eMedicine Specialties > Emergency Medicine > Cardiovascular

Tetralogy of Fallot: Treatment & Medication

Author: Mark Spektor, DO, Medical Director, Department of Emergency Medicine, Maimonides Medical Center
Coauthor(s): David A Donson, MD, Assistant Medical Director, Department of Emergency Medicine, Maimonides Medical Center; Kurt Pflieger, MD, FAAP, Active Staff, Department of Pediatrics, Lake Pointe Medical Center
Contributor Information and Disclosures

Updated: Dec 22, 2008

Treatment

Prehospital Care

  • Any infant with cyanosis and/or respiratory distress requires oxygen.
  • Blow-by O2 (BBO2) is the least objectionable. Use the open-end of a cannula or tube.
  • Permit the baby to remain with the mother or father.
  • Do not provoke the infant by attempting to start an intravenous (IV) line, especially if not skilled in pediatric IV placement.
  • An intraosseous insertion could be an immediate lifesaving tool.

Emergency Department Care

The emergency physician should be able to recognize and treat a hypercyanotic episode (tet spell) as one of the very few pediatric cardiology emergencies that may present to the ED.

  • Hypoxic tet spell: Hypercyanotic episodes are characterized by paroxysms of hyperpnea, prolonged crying, intense cyanosis, and decreased intensity of the murmur of pulmonic stenosis.
    • Mechanism - Secondary to infundibular spasm and/or decreased SVR with increased right-to-left shunting at the VSD, resulting in diminished pulmonary blood flow
    • If left untreated, may result in syncope, seizure, stroke, or death
  • Treatment for the acute setting of hypercyanosis includes the following:
    • Knee-chest position: Place the baby on the mother's shoulder with the knees tucked up underneath. This provides a calming effect, reduces systemic venous return, and increases SVR.
    • Oxygen is of limited value since the primary abnormality is reduced pulmonary blood flow.
    • Morphine sulfate, 0.1-0.2 mg/kg IM/SC, may reduce the ventilatory drive and decrease systemic venous return.
    • Phenylephrine, 0.02 mg/kg IV, is used to increase SVR.
    • Dexmedetomidine infusion, 0.2 mcg/kg/min, has recently shown promise in ameliorating symptoms in hypercyanotic neonates.1
    • Treating acidosis with sodium bicarbonate may reduce the respiratory center stimulating effect of acidosis.
    • General anesthesia is a last resort.

Consultations

Consult a pediatric cardiologist/surgeon.

Medication

The goals of therapy are to reduce the ventilatory drive, decrease systemic venous return, and increase peripheral venous return.

Analgesics

These agents reduce ventilatory drive. Pain control ensures patient comfort and promotes pulmonary toilet. Most analgesics have sedating properties, which are beneficial for patients who are having hypercyanotic episodes.


Morphine sulfate (Duramorph, Astramorph, MS Contin)

DOC for narcotic analgesia because of its reliable and predictable effects, safety profile, and ease of reversibility with naloxone.
Administered IV, may be dosed in number of ways and commonly titrated until desired effect obtained.

Adult

Pediatric

0.05-0.2 mg/kg dose IV prn; not to exceed 15 mg/dose

Phenothiazines may antagonize analgesic effects; TCAs, MAOIs, and other CNS depressants may potentiate adverse effects

Documented hypersensitivity; hypotension; potentially compromised airway with uncertain rapid airway control; respiratory depression; nausea; emesis; constipation; urinary retention

Pregnancy

B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals

Precautions

Avoid in hypotension, respiratory depression, nausea, emesis, constipation, and urinary retention; caution in atrial flutter and other supraventricular tachycardias; has vagolytic action and may increase ventricular response rate

Alpha-adrenergic Agonist

These agents improve hemodynamic status by improving myocardial contractility and increasing heart rate, resulting in increased cardiac output. Peripheral resistance is increased by vasoconstriction. Increased cardiac output and increased peripheral resistance increase blood pressure.


Phenylephrine (Neo-Synephrine)

Strong postsynaptic alpha-receptor stimulant with little beta-adrenergic activity; produces vasoconstriction of arterioles, increasing peripheral venous return.

