Congenital heart disease and acquired valvular lesions in pregnancy

In Germany, about 6,000 pregnancies in women with grown-up congenital heart disease or acquired valvular lesions are expected per year. The pregnancy-related physiology is characterized by a 50% increase in plasma volume and a 25% increase in erythrocyte volume. The cardiac output increases by 40% due to 30% increase in stroke volume and 10% increase in heart rate during the first half, and 10% increase in stroke volume but 30% increase in heart rate during the second half of pregnancy. As a consequence of the decrease of systemic vascular resistance, the systolic and, even more, the diastolic blood pressures are reduced during approximately the first 20 weeks of pregnancy. Women with uncorrectable congenital heart disease, congestive heart failure (NYHA III and IV) despite optimized medical treatment after palliative surgery, or pulmonary vascular resistances > 800 dyn x s x cm(-5) should be advised against pregnancy. The presence of congestive heart failure or persistent cyanosis in the mother are the most important predictors of fetal hypoxia. Patients with pretricuspid shunts (e.g., atrial septal defect [ASD]) are at low risk of a hemodynamic deterioration or first onset of arrhythmias. In the rare case of a marked clinical deterioration, catheter-based closing of the shunt is the first-line treatment. Also, ventricular septal defects and persistent ducti arteriosi are usually well tolerated during pregnancy, as they are highly resistant to flow. In some cases, arrhythmias may occur. The prognosis is less favorable, if myocardial compromise has already been present before pregnancy. The fatal complication rate correlates closely with the degree of congestive heart failure. In aortic coarctation, development of severe hypertension, myocardial decompensation, aortic dissection, and cerebral hemorrhage have been reported in 2.3% of cases. To prevent aortic dissection and rupture of cerebral vascular aneurysms, patients should be advised to reduce their physical activity and have their blood pressure controlled closely. If, during pregnancy, a therapeutic intervention is unavoidable, stent placement is the therapy of choice. The maternal complication rate is low in pulmonary artery stenosis. Hemodynamically significant stenoses should be treated before pregnancy. In the rare case of progressive right heart failure or cyanosis during pregnancy, balloon valvotomy is the first-line therapeutic option. Women with incomplete correction of a tetralogy of Fallot