 Intercostals retractions Hepatomegaly
 Growth failure

DIAGNOSIS

 Chest X-ray
 ECG
 Echocardiogram

CLINICAL THERAPY

 Surgical ligation of PDA.

 Intravenous indomethacin helps closure of ducts arteriosus .
 Transcatheter closure by obstructive device is attempted in children over 18 months of
age.
2. ATRIAL SEPTAL DEFECT: The opening in the atrial septum permits left to right
shunting of blood.

CLINICAL FEATURES:
 CHF
 Easy tiring
 Poor growth

DIAGNOSIS
  Echocardiogram
 Chest X- ray
 ECG

CLINICAL THERAPY

 Surgery to close or patch the ASD is performed.

 Secundum ASD may be closed by a transcatheter device ( Septal occluder)
during cardiac catheterization.
3. VENTRICULAR SEPTAL DEFECT: An opening in the ventricular septum causes
increased pulmonary blood flow.

CLINICAL FEATURES :
 Systolic murmur is auscultated at 3rd or 4th left intercostal space at sterna border.

DIAGNOSIS

 Echocardiogram
 Chest Xray
 ECG

CLINICAL THERAPY

 Surgical patching of VSD during infancy is performed.

 Closure of VSD by transcatheter device during cardiac catheterization.

4. ATRIOVENTRICULAR CANAL DEFECT : It refers to a combination of defects in

atrial and ventricular septa and portions of tricuspid and mitral valves
CLINICAL FEATURES :
 Tachypnea
 Tachycardia
 Poor growth
 Recurrent respiratory infections
 Repeated respiratory failure

DIAGNOSIS :

 Chest Xray
 ECG
 Echocardiogram
 Cardiac catheterization

CLINICAL THERAPY:

 Palliative pulmonary artery banding to reduce blood flow to lungs and CHF.
 Oxygen may be required until surgery.
  Patches are placed over septal defects and valve tissue is used to form functioning
valves.

Defects That Decreases Pulmonary Blood Flow:

5. PULMONIC STENOSIS: It is narrowing of the valve , valve area or great artery above
the valve, obstructing the blood flow into pulmonary artery thus increasing preload and
results in ventricular hypertrophy.

CLINICAL FEATURES:

 Dyspnea
 Fatigue on excretion, Heart failure
 Chest pain

DIAGNOSIS :

 Chest Xray
 ECG
 Echo
 Cardiac catheterization

CLINICAL THERAPY :

 Dilation by balloon valvuloplasty during cardiac catheterization.

 Surgical valvotomy when VSD is present.
 Surgical resection may be needed for narrowing above the valve area.

6. TETRALOGY OF FALLOT ( TOF): it consists of four defects – v

Pulmonic stenosisv, Right ventricular hypertrophyv, VSDv, Overriding of aorta

CLINICAL FEATURES :

 Hypoxic episodes
 Cyanotic
 Systolic murmur
 Polycythemia
 Metabolic acidosis
 Poor growth
 Clubbing
 Exercise intolerance

DIAGNOSIS :

 Chest Xray
 ECG
 Echocardiogram
  Blood tests elevated hematocrit , Hb levels

CLINICAL THERAPY:

 Place the child in a knee chest position.

 Provide oxygen.
 Administer morphine and propanlol intravenously .
 Monitor the metabolic acidosis.
 Palliative shunt procedure ( Blalock – Taussig) may be performed.

7. PULMONARY OR TRICUSPID ATRESIA: it is the absence of communication between

the right ventricle and pulmonary artery.

CLINICAL FEATURES:

 Cyanosis at birth
 Tachypnea
 CHF
 Pulmonary edema
 Hepatomegaly
 Acidosis
 Hypoxic episodes

CLINICAL THERAPY :

 PGE1 is given immediately to maintain a PDA.

 Digoxin and diuretics are also used.
 Balloon atrial septostomy is performed to increase atrial opening.

8. TOTAL ANOMALOUS PULMONARY VENOUS RETURN: The pulmonary veins

empty into right atrium or veins leading to right atrium rather than into left atrium. The
foramen ovale must remain patent for mixed blood form right atrium to pass to the
systemic circulation . any obstructon of pulmonary veins increase tha condition’s
severity.

