Tetralogy of Fallot: Difference between revisions
DrJeffRogers (talk | contribs) |
DrJeffRogers (talk | contribs) |
||
| Line 1: | Line 1: | ||
== Background == | == Background == | ||
*Most common cyanotic CHD manifesting in post-infancy period. Tet spells are acute episodes of hypoxia and cyanosis caused by right-to-left shunting across the VSD. Patients will present with irritability, agitation, grunting, crying, and central cyanosis. | *Most common cyanotic CHD manifesting in post-infancy period. Tet spells are acute episodes of hypoxia and cyanosis caused by right-to-left shunting across the VSD. Patients will present with irritability, agitation, grunting, crying, and central cyanosis. | ||
*During cyanotic spells, there is either: | |||
#Increased pulmonary outflow obstruction and/or | |||
#Decreased systemic vascular resistance leading to right-to-left shunting | |||
*During the spell there is hypercarbia and hypoxemia (which further increases pulmonary vascular resistance). The process compounds itself creating worsening right-to-left shunting, hyperpnea, right outflow tract obstruction and increased systemic venous return. | |||
===Tetralogy=== | ===Tetralogy=== | ||
#VSD | #VSD | ||
Revision as of 14:53, 16 April 2015
Background
- Most common cyanotic CHD manifesting in post-infancy period. Tet spells are acute episodes of hypoxia and cyanosis caused by right-to-left shunting across the VSD. Patients will present with irritability, agitation, grunting, crying, and central cyanosis.
- During cyanotic spells, there is either:
- Increased pulmonary outflow obstruction and/or
- Decreased systemic vascular resistance leading to right-to-left shunting
- During the spell there is hypercarbia and hypoxemia (which further increases pulmonary vascular resistance). The process compounds itself creating worsening right-to-left shunting, hyperpnea, right outflow tract obstruction and increased systemic venous return.
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[1]
- May squat to relieve symptoms: increases afterload and decreases shunt[1]
- Acute respiratory distress (Tet Spells) due to increased right outflow tract obstruction[1]
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[2]
- Increased pulmonary vascularity
- Decreased pulmonary vascularity
- Tetralogy of fallot
- Rare heart diseases with pulmonic stenosis
Treatment
- Definitive Treatment: Surgery
- Acute Presentation (Tet spell):
- 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
- Consider Intranasal Fentanyl
- 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
- ↑ 1.0 1.1 1.2 1.3 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