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Being born with a single cardiac ventricle: What do we tell prospective parents
Yves d'Udekem1, Darren Hutchinson2
1Division of Cardiac Surgery, Children's National Hospital and Children's National Heart Institute, Washington, District of Columbia, USA.
Insights
Single ventricle cardiac disease, a severe congenital heart defect, now allows longer survival into adulthood. This review covers diagnosis, treatment, outcomes, and future care for patients with single ventricle hearts.
Area of Science:
- Cardiology
- Pediatric Cardiology
- Congenital Heart Disease
Background:
- Single ventricle cardiac disease is a severe congenital heart defect characterized by a single ventricular cavity.
- While historically associated with poor prognosis, current medical techniques have significantly improved survival rates, with many patients now living into adulthood.
Purpose of the Study:
- To provide an up-to-date review of single ventricle cardiac disease.
- To discuss prenatal assessment, treatment pathways, and short- and long-term outcomes.
- To offer a contemporary perspective on patient and family counseling.
Main Methods:
- Comprehensive literature review of single ventricle cardiac disease.
- Synthesis of information on diagnosis, treatment, and outcomes.
- Analysis of current approaches to patient and family counseling.
Main Results:
- Patients with single ventricle defects demonstrate improved long-term survival into adulthood.
- A significant burden of disease persists for many adult survivors.
- Updated information is crucial for effective prenatal and lifelong care planning.
Conclusions:
- Improved survival necessitates a contemporary approach to managing single ventricle cardiac disease.
- Effective counseling, starting from prenatal diagnosis, is vital for families.
- Ongoing research and multidisciplinary care are essential for optimizing outcomes.
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.
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