A Prospective Study of Patients With Hypoplastic Left Heart Syndrome (HLHS) Following Stage II Surgical Palliation

October 2, 2020 updated by: Timothy J Nelson, MD, PhD

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.

Study Overview

Status

Completed

Conditions

Study Type

Observational

Enrollment (Actual)

4

Contacts and Locations

This section provides the contact details for those conducting the study, and information on where this study is being conducted.

Study Locations

  • United States
    • Minnesota
      • Rochester, Minnesota, United States, 55901
        • Mayo Clinic
    • Oklahoma
      • Oklahoma City, Oklahoma, United States, 73104
        • Oklahoma University Children's Hospital
    • Pennsylvania
      • Philadelphia, Pennsylvania, United States, 19104
        • Children's Hospital of Philadelphia

Participation Criteria

Researchers look for people who fit a certain description, called eligibility criteria. Some examples of these criteria are a person's general health condition or prior treatments.

Eligibility Criteria

Ages Eligible for Study

1 second to 1 year (Child)

Accepts Healthy Volunteers

No

Genders Eligible for Study

All

Sampling Method

Non-Probability Sample

Study Population

Children with HLHS up to 18 months of age requiring a planned Stage II bi-directional Glenn surgery

Description

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.

Study Plan

This section provides details of the study plan, including how the study is designed and what the study is measuring.

How is the study designed?

Design Details

  • Observational Models: Cohort
  • Time Perspectives: Prospective

Cohorts and Interventions

Group / Cohort
Children with Hypoplastic Left Heart Syndrome (HLHS)
HLHS patients requiring Stage II Glenn surgery

Collaborators and Investigators

This is where you will find people and organizations involved with this study.

Sponsor

Timothy J Nelson, MD, PhD

Collaborators

Mayo Clinic
Children's Hospital of Philadelphia
University of Oklahoma

Investigators

  • Principal Investigator: Muhammad Y Qureshi, MBBS, Mayo Clinic
  • Study Director: Timothy J Nelson, MD, PhD, Mayo Clinic

Publications and helpful links

The person responsible for entering information about the study voluntarily provides these publications. These may be about anything related to the study.

Helpful Links

Study record dates

These dates track the progress of study record and summary results submissions to ClinicalTrials.gov. Study records and reported results are reviewed by the National Library of Medicine (NLM) to make sure they meet specific quality control standards before being posted on the public website.

Study Major Dates

Study Start (Actual)

October 1, 2012

Primary Completion (Actual)

May 1, 2020

Study Completion (Actual)

May 15, 2020

Study Registration Dates

First Submitted

October 15, 2012

First Submitted That Met QC Criteria

October 16, 2012

First Posted (Estimate)

October 17, 2012

Study Record Updates

Last Update Posted (Actual)

October 5, 2020

Last Update Submitted That Met QC Criteria

October 2, 2020

Last Verified

October 1, 2020

More Information

Terms related to this study

Keywords

Additional Relevant MeSH Terms

Other Study ID Numbers

  • 12-002887

This information was retrieved directly from the website clinicaltrials.gov without any changes. If you have any requests to change, remove or update your study details, please contact register@clinicaltrials.gov. As soon as a change is implemented on clinicaltrials.gov, this will be updated automatically on our website as well.

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