tetralogy of fallot

Tetralogy of Fallot

A congenital heart defect characterized by four structural defects of the heart.

Cyanotic heart defect

A heart defect causing cyanosis due to decreased pulmonary blood flow.

RAPS

Mnemonic for the four defects in Tetralogy of Fallot: Right ventricular hypertrophy, Aorta displacement, Pulmonary stenosis, Septal defect.

Right ventricular hypertrophy

Enlargement of the right ventricle caused by overworking due to increases in pressure.

Pulmonary stenosis

Narrowing of the pulmonary valve and artery, impeding blood flow to the lungs.

Ventricular Septal Defect (VSD)

A hole in the ventricular wall allowing mixing of oxygenated and unoxygenated blood.

Normal blood flow sequence

Pathway of unoxygenated blood from body to the lungs and the oxygenated blood from the lungs to the body.

Knee-to-chest position

Position used in managing a 'Tet spell' to decrease right to left shunting and increase systemic vascular resistance.

Tet spell

An episode of cyanosis and breathing difficulty that occurs during activity due to TOF.

Activity intolerance

A term describing the reduced ability to engage in normal activities due to low oxygen levels.

Clubbing of fingernails

Physical sign arising from chronic hypoxia often seen in patients at about six months.

Failure to thrive

A clinical condition in which a child cannot maintain an adequate growth trajectory due to health issues.

Systolic murmur

A heart sound associated with pulmonary stenosis, typically heard left of the sternal border.

Palliative surgery

Temporary surgical procedures to improve symptoms and blood flow, not a permanent solution.

Prostaglandin E (Aprostadil)

Medication that keeps the ductus arteriosus open to improve blood flow to the lungs.

Shunt placement

A temporary surgical procedure to direct blood flow and increase oxygen levels in the body.

Complete repair surgery

A definitive surgical procedure usually performed when the child is old enough to tolerate it.

Aorta displacement

A condition in TOF where the aorta is positioned over the ventricular septal defect instead of the left ventricle.

Stenosis

Narrowing of a passage or opening, specifically in the context of blood vessels.

Failure to thrive symptoms

Not gaining weight, being small for age, and not achieving developmental milestones.

Cyanosis

Bluish discoloration of the skin or mucous membranes due to insufficient oxygen in the blood.

Signs of Tetralogy of Fallot

Cyanosis, fatigue, activity intolerance, and harsh systolic murmur.

Importance of systemic vascular resistance

Increasing resistance to improve oxygen levels by decreasing right to left shunting.

Hypoxemia

A condition characterized by low oxygen levels in the blood.

Management of 'Tet spells'

Involves calming the infant, placing them in knee-to-chest position, and administering oxygen.

Risk of heart failure in TOF

Due to chronic strain on the right ventricle from increased workload caused by defects.

Surgical intervention timing for TOF

Typically performed when the child is between 6-12 months of age.

Tricuspid valve

The heart valve that controls blood flow from the right atrium to the right ventricle.

Aortic valve

The heart valve that regulates blood flow from the left ventricle into the aorta.

Pulmonic valve

The valve that controls blood flow from the right ventricle to the pulmonary artery.

Heart defect prevalence in US

1 in 2518 babies born in the US will be diagnosed with Tetralogy of Fallot.

Developmental milestones

Goals for children’s physical and cognitive skills that occur at various ages.

Intercostal space

The spaces between the ribs where specific heart sounds can be auscultated.

Pulmonary vein

The blood vessels carrying oxygenated blood from the lungs to the left atrium.

Right atrium

The chamber of the heart that receives unoxygenated blood from the body.

Bicuspid valve (Mitral valve)

The valve that controls blood flow from the left atrium to the left ventricle.

Aorta

The large artery that carries oxygenated blood from the heart to the body.