Hypoplastic Left Heart Syndrome
Conditions
Keywords
Hypoplastic Left Heart Syndrome, Stage II palliative surgery, Glenn procedure, Congenital heart defect, Congenital heart disease, Underdeveloped left ventricle
Brief summary
Hypoplastic left heart syndrome (HLHS) is a severe form of congenital heart disease that consists of multiple obstructions to flow through the left heart and aorta, as well as hypoplasia of the left ventricle. Most patients require a three-stage surgical protocol starting within days of birth. Stage I of this process is the Norwood reconstruction (within the first few days of life), Stage II (usually required within 3-8 months) involves creation of a direct connection between the patient's superior vena cava and the pulmonary arterial confluence (bidirectional Glenn anastomosis), and the last stage is creation of a Fontan circulation (typically within the first 2-4 years). This single ventricle approach requires the right ventricle to perform as the only circulatory pump for the entire body. Our long-term goal is to develop regenerative strategies to strengthen and augment the right ventricular muscle of the single-ventricle heart following surgical palliation in HLHS patients. To determine the safety and feasibility of a cell-based therapeutic intervention at the Stage II surgery, we aim to document the natural history of post-surgical care in HLHS patients having undergone standard of care with protocol specific follow-up over the course of a 6-month period. This prospective study will document the natural history in patients with HLHS after planned Stage II surgical palliation with a focus on cardiovascular parameters within 6 months following surgery in 10 patients.
Interventions
None listed
Sponsors
University of Oklahoma
Children's Hospital of Philadelphia
Mayo Clinic
Timothy J Nelson, MD, PhD
Study design
Observational model
COHORT
Time perspective
PROSPECTIVE
Eligibility
Sex/Gender
ALL
Age
0 Months to 18 Months
Healthy volunteers
No
Inclusion criteria
* Individuals with HLHS having undergone Stage I surgical palliation and undergoing planned Stage II palliative Glenn surgery. * Individuals up to 18 months of age are eligible if written informed consent can be obtained from both parents and/or legal guardians, unless one parent is not reasonably available.
Exclusion criteria
* Severe chronic diseases, extensive extra-cardiac syndromic features, or history of cancer The following complications of congenital heart disease: * Any condition requiring urgent, or unplanned procedure within 15 days prior to Stage II surgical repair. * Severe pulmonary hypertension (reported in the medical record as \>70% systemic pressure). * Other clinical concerns as documented by a site investigator that would predict (more likely to happen than not to happen) a risk of severe complications or very poor outcome during or after Stage II surgical repair.
Countries
United States
Outcome results
None listed