CONGENITAL HEART DISEASE

FOCUS: Pregnancy

Pregnancy in Women with Congenital Heart Defects

Andrej Robida, MD, FACC* An increasing number of children with congenital heart disease survive into adulthood, either because of natural selection or due to operation. They usually have mild forms of common malformations such as atrial septal defect of secundum type, small ductus arteriosus, mild to moderate pulmonary or aortic valve stenosis, coarctation of the aorta, and tetralogy of Fallot. The remarkable improvement in the surgical palliative treatment of complex congenital heart disease such as Mustard/Senning operation for transposition of the great arteries, and Fontan surgery for malformations with functional one ventricle, has led to the growing number of such patients reaching adulthood. For the young women with complex congenital heart disease, pregnancy represents a major risk for the mother and the fetus. The major risk for the mother is death, embolic events, and infective endocarditis. The fetus is exposed to immediate risk of death and remote risk of congenital cardiac malformation due to genetic transmission or teratogenic effect of cardiac drugs (1). Physiologic changes during pregnancy increase the demand on the already compromised cardiovascular system of a pregnant woman with a congenital heart defect (table 1). Further variations occur during labor and in the postpartum period. The right and left ventricles eject an increased stroke volume against a decreased pulmonary and vascular resistance. These physiologic changes influence maternal and fetal outcome in women with congenital heart defects. In the group of acyanotic congenital heart defects, the risk for the mother is small if her functional class is favorable and the ventricular function is preserved. However, adverse effects on the fetus include a high incidence of miscarriage, smaller infant size, and the recurrence risk of congenital heart disease is higher than in the normal population (3). Cyanotic congenital heart disease, excluding Eisenmenger syndrome, represents a low mortality risk for mothers, but there is a high risk of miscarriage, as well as a high incidence of prematurity, and low birth weight infants. In a study of 96 pregnancies in 44 women with cyanotic congenital heart defects, cardiovascular complications occurred in 32% with 1 death from endocarditis. Only 43% of fetuses were born alive and 37% were premature. Mean weight of full-term infants was 2575 grams. The strongest predictors for fetal outcome were hemoglobin level and arterial oxygen saturation (4). Eisenmenger syndrome is the most serious congenital heart disease during pregnancy, with poor prognosis for the mother and the fetus. Maternal death can exceed 50% and may occur during pregnancy, labor, delivery or the puerperium period. Death is mainly due to thrombembolism and excessive blood loss (5). The prognosis has not improved over the last two decades for the mother with Eisenmenger

Table 1. Cardiovascular changes during pregnancy

Heart rate Increase around 10 beats/ minute Increase 30 to 50% Increase 40 to 50% Decrease

Maternal and Fetal Risks

Functional class correlates directly with maternal and fetal outcome (2). Relatively simple heart defects and less severe heart dysfunction have better prognosis (table2).
Consultant Pediatric Cardiologist, Pediatric Cardiology Section Department of Cardiology and Cardiovascular Surgery, Hamad Medical Corporation, P.O. Box 3050, Doha, Qatar. E-mail:ARobida@excite.com

Cardiac output Blood cell volume Systemic vascular resistance Pulmonary vascular resistance

Decrease

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Pregnancy in Women with Congenital Heart Defects

Table 2. Mortality risk for mothers with congenital heart defects

Mortality <1% Smal atrial septal defects Small ventricular septal defects Small ductus arteriosus Mild pulmonary/aortic stenosis Operated tetralogy of Fallot NYHA class I and II Mortality 5% to15% Moderate to severe aortic stenosis Coarctation of the aorta Mortality 25% to 50% Eisenmenger syndrome

Marfan syndrome with the aortic involvement

Tetralogy of Fallot Marfan syndrome with normal aorta NYHA class III and IV

Abbreviation: NYHA = New York Heart Association syndrome and for the fetus. In 73 women with Eisenmenger syndrome, death occurred in 36%. Premature births were high (53%) and there was a 13% death rate among fetuses and neonates (6). A scoring system of these predictors to estimate the probability of cardiac complication in the mother was used. The rate of cardiac complications in a mother with a score of 0, 1, >1 was 3%, 30%, and 66%, respectively. Neonatal complications occurred in 42 (17%) completed pregnancies. The complications were death, respiratory distress syndrome, intraventricular hemorrhage, premature birth, and small for gestational age birth weight (7).

Risk Assessment
To improve the prediction of adverse effect of pregnancy in mothers with congenital or acquired heart disease the outcome of 221 women with 252 completed pregnancies were examined. The following predictors of cardiac events were found: poor maternal functional class or cyanosis myocardial dysfunction left heart obstruction prior arrhythmia prior cardiac events

CONCLUSION
Pregnancy in women with congenital heart disease, particularly with Eisenmenger syndrome, represents a major risk for the mother and the fetus. The number of women with complex congenital heart disease is small, hence, a prospective multicenter design is needed to improve prenatal care as well as preconception counseling.

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