Atrial septal defect Ventricular septal defect Patent ductus arteriosus

Definition Abnormal opening between the atria,
allowing blood from the higher pressure left
atrium to flow into the lower pressure right
atrium
THREE TYPES
-Osteum primum (ASD)
opening at lower end of the septum; may be
associated with mitral valve abnormalities
-Osteum secundum (ASD 2) opening near
center of septum
- Sinus venous defect:
opening near junction of superior vena cava
and right atrium: may be associated with
partial anomalous pulmonary venous
connection
Abnormal opening between right and left ventricle
-Classified according to location: - membranous
(80%)
- muscular
- Vary in size from a small pinhole to absence of
septum
-20-60% of VSDs are closed spontaneously during
1styear of life
•Frequently associated with other defects such as
pulmonary stenosis or PDA.
•Failure of the fetal ductus arteriosus
(artery connecting the aorta and
pulmonary artery) to close within the first
weeks of life.
•It allows blood to flow from the higher
pressure aorta to the lower pressure
pulmonary artery causing left-to-right
shunt.

Cause Acynotic: ↑Pulmonary blood flow leads to Left to right shunt:-Abnormal connection exists between the chambers, blood will flow from an area of
/Pathophysi higher pressure to lower pressure
ology

Clinical -Asymptomatic and may develop Heart
manifestatio failure
n - Characteristic systolic murmur with a fixed
split-second heart sound
- Diastolic murmur
- Atrial dysrhythmias(atrial enlargement and
stretching of conduction fibers
- Pulmonary vascular obstruction disease
- Emboli formation in later life (from
chronically increased blood flow)
•Heart failure CLINICAL MANIFESTATIONS
•Loud systolic murmur heard best at the left 1.asymptomatic/Show signs of HF
sternal border 2.Machinery like murmur
•Patients are at risk for BE and pulmonary vascular 3.Widened pulse pressure and bounding
obstructive disorder pulses
4.Later life risk for BE and pulmonary
vascular obstructive disordes

Diagnostic -Chest radiography(x-ray) -ECG -Echocardiography -Transthoracic -M-mode -Two-dimensional

evaluation -Doppler -Fetal -TEE -Cardiac catheterization -Hemodynamics -Angiography -Biopsy
-EPS -Exercise stress test -Cardiac MRI
Medical Non surgical treatment:
managemen
t -ASD 2 closure with a device (The Amplatzer
Septal Occluder) during cardiac
catheterization.
-Patient receives low-dose aspirin for 6
months.
Surgical treatment: Open repair with
cardiopulmonary bypass performed before
school age
-Patch closure (pericardial patch or Dacron
patch) for moderate to large defects
-Open cardiopulmonary bypass is performed
before school age.
-Patch placement for sinus venous defect to
direct the right pulmonary venous return to
the left atrium.
-ASD type 1 may require mitral valve repair
or placement of the mitral valve.
-Operative mortality is very low >1%
•Nonsurgical:
•Device closure during cardiac catheterization
-simple defects <2%, multiple defect carry higher
risks
•Surgical:
•Palliative: pulmonary artery banding (placement
of a band around the main pulmonary artery to
decrease pulmonary blood flow) for infants with
multiple muscular VSDs or complex anatomy.
•Complete repaire (procedure of choice): sutures
to repaire small defects and a knitted Dacron
patch for larger defects.
•Postoperative complications include residual VSD
and conduction disturbances.
•Nonsurgical management
•Indomethacin (prostaglandin inhibitor)
•Coils to occlude PDA through
catheterization
•Surgical treatment
•Division or ligation of the patent vessels
through left thoracotomy
•Video assisted thoracoscopic surgery
•Less than 1% mortality rate with both
surgical and nonsurgical management.
•PDA closure has higher mortality rate in
very preterm infants because of additional
medical problems.
 Coarctation of the aorta Aortic stenosis

Definition •Localized narrowing near the insertion of the ductus arteriosus
leading to increased pressure proximal to the defect (head and
upper extremities) and decreased pressure distal to the
obstruction (body and the lower extremities).
Cause Acynotic: Obstruction to blood flow from ventricles
/Pathophysiology
•Narrowing or stricture of the aortic valve leading to resistance to blood
flow in the left ventricle, decreased cardiac output, left ventricular
hypertrophy and pulmonary vascular congestion.
•Types:
•Valvular stenosis: the most common type
•Subvalvular stenosis

Clinical •High blood pressure in the arms CLINICAL MANIFESTATIONS

manifestation •Bounding pulse in the arms, weak or absent femoral pulse, •Newborns:
•Cool lower extremities -decreased cardiac output with faint pulse, -hypotension
•Decreased pressure in the lower extremities -tachycardia -poor feeding
•Signs of HF in infants •Children:
•Older children will have dizziness, headache, fainting, and -exercise intolerance -chest pain
epistaxis -dizziness when standing for long period
•Systolic ejection murmur
•At risk for BE
•Coronary insufficiency
•Ventricular dysfunction Hypertrophy

