Research Program

In keeping with its mission, the ACHD Center maintains a dedicated commitment to its research program, resulting in numerous medical publications as well as national and international presentations.

Faculty members examine the many issues in this growing field and place an emphasis on studies that can readily translate to patient care.

Inspiratory Muscle Training in Fontan survivors- How to use the inspiratory muscle trainer to improve lung capacity

Current areas in which research endeavors are focused include:

• Transcatheter pulmonary valve replacement, including risk factors for and management of infective endocarditis
• Transcatheter pulmonary valve replacement in native pulmonary outflow tracts
• Management of Fontan patients with inspiratory muscle training
• Transcriptomics, liver fibrosis, and clinical outcomes research in patients with a Fontan circulation
• Socially determined barriers to accessing congenital heart disease care
• The effect of SARS-CoV-2 on patients with congenital heart disease
• Evaluating pulmonary microcirculation using novel devices
• Management of patients with congenital heart disease and heart failure

Clinical Trials 

1. The SAPIEN 3 valve with the ALTERRA Adaptive Pre-stent: Performance in Patients with Congenital Pulmonary Dysfunction

This multicenter, single-arm, prospective study evaluates the performance of the ALTERRA adaptive pre-stent, coupled with the SAPIEN 3 transcatheter heart valve. These devices were implanted into patients with native pulmonary outflow tracts. There were six patients enrolled at UCLA, and follow-up is expected to continue for 10 years. The ALTERRA device is now being used for commercial purposes.

2. The Medtronic Harmony Transcatheter Pulmonary Valve Clinical Study

Another multicenter, single-arm, prospective study, the Medtronic Harmony study tests the safety and efficacy of another catheter-deployed heart valve in the pulmonic position. UCLA enrolled seven patients in this trial, and follow-up is also expected to continue for 10 years. This device is now being used commercially.

3. The Edwards Compassion SAPIEN 3 valve Study: Evaluating Device Performance in Patients with Non-Native Right Ventricular Outflow Tracts

This multicenter, single-arm, prospective study evaluates the performance of the SAPIEN 3 transcatheter heart valve, a device originally designed for use in the aortic position. UCLA was a leading center in the investigation, enrolling a total of 13 patients. The study is currently in follow-up, and the device is now regularly used commercially.

4. The GORE CARDIOFORM ASD Occluder Clinical Study: Evaluating the Safety and Efficacy of Transcatheter Closure of Ostium Secundum Atrial Septal Defects

Another prospective, device-based study, the GORE investigation considered the safety and efficacy of the CARDIOFORM ASD Occluder in patients with an ostium secundum atrial septal defect. The study has completed all patient follow-up, and six participants completed the three-year final study visit at UCLA.

Other Multi-Center Collaborations 

1. SERVE Registry: Self-Expanding RVOT Valve Evidence (SERVE) Registry Protocol

The arrival of the Medtronic Harmony transcatheter heart valve and the Edwards ALTERRA adaptive pre-stent has facilitated the process of delivering transcatheter prosthetics into the pulmonic position. This registry, led by Aimee Armstrong of Nationwide Children’s Hospital, seeks to evaluate the early outcomes of these devices.

2. COVID-19 and Adults with Congenital Heart Disease: A Multicenter Registry

Via the Alliance for Adult Research in Congenital Cardiology, the Adult Congenital Heart Association, and International Society for Adult Congenital Heart Disease, UCLA has compiled a multicenter, retrospective cohort study tracking the outcomes of patients with congenital heart disease and COVID-19. Data collection is ongoing and early results have been published. An initial paper included 58 adult centers, and it found that COVID-19 mortality in adults with congenital heart disease is comparable to that of the general population.

3. Social Needs Assessment in Adult Congenital Heart Disease Patients

This study, led in partnership with the Adult Congenital Heart Association and Dr. Jamie Jackson from Nationwide Children’s Hospital, seeks to identify what social barriers prevent congenital heart disease patients from seeking specialized care, and what type of care different demographics receive for their condition. Study assessment tools include patient questionnaires, and they will be distributed in the Los Angeles and Houston areas. Data will be compiled by UCLA and shared with Dr. Jackson for analysis.

4. ENTRUST Adult Congenital Heart Disease and Heart Failure

This international, multicenter study evaluates the use of sacubitril and valsartan in treating patients with dual presentations of congenital heart disease and systemic ventricular dysfunction. The safety and efficacy of these medications will be assessed by considering all-cause mortality, hospitalization, serum biomarkers, subjective patient questionnaires, and objective functional outcomes. Data will be collected retrospectively. The initiative is being led by the University of Queensland in Australia.

5. Implementation of a Sexual Orientation and Gender Identity Survey during Routine Clinic Visits: A Quality Improvement Project.

During routine clinic visits for congenital cardiology care, a survey containing questions related to sexual orientation and gender identity will be administered to patients. Surveys will also ask patients about their opinion of the questionnaire as well. This initiative is led by Dr. Stephen Cook from Indiana University. UCLA will administer this survey to approximately 50 patients and share patient responses with the lead center.

For more information on research studies being conducted at the Ahmanson/UCLA Adult Congenital Heart Disease Center, please email us at [email protected].