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General principles to discuss with the patient include preconceptual counseling, pregnancy risk assessment, genetic risks, environmental risks, and pregnancy management. For some patients, it may also include a discussion regarding contraception, termination of a pregnancy, and a conversation about not only the delivery but what will happen post-pregnancy (including issues such as an eventual need for heart surgery or transplantation).

The Cardiac Disease in Pregnancy Investigation (CARPREG I) scoring system for risk from cardiac events for women with heart disease noted four major risk factors: (1) NYHA FC III or IV heart failure, (2) prior cardiac events, (3) mitral or aortic obstruction, and (4) LVEF less than 40%. One point is assigned to each. Patients with no points had a 5% risk, those with 1 point had a complication rate of 27%, while for those with 2 or more points, the risk was 74%. Other reviews have suggested that the major risk for adverse outcomes or death to either the mother or fetus include pulmonary hypertension (with pulmonary pressure greater than three-quarters of systemic pressure), maternal cyanosis, systemic ventricular dysfunction, poor maternal functional class, severe left-sided valvular obstruction, aortic coarctation, significantly dilated aortic root, significant unrepaired heart defects, and warfarin therapy in patients with mechanical valves. In 2018, this group reported the results from a follow-up study (CARPREG II). Cardiac complications occurred in 16% of pregnancies and were primarily related to arrhythmias and heart failure. Although the overall rates of cardiac complications during pregnancy did not change over the years, the frequency of pulmonary edema decreased (8% from 1994 to 2001 vs. 4% from 2001 to 2014). Ten predictors of maternal cardiac complications were identified: five general predictors (prior cardiac events or arrhythmias, poor functional class or cyanosis, high-risk valve disease/LV outflow tract obstruction, systemic ventricular dysfunction, no prior cardiac interventions); four lesion-specific predictors (mechanical valves, high-risk aortopathies, pulmonary hypertension, CAD); and one delivery of care predictor (late pregnancy assessment). These 10 predictors were incorporated into a new risk index (CARPREG II) shown in Figure 10–10.

Figure 10–10.

Risk index for material cardiac complications in pregnancy (CARPREG II). The risk index is divided into five categories based on the sum of the points for a given pregnancy: 0 to 1 point; 2 points; 3 points; 4 points; and > 4 points. The predicted risks for primary cardiac events stratified according to point score were 0 to 1 point (5%), 2 points (10%), 3 points (15%), 4 points (22%), and > 4 points (41%). (Modified, with permission, from Silversides CK et al. Pregnancy outcomes in women with heart disease: the CARPREG II Study. J Am Coll Cardiol. 2018 May 29;71(21):2419–30. Copyright © 2018 by the American College of Cardiology Foundation. Published by Elsevier.)

In 2011, the World Health Organization outlined guidelines for the management of pregnancy in patients with congenital heart disease. This guideline also outlines risks to the fetus. ...

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