Cyanotic Heart Disease is a defect or group of defects in the structure or function of the heart or the

great vessels, present at birth, consisting of abnormal blood flow from the right to the left part of the
circulatory system (either at the level of the atria, the ventricles, or the great vessels). This abnormal
communication (called right-to- left shunt) results in poor oxygenation of the body and therefore
cyanosis.
Symptoms & Signs
Cyanosis Dyspnea. Failure to thrive, or failure to grow properly Fatigue Squatting position after
physical activity to relieve breathlessness. Hypoxic spells, characterized by: -Anxiety.
-Hyperventilation. -Sudden increase in cyanosis. Syncope. Chest pain, Arrythmias.
Diagnosis
*Clinical:
-Upper left sternal border ejection murmur of RV outflow tract obstruction: TOF (Tetralogy of Fallot)
- Newborns present with severe cyanosis and a continuous murmur of ductal flow: pulmonary valve
atresia and ductus arteriosus-dependent pulmonary blood flow .
-Present immediately after birth with severe cyanosis that progresses rapidly to metabolic acidosis: TGA
(Transposition of Great Arteries)
-Days to weeks after birth with heart failure and mild hypoxemia, A hyperdynamic precordium, wide
pulse pressure, a normal S1 with a frequent ejection click, and a loud, usually single S2 are characteristic:
persistant truncus.
- In persistent truncus, Heart murmurs vary and may include a flow murmur at the base, a loud regurgitant
murmur at the lower left sternal border, and a mid-diastolic mitral flow murmur. With truncal valve
insufficiency, a high-pitched diastolic murmur over the mid-sternum is present
*ECG
* X-Ray
*Echocardiography
Management/Treatment:
*TET SPELLS: treatment
1. knee-chest position or over parent's shoulder with knees bent
2. supplemental oxygen (effectiveness is questionable in the absence of pulmonary blood flow)
3. sedation: intravenous or subcutaneous morphine, 0.1 mg/kg
4. intravascular volume expansion
5. prolonged cyanosis: an alpha agonist (phenylephrine, 5-10 mcg/kg IV)
6. for prevention of spells: propranolol (0.5-1 mg/kg po QID)
* The infants need to be monitored because of the resultant polycythemia, which may lead to
hyperviscosity. Hyperviscous blood flows poorly through the circulatory bed and results in poor tissue
perfusion.
*If blood flow to the systemic or pulmonic circulation is not sufficient to sustain life, prostaglandin E1
(PGE1) (0.05 to 0.1 g/kg/min IV) can be administered to maintain patency of the ductus arteriosus.
*When a PGE1 infusion is being administered, blood pressure must be monitored and hypotension
corrected.
Transposition of the Great Vessels

*The arterial switch procedure is the surgical intervention of choice, since it returns blood flow to its
normal pattern. This procedure involves cutting the great vessels above the valves and switching their
positions with reimplantation of the coronary arteries.
*When corrective surgery is not possible, a palliative balloon or surgical septostomy is performed.
*If a balloon septostomy is not possible or not effective, a surgical septostomy (Blalock-Hanlon
operation) can be performed.
*Corrective surgery involves partitioning the atrium and dividing the chamber into a front and a back
section (Atrial Switch).
*Systemic venous blood is redirected in front of the partition toward the left ventricle and pulmonary
venous blood is directed behind the partition toward the right ventricle.
*This partition can be made of a synthetic material (Mustard procedure) or of the childs atrial septum
(Senning procedure).
*The most common long-term complications of the Mustard and Senning procedures are arrhythmias
Tetralogy of Fallot
*Corrective operations are often performed by 18 months of age or earlier if the child has recurrent
hypoxic episodes or progressive cyanosis.
*If pulmonary stenosis is severe, and supplemental blood flow through the ductus arteriosus is required to
support oxygenation during the neonatal period.
*A palliative shunt is often placed, the most common of which is the Blalock-Taussig shunt. The shunting
procedure involves anastomosis of the subclavian artery to the pulmonary artery, which will direct blood
from the systemic circuit into the pulmonary bed and improve pulmonary blood flow.
*Corrective surgery involves closing the VSD with a patch, relieving the right ventricular outflow
obstruction, and closing any previous palliative shunts.
*Early corrective surgery is preferable to a palliative procedure and can be performed in infancy if the
pulmonary arteries are of sufficient size and the coronary artery connection is in a normal position.
*Without surgery, life expectancy is markedly reduced. Even with successful surgery, heart block,
aneurysm formation, and late sudden death are possible

Acyanotic Heart Defects a congenital disorder manifested with left to right

shunting and obstructive lesions. Clinical signs are not always apparent at birth,
they manifest anytime during infancy or early childhood.
Defects:
a.

