Funds raised through Philly Spin-In are used to propel research and advance care for young patients with heart disease — here at Children’s Hospital of Philadelphia (CHOP) and around the world.
Recently, three cardiac research projects at CHOP were awarded two-year, $100,000 grants (for a total of $200,00 per program over the two-year period) to explore specific challenges facing children with pulmonary hypertension, single ventricle disease and hypoplastic left heart syndrome (HLHS).
Pulmonary Hypertension Research
Attending Cardiologist David B. Frank, MD, PhD, and Researcher Michael Chorny, PhD, are investigating new advances in the treatment of pulmonary hypertension, a severe disease that affects children and adults with congenital heart disorders and currently has no cure.
A collaborative cross-disciplinary team composed of experts in nano-medicine and drug delivery are collaborating to develop and test novel medications — using biological materials, chemical synthesis and site-specific drug delivery strategies — to target pulmonary vascular disease. The focus of their research is on site-specific drug delivery strategies to enhance pulmonary vascular permeability with macro-molecular drug carriers.
Single Ventricle Disease Research
Interventional Cardiologist Andrew C. Glatz, MD, and Cardiologist Carlo Renato G. Bartoli, MD, PhD, are conducting research to better understand the cause of vascular anomalies in children born with single ventricle heart defects. Children with single ventricle disease who undergo the Glenn procedure as a bridge to Fontan (at 3- to 5-months of age), often develop abnormal angiogenesis (development of blood vessels), and angiodysplasia (vascular malformations in the gastro-intestinal system).
Dr. Glatz and his team is investigating potential causations — and hope to eventually develop potential therapeutic interventions — by targeting specific proteins in the von Willebrand factor-Angiopoietin Axis, which are identified in the development of these vascular anomalies.
Hypoplastic Left Heart Syndrome Research
Tricuspid valve regurgitation in patients with hypoplastic left heart syndrome (HLHS) is highly associated with death, and necessitates surgical intervention in more than 30 percent of patients. To maximize successful surgical outcomes, accurate assessment of the complex mechanisms of valve dysfunction are essential. While 3-D modeling is available for adult valve repair and has revolutionized mitral valve surgery, there is currently no commercially or readily available method for modeling the tricuspid valve in pediatric patients with HLHS.
Matthew A. Jolley, MD, an Attending Anesthesiologist in the Division of Cardiothoracic Anesthesiology, is leading an effort to develop, validate and optimize 3-D-based computer modeling software to visualize and quantify tricuspid valve structure in patients with HLHS. Then, by working with surgeons, Dr. Jolley wants to apply that knowledge and insight to improve tricuspid valve repair and outcomes for patients with HLHS.