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.
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http://dx.doi.org/10.1080/14767058.2025.2470411 | DOI Listing |
J Matern Fetal Neonatal Med
December 2025
Dipartimento di Scienze Cardiovascolari, Fondazione Policlinico Universitario A. Gemelli IRCCS, Roma.
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.
View Article and Find Full Text PDFCJC Pediatr Congenit Heart Dis
December 2024
Department of Cardiac Sciences, Division of Cardiology, University of Calgary and Alberta Health Services, Calgary, Alberta, Canada.
Background: Adults with congenital heart disease (CHD) are growing in number and living longer with complex lesions; however, many are at risk of death in midlife. Conversations about advance care planning (ACP) in this population have been found to be infrequent and not part of regular outpatient care. The intent of this study was to explore readiness of patients with adult CHD (ACHD) to discuss ACP and assess the impact of interventions to support ACP conversations.
View Article and Find Full Text PDFCardiovasc Diagn Ther
December 2024
Cardiovascular Center, St. Luke's International Hospital, Tokyo, Japan.
Right ventricular (RV) dysfunction after biventricular repair is critical in most adults with congenital heart disease (ACHD). Conventional 2D magnetic resonance imaging (MRI) measurement is considered as a 'gold standard' for RV evaluation; however, addition information on ACHD after biventricular repair is sometimes required. The reasons why adjunctive information is required is as follows: (I) to evaluate the severity of cardiac burden in symptomatic patients with normal RV size and ejection fraction (EF), (II) to determine the optimal timing of invasive treatments in asymptomatic ones, and (III) to detect proactively a potential cardiac burden leading to ventricular deterioration, from a fluid dynamics perspective.
View Article and Find Full Text PDFCardiovasc Diagn Ther
December 2024
Department of Cardiology, University Heart & Vascular Center, University Medical Center Hamburg-Eppendorf (UKE), Hamburg, Germany.
Background: Cardiovascular disease (CVD) remains the leading cause of death in pregnant and peripartal women in western countries. Physiological changes during pregnancy can lead to cardiovascular complications in the mother; women with pre-existing heart disease may not tolerate these changes well, increasing their susceptibility to adverse cardiovascular outcomes during pregnancy. The aim of this study is to characterize pregnancy-induced changes in cardiac function, biomarker concentrations and cardiovascular outcomes in women with CVD during pregnancy at a tertiary care hospital in Germany.
View Article and Find Full Text PDFFront Cardiovasc Med
December 2024
Department of Congenital and Pediatric Cardiac Surgery, German Heart Center Munich, Technical University of Munich, Munich, Germany.
Objective: To investigate the long-term impact of cardiac surgery on the quality of life in adults with congenital heart disease (ACHDs).
Methods: Patients who had undergone cardiac surgery for congenital heart disease (CHD) at the age of 18 years or more were recruited in a single-center, cross-sectional study. The enrolled subjects completed online questionnaires to assess patient-reported outcomes: perceived health status and life satisfaction, psychological functioning, health behaviors, and illness perception.
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