Tetralogy of fallot
- 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.
- RV outflow obstruction (pulmonic stenosis)
- Overriding aorta
- RV hypertrophy
- Systolic ejection murmur along the left sternal border
- Cyanosis worse during feeding and crying
- May squat to relieve symptoms: increases afterload and decreases shunt
- Acute respiratory distress (Tet Spells) due to increased right outflow tract obstruction
Congenital Heart Disease Types
- Differentiation by pulmonary vascularity on CXR
- CXR: shows the classic “boot-shaped” heart
- Acute Presentation (Tet spell):
- 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
- 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 
- Only one case report but IN administration may avoid the pain from a needle stick
- Dose: 0.2 mg/kg IV
- Increases SVR similar to knee to chest positioning
- Improves RV filling
- Propranolol IV
- Will relax the spasm causing right-sided ventricular outflow obstruction.
- Should be administered in consulation with cardiology and pediatric surgery.
- Start infusion at 0.05 mcg/kg/min IV and titrate up to 0.1 mcg/kg/min, monitoring for hypotension (and apnea)
- Maintains the ductus
- Side Effects: Hypotension, Bradycardia, Seizures and Apnea
- Cardiothoracic surgery to repair the defects early before significant pulmonary hypertension develops
- Optimal age for correction controversial, but if elective repair, 3-11 months of age may be best
- 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.
- Donofrio MT, Moon-Grady AJ, Hornberger LK, Copel JA, Sklansky MS, Abuhamad A, Cuneo BF, Huhta JC, Jonas RA, Krishnan A, Lacey S, Lee W, Michelfelder EC Sr, Rempel GR, Silverman NH, Spray TL, Strasburger JF, Tworetzky W, Rychik J. Diagnosis and treatment of fetal cardiac disease: a scientific statement from the American Heart Association. American Heart Association Adults With Congenital Heart Disease Joint Committee of the Council on Cardiovascular Disease in the Young and Council on Clinical Cardiology, Council on Cardiovascular Surgery and Anesthesia, and Council on Cardiovascular and Stroke Nursing.Circulation. 2014 May 27;129(21):2183-242. doi: 10.1161/01.cir.0000437597.44550.5d. Epub 2014 Apr 24.
- Van Arsdell GS et al. What is the Optimal Age for Repair of Tetralogy of Fallot? Circulation. 2000; 102: Iii-123-Iii-129.