CLINICAL FEATURES :

 Mild cyanosis
 Frequent respiratory infections
 Precordial bulge may be palpated
 Murmur
 Gallop rhythm is heard

DIAGNOSIS :

 Chest radiographs
 ECG – hypertrophy of rt. Atrium and ventricle
  Echocardiogram

CLINICAL THERAPY :

 Prostaglandin E1 is given to maintain patent ducts arteriosus.

 Hypoxemia and CHF are treated.
 Balloon atrial septostomy may be performed to promote better mixing of blood.
 Surgery to reconnect or baffle the pulmonary veins to the left atrium is performed.

Defects Obstructing Systemic Blood Flow:

9. AORTIC STENOSIS: Narrowing of the aortic valve obstructs blood flow to systemic
circulation.

CLINICAL FEATURES :

 Peripheral pulses may be weak.

 Syncope
 Dizziness
 Systolic heart murmur
 Chest pain diagnosis
 Chest Xray – normal
 ECG -it ventricular hepertrophy
 Echocardiogram

CLINICAL THERAPY:

 Pge1 is given to maintain a patent ductus arteriosus until aortic valve can be dilated.
 Balloon valvuloplasty is done during cardiac catheterization.
 Aortic valve replacement is performed.

10. COARCTATION OF AORTA ( COA) : narrowing or constriction in the decending aorta,

often near the ductus arteriosus or left subclavian artery, obstructing the systemic blood
flow.

CLINICAL FEATURES:

 Asymptomatic
 Bounding brachial and radial pulses
 Weak or absent femoral pulses
 Weakness and pain

DIAGNOSIS:

 Chest radiographs- cardiomegaly,pulmonary venous congestion.

 MRI shows site of coarctation.
 ECG it ventricular hypertrophy
 Echocardiogram size of aorta actual coarctation.
 CLINICAL THERAPY:

 Balloon dilation.
 Surgical resection with end to end anastomosis.

11. HYPOPLASTIC LEFT HEART SYNDROME: this is a defect with absence or stenosis
of mitral or aortic valves, abnormally small left ventricle , small aorta or mitral stenosis or
atresia.

CLINICAL FEATURES :

 Progressive cyanosis
 Tachycardia
 Tachypnea
 Retractions
 Decreased peripheral pulses
 Systolic murmur
 Poor peripheral perfusion
 Pulmonary edema
 CHF

DIAGNOSIS:

 Chest radiograph: cardiomegaly , increased pulmonary vascularity

 Echocardiogram – small left ventricle

CLINICAL THERAPY :

 Prostaglandin E1 is given to maintain a PDA.

 Supplemental oxygen is avoided.
 Norwood procedure: it is performed in 1st week of life, followed by glenn
procedureat 3- 8 months, and fontain procedure at 18 months – 3 years of age.
 Heart transplantation.

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 vlvular disease and coronary artery disease
 Chestrediograph study: radiograph revels the size ant contour of the heart and
charecteristics of pulmonary vascular marking
 Electrocardiogram: it identifies the heart size, structure pattern of movement,
hemodynamics, blood flow and blood flow disterbences. It detect cardiac
dysrhythmia, identification of electrolyte imbalances etc.
 Hyperoxitest: arterial blood is collected before and atleast 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 disease, pulmonary disease or
CNS dipression
 MRI: it provide images for heart myocardium, value function , blood vessels etc.
 Laboratory test: ABG, CBC, serum digoxin levels, ASO antibody titer, ESR, C-
reactive protein, serum lipid profile

NURSING MANAGEMENT

Prior to surgery / pre –operative care

 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 parent’s 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 calorie formula may be used in
case the infant does not gain weight.
 Instruct the parents to notify any signs of respiratory infections, fever, vomiting
and diarrhea, poor feeding, etc..
 Provide all immunizations according to the recommended schedule.
 Prepare the infant for surgery.

Post – operative care

 Assess the child’s behavioral patterns, heart functions, respiratory functions,
weight and fluid status.
 Assess the signs of surgical complications like infections, 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 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