Cyanotic Congenital Heart Defects Lecture

Types of Cyanotic Congenital Heart Defects

• Decreased Pulmonary Blood Flow:
  • Tetralogy of Fallot (Tet)
  • Tricuspid Atresia
  • Truncus
• Mixed Blood Flow:
  • Transposition of the Great Arteries
  • Total Anomalous Pulmonary Venous Return (TAPVAR)
  • Hypoplastic Left Heart Syndrome (HLHS)

Signs and Symptoms

• Cyanosis
• Desaturation
• Congestive Heart Failure
• Feeding Intolerance

General Care

• Air emboli precautions
• Expect lower saturation (usually <90%)
• Decrease oxygen and metabolic demands
• Maintain cardiac output (heart rate x stroke volume)
• Cautious oxygen management (maintain patent ductus arteriosus)

Tetralogy of Fallot

• Four Defects:
  • Large Ventral Septal Defect (VSD)
  • Right Ventricular Outflow Tract Obstruction
  • Overriding Aorta
  • Right Ventricular Hypertrophy
• Cyanosis depends on pulmonary obstruction severity
• Tet Spells Management:
  • Calm environment
  • Knee-to-chest position
  • 100% oxygen
  • Possible medication use

Tricuspid Atresia

• Tricuspid valve fails to develop
• Blood flows through atrial septal defect or patent foramen ovale
• Patent ductus arteriosus required
• Symptoms: Cyanosis, tachycardia, dyspnea

Truncus Arteriosus

• Aorta and pulmonary artery form common trunk
• Complete blood mixing
• Common with DeGeorge syndrome
• Monitor for pulmonary overcirculation

Transposition of the Great Arteries

• Two parallel circulations
• Requires patent foramen ovale or ductus arteriosus for blood mixing
• Reverse differential cyanosis

Total Anomalous Pulmonary Venous Return (TAPVAR)

• Pulmonary veins fail to drain into left atrium
• Requires atrial septal defect for mixing
• Obstructive cases are surgical emergencies

Hypoplastic Left Heart Syndrome

• Diagnosed via 20-week ultrasound
• Small left ventricle and atrium
• Patent ductus arteriosus is necessary
• Symptoms: Respiratory distress, mild cyanosis

Prostaglandin Use

• Maintains ductal patency
• Important for ductal dependent lesions
• Dosage calculation: Patient weight and infusion rate

Congestive Heart Failure (CHF)

• Inability of heart to pump adequate blood
• Right-Sided Failure:
  • Venous hypertension
  • Hepatosplenomegaly
  • Edema
• Left-Sided Failure:
  • Pulmonary congestion
  • Pulmonary edema
• Symptoms:
  • Tachypnea, tachycardia
  • Dyspnea, retractions
  • Feeding intolerance, weight gain

Management of Heart Failure

• Improve cardiac function
• Remove fluid and sodium
• Decrease cardiac demands
• Medications:
  • Digoxin (improves contractility)
  • ACE inhibitors (reduce afterload)
  • Beta blockers
• Digoxin toxicity signs: Nausea, vomiting, anorexia, bradycardia, dysrhythmias

These notes provide a comprehensive overview of cyanotic congenital heart defects, their management, and related heart failure conditions discussed in the lecture. They are designed to serve as a useful study aid for understanding the key concepts and interventions.