Clinical Diagnosis, Management and Treatment of Congenital Heart Disease

Dear Colleagues,

Congenital heart disease (CHD) refers to a group of structural heart abnormalities that are present at birth. It is a complex and diverse condition, affecting individuals of all ages and requiring specialized medical attention. This Special Issue aims to explore the latest advancements in the clinical understanding, management, and treatment of CHD.

Over the years, significant progress has been made in the diagnosis and evaluation of CHD, facilitated by advanced imaging techniques and genetic testing. Improved screening programs and early detection have enhanced patient outcomes, leading to timely interventions and better management strategies. Cutting-edge technologies and innovative approaches have revolutionized the management of CHD. Minimally invasive procedures, transcatheter interventions, and 3D printing have expanded treatment options and offered personalized solutions for complex cases. Moreover, advances in medical devices and surgical techniques continue to enhance quality of life for CHD patients.

We invite original research articles, comprehensive reviews, and clinical studies that contribute to the existing knowledge of CHD. Submissions should focus on clinical insights, recent progress, and the application of new technologies in the field.

Dr. Xavier Iriart
Guest Editor

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• congenital heart disease
• pediatric cardiology
• comorbidities
• echocardiography
• cardiac function
• heart valve diseases
• cardiac imaging

Title: Pregnancy outcomes in Women with Biventricular circulation and Systemic Right Ventricle: A systematic Review
Authors: Triantafyllia Grantza1; Alexandra Arvanitaki1,2; Amalia Baroutidou1; Ioannis Tsakiridis3; Apostolos Mamopoulos3; Andreas Giannopoulos4; Antonios Ziakas1; George Giannakoulas1
Affiliation: 1 First Department of Cardiology, AHEPA University General Hospital, School of Medicine, Aristotle University of Thessaloniki, Thessaloniki, 54636, Greece. 2 Adult Congenital Heart Centre and National Centre for Pulmonary Hypertension, Royal Brompton and Harefield Hospitals, Guy’s and St Thomas’s NHS Foundation Trust, Imperial College, London, SW3 5NP, United Kingdom. 3 3rd Department of Obstetrics and Gynecology, Hippokratio General Hospital, School of Medicine, Aristotle University of Thessaloniki, Thessaloniki, 54642, Greece. 4 Pediatric Department, Aristotle University of Thessaloniki, AHEPA University General Hospital, School of Medicine, Aristotle University of Thessaloniki, Thessaloniki, 54636, Greece.
Abstract: Background: Pregnancy in women with a biventricular circulation and a systemic right ventricle (sRV) is considered high risk, with limited data available on pregnancy outcomes. This study aims to investigate maternal and fetal outcomes in this population. Materials and Methods: A systematic literature review was conducted through four major electronic databases (PubMed, CENTRAL (Cochrane Central Register of Controlled Trials), Scopus and Web of Science), article references and grey literature. Pregnant women with complete transposition of great arteries (d-TGA) after atrial switch operation or a congenitally corrected transposition of the great arteries (ccTGA) were included. Results: In total, 15 studies including 632 pregnancies in 415 women with sRV and a biventricular circulation were identified, of whom 299 (72%) had d-TGA and 116 (28%) ccTGA. Maternal mortality, caused by sudden cardiac death, occurred in 0.8% of pregnancies. The most frequent maternal complications were all-cause hospitalization [pooled estimate (PE): 16%, 95%CI: 5;26], worsening of systemic atrioventricular valve regurgitation (PE: 14.5%, 95%CI: 5;26) and sRV function (PE: 15%, 95%CI: 2;27), worsening of NYHA class (PE: 13%, 95%CI: 6;20), symptomatic heart failure (PE: 6%, 95%CI: 3;10) and arrhythmias (PE: 8%, 95%CI: 5;11). Stillbirth occurred in 0.6% of pregnancies and neonatal death in 0.5%. Small for gestational age neonates were encountered in 36% (95%CI: 21;52) of pregnancies, preterm birth in 22% (95%CI: 14;30) and vaginal delivery in 51% (95%CI: 37;65). Subgroup analysis showed no significant difference in outcomes between women with d-TGA and those with ccTGA, except from worsening of NYHA class, which occurred more often in d-TGA (18%, 95%CI: 12;27 vs 6%, 95%CI: 3;15, respectively, p =0.03). (Figure) Conclusion: Maternal and fetal mortality is low among pregnant women with biventricular circulation and a sRV. However, significant maternal morbidity and worse fetal outcomes are frequently encountered, rendering management in specialized centers an imperative.