Tetralogy of Fallot
Background
- Most common cyanotic CHD manifesting in post-infancy period
- Tetralogy:
- VSD
- RV outflow obstruction (pulmonic stenosis)
- Overriding aorta
- RV hypertrophy
Clinical Presentation
- Systolic ejection murmur along the left sternal border[1]
- Cyanosis worse during feeding and crying[2]
- May squat to relieve symptoms: increases afterload and decreases shunt[3]
- Acute respiratory distress (Tet Spells) due to increased right outflow tract obstruction[4]
Work-Up
- Echo
- CXR: shows the classic “boot-shaped” heart
Differential Diagnosis
Congenital Heart Disease Types
- Cyanotic
- Acyanotic
- AV canal defect
- Atrial septal defect (ASD)
- Ventricular septal defect (VSD)
- Cor triatriatum
- Patent ductus arteriosus (PDA)
- Pulmonary/aortic stenosis
- Coarctation of the aorta
- Differentiation by pulmonary vascularity on CXR[5]
- Increased pulmonary vascularity
- Decreased pulmonary vascularity
- Tetralogy of fallot
- Rare heart diseases with pulmonic stenosis
Treatment
- Definitive Treatment: Surgery
- Acute Presentation:
- Valsalva
- Place in knee-chest position
- Increases SVR > more blood into pulm ciruclation
- Morphine 0.1-0.2Mg/kg IV or IM
- Mechanism of action unclear
- Fluids IV
- Improves RV filling
- Beta blockers IV
- Relaxation of RVOT
- Phenylephrine
- Similar to knee-chest position
- Prostaglandin E1 0.1 mg/kg bolus followed by infusion 0.05 to 0.1 mg/kg/min
- Maintains the ductus
- Side Effects: Hypotension, Bradycardia, Seizures and Apnea
See Also
Source
- ↑ Horeczko T, Inaba AS: Cardiac Disorders; in Marx JA, Hockberger RS, Walls RM, et al (eds): Rosen’s Emergency Medicine: Concepts and Clinical Practice, ed 8. St. Louis, Mosby, Inc., 2014, (Ch) 171: p 2139-2169.
- ↑ Horeczko T, Inaba AS: Cardiac Disorders; in Marx JA, Hockberger RS, Walls RM, et al (eds): Rosen’s Emergency Medicine: Concepts and Clinical Practice, ed 8. St. Louis, Mosby, Inc., 2014, (Ch) 171: p 2139-2169.
- ↑ Horeczko T, Inaba AS: Cardiac Disorders; in Marx JA, Hockberger RS, Walls RM, et al (eds): Rosen’s Emergency Medicine: Concepts and Clinical Practice, ed 8. St. Louis, Mosby, Inc., 2014, (Ch) 171: p 2139-2169.
- ↑ Horeczko T, Inaba AS: Cardiac Disorders; in Marx JA, Hockberger RS, Walls RM, et al (eds): Rosen’s Emergency Medicine: Concepts and Clinical Practice, ed 8. St. Louis, Mosby, Inc., 2014, (Ch) 171: p 2139-2169.
- ↑ Knipe K et al. Cyanotic congenital heart diseases. Radiopaedia. http://radiopaedia.org/articles/cyanotic-congenital-heart-disease