Tetralogy of Fallot: Difference between revisions
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#RV hypertrophy | #RV hypertrophy | ||
==Clinical | ==Clinical Features== | ||
*Systolic ejection murmur along the left sternal border<ref name="horeczko">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.</ref> | *Systolic ejection murmur along the left sternal border<ref name="horeczko">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.</ref> | ||
*Cyanosis worse during feeding and crying<ref name="horeczko"></ref> | *Cyanosis worse during feeding and crying<ref name="horeczko"></ref> | ||
*May squat to relieve symptoms: increases afterload and decreases shunt<ref name="horeczko"></ref> | *May squat to relieve symptoms: increases afterload and decreases shunt<ref name="horeczko"></ref> | ||
*Acute respiratory distress (Tet Spells) due to increased right outflow tract obstruction<ref name="horeczko"></ref> | *Acute respiratory distress (Tet Spells) due to increased right outflow tract obstruction<ref name="horeczko"></ref> | ||
==Differential Diagnosis== | ==Differential Diagnosis== | ||
{{Congenital heart disease DDX}} | {{Congenital heart disease DDX}} | ||
== | ==Diagnosis== | ||
*Echo | |||
*CXR: shows the classic “boot-shaped” heart | |||
==Management== | |||
*Acute Presentation (Tet spell): | *Acute Presentation (Tet spell): | ||
===Knee-to-Chest Position=== | ===Knee-to-Chest Position=== | ||
| Line 32: | Line 31: | ||
*Increasing the SVR causes more blood to flow to the pulmonary circulation | *Increasing the SVR causes more blood to flow to the pulmonary circulation | ||
===Analgesia=== | ===Analgesia=== | ||
#Morphine 0.1-0.2Mg/kg IV or IM | #[[Morphine]] 0.1-0.2Mg/kg IV or IM | ||
#*Goal is to ideally avoid IV placement if possible | #*Goal is to ideally avoid IV placement if possible | ||
#[[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> | #[[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> | ||
| Line 43: | Line 42: | ||
*Improves RV filling | *Improves RV filling | ||
===Beta blockers=== | ===[[Beta blockers]]=== | ||
*Propranolol IV | *Propranolol IV | ||
**Will relax the spasm causing right-sided ventricular outflow obstruction. | **Will relax the spasm causing right-sided ventricular outflow obstruction. | ||
**Should be administered in consulation with cardiology and pediatric surgery. | **Should be administered in consulation with cardiology and pediatric surgery. | ||
===Prostaglandin E1=== | ===[[Prostaglandin E1]]=== | ||
*0.1 mg/kg bolus followed by infusion 0.05 to 0.1 mg/kg/min | *0.1 mg/kg bolus followed by infusion 0.05 to 0.1 mg/kg/min | ||
*Maintains the ductus | *Maintains the ductus | ||
Revision as of 12:20, 12 May 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 Features
- 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]
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
Diagnosis
- Echo
- CXR: shows the classic “boot-shaped” heart
Management
- 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
- Morphine 0.1-0.2Mg/kg IV or IM
- Goal is to ideally avoid IV placement if possible
- 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
- 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
- ↑ 1.0 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
- ↑ 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.