Congenital heart disease in the ESC EORP Registry of Pregnancy and Cardiac disease (ROPAC)

Abstract

Pregnancy is a major burden on the maternal cardiovascular system. Hormonal changes cause a drop in systemic vascular resistance and a compensatory volume expansion, leading to a 30–50% increase in cardiac output. Cardiac output peaks at the end of the second trimester and remains on a plateau thereafter, until delivery. During labour, pain, stress and the uterine contractions increase cardiac output by another 25%. These changes, with the abrupt cessation of the increased utero-placental blood flow at delivery with the return of 500–700 mls into the systemic circulation, make the postpartum period high risk for the development of heart failure. Additionally, the risk of arrhythmias, thrombosis and aortic dissection is increased during and shortly after pregnancy [3]. The impact of pregnancy on the cardiovascular system may explain why cardiac disease is the leading cause of maternal mortality in high income countries and why women with pre-existing cardiac disease, including congenital heart disease (CHD), are at particular risk.<br/>For women with cardiac disease, hard data are very limited and the clinical management of pregnancy often remains based on expert opinion derived from clinical experience. The inherent difficulty in performing randomized controlled trials in pregnant women further compounds the situation. However, advances in medical and surgical treatment have increased the number of adult CHD patients, and consequently the number of pregnant CHD patients. CHD is now the most common form of heart disease seen in pregnancy. Advances in care mean that even women with complex CHD, such as a Fontan circulation, are now becoming pregnant and the need for data upon which to base clinical management is becoming urgent.<br/>In an attempt to define optimal care for women with heart disease the ESC EORP Registry Of Pregnancy And Cardiac disease (ROPAC), which is a prospective, observational worldwide registry, was initiated in 2007. Patients with structural heart disease, congenital heart disease (CHD), valvular heart disease (VHD), cardiomyopathy (CMP), ischemic heart disease (IHD), aortic pathology (AOP) and pulmonary hypertension (PH) were included.<br/>Between 2007 and 2018, 5739 pregnancies from 138 centres in 53 countries were included in the ROPAC, and in 2019 the main results were published for the entire cohort. There have been earlier ROPAC publications for specific CHD diagnoses, including aortic coarctation, transposition of the great arteries (TGA), tetralogy of Fallot, aortic stenosis and uncorrected CHD. However, a detailed overview of the ROPAC data on characteristics and pregnancy outcomes for women with CHD has not been published previously and is the focus of this manuscript.