Clinical Trial: Pulmonary Arteriopathy-Diagnostics and Therapy

Study Status: Completed
Recruit Status: Completed
Study Type: Observational

Official Title: Pulmonary Arteriopathy; Evaluation of Invasive Treatments of Peripheral Pulmonary Stenosis in Terms of Right Ventricular Function and Patient Exercise Tolerance

Brief Summary:

Postoperative stenoses of the pulmonary artery vascular system seldom occur alone; they are frequently found in connection with congenital heart defects or malformation syndromes. The resulting increase of afterload represents a serious pressure load for the right ventricle. Depending on the number and severity of the stenoses, gradual functional right ventricular failure is to be expected. Due to limited clinical experience, there has not yet been a consensus concerning the indications for the different therapeutic strategies (balloon dilatation, stent implantation, surgical dilatation techniques). Up to now, only few investigators in few centres use stents as therapy. Therefore, systematic multicenter investigations assessing larger groups of patients undergoing this procedure are not yet available. The same applies to other novel dilatation techniques, such as the use of the "cutting balloon" as therapy for rigid valve stenoses.

By comparing and analysing different invasive forms of treatment (balloon dilatation, stent implantation and surgery), we expect to achieve an optimisation of therapy.

In the study, the outcomes of different strategies as practiced now in German cardiological centers will be compared and the main factors influencing the results will be determined. On the basis of a standardized investigation before and one year after the intervention, these comparisons with respect to the reduction of stenosis and corresponding changes of right ventricular functional and anatomical changes are carried out correcting for known confounders. The assessment of the different included invasive and non-invasive diagnostical procedures with respect to their ability to detect pathological findings and their changes as result of the treatment is an important secondary target of the study.

Detailed Summary:

For patients with untreated peripheral pulmonary stenoses, the course of disease is characterised by a chronic pressure load of the right ventricle. In the medium to long term this can lead to right heart failure and cardiac arrhythmia unresponsive to therapy. The probability of these events as well as the time of their occurrence depends on the severity of the stenoses, the duration of stress and a possible additional volume load. Within the scope of the prospective study introduced here, we intend to characterise the stenoses with respect to degree and type and to document the treatment success in terms of decrease of afterload of the right ventricle. In this context, imaging and catheter-based methods as well as respective functional analyses are to be used to provide evidence of a reduction or neutralisation of the pulmonary artery stenoses.

Accordingly, the following questions, or corresponding objectives, can be identified as the base for the conduct of this clinical trial:

• Morphological and functional categorisation of the angiostenoses and standardisation of the diagnostic procedures. For this purpose, the quantitative criteria for both the morphological degree of stenosis (lumen constriction) and the functional degree of stenosis (pressure gradient) are established by means of cardiac catheterisation and angiography. Non-invasive imaging methods, which have as yet been restricted primarily to echocardiography, are complemented by the method of spin angiography. Compared to conventional angiography, this has the advantage of being less invasive and not involving radiation exposure.
• On the strength of past experience, the implantation of stents can be viewed as a very promising approach to a treatment with lasting effectiveness of pulmonary artery st
  Sponsor: Competence Network for Congenital Heart Defects
  

  Current Primary Outcome:

  • Primary outcome measure:
  • To assess the efficacy of the administered therapy, both the ectasia of the stenosed vessels in
  • relation to the target diameter and the reduction of the pressure gradient in the stenosed vessel
  • are indicated in percent. An evaluation of the therapeutic success takes place after twelve months.
  • Definition of the primary target of therapy:
  • Morphometric:
  • • Expansion of the peripheral pulmonary artery stenosis to at least 80% of the vascular
  • lumen.
  • Manometric:
  • • Gradient reduction over the stenosis of at least 50%.
  • • Reduction in right ventricular systolic pressure, indicated by a reduced ratio of systolic
  • pressure of LV to RV. In this context, there is no definition in terms of a primary target,
  • as right ventricular pressure reduction depends on a variety of parameters (such as
  • number of stenoses of the entire peripheral pulmonary vasculature, potential
  • additional volume load and right ventricular overall function).
  
  
  

  Original Primary Outcome:
  
  

  Current Secondary Outcome:

  • Secondary outcome measures:
  • The improvement of the right ventricular systolic and diastolic function as well as reduction of
  • ventricle size and of tricuspid valve incompetence are quantified by means of echocardiography,
  • angiography and, where required, nuclear magnetic resonance tomography.
  • An improvement in capability/quality of life is assessed by means of bicycle ergometer
  • examinations as well as the SF-36 (New England Hospital Inc.) and KINDL questionnaires
  • (Ravens-Sieberer & Bullinger) for adults and children respectively.
  • In assessing the organ-related outcome measures, the different diagnostic imaging methods are
  • compared with each other.
  
  
  

  Original Secondary Outcome:
  
  Information By: Competence Network for Congenital Heart Defects
  

  Dates:
  Date Received: December 15, 2005
  Date Started: May 2005
  Date Completion:
  Last Updated: October 14, 2008
  Last Verified: October 2008