The following are key points to remember about this American Heart Association (AHA) Scientific Statement on Management of Pregnancy in Patients With Complex Congenital Heart Disease (CHD):

1. The preconception evaluation of a patient with complex congenital heart disease (CHD) should include a thorough review of medical records that include information on the primary defect, surgical history including both palliative and reparative procedures, and the presence of comorbidities and any residua or sequelae associated with the specific cardiac lesions or surgery.
2. The initial diagnostic evaluation includes measurement of arterial oxygen saturation, electrocardiogram, and echocardiogram.
3. A three-generation family history, including any consanguinity, should be completed. Genetic evaluation should be made available to all women with CHD to determine recurrence, particularly in those patients with a family history of CHD and those with possible autosomal dominant lesions (e.g., 22q 11 deletion).
4. Patients considered low risk without specific clinical issues may receive prenatal care locally. However, evaluation should be performed early in pregnancy at a regional adult CHD center to establish a regimen for prenatal management, and then repeated in the third trimester to evaluate current maternal status and candidacy for local delivery. High-risk patients should be managed exclusively at a regional adult CHD center for prenatal care and delivery.
5. For patients considered exceedingly high risk for maternal morbidity or mortality such as those with elevated pulmonary pressures or important aortic root enlargement, therapeutic termination of the pregnancy should be offered.
6. Cardiac monitoring (direct vs. telemonitoring) is recommended during active labor and delivery for women with a history of preconception arrhythmias or those who have developed arrhythmias during pregnancy.
7. All women with mechanical valves should undergo prepregnancy counseling by a cardiologist with expertise in valvular heart disease and pregnancy in accordance with the American College of Cardiology/AHA guidelines. The risks to both mother and baby of embarking on a pregnancy should be reviewed in detail, as well as the risks and benefits of each anticoagulation strategy.
8. Preconception counseling is important for women at risk of developing heart failure during pregnancy. Pregnancy is contraindicated in women with severe systemic ventricular dysfunction (left ventricular ejection fraction <30%, New York Heart Association class III or IV).
9. Prepregnancy counseling should be provided by a cardiologist with expertise in the management of patients with cyanosis. Because of the increased risk associated with cyanosis to the mother and high risk of fetal loss, pregnancy termination should be considered.
10. Delivery of patients with cyanotic CHD should be managed by a multidisciplinary team in a tertiary care center.

Keywords: Arrhythmias, Cardiac, Consanguinity, Cyanosis, Echocardiography, Electrocardiography, Genetic Testing, Heart Defects, Congenital, Heart Failure, Heart Valve Diseases, Infant, Pregnancy, Pregnancy Trimester, Third, Primary Prevention, Risk Assessment, Stroke Volume, Tertiary Care Centers, Ventricular Dysfunction

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