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Page 2 of 17                  Saxena et al. Vessel Plus 2022;6:15  https://dx.doi.org/10.20517/2574-1209.2021.96

               INTRODUCTION
               Maternal cardiac diseases complicate 1%-4% of all pregnancies and are the leading cause of maternal
                                                                                      [1,2]
               mortality in high-income countries (HIC), constituting about 15% of all causes . Among the cardiac
               diseases, congenital heart diseases (CHDs) are now the most common form seen in four-fifths of all
                                     [1,3]
               maternal cardiac diseases . The advancements in medical and surgical treatment of CHDs have led to
                                                        [4]
               improved survival of these patients to adulthood . This increased survival coupled with significant decline
               in incidence of rheumatic heart disease (RHD) has resulted in CHD becoming the most common
               cardiovascular problem during pregnancy in HIC. Despite a relative increase in pregnancy related cardiac
               deaths over the last two decades, the proportion of CHD-related deaths has remained relatively low . On
                                                                                                     [5]
               the other hand, in low- and middle-income countries (LMIC), RHD continues to be the most common
               cardiovascular disease during pregnancy . Though the data regarding maternal cardiac diseases in LMIC
                                                  [6-8]
               are scarce, global progress reports have emphasized the increasing importance of indirect maternal deaths
               due to preexisting heart diseases . Therefore, as the incidence of RHD declines and standards of medical
                                           [9]
                                                                                   [7,8]
               care improve in these countries, maternal CHD is likely to gain more importance .
               Unlike HIC where patients with CHD have excellent access to robust surgical centers, most patients with
               CHD in LMIC have no access to surgery [10,11] . Therefore, most patients have uncorrected CHD in LMIC.
                                                                                              [7,8]
               Moreover, many patients are diagnosed to have CHD for the first time during pregnancy . This is the
               cause of high morbidity and worse outcomes in pregnant females with CHD in LMIC [6,7,12] . A recent study
               reported that the risk of maternal mortality was seven times higher in women with uncorrected CHD than
               corrected CHD (P = 0.01) .
                                     [12]

               CHDs comprise of a broad spectrum of anatomic abnormalities but have a limited spectrum of
               pathophysiologic states. As pregnancy has a marked impact on the cardiovascular system, women with
               CHD are at high risk of developing cardiovascular complications, which affect the maternal and fetal
               outcomes. In one of the largest studies on pregnant women with CHD, Schlichting et al.  reported that the
                                                                                         [13]
               odds of occurrence of an adverse maternal cardiac event during delivery were 2.4-27.6 times higher for
                                                                            [14]
               women with CHD compared with women without CHD. Ramage et al.  studied 2114 women with CHD
               and reported the risk of serious maternal morbidity during pregnancy to be 4.0% in comparison to 1.1% in
               women without CHD.


               Optimum care for these patients requires a coordinated effort by a cardio-obstetric team as they need
               careful antenatal and postnatal surveillance and an individualized plan for labor and delivery. A recent large
               multicenter study of pregnant patients with maternal cardiac disease found that almost half of serious
               complications in patients are preventable and are due to failure on the part of healthcare provider to identify
               the high-risk patients and institute timely appropriate management . Therefore, these women require
                                                                           [15]
               appropriate preconception assessment, risk stratification, and counseling. There exists a wide gap in quality
               of care between HIC and LMIC which needs to be bridged [2,3,6,7] .


               SCOPE OF THE ARTICLE
               We highlight the current understanding of the issues related to pregnancy in women with congenital heart
               disease and describe the management of the issues as per existing literature.
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