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

This study is currently recruiting participants. (see Contacts and Locations)
Verified March 2016 by Mayo Clinic
University of Oklahoma
Information provided by (Responsible Party):
Timothy J. Nelson, Mayo Clinic Identifier:
First received: October 15, 2012
Last updated: March 11, 2016
Last verified: March 2016

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 10-20 patients.

Hypoplastic Left Heart Syndrome

Study Type: Observational
Study Design: Observational Model: Cohort
Time Perspective: Prospective

Resource links provided by NLM:

Further study details as provided by Mayo Clinic:

Estimated Enrollment: 20
Study Start Date: October 2012
Estimated Study Completion Date: January 2017
Estimated Primary Completion Date: January 2017 (Final data collection date for primary outcome measure)
Children with Hypoplastic Left Heart Syndrome (HLHS)
HLHS patients requiring Stage II Glenn surgery


Ages Eligible for Study:   up to 18 Months   (Child)
Sexes Eligible for Study:   All
Accepts Healthy Volunteers:   No
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

Inclusion Criteria:

  • Up to 18 months of age
  • Undergoing planned Stage II Glenn palliative surgery
  • History of successful modified Norwood procedure utilizing a RV-PA shunt
  • 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, extra-cardiac syndromes, or cancer
  • The following conditions within 15 days prior to the date of the Stage II Glenn surgery:

    • Cardiogenic shock
    • Pulmonary hypertension requiring chronic medical therapy (e.g. supplemental oxygen, vasodilator)
    • Arrhythmia that required medication for control
  • Any documented infection requiring treatment with IV antibiotics, and/or current infection being treated with antibiotics
  • The following complications of their congenital heart disease:

    • Any condition requiring urgent, or unplanned procedure 15 days prior to Stage II Glenn surgery
  • Tricuspid repair and/or aortic arch repair at the time of Stage II Glenn surgery
  • Length of hospitalization of more than 60 days for Stage I Norwood procedure

    • Chylothorax requiring dietary modifications
    • Seizure or neurological injury
    • Moderate to severe tricuspid regurgitation prior to Stage II Glenn surgery
    • History of extracorporeal membrane oxygenator (ECMO) support
  Contacts and Locations
Choosing to participate in a study is an important personal decision. Talk with your doctor and family members or friends about deciding to join a study. To learn more about this study, you or your doctor may contact the study research staff using the Contacts provided below. For general information, see Learn About Clinical Studies.

Please refer to this study by its identifier: NCT01708863

Contact: Karen S Miller 507-266-5510
Contact: Karen M Cavanaugh 507-538-8425

United States, Minnesota
Mayo Clinic Recruiting
Rochester, Minnesota, United States, 55901
Contact: Karen S Miller    507-266-5510   
Contact: Karen M Cavanaugh    507-538-8425   
Principal Investigator: Muhammad Y Qureshi, MBBS         
United States, Oklahoma
Oklahoma University Children's Hospital Recruiting
Oklahoma City, Oklahoma, United States, 73104
Contact: Stephanie Mitchell    405-271-2128   
Contact: Nicholas Farley    405-271-4411 ext 44403   
Principal Investigator: Harold M Burkhart, M.D.         
Sponsors and Collaborators
Timothy J. Nelson
University of Oklahoma
Study Director: Timothy J Nelson, MD, PhD Mayo Clinic
Principal Investigator: Muhammad Y Qureshi, MBBS Mayo Clinic
  More Information

Responsible Party: Timothy J. Nelson, Program Director, Mayo Clinic Identifier: NCT01708863     History of Changes
Other Study ID Numbers: 12-002887
Study First Received: October 15, 2012
Last Updated: March 11, 2016

Keywords provided by Mayo Clinic:
Hypoplastic Left Heart Syndrome
Stage II palliative surgery
Glenn procedure
Congenital heart defect
Congenital heart disease
Underdeveloped left ventricle

Additional relevant MeSH terms:
Hypoplastic Left Heart Syndrome
Pathologic Processes
Heart Defects, Congenital
Cardiovascular Abnormalities
Cardiovascular Diseases
Heart Diseases
Congenital Abnormalities processed this record on April 28, 2017