A PROSPECTIVE STUDY OF PATIENTS WITH HYPOPLASTIC LEFT HEART SYNDROME FOLLOWING STAGE II SURGICAL PALLIATION

Location:

Rochester, Minn.

Trial status:

Open for Enrollment

Why is this study being done?

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.

Who is eligible to participate?

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

Last updated:

8/29/2014

NCT ID:

NCT01708863

IRB Number:

12-002887