Why? Children are confident with the pediatric team, why should - - PowerPoint PPT Presentation

4/21/2018 1

Pediatric Pulmonary Hypertension

The transition in Paris (France) Marilyne Levy

Why?

• Children are confident with the pediatric team, why should

they change?

• Most pediatric cardiologist in France are also cardiologist

and could follow them as grown ups

• Risk of deterioration : less confidence at a difficult age-

break in FU or treatment (only 48% of adolescents with CHD

underwent successful transition)

• BUT in case of deterioration they will need an adult unit

Campbell F et al. Cochrane Database Syst Rev. 2016 Sable et al. Circulation. 2011;123:1454-1485.)

At what age?

WHO : 10 - 19 yrs old

Early adolescence refers to 10–14 years, late adolescence to 15–19 years, and young adulthood to 20–24 years. Adolescence is characterised by dynamic brain development in which the interaction with the social environment shapes the capabilities an individual takes forward into adult life

Lancet commission 2016 : 10-24 yrs old

The American College of Cardiology task force and the recent “ACC/AHA Guidelines on the Management of Adults With Congenital Heart Disease” recommended that the transition process start at 12 years of age to prepare the patient for transfer to adult care.

AHA recommandations

Sable et al. Circulation. 2011;123:1454-1485

Pediatric « cocooning » Child + parents + doctor

• At diagnosis : difficult moments spent together
• Initiation of the treatment, regular visits
• Minimal invasive explorations (Cath at diagnosis and in

case of deterioration)

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Catheterization in children

Repeat cardiac catheterization is recommended within 3 to 12 months after initiation of therapy to evaluate response or with clinical worsening (Class I; Level of Evidence B). Serial cardiac catheterizations with AVT are recommended as follows:

• a. Serial cardiac catheterizations should be done

during follow-up to assess prognosis and potential changes in therapy (Class I; Level of Evidence B)

• b. Intervals for repeat catheterizations should be

based on clinical judgment but include worsening clinical course or failure to improve during treatment (Class I; Level of Evidence B). Cardiac catheterization is recommended before initiation

• f PAH-targeted therapy (Class I; Level of

Evidence B). Exceptions may include critically ill patients requiring immediate initiation of empirical therapy (Class I; Level of Evidence B). Cardiac catheterization should include acute vasoreactivity testing (AVT) unless there is a specific contraindication (Class I; Level of Evidence A). Abman S et al. Circulation. 2015;132:2037-2099.

To adult doctors

• Yesterday a child with parents
• Today « almost » an adult but

still with parents…. The « adult » doctors are not used to..

• Patient already treated but by

another team

• New evaluation with cath in
• rder to adapt the treatment

The initial experience

• First patients did not want to

return to the adult department

From initial experience up to now

• Among 79 « adolescent »

patients

• 31 refused the transition

program

• 7 were referred for

transplantation

• 34 in discussion….
• 7 died (one should have been

transferred before but transition program was delayed)

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From initial experience up to now

• Many patients refused to

return to adult clinics

• Common staff in the pediatric

department to present the case to the adult doctors

From initial experience up to now

• Patients refused to return to

adult clinics

• Common staff to present the

case to the adult doctors

• First consultation in the adult

PH department with the pediatric team

The new team

• First consultation around 16-

18 >> adult

• Different approach
• Hemodynamic evaluation

more often (serial catheterizations intervals 4- 12months)

Soizig: adult team at 17

• 2010-2015 : stable with sildenafil FC 1-2 - Cath at diagnosis (mPAP 65) and in 2012

(mPAP 46mmHg)

• Transition feb 2015 aged 17 : Stable FC till 2016 with sildenafil but increased PAP at

echo

• Cath (Jul 2016): mPAP 86 - CI 4 - PVR 14; NT-Pro BNP 730 : Bitherapy
• Cath (Fev 2017) : mPAP 79 - CI 4.1 - PVR 12.3 - NT-ProBNP 700 : + Selexipag - FC 2
• Cath (Oct 2017) : mPAP 76 - CI 3.3 - PVR 14.3 - NT-ProBNP 715 : switch to SC Trepro
• Cath (Feb 2018) : mPAP 85 - CI 4.4 - PVR 12.2 - NTProBNP 1000: switch to IV Prosta
• still in FC 2 but dropped out of university because of treatment
• Cath (Mai 2018) : STABLE FC but hemodynamic data unchanged : EVERY 4 MONTHS

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Soreya adult team at 18

• Diagnosis at 13 syncope, chest pain
• Cath 1 (2009) : 59/39-m49 and 28 after NO
• CCB Flush ++++ switch to sildenafil - FC 1 and stopped the treatment at 18,

syncope

• Transition at 18 (jan 2015): Cath2 PAP 81/41-m59 - IC 3.4 - PVR 8.4 AFTER NO :

PAP 57/26-m38 - IC 3.7 - PVR 6

• CCB low doses (flush, nausea, headache) : FC 1 - low PAP at echo
• KT 3 (jan 2016) : PAP 51/25-m36 - IC 3.9 -PVR 3.7
• Dec 2017 : FC 1 - NTProBNP normal, good echo parameters
• STABLE clinicat and hemodynamically : EVERY YEAR

Catheterization in adults

Galiè et al. Eur Respir J 2015; 46: 903–975

What do the parents feel?

• More technical, less listening
• « I have the feeling that the new team doesn’t know what

has been done previously »

• Different doctors during hospitalization
• Explanations but only catheterization is taken into account

What do the patients feel?

• More technical
• Too many KT
• More agressive treatments
• Less dialogue
• « I miss the cocooning » of your team
• We were known from everyone, now we are anonymous,

we feel a « number »

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Paris aftermath today

• 20 treated children were referred to the adult department
• 7 transferred for transplantation
• 2 refused to return
• 11 succeeded
• 6 complain but know they have no other option
• Patients who were transferred when deteriorating complained the

most

• All but one think the approach was very different

Why?

• Children are confident with the pediatric team, why should

they change?

• Most pediatric cardiologist in France are also cardiologist

and could follow them as grown ups

• Risk of deterioration : less confidence at a difficult age-

break in FU or treatment (only 48% of adolescents with CHD

underwent successful transition)

• BUT in case of deterioration they will need an adult

unit

Campbell F et al. Cochrane Database Syst Rev. 2016 Sable et al. Circulation. 2011;123:1454-1485.)

Conclusion

• Preparing the patient and the family early enough
• Transition in a stable clinical situation
• Idealy the patient should meet the « adult » doctor in

the pediatric department

• First visit in the adult department with the pediatric

team

• Periode of transition alternation between adult and

pediatric consultation (1year?)

Thanks

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