The overall goal of this application is to examine treatment modalities which may improve the clinical care of two groups of patients with congenital heart disease: infants born with a single ventricle supplying blood flow to the lungs and body and children with pulmonary hypertension associated with congenital heart disease. The primary hypothesis in infants with single ventricle is that chronic angiotensin converting enzyme (ACE) inhibition favorably modifies the ventricular remodeling response to volume overload and improves ventricular function over the first year of life. Serial changes in ventricular geometry will be assessed using magnetic resonance imaging and compared with measurements of systolic and diastolic function, including the pressure/volume relation and the Tei index, and clinical outcome measures including post-operative course and changes in the Ross? heart failure classification. The beneficial effect of ACE inhibition is expected to occur prior to and following volume unloading surgery with the bidirectional Glenn shunt or hemi-Fontan. The primary hypothesis of the study in congenital heart disease associated with pulmonary hypertension is that the effect of long-term treatment with an oral prostacyclin analogue or an oral endothelin receptor blocker has a salutory effect on exercise capacity, longevity, and quality of life. It will also be determined whether any of these patients carry a defect of the primary pulmonary hypertension-1 gene. Each of these studies could potentially lead to a significant improvement in prognosis: in the single ventricle group by preventing a long-term deterioration in ventricular function and in the pulmonary hypertension patients by improving quality of life and survival without transplantation.

National Institute of Health (NIH)
National Heart, Lung, and Blood Institute (NHLBI)
Research Project--Cooperative Agreements (U01)
Project #
Application #
Study Section
Special Emphasis Panel (ZHL1-CSR-L (M3))
Program Officer
Pearson, Gail D
Project Start
Project End
Budget Start
Budget End
Support Year
Fiscal Year
Total Cost
Indirect Cost
Columbia University (N.Y.)
Schools of Medicine
New York
United States
Zip Code
Newburger, Jane W; Sleeper, Lynn A; Gaynor, J William et al. (2018) Transplant-Free Survival and Interventions at 6 Years in the SVR Trial. Circulation 137:2246-2253
Hoskoppal, Arvind; Menon, Shaji; Trachtenberg, Felicia et al. (2018) Predictors of Rapid Aortic Root Dilation and Referral for Aortic Surgery in Marfan Syndrome. Pediatr Cardiol 39:1453-1461
Mahle, William T; Hu, Chenwei; Trachtenberg, Felicia et al. (2018) Heart failure after the Norwood procedure: An analysis of the Single Ventricle Reconstruction Trial. J Heart Lung Transplant 37:879-885
Saarel, Elizabeth V; Granger, Suzanne; Kaltman, Jonathan R et al. (2018) Electrocardiograms in Healthy North American Children in the Digital Age. Circ Arrhythm Electrophysiol 11:e005808
Selamet Tierney, Elif Seda; Levine, Jami C; Sleeper, Lynn A et al. (2018) Influence of Aortic Stiffness on Aortic-Root Growth Rate and Outcome in Patients With the Marfan Syndrome. Am J Cardiol 121:1094-1101
Mussatto, Kathleen A; Hollenbeck-Pringle, Danielle; Trachtenberg, Felicia et al. (2018) Utilisation of early intervention services in young children with hypoplastic left heart syndrome. Cardiol Young 28:126-133
Ramroop, Ronand; Manase, George; Lu, Danny et al. (2017) Adrenergic receptor genotypes influence postoperative outcomes in infants in the Single-Ventricle Reconstruction Trial. J Thorac Cardiovasc Surg 154:1703-1710.e3
Lopez, Leo; Colan, Steven; Stylianou, Mario et al. (2017) Relationship of Echocardiographic Z Scores Adjusted for Body Surface Area to Age, Sex, Race, and Ethnicity: The Pediatric Heart Network Normal Echocardiogram Database. Circ Cardiovasc Imaging 10:
Burch, Phillip T; Ravishankar, Chitra; Newburger, Jane W et al. (2017) Assessment of Growth 6 Years after the Norwood Procedure. J Pediatr 180:270-274.e6
Lambert, Linda M; Trachtenberg, Felicia L; Pemberton, Victoria L et al. (2017) Passive range of motion exercise to enhance growth in infants following the Norwood procedure: a safety and feasibility trial. Cardiol Young 27:1361-1368

Showing the most recent 10 out of 110 publications