Medical •Surgical: •Stabilization with beta-blocker or a calcium channel blocker

management •The treatment of choice for infants less than 6 months and for •Surgical treatment:
patients with long- segment stenosis or complex anatomy •Aortic valvotomy
•Resection of the coaracted portion or enlargement of the •Valve replacement with lifelong antiplatelet or anticoagulation or
constricted section(the aorta is removed and the new ends of antibiotic therapy
the aorta are anastomosed with subclavian artery graft) -Valvular aortic stenosis
•Nonsurgical treatment: •Nonsurgical treatment:
•Balloon angioplasty in older infants and children •Balloon angioplasty (the first line procedure)
•Stents in adolescent ••Balloon valvuloplasty
•Therapy with digoxin and diuretics before surgery Subvalvular aortic stenosis
•Continues treatment with anti hypertensive agents •Surgical treatment :
PROGNOSIS:-Mortality is <5% •Konno procedure
 Tetralogy of fallots Tricuspid atresia

Definition The classic form includes four defects •It is usually associated with PS and TGA.
Pulmonary stenosis Description:
VSD 1- Tricuspid valve fails to develop
Overriding of the aorta 2- No communication from RA to the RV
Hypertrophy of right ventricle 3- Blood flows through the ASD to the Left side of heart and
through VSD to the RV and out of the lungs
4- Complete mixing of unoxygenatedand oxygenated blood
5- Leads to systemic desaturation and Varying amounts of
pulmonary obstruction
6- Decreased pulmonary blood flow

Cause cyanotic: ↓Pulmonary blood flow

/Pathophysiology

Clinical •Cyanosis Cyanosis in new born period

manifestation •Systolic murmur •Tachycardia
•Blue spells or tet spells (acute episodes of cyanosis and hypoxia) •Dyspnea
•Anoxic spells occur during crying or feeding (oxygen requirements •Older children have signs of hypoxemia with clubbing
exceed the blood supply)
•Children at risk for emboli, seizures and loss of consciousness or
sudden death

Medical Surgical management •Infusion of Prostaglandin: E1 is started at 0.1 mcg/kg/min until

management •Palliative shunt –provides blood flow to the pulmonary arteries surgery is arranged
from the rt. subclavian artery via a tube graft .Procedure is called •Surgical treatment:
Blalock Taussing shunt •Placement of a shunt at 4-9months as second stage
•Complete repair : Performed in the first year of life -If ASD is small-atrial septostomy
-Indication : increase in cyanosis and development of hyper •Pulmonary artery banding in case of increased pulmonary blood
cyanotic spells flow
•Procedure : complete repair involves closure of the VSD and •Bidirectional Glenn shunt
resection of infundibular stenosis with placement of pericardial •Fontan procedure
patch •Pulmonary to systemic artery anastomosis
•PROGNOSIS: Operative mortality is less than 3 % •Prognosis:
•HF may occur post operatively •Surgical mortality is less than 5%
 Transposition of the great arteries (TGA) or transposition of Heart Failure
the great vessels

Definition The pulmonary artery leaves the LV, and the aorta exits from HFis an inability of the heart to pump an adequate amount of blood to the
the RV with no communication between the systemic and systemic circulation
pulmonary circulations
Two categories: Right sided and left sided failures

Cause •It is secondary to structural abnormalities (increased blood volume and

/Pathophysiol •Associated defects such as septal defects or PDA allow blood pressure in the heart), myocardial failure (contractility of the ventricle
ogy to enter the systematic circulation or the pulmonary impaired), sepsis or severe anemia (excessive demands on a normal heart
circulation for mixing of saturated and desaturated. muscle) .
RIGHT SIDE FAILIURE LEFT SIDE FAILIURE

definition The right ventricle is unable to The left ventricle is unable

pump blood in to the
pulmonary artery, results
increased pressure in the right
atrium and systemic venous
circulation
to pump blood in to the
systemic circulation
resulting in increased
pressure in the left atrium
and pulmonary edema

S&S Venous hypertension Lungs become congested

Hepatosplenomegaly with blood
Edema Pulmonary hypertension
Pulmonary edema

Clinical Severely cyanotic and have depressed functions at birth
manifestation •Large septal defects or PDA-less cyanotic but have
symptoms of HF.
•Heart sounds depends on defects present
•Cardiomegaly few weeks after birth.
•Impaired myocardial function
•Tachycardia - weak peripheral pulses •Sweating - decreased BP
•Decreased urinary output •Fatigue -gallop rhythm •Anorexia
•Weakness - cardiomegaly •Restlessness •Pale , cool extremities
•Systemic venous congestion
•Weight gain •Hepatomegaly •Peripheral edema, periorbital •Ascites
•Neck vein distention (children)
•Pulmonary congestion
•Tachypnea - cyanosis •Dyspnea - wheezing •Flaring nares •Orthopnea
•Retractions (infants) - grunting •Exercise intolerance •Cough, hoarseness
Diagnostic •Chest radiograph
evaluation •Cardiomegaly/•Increased pulmonary blood flow
•ECG •Ventricular hypertrophy
•Echocardiography
•Cause of HF (congenital heart defect or poor ventricular function)

