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.

Prenatal Diagnosis
|March 12, 2022
PubMed

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.

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