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Tetralogy of Fallot (Newborn Presentation)

A cyanotic congenital heart disease consisting of ventricular septal defect, pulmonary stenosis, overriding aorta and right ventricular hypertrophy.

Written by: Saygı Hospital Health Guide Editorial Board
Last updated:

This content has been compiled by the Saygı Hospital Health Guide Editorial Board and is periodically reviewed by a specialist physician.

References (5)

This content is for informational purposes only and does not constitute medical advice. You can book an appointment at our Çocuk Sağlığı ve Hastalıkları department. Book Appointment →

What is Tetralogy of Fallot (Newborn Presentation)?

Tetralogy of Fallot is formed by the combination of four lesions: large ventricular septal defect, right ventricular outflow tract obstruction (pulmonary stenosis), overriding of the aorta over the septum, and right ventricular hypertrophy. Embryologically it results from abnormal displacement of the conotruncal septum.

In the newborn period the degree of pulmonary stenosis determines the clinical picture. In severe stenosis, cyanosis is prominent at birth and duct-dependent pulmonary circulation may be seen. In mild-moderate stenosis the baby can be pink in the newborn period and 'tet spells' appear over time.

Diagnosis is made by history, physical examination (squatting position, ejection murmur), echocardiography and, when necessary, cardiac CT/MRI. Surgical repair is generally planned as complete correction between 4-12 months. In severe cases, palliative shunt or corrective surgery in the newborn period is considered.

Symptoms

Cyanosis (prominent during crying, feeding and tet spells)
Left parasternal ejection murmur
Feeding difficulty
Growth retardation
Squatting position
Tet spell (paroxysmal hyperpnea attacks)
Digital clubbing

Risk Factors

22q11.2 deletion syndrome (DiGeorge)
Down syndrome
Maternal diabetes
Untreated maternal PKU
Maternal retinoid
Family history of congenital heart disease

When to See a Doctor?

If you experience any of the following symptoms, seek medical attention promptly:

  • When cyanosis is detected at birth or in early infancy
  • When a murmur is detected
  • In the development of tet spells (hyperpnea, cyanosis attacks)
  • When cyanosis occurs during feeding
  • When pulse oximetry is low

Treatment Methods

01
Definitive diagnosis by echocardiography
02
Oxygen, knee-chest position, morphine and phenylephrine during tet spell
03
Propranolol prophylaxis
04
Prostaglandin E1 infusion in severe cases
05
Palliative B-T shunt (severe newborn)
06
Complete corrective surgery (4-12 months)

Which Department to Visit?

You can visit our Çocuk Sağlığı ve Hastalıkları department for these complaints. Our specialist physicians will create the most suitable treatment plan for you.

Learn About Çocuk Sağlığı ve Hastalıkları Department

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Health Disclaimer: The information on this page is prepared for general informational purposes only. It does not replace medical diagnosis and treatment. Please consult your physician for your complaints. Saygı Hospital does not accept responsibility for actions taken based on the information on this page.