Pulmonary Arteriopathy-Diagnostics and Therapy

NCT00266175 | Completed

• 2 other identifiers: MP 101G10210
• Study Type: observational
• Target: 53
• Locations: 1 country
• Sites: 5

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.

Conditions

Pulmonary Stenosis

Keywords

Pulmonary heart disease; Pulmonary hypertension; Stent implantation; Balloon dilatation; Surgery

Outcome Measures

Primary Outcomes (15)

• 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).

Secondary Outcomes (9)

• 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

Eligibility Criteria

• Age: 6 Years+
• Sex: all
• Healthy Volunteers: No
• Age Groups: Child (0-17), Adult (18-64), Older Adult (65+)

You may qualify if:

• 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. -

You may not qualify if:

• 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. -

Sponsors & Collaborators

• Competence Network for Congenital Heart Defects: lead
• German Federal Ministry of Education and Research: collaborator

Study Sites (5)

Medizinische Hochschule Hannover

Hanover, Lower Saxony, D-30625, Germany

Location

Herz-und Diabeteszentrum NRW

Bad Oeynhausen, North Rhine-Westphalia, D-32545, Germany

Location

Deutsches Kinderherzzentrum

Sankt Augustin, North Rhine-Westphalia, D-53757, Germany

Location

Universitätsklinikum des Saarlandes

Homburg, Saarland, D-66421, Germany

Location

Deutsches Herzzentrum Berlin

Berlin, State of Berlin, D-13353, Germany

Location

MeSH Terms

Conditions

Pulmonary Valve Stenosis; Pulmonary Heart Disease; Hypertension, Pulmonary

Condition Hierarchy (Ancestors)

Heart Valve Diseases; Heart Diseases; Cardiovascular Diseases; Ventricular Outflow Obstruction; Lung Diseases; Respiratory Tract Diseases; Hypertension; Vascular Diseases

Study Officials

• Martin Schneider, MD

  Deutsches Kinderherzzentrum

  PRINCIPAL INVESTIGATOR

Study Design

• Study Type: observational
• Time Perspective: PROSPECTIVE
• Sponsor Type: OTHER GOV

Study Record Dates

• First Submitted: December 15, 2005
• First Posted: December 16, 2005
• Study Start: May 1, 2005
• Study Completion: July 1, 2008
• Last Updated: October 15, 2008

Record last verified: 2008-10

Locations

DE (5)