List of Topics | Congenital Heart Disease - Cove Point Foundation | Johns Hopkins Children's Hospital

Cyanotic Congenital Heart Disease

• Cyanotic CHD Scenarios
• Persistent Truncus Arteriosus
• Transposition of the Great Arteries (TGA)
• Total Anomalous Pulmonary Venous Return (TAPVR)
• Tetralogy of Fallot (TOF)
• Tricuspid Valve Atresia
• Pulmonic Stenosis
• Hypoplastic left heart syndrome
• Ebstein Anomaly

Remember the cyanotic congenital heart lesions as the 1-2-3-4-5 T’s, each number corresponding to the defect:

1. T**wo separate circuits (parallel) 2. Give PGE1 (duct dependent) 3. Arterial switch is urgent | 3/Tricuspid valve fails to form | 4/Tetrad of cardiac defects PROVe/Tetra 4:
2. Pulmonary infundibular stenosis
3. Right ventricular hypertrophy
4. Overriding aorta
5. Ventricular septal defect | 5 words; Pulmonary veins do not connect to left atrium | | Cyanosis onset | Mild to moderate from birth | Severe cyanosis within first hours of life | At birth or as ductus closes | After ductus closes (hours–days) | Early, especially in obstructed type | | Heart sounds | Loud single S2, systolic ejection murmur | Loud single S2, usually no murmur | Single S2, holosystolic murmur (VSD) | Single S2, harsh systolic ejection murmur (PS) | Wide and fixed split S2, soft systolic murmur | | ECG findings | Biventricular hypertrophy | Normal or RAD, RVH | Left axis deviation, LVH | Right axis deviation, RVH | Right axis deviation, RVH | | CXR findings | Cardiomegaly, ↑ pulmonary markings | Egg-on-a-string, ↑ pulmonary markings | Small heart, ↓ pulmonary markings | Boot-shaped heart, ↓ pulmonary markings | "Snowman" sign in supracardiac type, ↑ markings | | Pulmonary blood flow | ↑ | ↑ (unless VSD/PS present) | ↓ (depends on associated defects) | ↓ (due to RV outflow obstruction) | ↑ if unobstructed, ↓ if obstructed | | Associated lesions | VSD present by definition | Need ASD/PFO and PDA for mixing | ASD/PFO + VSD essential for survival | VSD, RVOTO, overriding aorta, RVH | ASD required for mixing | | Surgical urgency | Early surgery in infancy | Emergent PGE1 then arterial switch surgery | PGE1 + staged surgery (Glenn/Fontan) | Elective repair at 4–6 months | Emergent in obstructed type | | Note(s) | Associated w/ DiGeorge syndrome (22q11.2 del), avoid O2 (pulm vasodilator) | Cyanosis without respiratory distress, severe acidosis | | Tet spells; degree of RVOT obstruction is the primary driver of symptoms | Prostaglandin contraindicated in obstructive type |

Acyanotic Congenital Heart Disease

• Patent Ductus Arteriosus (PDA)

• Atrial Septal Defect (ASD)

• Ventricular Septal Defect (VSD)

• Coarctation of the Aorta