Adult

Pediatric

5-20 mcg/kg/dose IV bolus q10-15min prn
0.1 mg/dose IV/SC q1-2h prn
0.1-0.5 mcg/kg/min IV infusion

Bretylium may potentiate action on adrenergic receptors, possibly resulting in arrhythmias; MAOIs may significantly enhance adrenergic effects, and pressor response may be increased 2- to 3-fold; guanethidine may increase pressor response of direct-acting vasopressors, possibly resulting in severe hypertension

Documented hypersensitivity; severe hypertension; ventricular tachycardia

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

Caution in elderly patients, hyperthyroidism, myocardial disease, bradycardia, partial heart block or severe arteriosclerosis; in hypovolemia, not substitute for replacement of blood, fluids and electrolytes, and plasma (these should be restored promptly when loss has occurred)

More on Tetralogy of Fallot

Overview: Tetralogy of Fallot
Differential Diagnoses & Workup: Tetralogy of Fallot
Treatment & Medication: Tetralogy of Fallot
Follow-up: Tetralogy of Fallot
Multimedia: Tetralogy of Fallot
References
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References

  1. Senzaki H, Ishido H, Iwamoto Y, et al. Sedation of hypercyanotic spells in a neonate with tetralogy of Fallot using dexmedetomidine. J Pediatr (Rio J). Jul-Aug 2008;84(4):377-80. [Medline].

  2. Kantorova A, Zbieranek K, Sauer H, et al. Primary early correction of tetralogy of Fallot irrespective of age. Cardiol Young. Apr 2008;18(2):153-7. [Medline].

  3. Miatton M, De Wolf D, François K, et al. Intellectual, neuropsychological, and behavioral functioning in children with tetralogy of Fallot. J Thorac Cardiovasc Surg. Feb 2007;133(2):449-55. [Medline].

  4. Aboulhosn J, Child JS. Management after childhood repair of tetralogy of fallot. Curr Treat Options Cardiovasc Med. Dec 2006;8(6):474-83. [Medline].

  5. Anderson RH, Weinberg PM. The clinical anatomy of tetralogy of fallot. Cardiol Young. Feb 2005;15 Suppl 1:38-47. [Medline].

  6. Balaji S. Medical therapy for sudden death. Pediatr Clin North Am. Oct 2004;51(5):1379-87. [Medline].

  7. Goldmuntz E. The genetic contribution to congenital heart disease. Pediatr Clin North Am. Dec 2004;51(6):1721-37, x. [Medline].

  8. He GW. Current Strategy of Repair of Tetralogy of Fallot in Children and Adults: Emphasis on a New Technique to Create a Monocusp-Patch for Reconstruction of the Right Ventricular Outflow Tract. J Card Surg. Sep 5 2008;[Medline].

  9. Hövels-Gürich HH, Konrad K, Skorzenski D, et al. Long-term behavior and quality of life after corrective cardiac surgery in infancy for tetralogy of Fallot or ventricular septal defect. Pediatr Cardiol. Sep-Oct 2007;28(5):346-54. [Medline].

  10. Khairy P, Harris L, Landzberg MJ, et al. Implantable cardioverter-defibrillators in tetralogy of Fallot. Circulation. Jan 22 2008;117(3):363-70. [Medline].

  11. Kirklin JW, Barrett-Boyes BGF. Ventricular septal defect and pulmonary stenosis or atresia. Cardiac Surgery. 1993;2:861-1012.

  12. Nadas AS. Tetralogy of fallot. In: Nadas Pediatric Cardiology. Hanley & Belfus; 1992:471-93.

  13. Park MK. Tetralogy of fallot. In: Pediatric Cardiology for Practitioners. 3rd ed. Mosby-Year Book; 1996:168-75.

  14. Silverman NH. Tetralogy of fallot and related lesions. In: Pediatric Echocardiography. Lippincott Williams & Wilkins; 1992:195-214.

  15. Snider AR, Serwer GA. Defects in cardiac septation. In: Echocardiography in Pediatric Heart Disease. Mosby-Year Book; 1990:150-3.

