The answer is E: ASD
In 1888, Fallot described a congenital heart defect composed of four characteristics (a) large ventricular septal defect (VSD) (b) right ventricular outflow obstruction (pulmonary valve stenosis) (c) overriding aorta (d) right ventricular hypertrophy. The main characteristic of TOF is cyanosis. Hypercyanotic spells or “tet spells” are paroxysmal episodes in which the cyanosis acutely worsens. Crying, feeding, or defecating can bring on these episodes.
View blueprint lesson
Tetralogy of Fallot Pearls
Tetralogy of Fallot is the only cyanotic congenital heart disease on the NCCPA blueprint
Characterized by four defects (tetra = 4) that together cause oxygen-deficient blood to flow out of the heart and into the rest of the body.
Four features: PROVe
- P ulmonary Stenosis
- R ight ventricular hypertrophy
- O verriding aorta
- V entricular septal defect
TET SPELLS: hypercyanotic episodes that develop during crying or feeding
Murmur is classically described as a harsh SYSTOLIC ejection murmur heard best at the left sternal border
The pulmonary stenosis causes a RIGHT TO LEFT shunt through the VSD, thereby causing cyanosis.
Diagnosis
Echocardiography is the best diagnostic tool to confirm the diagnosis of Tetralogy of Fallot
- Chest x-ray classically shows a boot-shaped heart
- Squatting decreases the cyanosis in tetralogy of Fallot
- EKG may show enlarged RA and RV. Check the width of QRS annually via ECG to reduce the risk of sudden cardiac death
- Cardiac catheterization may be required in some patients to fully define the anatomy
Treatment
Surgical correction is the definitive treatment for tetralogy of Fallot
- Most patients have surgery within the first year of life
- Twenty-year survival rates after surgery are above 80%. The most common causes of death are sudden cardiac death and heart failure
- Complications after surgery include arrhythmias, pulmonary regurgitation, residual outflow obstruction, and heart failure
