• Hsu, Daphne T. (PI)
  • Gersony, Welton (PI)
  • Hellenbrand, William (PI)
  • Wyman, Lai (PI)
  • Rose, Susan A. (PI)
  • Rose, Susan A. (PI)
  • Rose, Susan A. (PI)
  • Rose, Susan A. (PI)

Project: Research project

Project Details


DESCRIPTION (provided by applicant)
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.
Effective start/end date9/5/018/31/11


  • Cardiology and Cardiovascular Medicine
  • Pediatrics, Perinatology, and Child Health
  • Surgery
  • Medicine(all)
  • Pulmonary and Respiratory Medicine


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