Medical IV Prostaglandin E1 to keep ductus open temporarily 1.Improve cardiac function(increase contractility and decrease afterload)
Management •Balloon atrial septostomy •Digitalis glycosides (digoxin)
•Rashkind procedure •Angiotensin-converting enzyme(ACE) inhibitor
SURGICAL MANAGEMENT (Captopril, enalapriland Lisinopril)
•Arterial switch procedure in neonates 2. Remove accumulated fluid and sodium (decrease preload)
•Intraatrial baffle repairs in above 15 years •Administer diuretics (Furosemide, chiorothiazide, spirinolactone)
•Rastelli procedure in infants 3. Decrease cardiac demands
•Prognosis: •Provide neutral thermal environment
•Operative mortality is less than 2%. •Treat any existing infection
•Reduce the effort of breathing •Semi fowlers position
•Administer sedation
•Provide rest
4.Improve tissue oxygenation
•Lessen tissue oxygen demands •Oxygen administration

NURSING 1.Assist in measures to improve cardiac function 2.Monitor afterload reduction

CARE 3.Decrease cardiac demands
MANAGEME 4.Reduce respiratory distress
NT 5.Maintain Nutritional status
6.Assist in measures to promote fluid loss
7.Support child and family
assessment Diagnosis

Tachypnea, Tachycardia, Cool extremities, Hypotension, edema, Rapid Decreased cardiac outputR/T structural defects, Myocardial
weight gain dysfunction, altered hemodynamics

Tachypnea, dyspnea, Retractions, crackles. Cyanosis, activity Ineffective breathing pattern R/T pulmonary congestion, decreased
intolerance cardiac output

Ineffective cardiopulmonary and peripheral tissue perfusion related

to inadequate heart function
 Hypoxemia

Definition •An arterial oxygen tension (pressure pao2) that is less than normal and can be identified by a decreased arterial saturation or decreased
pao2.
•Hypoxia – is a reduction in tissue oxygenation that results from low oxygen saturations and pao2 and results in impaired cellular
processes
•Cyanosis – is a blue discoloration in the mucous membranes, skin, and nail beds of the child with reduced oxygen saturation.

Clinical •Polycythemia – increased number of RBCs, increases the oxygen-carrying capacity of the blood.
manifestation •Polycythemia increases the viscosity of the blood and crowds out clotting factors
•Anemia may result if iron is not readily available for the formation of hemoglobin
•Clubbing – thickening and flattening of the tips of the fingers and toes.
•Infants with hypoxemia may be asymptomatic except for cyanosis and exhibit near-normal growth and development.
•Infants with more severe hypoxemia may exhibit fatigue with feeding, poor weight gain, tachypnea and dyspnea.
•Severe hypoxemia resulting in tissue hypoxia is manifested by clinical deterioration and signs of poor perfusion.
•Hypercyanotic spells (blue spells or TET spells)
•Most frequently seen in 1st year of life (rarely in 2months of age)
•Feeding, crying, defecation, stressful procedures
•Seen in TOF, obstruction to pulmonary blood flow, communication between ventricles.

Diagnostic •Hyperoxia test

evaluation •Infant will be placed in a 100% oxygen environment and blood parameters are monitored
•Pao2 of 100 mmhg suggests lung disease
•Pao2 lower than 100 mmhg suggests cardiac disease
•Chest radiography
•Echocardiogram

Medical •Prostaglandin E
Management •Causes vasodilation and smooth muscle relaxation
•Hydration (IVF)
•Keep the hematocrit and blood viscosity within acceptable limits to reduce the risk of cva’s
•Anemia monitoring
•Reduced arterial-oxygen carrying capacity and reduced risk of cvas
•Iron supplementation •Blood transfusion
•Improve pulmonary function
•Aggressive pulmonary hygiene •Chest physical therapy •Administration of antibiotics •Oxygen to improve arterial saturations
NURSING •They need simple explanation of hypoxemia and cyanosis and reassurance that cyanosis does not imply a lack of oxygen to the brain.
CARE •Questions and fears need to be addressed in a calm, supportive manner and positive aspects of child’s growth and development are
MANAGEMEN emphasized.
T •Treating hypercyanotic spells
•Place infant in knee-chest position •Use a calm, comforting approach •Administer 100% oxygen by blow-by
•Give morphine subcutaneously or through an existing IV line
•Begin IV fluid replacement and volume expansion if needed •Repeat morphine administration
•Preventive measures and accurate assessment of respiratory infection
•Good hand washing•Stay away from person with obvious respiratory infection•Aggressive pulmonary hygiene •Treatment with antibiotics
Decreased cardiac output related to congenital structural disorder
Ineffective tissue perfusion related to inadequate cardiac output
Deficient knowledge related to care of the child pre and postoperatively
Fear related to lack of knowledge about child’s illness
Interrupted family processes related to stresses of the diagnosis and care responsibilities.
Ineffective coping related to lack of adequate support people.
Impaired parenting related to inability to bond with critically ill newborn.