Functional class in children

Transcription

1 Functional class in children WHO-FC versus Panama Pediatric FC Astrid E Lammers Paediatric Cardiology, Münster University Hospital Astrid.Lammers@ukmuenster.de

2 Disclosures Consulting activity for Actelion No other conflict of interests. 2

3 Why do we need a functional classification? Escalation of therapy Intervention/ Operation Palliative care Hospice 3

4 Why do we need a functional classification? Evaluation of ability of physical performance/ functioning in daily life Comparison of patients to healthy peers Monitoring following a therapeutic intervention ( goal-orientated therapy ) Evaluate prognosis and risk stratification for the individual patient Group patients with comparable status of disease for therapeutic guidance and studies 4

5 Functional class prognosis McLaughlin VV. Circulation 2002;106:

6 Change of functional class Impact on Prognosis Barst RJ. Chest 2013

7 Ploegstra IJC, 184 (2015)

8 Conventional functional classifications NYHA WHO Not very specific and easy to apply particularly in young children 8

9 FC I-IV 5 age groups 9 Lammers AE. Pulm Circ. 2011; 1(2):

10 Introduction of 3 new categories 1. Motorical developmental milestones Regression of already learned motorical abilities 10

11 Panama classification, PVRI pediatric taskforce, Panama Lammers AE. Pulm Circ. 2011; 1(2):

12 Milestones of motorical develoment: 0-15/12 12

13 Introduction of 3 new categories 1. Motorical developmental milestones Regression of already learned motorical abilities 2. Growth and physical development 13

14 Functional class I II III IV

15 ? Functional class I II III IV

16 UK Pulmonary Hypertension Service for Children 39 IPAH on continuous iv epoprostenol IPAH z-score for weight from -1,6-1,2 Moledina, Heart. 2010;96: Lammers AE, Heart 2007;93:

17 Panama classification, PVRI pediatric taskforce, Panama 2011 Lammers AE. Pulm Circ. 2011; 1(2):

18 18 Ploegstra MJ et al. Lancet, Lancet Respir Med 2016; 4:

19 19 Ploegstra MJ et al. Lancet, Lancet Respir Med 2016; 4:

20 Introduction of 3 new categories 1. Motorical developmental milestones Regression of already learned motorical abilities 2. Growth and physical development 3. School/ Kindergarten attendance, participation in sports/pe 20

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23 Panama classification, PVRI pediatric taskforce, Panama 2011 Lammers AE. Pulm Circ. 2011; 1(2):

24 Limitations of Panama classifcation Parameters are not objective Overlap between functional classes School and Kindergarten attendence is dependent on many factors: type of school (inclusion/ special needs) distance to school and mode of transport family structure Actual school attendance difficult to quantify (?diary) Syndromatic children or children with motordevelopmental delay may not be properly reflected in this classification. Not validated - First use in clinical trial Tomorrow Study: PediaTric Use Of Macicentan to Delay Disease progression in PAH Worlwide. 24

25 Discussion for a revised paediatric classification More specific? add height centiles/ z-scores Absolute values or centile change? Define cut-off values? Growth compromise when negative z-score? Severe growth compromise z >-2? Increase in strength of FC by combining other tools reflecting exercise capacity and physical functioning in daily life: e.g accelerometry 25

26 Time for a revision of the Panama classification? 26

27 Peak systo CPET Functional class MRI variables PA compliance/ distensibility 27

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29 Nice

30 Bedankt vor uw aandacht! Contact: