Table Of Contents

Misc Defects

🧠 Presentation is typically delayed until a few days after birth when the ductus arteriosus closes. | 🔹 TTE | 🔹 Alprostadil and transcatheter septoplasty to maintain shunting 🔹 Surgical repair | | Ebstein's Anomaly | 🔹 Malformed and displaced tricuspid valve, attached to the RV endocardium 🔹 Associated with Lithium use | 🔹 Severe disease presents with cyanosis and heart failure in infants | 🔹 TTE (Tricuspid regurgitation and RV dilatation) | 🔹 Surgical repair | | Supravalvular Aortic Stenosis | 🔹 Thickening of the ascending aorta, causing aortic stenosis 🔹 Associated with Williams Syndrome | 🔹 Variable features depending on obstruction degree 🔹 Systolic ejection murmur (FIRST right space) 🔹 BP differential between arms | 🔹 TTE | 🔹 Surgical repair for high-grade stenosis |

Illustrations

• Ebstein anomaly

• Hypoplastic left heart syndrome

Persistent Pulmonary Hypertension of the Newborn (PPHN)

General

• Occurs when pulmonary vascular resistance remains abnormally high after birth, leading to right-to-left shunting through fetal structures (e.g., foramen ovale, ductus arteriosus) and impaired oxygenation.

Etiology

• Elevated pulmonary vascular resistance results in right-to-left shunting of blood.
  • This bypasses the lungs, leading to a pre- and postductal oxygenation gradient (preductal oxygen saturation is often higher than postductal).

Risk Factors

• Meconium aspiration syndrome.
• Neonatal pneumonia.
• Lung hypoplasia congenital diaphragmatic hemia

Clinical Features

• Usually appears within 24 hours after birth.
• Low APGAR scores.
• Cyanosis and signs of respiratory distress (e.g., tachypnea, grunting).
• Prominent S2

Imaging