Tetralogy of Fallot and Pregnancy
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Tetralogy of Fallot is a birth defect of the heart consisting of :

1.  Pulmonic stenosis (narrowing of the pulmonary artery).
2. A ventricular septal defect (VSD). The VSD causes cyanosis (bluish discoloration of the skin due to lack of oxygen) by allowing blood to flow from the right side of the heart to the left side without passing through the lungs.
3. Malignment of the aorta so that it arises from the VSD  or the right ventricle instead of directly from the left ventricle
4. Right ventricular hypertrophy (thickening of the right heart chamber that pumps blood to the lungs).

Tetralogy of Fallot is typically an isolated sporadic malformation. If found with other abnormalities then congenital syndromes such as trisomy 18, trisomy 13, de Lange , Klippel-Feil, and  CHARGE,  should be considered. Up to 15% of patients with tetralogy of Fallot have a chromosome 22q11 deletion.

Some severely affected infants will require an operation shortly after birth to divert (shunt) blood from the aorta to one of the pulmonary arteries in order to improve oxygenation. Corrective surgery to close the VSD and enlarge the narrowed areas of the right ventricle and the pulmonary valve and arteries is usually performed at 6 months. Overall survival after operative repair is excellent.

Persons with tetralogy of Fallot generally receive endocarditis prophylaxis before dental or other surgical procedures. Long term, congestive heart failure and abnormal atrial or ventricular arrhythmias (irregular heart beats) may require treatment with medications in some patients.

Women with tetralogy of Fallot who intend to become pregnant are usually assessed for functional status by their cardiologist. In addition they usually will have had an echocardiogram to assess for residual ventricular dysfunction, valvular function, and pulmonary hypertension. Women who have had repair and have good hemodynamic status generally have successful term pregnancies. During pregnancy the mother is examined regularly for complications such as anemia, hypertension, and infection. The fetus is evaluated for congenital heart disease and may be evaluated periodically for growth restriction .  Vaginal delivery is preferred.

 In general, advanced New York Heart Association (NYHA) functional class and a prior history of heart failure are risk factors  for adverse cardiac outcomes in women with congenital heart disease. In unoperated patients hypotension and large decreases in blood volume (as occurs with cesarean delivery) will exaggerate the right to left shunt worsening cyanosis. Unoperated pregnant patients or repaired patients with significant residual right ventricular outflow tract (RVOT) obstruction, valve dysfunction, or ventricular dysfunction are at high risk for right heart failure and arrhythmias due to the increased volume load of pregnancy.


Khairy P, et al., Pregnancy outcomes in women with congenital heart disease.Circulation. 2006 ;113(4):517-24. PMID: 16449731
Veldtman GR, et al.,Outcomes of pregnancy in women with tetralogy of Fallot.J Am Coll Cardiol. 2004 ;44(1):174-80. PMID: 15234429
Meijer JM, et al., Pregnancy, fertility, and recurrence risk in corrected tetralogy of Fallot.Heart. 2005 ;91(6):801-5.
PMID: 15894783

Created 6/29/2007 Mark Curran, M.D. FACOG


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