Difference between revisions of "Tetralogy of fallot"

(Background)
(Treatment)
Line 26: Line 26:
  
 
== Treatment ==
 
== Treatment ==
*Definitive Treatment: Surgery
+
 
 
*Acute Presentation (Tet spell):
 
*Acute Presentation (Tet spell):
#Valsalva
+
===Knee-to-Chest Position===
#Place in knee-chest position
+
*The knee-to-chest position increases SVR. Dr. This can be done in the parent's arms or while lifting the patient onto the parents shoulders and tucking the knees underneath the chest.
##Increases SVR > more blood into pulm ciruclation
+
*Increasing the SVR causes  more blood to flow to the  pulmonary circulation
 +
===Analgesia===
 
#Morphine 0.1-0.2Mg/kg IV or IM
 
#Morphine 0.1-0.2Mg/kg IV or IM
##Mechanism of action unclear
+
#*Goal is to ideally avoid IV placement if possible
##Consider [[Intranasal Sedation#Intranasal Medications|Intranasal Fentanyl]]
+
#[[Intranasal Sedation#Intranasal Medications|Intranasal Fentanyl]] 1.5-2mcg/kg range <ref>Tsze DS, Vitberg YM, Berezow3 J, Starc TJ, Dayan PS. Treatment of tetrology of Fallot hypoxic spell with in- tranasal fentanyl. Pediatrics. 2014 Jul;134(1):e266-9.</ref>
#Fluids IV
+
#*Only one case report but IN administration may avoid the pain from a needle stick
##Improves RV filling
+
===Phenylephrine===
#Beta blockers IV
+
*Dose: 0.2 mg/kg IV
##Relaxation of RVOT
+
*Increases SVR similar to knee to chest positioning
#Phenylephrine
+
 
##Similar to knee-chest position
+
===Fluids IV===
#Prostaglandin E1 0.1 mg/kg bolus followed by infusion 0.05 to 0.1 mg/kg/min
+
#Improves RV filling
## Maintains the ductus
+
 
## Side Effects:  Hypotension, Bradycardia, Seizures and Apnea
+
===Beta blockers===
 +
*Propranolol IV  
 +
**Will relax the spasm causing right-sided ventricular outflow obstruction.
 +
**Should be administered in consulation with cardiology and pediatric surgery.
 +
 
 +
===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
 +
 
 +
===Definitive Treatment===
 +
*Cardiothoracic surgery to repair the defects early before significant pulmonary hypertension develops.
  
 
== See Also ==
 
== See Also ==

Revision as of 15:17, 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:
  1. Increased pulmonary outflow obstruction and/or
  2. 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

  1. VSD
  2. RV outflow obstruction (pulmonic stenosis)
  3. Overriding aorta
  4. 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

Treatment

  • Acute Presentation (Tet spell):

Knee-to-Chest Position

  • The knee-to-chest position increases SVR. Dr. This can be done in the parent's arms or while lifting the patient onto the parents shoulders and tucking the knees underneath the chest.
  • Increasing the SVR causes more blood to flow to the pulmonary circulation

Analgesia

  1. Morphine 0.1-0.2Mg/kg IV or IM
    • Goal is to ideally avoid IV placement if possible
  2. Intranasal Fentanyl 1.5-2mcg/kg range [3]
    • Only one case report but IN administration may avoid the pain from a needle stick

Phenylephrine

  • Dose: 0.2 mg/kg IV
  • Increases SVR similar to knee to chest positioning

Fluids IV

  1. Improves RV filling

Beta blockers

  • Propranolol IV
    • Will relax the spasm causing right-sided ventricular outflow obstruction.
    • Should be administered in consulation with cardiology and pediatric surgery.

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

Definitive Treatment

  • Cardiothoracic surgery to repair the defects early before significant pulmonary hypertension develops.

See Also

Source

  1. 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.
  2. Knipe K et al. Cyanotic congenital heart diseases. Radiopaedia. http://radiopaedia.org/articles/cyanotic-congenital-heart-disease
  3. Tsze DS, Vitberg YM, Berezow3 J, Starc TJ, Dayan PS. Treatment of tetrology of Fallot hypoxic spell with in- tranasal fentanyl. Pediatrics. 2014 Jul;134(1):e266-9.