Pulmonary Arteriopathy-Diagnostics and Therapy

Brief Title

Pulmonary Arteriopathy-Diagnostics and Therapy

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 Description

      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
           stenoses. The stents have the advantage of high radial forces that can be offset against
           the elastic recoil of the vessels occurring after sole dilatation. Furthermore,
           constrictions that are caused by either kinking of vessels or their compression by
           neighbouring structures can be dilated sufficiently by the use of stents.

        -  With respect to the interconnectedness of all the projects of the Competence Network for
           Congenital Heart Defects, one essential aspect is the standardisation of diagnostic
           methods of evaluating right ventricular function. The data obtained during the study are
           to provide the basis for the development of diagnostic guidelines.

        -  Concerning evaluation, particular attention is paid to the patients' self-assessment
           with respect to their exercise tolerance. In this context, it is a matter of interest to
           what degree the patients' subjective maximum stress and therefore their quality of life
           correlate with the objective measurement parameters. The anticipated results are of
           direct clinical significance for the patients, as they aim at medium to long-term relief
           of the right ventricle, which may effect a decrease in morbidity and therefore an
           improvement of the quality of life. This is of particular importance with regard to the
           fact that there will be a continuously increasing number of patients with congenital
           heart defects reaching adulthood.
    


Study Type

Observational


Primary Outcome

Primary outcome measure:

Secondary Outcome

 Secondary outcome measures:

Condition

Pulmonary Stenosis



Publications

* Includes publications given by the data provider as well as publications identified by National Clinical Trials Identifier (NCT ID) in Medline.

Recruitment Information



Estimated Enrollment

53

Start Date

May 2005

Completion Date

July 2008


Eligibility Criteria

        Inclusion Criteria:

        Patients with native or postoperative peripheral pulmonary stenoses (PPS; any stenoses that
        are located distally to the RVOT). Definition of PPS: 40% lumen constriction, or 30%
        constriction if volume stress is also present. Informed consent of the patient or his/her
        legal representative is given after instruction. -

        Exclusion Criteria:

        Patients unable to undergo MRI or spiroergometry for physical or psychological reasons.

        Pregnant or nursing patients. Patients affected by other clinically relevant diseases
        (malignant tumours, infectious diseases, metabolic disorders etc.). Patients with known
        intolerance of contrast media. Patients with syndromal diseases such as Alagille's
        syndrome, rubella embryopathy or elfin face syndrome. -
      

Gender

All

Ages

6 Years - N/A

Accepts Healthy Volunteers

No

Contacts

Martin Schneider, MD, , 

Location Countries

Germany

Location Countries

Germany

Administrative Informations


NCT ID

NCT00266175

Organization ID

MP 1

Secondary IDs

01G10210


Study Sponsor

Competence Network for Congenital Heart Defects

Collaborators

 German Federal Ministry of Education and Research

Study Sponsor

Martin Schneider, MD, Principal Investigator, Deutsches Kinderherzzentrum


Verification Date

October 2008