Congenital Heart Valve Program

We have created the first prosthetic pulmonary valve replacement that is specifically designed for pediatric patients and can expand over time inside a child’s anatomy. Known as the Autus Valve, it can be adjusted to match a patient’s heart anatomy before it is implanted. Once the device is implanted, a catheter balloon can expand the valve to align with a child’s growth.

We evaluated the patient records of more than 150 patients with Ebstein's anomaly who had a Cone procedure to repair diseased tricuspid valve. We found that the time to tricuspid valve reoperation may be shorter when Cone repair is performed before age four years, but this result requires confirmation in a larger sample. An annuloplasty ring should be considered when appropriate.

We were one of the first hospitals in the world to perform a partial heart transplant as an alternative to a heart valve replacement procedure. We removed a 4-year-old boy’s diseased aortic valve and replaced it with a donor’s healthy valve, which is expected to grow along with the boy, Jack.

Our recent study of more than 200 patients with hypoplastic left heart syndrome (HLHS) and significant tricuspid valve regurgitation showed they were more likely to have successful tricuspid valve repair if they had less tricuspid annular dilation, less-severe RV enlargement, and predominantly anterior leaflet prolapse. Successful tricuspid valve repair was associated with improved mid- and long-term outcomes.

Working with the Cardiovascular 3D Modeling and Simulation Program, we are evaluating the short- and long-term effects of using different types of patch material to repair deficient mitral valves that require additional leaflet tissue to facilitate appropriate valve coaptation.

We’re also helping to develop a clinical trial for a device that may be beneficial in reducing the amount of regurgitation in patients with HLHS who have had a single-ventricle surgery (Norwood, bi-directional Glenn, or Fontan).

Here is a sample of published research that documents our efforts to better understand valve disease and how we can enhance care for patients:

Aortic valve disease

Simple leaflet repair

Publication: Aortic Valve Repair in the Pediatric Population: Midterm Outcomes Comparing Leaflet Repair Using Autologous Pericardium Versus Porcine Intestinal Submucosa (Cormatrix ®). The Journal of the American College of Cardiology. March 2017; Volume 69, Issue 11: Page 557. https://www.sciencedirect.com/science/article/pii/S0735109717339463?via%3Dihub 

Complex leaflet repairs/bicuspidization

Publication: Symmetric Bicuspidizing Repair for Patients with Congenital Aortic or Truncal Valve Disease. The Journal of Thoracic Cardiovascular Surgery. Aug. 2023; Volume 166, Issue 22: Pages 283-291. https://www.jtcvs.org/article/S0022-5223(22)01131-X/abstract

Valve-sparing aortic root repair

Publication: Valve-sparing aortic root replacement and remodeling with complex aortic valve reconstruction in children and young adults with moderate or severe aortic regurgitation. The Journal of Thoracic Cardiovascular Surgery. June 2014; Volume 147, Issue 6: Pages 1768-1776. https://www.sciencedirect.com/science/article/pii/S0022522314002621?via%3Dihub

Ozaki procedure

Publication: Congenital Aortic and Truncal Valve Reconstruction Using the Ozaki Technique: Short-Term Clinical Results. The Journal of Thoracic and Cardiovascular Surgery. May 2021; Volume 161, Issue 5: Pages 1567-1577. https://www.jtcvs.org/article/S0022-5223(20)30438-4/fulltext

Bioprosthetic aortic valve replacement

Publication: Valve Replacement with an Expandable Bioprosthetic Valve in Neonates and Infants. Journal of the American College of Cardiology. March 2013; Volume 61, Issue 10: Supplement. https://www.jacc.org/doi/10.1016/S0735-1097%2813%2960426-X

Mechanical aortic valve replacement

Publication: Outcomes After Mechanical Aortic Valve Replacement in Patients with Congenital Heart Disease. The Journal of Thoracic and Cardiovascular Surgery. Jan. 2019; Volume 157, Issue 1: P 329-340. https://www.sciencedirect.com/science/article/pii/S0022522318323535?via%3Dihub

Mitral valve disease

Evolving techniques and a focus on mitral valve repair

Publication: Mitral Valve Operations at a High-volume Pediatric Heart Center: Evolving Techniques and Improved Survival with Mitral Valve Repair Versus Replacement. Annals of Pediatric Cardiology. Jan.-June 2012; Volume 5, Issue 1. https://journals.lww.com/aopc/fulltext/2012/05010/mitral_valve_operations_at_a_high_volume_pediatric.3.aspx

Tricuspid valve disease

Tricuspid valve repair with Norwood procedure for babies with HLHS

Publication: Tricuspid Valve Repair Concomitant with the Norwood Operation Among Babies with Hypoplastic Left Heart Syndrome. European Journal of Cardiothoracic Surgery. Oct. 2022; Volume 62, Issue 4.  https://doi.org/10.1093/ejcts/ezac033

Tricuspid valve repair with Cone repair for Ebstein’s

Publication: The Association of Age and Repair Modification with Outcome after Cone Repair for Ebstein's Malformation. Seminars in Thoracic Cardiovascular Surgery. Spring 2022; Volume 34, Issue 1: P 205-212. https://www.sciencedirect.com/science/article/abs/pii/S1043067921001829

Truncal valve disease

Simple leaflet repair

Publication: Aortic Valve Repair in the Pediatric Population: Midterm Outcomes Comparing Leaflet Repair Using Autologous Pericardium Versus Porcine Intestinal Submucosa (Cormatrix®). The Journal of the American College of Cardiology. March 2017; Volume 69, Issue 11: P 557. https://www.sciencedirect.com/science/article/pii/S0735109717339463?via%3Dihub

Complex leaflet repair/bicuspidization

Publication: Symmetric Bicuspidizing Repair for Patients with Congenital Aortic or Truncal Valve Disease. The Journal of Thoracic Cardiovascular Surgery. Aug. 2023; Volume 166, Issue 22: P 283-291. https://www.jtcvs.org/article/S0022-5223(22)01131-X/abstract

Valve-sparing aortic root repair

Publication: Valve-sparing aortic root replacement and remodeling with complex aortic valve reconstruction in children and young adults with moderate or severe aortic regurgitation. The Journal of Thoracic Cardiovascular Surgery. June 2014; Volume 147, Issue 6: P 1768-1776. https://www.sciencedirect.com/science/article/pii/S0022522314002621?via%3Dihub

Ozaki procedure

Publication: Congenital Aortic and Truncal Valve Reconstruction Using the Ozaki Technique: Short-Term Clinical Results. The Journal of Thoracic and Cardiovascular Surgery. May 2021; Volume 161, Issue 5: P 1567-1577. https://www.jtcvs.org/article/S0022-5223(20)30438-4/fulltext

Pulmonary valve disease

Ozaki procedure

Publication: Pulmonary Valve Reconstruction Using the Ozaki Leaflet Reconstructive Techniques. The Annals of Thoracic Surgery. Jan. 2021; Volume 111, Issue 1: P. E19-E21. https://www.annalsthoracicsurgery.org/article/S0003-4975(20)30887-0/fulltext