Abstract Background Individuals with congenital heart disease (CHD) are at increased risk of cardiovascular complications. There is limited available data on burden of acute cardiovascular complications in pregnant women with CHD. Purpose To study the acute cardiovascular complications in pregnant women with CHD. Methods We conducted a retrospective observational analysis using the Nationwide Inpatient Sample (NIS) database. Using ICD-9 and ICD-10-CM codes, we identified pregnant women aged 18 years or older with and without CHD hospitalized between January 1st, 2010, and December 31st, 2019. The outcomes of the study were peripartum cardiomyopathy, hypertensive disorders of pregnancy (gestational hypertension, pre-eclampsia, and eclampsia), acute myocardial infarction, cardiac arrhythmias, cardiac arrest, stroke or transient ischemia, and in hospital mortality. Results During the study period, there were a total of 41,114,105 hospitalizations for pregnancy. Of these, 43,131 (0.1%) had CHD. The mean age of the population was 28 years in both CHD and non-CHD groups. The majority were white patients, followed by black patients and then Asians in both groups. There was a higher proportion of white patients in the CHD group compared to the non-CHD group. Comorbidities including diabetes mellitus, chronic pulmonary disease, chronic kidney disease, hyperlipidemia, smoking, depression, and drug abuse were higher in the CHD group compared to the non-CHD group (Table 1). Pregnant women with CHD had a significantly higher incidence of peripartum cardiomyopathy (1905 vs 89 per 100,000 pregnancy hospitalizations; p<0.001), hypertensive disorders of pregnancy (9332 vs 7802 per 100,000 pregnancy hospitalizations ; p<0.001), acute coronary syndrome (253 vs 12 per 100,000 pregnancy hospitalizations; p<0.001), cardiac arrhythmias (3480 vs 176 per 100,000 pregnancy hospitalizations; p<0.001), cardiac arrest (127 vs 6 per 100,000 pregnancy hospitalizations; p<0.001), stroke or transient ischemic attack (1648 vs 32 per 100,000 pregnancy hospitalizations; p<0.001), and venous thromboembolism (816 vs 124 per 100,000 pregnancy hospitalizations; p<0.001) (Table 2). Similarly, pregnant women with CHD had significantly higher in hospital mortality compared to pregnant women with no CHD (116 vs 11 per 100,000 pregnancy hospitalizations; p<0.001). Conclusion Pregnant women with CHD are at significantly increased risk of acute cardiovascular complications compared to those without CHD. Our study underscores the importance of close monitoring and specialized care for this high-risk population during pregnancy and delivery. Further research is warranted to better understand the risks associated with these complications and to optimize management strategies for pregnant women with CHD.Baseline CharacteristicsOutcomes