Patient care— Heart and Lungs Center— Adult congenital heart disease
Heart and Lungs Center
Hart Long Centrum

Adult congenital heart disease

Several studies have been completed on the fate of the right ventricle in patients operated for tetralogy of Fallot using MRI as a gold standard. In cooperation with the AMC, a study is conducted on the optimal timing of pulmonary valve replacement (PVR) in Fallot patients, and the durability of a homograft in pulmonary position.

Detailed measurement of the electrocardiogram has provided new insight into reducing electrical instability in Fallot patients after PVR. These measurements are now being extended to other groups of congenital heart disease.

Pulmonary hypertension in congenital heart disease has become a disease with therapeutical options. Therefore a specific pulmonary hypertension programme has been developed, in cooperation with the departments of Pulmonology, Reumatology and Internal Medicine of the LUMC, and in cooperation with the department of Pulmonology in the VUMC.

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Detailed measurement of the electrocardiogram has provided new insight into reducing electrical instability in Fallot patients after PVR. These measurements are now being extended to other groups of congenital heart disease.

Pulmonary hypertension in congenital heart disease has become a disease with therapeutical options. Therefore a specific pulmonary hypertension programme has been developed, in cooperation with the departments of Pulmonology, Reumatology and Internal Medicine of the LUMC, and in cooperation with the department of Pulmonology in the VUMC.

Main topics in this cluster are:

  • Tetralogy in Fallot;
  • Transposition of the great arteries;
  • Hypertrophic Cardiomyopathy;
  • Arrhythmias in adult congenital heart disease;
  • Pulmonary hypertension.

Clinical trials

PROCAS
Early diagnosis of right ventricular dysfunction in patients operated for tetralogy of Fallot
High dose statins in post-coarctectomy patients
Tetralogy of Fallot
CONCOR Study
Angiotensin II receptor blockers in patients with systemic right ventricles

PROCAS

Prospective randomized trial of the effects of Rosuvastatin on the progression of Stenosis in adult patients with Congenital Aortic Stenosis.

Aim of the study is to determine the effect of the statin rosuvastatin on the progression of stenosis in adult patients with congenital aortic stenosis.

Early diagnosis of right ventricular dysfunction in patients operated for tetralogy of Fallot

In patients who have undergone surgical repair of tetralogy of Fallot, we aim to:

  • Assess the development of right ventricular dimensions and function in relation to clinical outcome, in a time dependent manner.
  • Identify, characterize and quantify markers in blood samples, using high density antibody arrays and ELISA’s, obtained from patients at different stages of the development of right ventricular overload and hypertrophy.
  • Correlate circulating levels of identified markers with cardiac and systemic parameters, and to identify (a) potential biomarker(s) as a diagnostic and prognostic tool(s).

High dose statins in post-coarctectomy patients

Aim of the study is to investigate whether statines reduce atherosclerotic progression in post-coarctectomy patients.

Tetralogy of Fallot

Early diagnosis of right ventricular dysfunction in patients operated for tetralogy of Fallot. The project aims to improve early diagnosis of right ventricular failure during long-term follow-up of patients with congenital heart disease, by integrating information from history, physical examination, imaging technics and blood tests. This will ultimately result in improved guidelines for treatment of potential right ventricular failure, required for prevention of heart failure in patients with tetralogy of Fallot.

CONCOR Study

A national registry and DNA-bank of adults with congenital heart disease in the Netherlands to investigate long term outcome in these patients and to allow investigation of the molecular basis of congenital heart defects.

Angiotensin II receptor blockers in patients with systemic right ventricles

The primary objective of this study is to determine whether the Angiotensin II Receptor Blockers valsartan improves the functional (contractile, electrophysiologic) performance of the right ventricle in adult patients with a systemic right ventricle.

The secondary objectives of this study are to study whether the use of Angiotensin II Receptor Blockers valsartan:

  • changes congestive heart failure
  • changes the prevalence of supra-ventricular arrhythmias
  • changes in right ventricular function, determined by body surface mapping
  • changes the right ventricular volume
  • changes the peak oxygen consumption during exercise
  • changes the serum neurohormone levels
  • changes the quality of life and sport activity
  • changes the cardiac output and microcirculation
  • changes the number of deaths

Relevant departments

Pulmonology
Rheumatology
Internal medicine