congenital heart disease
Conditions
Interventions
None listed
Sponsors
Erasmus MC, Universitair Medisch Centrum Rotterdam
Eligibility
Age
2 Years to 99 Years
Inclusion criteria
Inclusion criteria: Prospective as well as cross-sectional patient-based study. A total of 400 patients will be included, with 1 of following diagnoses: - tetralogy of Fallot (ToF), - atrial septal defect (ASD), - univentricular heart - pulmonary arterial hypertension (PAH). ;These patients will be selected from 2 groups of patients: ;1) Patients with a recent indication for cardiac intervention a. with a recent diagnosis fitting the inclusion criteria, primarily infants and (young) children scheduled for surgical correction / palliation or heart catherization (target: 100 patients ( 4 diagnostic categories)). b. with an indication for re-intervention (target 100 patients ( 4 diagnostic categories)). ;2) Patients at mid- to long-term after intervention a. that have had previous systematic evaluation in an earlier research project (Dutch Heart Foundation (DHF) 2006B026 (ToF)/ DHF 2008B026 (ToF)/ WAKF 2007 (ToF)/ DHF 2008B095 (Fontan) / PhDLUMC2009 (ToF, Fontan, ASD) and pulmonary hypertension research UMCG). b. additional patients with similar diagnoses to provide balanced numbers between the groups (target 200 patients in category 2a and b ( 4 diagnostic categories)). ;In patients from group 1 changes in outcome parameters prior to and after intervention (surgery, catheter intervention, medical) will be assessed. In patients from group 2a changes in these parameters between current and available baseline measurements will be assessed. The different groups of ConHD will be age-matched which will allow identification of factors (in blood and myocardium) related to maintenance of homeostasis, in relation to type of diagnosis, residual loading abnormality, clinical state and age. ;Ad group 1: These patients will be studied prior to intervention and at regular time points until approximately 1 year after intervention (no longer because of time restrictions imposed by the format of the funding grant). ;Ad group 2: In category 2a data that is highly relevant for the current project of a total of approximately 400 patients with a diagnosis of ToF, ASD,Fontan or PAH, more than 1 year after intervention is already available. This data includes detailed information on medical history, past and current clinical status, ECG, regional ventricular function and ejection fraction (using TDi and speckle tracking echocardiography), metabolic exercise testing and of MRI-derived RV and LV volumes, wall mass and ejection fraction (at rest and with stress) and stored blood samples.
Exclusion criteria
Exclusion criteria: patients with mental retardation, patients who have contra-indications for exercise testing, patients with contra-indications for MRI.
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| 1. Right and/or left ventricular or single ventricular end-systolic volume indexed to body surface area (ml/m2 BSA) (patients > 7 years of age) 2. 2. Maximal oxygen uptake (adjusted for age, gender and weight) (children > 5 years of age, adults) (in VSD with PAH, ASD II, Fallot en Fontan patients not in PAH patients) | — |
Secondary
| Measure | Time frame |
|---|---|
| a. Maximal work load (patients > 5 years of age)(in VSD, ASD II, Fallot en Fontan patients) / walking distance in 6 min (only PAH patients) b. Right and/or left ventricular or single ventricular ejection fraction, c. Regional right and/or left ventricular or single ventricular strain / strain rate, d. NT-pro-BNP levels in blood | — |
Countries
Netherlands
Outcome results
None listed