Background. Pregnancy is a crucial moment in women’s life, characterized by changes in the cardiovascular system, that may translate into risks for patients with congenital or acquired heart disease. Methods. In this retrospective observational study, we evaluated the clinical-instrumental trends of heart disease patients referred to a single Centre of Pediatric Cardiology from 1970 to 2024 during pregnancy. Baseline characteristics, maternal gestational complications, obstetrical and fetal complications were studied. Then, the population was divided into three groups based on the modified World Health Organization classification, and the above characteristics and complications were analyzed within each study group. Results. A total of 287 pregnancies were examined from 139 patients with congenital or acquired heart disease, most of whom were already aware of the condition before gestation. More gestations were carried by patients with mild heart diseases. The rates of clinical-instrumental worsening of maternal heart disease during pregnancy correlate positively with its severity: from 8% worsening in the mild heart disease group to 32% in the moderate group to 46% in the severe group. Similarly, severe heart disease required more often introduction or enhancement of cardiology therapy during gestation. Further features that correlate positively with the severity of maternal heart disease are: the need for postpartum hospitalization of the mother in intensive care, neonatal prematurity, low neonatal birth weight, indication for delivery by cesarean section and abortions. However, the incidence of obstetric complications is similar among the study populations. The recurrence of heart disease in the newborn does not correlate with the severity of maternal cardiopathy. Conclusions. Today, with the evolution of cardiac surgery and increasing migration flows, more patients with congenital or acquired heart disease are facing pregnancy. Knowledge of cardiovascular adaptation in pregnancy and during childbirth and the pathophysiology of heart disease are fundamental to treat such patients. Adequate preconception counseling, maternal risk stratification, and the establishment of a multidisciplinary medical pregnancy team are crucial in their management.