Left to right shunting lesions, increased pulmonary blood flow

Pulmonary blood flow increases because of the extra volume in the right side.
There is a step-up 02 saturation in the right side of the heart (abnormal
increased) because of the addition of more highly saturated blood.
Physiologic effects include increased pulmonary blood flow, increased cardiac
workload (including ventricular strain, dilation, and hypertrophy)

Examples:
1. Atrial Septal Defect (ASD)

Most infants and children are asymptomatic but over years to decades
may experience:
o Fatigue and SOB
o Palpitations or atrial dysrythmias result of atrial enlargement
o Recurrent respiratory infections can occur when there is a large
amount of pulmonary blood flow
o Systolic murmur is produced by increased blood flow across the
pulmonary valve.
o Diastolic murmur is present with large shunts
o Stroke or major organ damage can occur because of embolization
of thrombus, air or other materials PARADOXIMAL EMBOLISM

Therapeutic Management:
1. Asymptomatic child is followed by cardiologist. Spontaneous closure can occur
in the first years of life for smaller size secundum ASDs.
2. Elective surgical repair is performed around 2-5 years of age
3. Surgical repair is recommended for all sinus venosus and ostium primum
defects.
Medical Management:
1. Asymptomatic patients with moderate size secundum ASDs are monitored for
spontaneous closure in the first years of life with medication.
2. Symptomatic infants and children are treated with diuretics and digoxin as
indicated
3. Atrial dysrythmias are treated with appropriate antidysrythmics

Surgical Management:
o

o
o

Surgical closure using either sutures or a pericardial prosthetic patch is

performed on an elective basis early in childhood. This is an open heart
procedure, through a sternal incision.
Mortality rate is <2%, with most centers near 0%. For the young adult with
ventricular dysfunction or pulmonary, the risk can be significantly higher.
Complications include sinus node and atrial dysrhythmias

Diagnostics:
1. Echocardiogram

2. EKG
3. CXR
4. Cardiac Catheterization
Ventricular Septal Defect (VSD)
Manifestations:
o

Signs and symptoms vary with the size of the defect and the presence of
associated cardiac lesions. Clinical symptoms are usually not seen at birth
because of continued high pulmonary vascular resistance in the newborn.
Infants with moderate to large defects will become symptomatic within the
first few weeks of life.
Children with small defects will remain asymptomatic.

Diagnostics:

Chest x-ray -- looks to see if there is a large heart with fluid in the lungs

ECG -- shows signs of an enlarged left ventricle

Echocardiogram -- used to make a definite diagnosis

Cardiac catheterization (rarely needed, unless there are concerns of high

blood pressure in the lungs, in which case surgery to close the defect is
generally not recommended)

MRI of the heart -- used to find out how much blood is getting to the lungs

Therapeutic Management:

20%-80% of all VSDs closed spontaneously.

Many small lesions do not require surgical intervention.

If there is aortic valve regurgitation related to VSD position near the valve
and even if the defect is small, surgery is indicated to reduce the progression
of valve insufficiency.

Antibiotic prophylaxis is indicated for all VSDs.

Medical Management:

Infants who develop CHF- digoxin diuretics, ACE inhibitors to reduce

afterload.

Nutritional supplements are added to infant formula to increase caloric

intake.NGT feeding or gastrostomy tube feeding for infants who are unable to
obtain adequate calories orally

Avoid exposure to respiratory infections.

Surgical Management:
1. Pulmonary artery banding for children with multiple muscular VSDs. In this
palliative procedure, a band is placed around the main pulmonary artery,
decreasing blood flow, reducing the severity of CHF and decreasing the risk of
pulmonary vascular disease.
2. The current trend is to perform corrective surgery earlier in life, and
consequently, pulmonary artery banding is performed less frequently than in
the past.
3. Total correction is accomplished by placing sutures to close small defects or
by placing a pericardial or prosthetic patch over moderate to large defects.
4. The surgical approach is usually through the RA to avoid a right ventricular
incision which could impair the contractility of the ventricle.
5. VSDs just below the pulmonary valve are closed through an incision in the
main pulmonary artery. Mortality is 5%-8%, depending on the age and type of
VSD.
6. Complications include residual VSDs, pulmonary hypertension in the
postoperative period, heart block that may require a pacemaker and an
abnormal rhythm called junctional ectopic tachycardia.
CO can be significantly decreased if dysrythmias are persistent. Post
pericardiotomy syndrome can also occur.
Other examples: Patent Ductus Arteriosus (PDA), and Atrioventricular Septal
Defect (AVSD).

b.

Obstructive or stenotic lesions stenosis of an opening can occur in a

valve or vessel constricting or obstructing blood flow through the area. Pressure
rises in the area behind the obstruction; blood flow distal to the obstruction may
be decreased or absent.
Example: Pulmonary stenosis, aortic stenosis, Coarctation of Aorta, and
interrupted aortic archs.

NURSING INTERVENTIONS FOR SPECIFIC NURSING DIAGNOSIS:

*Impaired gas exchange

Monitor intake and output

Limit fluids as ordered

Administer diuretics as ordered

Position changes every 2 hours or as ordered

*Risk for impaired growth and development

Treat child as normally as possible

Teach parents that children are more comfortable when they know what to
expect

Promote age-appropriate activities as condition allows

*Altered nutrition: less than body requirements

Offer small, frequent feedings

Use soft nipple for infant to ease the stress

of sucking

Organize care to allow for rest

*Risk for infection

Limit exposure to individuals with infections

Promote good pulmonary hygiene

Prophylactic antibiotics when undergoing surgical or dental treatment to

prevent subacute bacterial endocarditis