PATHOPHYSIOLOGY (TETRALOGY OF FALLOT)

The initial defect in TOF is a narrowing of the right ventricular outflow tract into the pulmonary artery. This prevents deoxygenated blood from entering
the pulmonary circuit. In response to this outflow obstruction, the myocardium of the right ventricle hypertrophies in order to contract forcefully enough to push
blood past the stenosis. Additionally, patients have a large ventricular septal defect, which allows shunting of blood between the ventricles. In a patient with an
isolated VSD, the blood flow is shunted initially from left-to-right. However, in TOF, the right ventricular outflow obstruction may impede the normal blood flow
so significantly that the left side of the heart becomes the path of least resistance. Blood from the right ventricle is then forced into the left ventricle, creating a
right-to-left shunt and subsequent cyanosis. Finally, the aorta overrides the ventricular septal defect, straddling the VSD. This allows deoxygenated blood
shunted from the right ventricle to immediately exit the heart mixed with blood from the left ventricle.

The most important determinant of the severity and clinical consequences of TOF is the degree of right ventricular outflow obstruction. With a lesser
obstruction, blood is shunted from left-to-right and permitted to preferentially enter the pulmonary circulation, allowing for oxygenation. With a greater degree
of obstruction, however, blood is forced in the opposite direction, away from the pulmonary circulation, leftward across the VS and ultimately blood exits the
heart before being oxygenated. Patients will present with differing degrees of outflow obstruction and this may fluctuate throughout the course of the illness.
 Predisposing Factors:
 Genetics (Father was diagnosed with
Precipitating Factors:
ALGS, heaptic ductular hypoplasia
 Stress
as a child.
 Medications taken by the mother during
 He has congenital spinal
pregnancy (anti-depressant: Fluoxetine;
abnormalities, cardiac defects inhalers: Becctide and Ventolin)
 Age = at birth

ALTERATION IN FETAL DEVELOPMENT

- Unequal growth of the artico-
pulmonary septum

Aorta too large C. Overriding Aorta

Steals space from B. Pulmonary Stenosis

Pulmonary artery

Prevents ventricular A. Ventricular Septal

wall closure Defect

Right Ventricular
Outflow Obstruction Mixing of oxygenated Increase ventricular
and deoxygenated contraction
blood

Decrease 02 supply to D. Right Ventricular

systemic circulation Hypertrophy

 Cyanosis
Hypoxia
 Tet spells
 Dyspnea on exertion
 Clubbing of fingers and toes
 Heart murmurs
 Low birth weight
 Poor feeding
 Growth and development may be delayed
 Polycythemia

IF TREATED IF NOT TREATED

Diagnostic exam: Transient increase in resistance to
 CBC blood flow to the lungs
 CXR
 Echocardiogram
 ABG
 Cardiac catherization Worsen tet spells

Treatment:
 Medications
*Aspirin 15mg OD Decrease organ perfusion including
“brain”
*Frusemide 3mg BD
*Spironolactone 3mg BD
*Paracetamol PRN
 Hypoxic brain injury
 CPAP (Continuous positive Airway
 Organ failure
Pressure)

 PEEP (Positive End-Expiratory

Pressure) DEATH
 Surgical Interventions

*Temporary or Palliative Surgery

*Complete Intracardiac Repair

•
Partially improved
Position oxygenation
the patient in a semi-upright position.
• Suction oral and nasal secretions.
• Provide bed rest to the patient. Ensure uninterrupted period of rest and sleep.
• Organize periods of medication and nursing care to give uninterrupted rest.
• Provide feeding, tactile stimulation and change wet diaper to prevent the patient from crying.
• Administer medication prescribed.
• Administer oxygen as required. Monitor oxygen saturation through pulse oximeter.
• Assess the patient for developmental milestones. Provide visual, tactile and auditory stimulations
appropriate to the patient’s age.
• Encourage parents to provide tender loving care.
• Assure the parents

• Partially improved oxygenation

• May improved length and quality of
life
Clubbing of finger nails