  16. Tanel RE. ECGs in the ED. Pediatr Emerg Care. Jun 2007;23(6):428-9. [Medline].

  17. Woods WA, Schutte DA, McCulloch MA. Care of children who have had surgery for congenital heart disease. Am J Emerg Med. Jul 2003;21(4):318-27. [Medline].

  18. Zuberhuler JH. Tetralogy of Fallot. In: Heart Disease in Infants, Children and Adolescents. 2nd ed. 1995:998-1026.

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Further Reading

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Keywords

tetralogy of Fallot, TOF, Fallot tetrad, Fallot's tetrad, right ventricular outflow tract obstruction, congenital heart disease, maldevelopment of right ventricular infundibulum, subaortic ventricular septal defect, right ventricular infundibular stenosis, aortic valve positioned to override the right ventricle, right ventricular hypertrophy, right-to-leftshunting, cyanosis, hypertrophy of the infundibular septum, dyspnea, retarded growth, aortic ejection click, systolic thrill, systolic ejection murmur, clubbing, scoliosis, squatting position, retinal engorgement, hemoptysis, conotruncal abnormalities, DiGeorge syndrome, branchial arch abnormalities, fetal hydantoin syndrome, fetal carbamazepine syndrome, fetal alcohol syndrome, maternal phenylketonuria birth defects

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Contributor Information and Disclosures

Author

Mark Spektor, DO, Medical Director, Department of Emergency Medicine, Maimonides Medical Center
Mark Spektor, DO is a member of the following medical societies: American College of Emergency Physicians, American College of Physician Executives, and Society for Academic Emergency Medicine
Disclosure: Nothing to disclose.

Coauthor(s)

David A Donson, MD, Assistant Medical Director, Department of Emergency Medicine, Maimonides Medical Center
David A Donson, MD is a member of the following medical societies: American College of Emergency Physicians and Society for Academic Emergency Medicine
Disclosure: Nothing to disclose.

Kurt Pflieger, MD, FAAP, Active Staff, Department of Pediatrics, Lake Pointe Medical Center
Kurt Pflieger, MD, FAAP is a member of the following medical societies: American Academy of Pediatrics, American College of Emergency Physicians, American Heart Association, and Texas Medical Association
Disclosure: Nothing to disclose.

Medical Editor

Theodore J Gaeta, DO, MPH, FACEP, Clinical Associate Professor, Department of Emergency Medicine, Joan and Sanford Weill Medical College at Cornell University; Vice Chairman and Program Director of Emergency Medicine Residency Program, Department of Emergency Medicine, New York Methodist Hospital; Academic Chair, Adjunct Professor, Department of Emergency Medicine, St George's University School of Medicine
Theodore J Gaeta, DO, MPH, FACEP is a member of the following medical societies: American College of Emergency Physicians, Council of Emergency Medicine Residency Directors, New York Academy of Medicine, New York Academy of Medicine, and Society for Academic Emergency Medicine
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

Gary Setnik, MD, Chair, Department of Emergency Medicine, Mount Auburn Hospital; Assistant Professor, Division of Emergency Medicine, Harvard Medical School
Gary Setnik, MD is a member of the following medical societies: American College of Emergency Physicians and National Association of EMS Physicians
Disclosure: Intellicare Salary Management position; South Middlesex EMS Consortium Salary Management position

CME Editor

John D Halamka, MD, MS, Associate Professor of Medicine, Harvard Medical School, Beth Israel Deaconess Medical Center; Chief Information Officer, CareGroup Healthcare System and Harvard Medical School; Attending Physician, Division of Emergency Medicine, Beth Israel Deaconess Medical Center
John D Halamka, MD, MS is a member of the following medical societies: American College of Emergency Physicians, American Medical Informatics Association, Phi Beta Kappa, and Society for Academic Emergency Medicine
Disclosure: Nothing to disclose.

Chief Editor

David FM Brown, MD, Assistant Professor, Department of Medicine, Division of Emergency Medicine, Harvard Medical School; Associate-Chief, Attending Physician, Department of Emergency Medicine, Massachusetts General Hospital
David FM Brown, MD is a member of the following medical societies: American College of Emergency Physicians and Society for Academic Emergency Medicine
Disclosure: Schering  Honoraria Speaking and teaching

 
 
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