Cardiac disease in pregnancy and repaired congenital heart disease


 * Associate Editor-In-Chief: ; Assistant Editor-In Chief: Ara Tachjian, MD [mailto:ara_tt@yahoo.com]

Overview
Most of the patients who undergo surgery for congenital heart disease repair will still have some residual of the defect. Therefore, these should be carefully evaluated prior to pregnancy planning.

Tetralogy of Fallot
Individuals with TOF usually present very early in life with cyanosis making it necessary to undergo surgical repair. The repair is is almost always done during infancy, particularly in developed countries. During the surgery, the pulmonary valve and right ventricular outflow tract are both surgically dilated. This usually leads to pulmonary valve regurgitation as well as arrhythmias originating from the right side of the heart. Therefore, subsequent right sided heart failure is sometimes observed. 20 years following repair of Tetralogy of Fallot, 10-15% of patients develop late complications, the most important of which is right sided heart failure.

Some TOF patients will have a palliative surgically-created systemic-pulmonary shunt such as Blalock-Taussig or Waterston, but without corrective surgery. In such situations, pregnancy might worsen an already existing right-to-left shunt (through a VSD) by reducing the systemic vascular resistance, leading to worsening cyanosis. In previous studies, maternal NYHA functional class >2 and cyanosis are identified as independent predictors of both maternal and fetal complications. Therefore, worsening cyanosis is not favored in such patients and pregnancy should be avoided.

Elements to be evaluated for through history, physical exam, EKG and echocardiogram prior to pregnancy (in women with surgical correction): 1. Residual pulmonary regurgitation and severity 2. Residual right ventricle outflow obstruction 3. Right sided heart failure 4. Tricuspid regurgitation 5. Atrial or Venctricular arrhythmias 6. VSD

Generally, it is accepted that most women with corrected TOF can have non-restricted lifestyle and tolerate pregnancy. However, they still need to be evaluated by a cardiologist prior to and during pregnancy. Women with severe pulmonary regurgitation as a complication of the corrective surgery might benefit from pulmonary valve replacement prior to pregnancy to reduce possibility of complications.