Being born with a single cardiac ventricle: What do we tell prospective parents. (7th April 2022)

• Record Type: Journal Article
• Authors: d'Udekem, Yves; Hutchinson, Darren
• Is Part Of: Prenatal diagnosis. Volume 42:Number 4(2022)
• Page Start: 411
• Page End: 418
• Publication Date: 2022-04-07
• Subjects: Prenatal diagnosis -- Periodicals; Fetus -- Diseases -- Diagnosis -- Periodicals; Electronic journals; 618.32075
• DOI: 10.1002/pd.6121
• Languages: English
• ISSNs: 0197-3851

Abstract

Abstract: Single ventricle cardiac disease remains one of the most extreme forms of congenital heart disease. It encompasses a wide variety of lesions characterised by the existence of one small ventricular cavity. We now realise that survival of this group is much longer than expected, well into adulthood for the majority with current techniques, albeit with a considerable burden of disease for many. Here we give a up to date review of single ventricle cardiac disease. We include discussion regarding prenatal assessment and planning, usual treatment pathways, short and long term outcomes and a discussion on the future for this patient group. We combine this information to give a contemporary perspective on our counselling approach appropriate for those who are the first contact and primary conveyors of this information to families. Key points: What is already known about this topic? Single ventricle cardiac disease is one of the most extreme congenital cardiac lesions. Survival depends upon a series of complex cardiac interventions in childhood with high morbidity and mortality compared to almost all other cardiac lesions. What does this review add? Prenatal management involves a broad multidisciplinary team approach. In addition to accurate cardiac diagnosis, it should include detailed extra cardiac assessment, psychosocial support and delivery planning to maximise postnatal outcomes. Counselling should include discussion regarding possible long term physical, psychological and neurodevelopmental effects in addition to survival rates. Improved long term survival, well into adulthood, is now expected for the majority of patients with a single cardiac ventricle.