PANCE Blueprint Cardiology (13%)

Patient will present as → a 2-week-old newborn brought to the ER by his mom who reports a sudden loss of consciousness during feeding and with crying. She also has noticed that the infant's lips have turned blue on three occasions during feeding.  blood pressure is 75/45 mmHg, a pulse is 170/min, and respirations are 44/min. A grade 3/6 harsh systolic ejection murmur is heard at the left upper sternal border. A CXR shows a small boot-shaped heart and decreased pulmonary vascular markings.

Cyanotic heart disorder that is 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

1. P ulmonary Stenosis
2. R ight ventricular hypertrophy
3. O verriding aorta
4. V entricular septal defect

The pulmonary stenosis causes a right to left shunt through the VSD, thereby causing cyanosis.

• 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

• Echocardiography is the diagnostic modality of choice. This test can clearly define the four abnormalities as well as provide important information about aortic arch anatomy
• A chest x-ray classically shows a boot-shaped heart
• 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
• Squatting decreases the cyanosis in tetralogy of Fallot

Treatment is surgical. 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