Prenatal diagnosis of functionally univentricular heart, associations and perinatal outcomes. (10th May 2016)

• Record Type: Journal Article
• Authors: Wolter, Aline; Nosbüsch, Sina; Kawecki, Andreea; Degenhardt, Jan; Enzensberger, Christian; Graupner, Oliver; Vorisek, Carina; Akintürk, Hakan; Yerebakan, Can; Khalil, Markus; Schranz, Dietmar; Ritgen, Jochen; Stressig, Rüdiger; Axt‐Fliedner, Roland
• Is Part Of: Prenatal diagnosis. Volume 36:Number 6(2016)
• Page Start: 545
• Page End: 554
• Publication Date: 2016-05-10
• Subjects: Prenatal diagnosis -- Periodicals; Fetus -- Diseases -- Diagnosis -- Periodicals; Electronic journals; 618.32075
• DOI: 10.1002/pd.4821
• Languages: English
• ISSNs: 0197-3851

Abstract

Abstract: Objective: Functionally univentricular hearts (UVHs) represent cardiac anomalies in which either the pulmonary or systemic circulation cannot be supported independently. The purpose of our study was to determine perinatal outcomes after prenatal diagnosis of functionally UVH. Methods: We retrospectively evaluated patients who presented between 2008 and June 2015 in our centre and in prenatal practice praenatal.de in Cologne. We included double inlet left ventricle (DILV), tricuspid valve atresia (TA), pulmonary valve atresia and intact ventricular septum (PA:IVS), unbalanced atrioventricular septal defect (AVSD), heterotaxy, hypoplastic left heart syndrome (HLHS) and hypoplastic left heart complex (HLHC). Results: Of initially 155 patients, 128 were liveborn (82.6%). Ten neonates (7.8%) were lost to follow‐up, in three (2.5%) neonates, parents decided for compassionate care. Overall survival after prenatal diagnosis of functionally UVH was 67.1%, and 90.4% on an intention‐to‐treat basis. Survival after surgery reached 93.7%. The majority of deaths occurred within the group of dominant RV (10/74, 13.5%). High risk HLHS with restrictive foramen ovale was associated with the lowest survival rate (13/17, 76.5%) with significant difference compared to survival rate in dominant LV (40/41, 97.6%, p < 0.05). Conclusion: These results should be explained to parents to ensure informed decisions and counselling. © 2016 John Wiley & Sons, Ltd. Abstract : What's Already Known About This Topic? Despite improvement in perinatal management, considerable mortality is still observed in patients with functionally single ventricle. The 15‐year survival for hypoplastic left heart syndrome and variants is reported to be 77% after 'Giessen‐Hybrid' approach in a recent single centre analysis and 60% in a study study using Norwood procedure. The 15‐year survival in functionally univentricular heart with dominant left ventricle (LV) was 85% according to previously published data. What Does This Study Add? Perinatal outcomes of fetuses diagnosed prenatally with functionally UVH are different between those with dominant LV and RV. Our results revealed that patients with dominant LV had higher neonatal survival than patients with dominant RV. The diagnosis of High‐risk HLHS was associated with highest mortality rates within 30 days postpartum and need for urgent intervention within 48 h of life.