Pulmonary stenosis

In many cases of complex congenital heart defects, the right heart muscle has to “work harder” to deliver blood through poorly functioning pulmonary vessels. The study group on pulmonary stenoses aims to develop diagnosis guidelines and new forms of treatment for peripheral pulmonary stenoses. The aim is to treat right ventricular dysfunction in good time by reducing pulmonary artery stenoses.

Patients with congenital heart defects frequently develop problems in the blood vessels leading to the lungs immediately after birth. These vessels may be too narrow or abnormally underdeveloped (stenosis or hypoplasia). Despite corrective surgery, further constriction of the pulmonary vessels may occur as the child grows older and the body matures. This natural growth places an increased burden on the right ventricle of the heart muscle.

The study investigates the extent to which the increased burden of stenosis affects cardiac output of the right ventricle. The evaluation is based on a set of observations not only obtained through the standard non-invasive examinations used in all primary research projects but also covering the various forms of therapy. These include both interventional types, such as balloon dilation and stent implantation, and surgical types. The organ of principal interest in this study is the right ventricle. This allows all relevant stenoses (graft stenoses, peripheral pulmonary stenoses) downstream from this organ to also be evaluated and treated as part of the same study.

This multi-centric study is being carried out in partnership with numerous clinics in Germany that provide medical care for patients with congenital heart defects. The results from each clinic and research group are entered in a central database and evaluated jointly by the members of the network – a more efficient way of obtaining high-quality research findings.

A detailed diagnosis is prepared during the patient’s first visit, including heart catheterisation in addition to measurements of right ventricle function and verification of the suspected increased burden. Following this set of examinations, an individual programme of treatment is defined to eradicate the stenosis and thereby reduce the burden on the right ventricle. A follow-up examination, performed one year later using the same standard methods, provides documented evidence of the outcome of the chosen therapy and its effect on right ventricular function.

The choice of treatment is based solely on medical necessity. Because this is a purely observational study, it does not set out to prove any specific hypothesis. All patients included in this study receive cardiac catheter examinations and interventional therapy.

The recruitment of patients for the study closed on 31 March 2007.

A number of clinics and heart centres all over Germany are taking part in the study. An up-to-date list of these participants can be found here …