LAUSR

Cardiovascular disease (CVD) complicating pregnancy is an increasingly frequent phenomenon with regional differences in numbers and causes and substantial changes over the last few decades. Between 0.2% to 4% of pregnancies are affected in countries with a developed health care system. Cardiac disease in the mother is the most important cause of maternal mortality and there have been no signs of decline in its incidence over the past few decades. On the contrary, there has been an increase, which is well documented for the United Kingdom and the United States. Aortic dissection, peripartum cardiomyopathy, and ischemic heart disease are rare but important causes of maternal mortality. The prevalence of ischemic heart disease has increased due to life style changes with more women with obesity, hypertension, and diabetes. In addition, the tendency to older age at first pregnancy, including the progressive rise in in vitro fertilization and multiple pregnancies, has increased the prevalence of CVD. One of the other major causes of CVD complicating pregnancy is the dramatic increase in patients with congenital heart disease (CHD) surviving to adulthood. In the 1950s only around 10% survived the first decades of life whereas today up to 85% to 90% reach adulthood. These women not only survive but, due to the tremendous advances in the diagnosis and treatment of even complex CHD, their quality of life has improved, enabling them to lead a normal life, including sexual activity and the desire for children. A studyconducted in the United States showed that, from 2000 to 2010, there was a linear increase in the prevalence of CHD from 6.4 to 9.0 per 10 000 of delivery hospitalizations and, furthermore, a mortality rate of 17.8 compared with 0.7/10 000 delivery hospitalizations in mothers without CHD. Like other investigations, that study showed the predominance of patients with an underlying left-to-right shunt lesion (atrial septal defect in 22.6%, ventricular septal defect in 14.5%) followed by valvular lesions (left-sided in 13.8%, right-sided in 7.5%). More complex lesions such as tetralogy of Fallot (2.6%) or transposition of the great arteries (1.5%) were seen only rarely. While many women with known CVD, whether treated or not, may be in a stable condition, pregnancy, with its significant hemodynamic changes, puts them at risk for deterioration. Stroke volume, heart rate, and cardiac output increase, whereas peripheral vascular resistance and hemoglobin decrease. All changes occur slowly, with a peak effect in the late second and early third trimester, normalizing only weeks after delivery. In addition to the hemodynamic effects, pregnancy has a thrombogenic effect and may affect connective tissue disease leading to a higher risk of aortic dissection during pregnancy. Therefore, pregnancy planning and contraceptive advice are essential for women with cardiac disease. Prior to pregnancy, all women with known CVD should be seen and counselled by well-trained specialists in a center with expertise. An experienced multidisciplinary team should be available to provide care before, during, and after pregnancy. Women with preexisting CVD are known to be at higher risk of developing cardiac complications in pregnancy, which are strongly influenced by the kind of heart defect and residual lesions. Efforts have been made in the field of risk assessment and stratification. The modified World Health Organization (mWHO) classification seems to be the most accurate measure in predicting these risks. It divides patients based on their underlying diagnosis in 4 groups from very low risk (mWHO I) to those in whom pregnancy poses a life-threatening risk and is therefore contraindicated (mWHO IV). The low-risk group (mWHO I) is composed of all patients with minor lesions not requiring treatment and those that have been treated without hemodynamically significant residuals. Pregnancy is contraindicated (mWHO IV) in women with pulmonary hypertension, severe cyanosis, diminished left ventricular function, previous peripartum cardiomyopathy with incomplete recovery, symptomatic left ventricular outflow tract obstruction, and Marfan syndrome with aortic root dilation. For a better understanding and management of women with CVD, guidelines have been developed covering most of the relevant issues. The guidelines are based on the available literature, which consists mainly of reports from single centers in different parts of the world and using distinct methods. They often focus on 1 lesion only and describe small groups of patients. Therefore, it used to be difficult to obtain reliable and relevant information. To gather up-to-date information about treatment during pregnancy and pregnancy outcomes, a large prospective observational registry has been initiated by the European Society of Cardiology and Association for European Paediatric and Congenital Cardiology: the Registry of Pregnancy and Cardiac Disease (ROPAC), which is web-based and open for inclusion to all Rev Esp Cardiol. 2017;70(2):78–80