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)