Pregnancy in ACHD women: crucial role of multidisciplinary clinical roadmap

Purpose: Pregnancy in women with adult congenital heart disease (ACHD), whether in its natural history or after surgical correction, represents a unique pathophysiological model that requires careful, multidisciplinary management to ensure favorable maternal, fetal and neonatal outcomes. Investigating the impact of congenital cardiac conditions on maternal and feto-neonatal health, the effect of pregnancy-related cardiovascular changes on maternal cardiac health, and the outcomes for offspring born from ACHD mothers is highly relevant, due to the increasing number of ACHD women reaching adulthood and the significant burden these pregnancies can pose. The aim of this article is to provide food for thought to those who have always been involved in ACHD and pregnancy, but also to provide a training tool for young doctors who are approaching at this wonderful world for the first time. Materials and methods: This article was conceived and structured as an “educational and debate”. In this article we describe our experience in the ACHD outpatient clinic and the High-Risk Pregnancies Division of Fondazione Policlinico A. Gemelli Hospital IRCCS from 2013 and now includes over 100 patients evaluated over a 10-year period. Results: In this article we describe our clinical pathway and the clinical history of our first patient, a 30-year-old woman with univentricular heart (criss-cross heart, double outlet right ventricle and pulmonary stenosis) who underwent a Glenn operation as a child. Our plan included scheduled cardiological and obstetrical follow-ups, as well as planned hospitalizations. An elective C-section was carried out at 38 gestational weeks under spinal anesthesia, with Extracorporeal Membrane Oxygenation and the heart surgery team stand by. It was an uncomplicated delivery. As a result, we developed a specific clinical pathway named “ACHD Pregnancy Pink Pathway.” Conclusions: The strength of this idea dwells in the synergy between different experts in deciding for the best decision regarding the required monitoring strictness and the more appropriate obstetric surveillance and delivery plan for the patient. The lesson we learned over the years is that to ensure the best diagnosis and treatment for our young unique patients, we must create a detailed “ROADMAP” for them. We propose a pioneering pathway divided into the three essential phases: maternal, obstetrics and fetal-neonatal.