Congenital heart disease Tetralogy of Fallot (TOF) Atrial septal defect (ASD) Univentricular heart Pulmonary arterial hypertension In het Nederlands: Aangeboren hartafwijkingen, Tetralogie van Fallot, atrium septum defect, pulmonale hypertensie, univentriculair hart.
Conditions
Interventions
All study procedures in patients in group 1 and 2 will be part of normal clinical follow-up and are explained in the subheading ‘timepoints’
Sponsors
Erasmus Medical Centre Rotterdam
Eligibility
Inclusion criteria
Inclusion criteria: In order to be eligible to participate in this study, a subject must meet all of the following criteria: Patients fitting the inclusion diagnoses (see 4.1) will be selected from 2 groups of patients: Group 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 start of medical therapy (target: 100 patients (divided among the 4 diagnostic categories)). b. with an indication for re-intervention or introduction of additional medical therapy (target 100 patients (divided among the 4 diagnostic categories)). Group 2) Patients at mid- to long-term after intervention a. children and adults 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 (divided among the 4 diagnostic categories)). Patients in category 2 will undergo repeat evaluation or new evaluation mid- to long-term after an intervention. Main objective of this study is to validate favorable factors that stimulate maintenance of myocardial homeostasis as well as harmful factors related to impending failure, as identified in the studies related to this project.
Exclusion criteria
Exclusion criteria: A potential subject who meets any of the following criteria will be excluded from participation in this study: For patients: - Patients with mental retardation, - Patients who have contra-indications for exercise testing, - Patients with contra-indications for MRI.
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| -Right and/or left ventricular or single ventricular end-systolic volume indexed to body surface area (ml/m2 BSA) (patients > 7 years of age) -Maximal oxygen uptake (adjusted for age, gender and weight) (children > 5 years of age, adults) (not in PAH patients) | — |
Secondary
| Measure | Time frame |
|---|---|
| - Maximal work load (in VSD, ASD, Fallot and Fontan patients) / walking distance in 6 min (only PAH patients). (adjusted for age, gender and weight) (if age permits). - Right and/or left ventricular or single ventricular ejection fraction. - Regional right and/or left ventricular or single ventricular strain / strain rate. - NT-pro-BNP levels in blood. | — |
Contacts
Public ContactW.A. Helbing
Erasmus Medical Centre — Sophia Children’s Hospital, Department of Paediatric Cardiology, Sp-2429
Outcome results
None listed