Clinical Features and Ultrasound Parameters in Pulmonary Arterial Hypertension, in Pediatric Cardiology

Author:Gáspár Hanga

1

Scientific coordinators:

Prof. Dr. Rodica TogănelDr. Carmen Șuteu

Introduction

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• Mean pulmonary artery pressure at rest

mPAP > 25 mmHg

• Pulmonary vascular resistance PVR > 3 Wood units (or 240 dyness/s/cm5)

• Pulmonary capillary wedge

pressure PCWP < 15 mmHg

Introduction Objective Material and method Results Conclusion

• Incidence: 1-2/ 1.000.000• Progressive disease• Vascular remodeling with luminal obliteration of small vessels

•Pulmonary vasoconstriction and thrombosis (endothelial dysfunction: imbalance favoring vasoconstriction)

•Pulmonary vascular inflammation and remodelling: intimal proliferation, medial thickening

• Injury to endothelial cells

• Right ventricular hypertrophy (compensatory)

Objective

3 Kórtörténet Fizikális vizsgálat Paraklinikum Differenciál

diagnózis Kezelés Eredmények

Evaluation and 1-year follow-up of clinical manifestationsand ultrasound parametersof children suffering from PAH,receiving Bosentan, Sildenafil or combination therapy.

Introduction Objective Material and method Results Conclusion

BPD

Post-o

pera

tive

PAH

Eise

nmen

ger s

yndr

ome

Idiopa

thic

PAH

0.00

2.00

4.00

6.00

8.00

Average agePeriod

• January 2008 -March 2013

41 children enrolled

• 21 from Târgu Mureș Emergency Institute for Cardiovascular Diseases and Transplantation

• 20 from the National Institute of Cardiology Gottsegen György, Budapest

Gender distribution

• 22 male:19 female• 53.7: 46.3 (%)

Material and method

Age

• children aged between 4 months-15 years • mean age: 5.16 years

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Material and method

Introduction Objective Material and method Results Conclusion

symptoms

functional class

results of the six-minute walk test

heart ultrasound parameters

Doppler echocardiography

Right heart catheterization

Acute vasodilator test

Diagnosis of PAH

Assessed at enrollment and one year layer:

Clinical classification* based on aethiology

6 Kórtörténet Fizikális vizsgálat Paraklinikum Differenciál

diagnózis Kezelés Eredmények

Idiopathic PAH

Post-operative PAH

Eisenmenger syndrome

Bronchopul-monary dys-plasia

4 groups created based on the causative disease*according to the Dana Point classification

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Disease targeted therapy

Introduction Objective Material and method Results Conclusion

BPD Post-operative Eisenmenger syndrome

Idiopathic0%

10%

20%

30%

40%

50%

60%

70%

80%

90%

100%

Bosentan Sildenafil Bitherapy

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Results

Introduction Objective Material and method Results Conclusion

Poor growth

Cyanosis

Dyspnea

Hepatomegaly

Fatigue

Recurrent respiratory infections

Hippocratic fingers

Tachypnea

Cough

Peripheral edema

Diaphoresis

Clinical symptoms

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Functional class

Introduction Objective Material and method Results Conclusion

Change in NYHA class

deteriorated improved stable• right- and biventricular hypertrophy had developed in 1-1 patient respectively

• 1 patient developed congestive cardiac insufficiency

• no syncopal episodes

Functional classification is strongly predictive of mortality, and is an important factor in the choice of PAH therapy.

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6-Minute Walk Test

Introduction Objective Material and method Results Conclusion

EisenmengerIPAH

75

80

85

90

95

Saturation at start

Eisen-menger IPAH

0

2

4

6

8

Drop in oxygen satu-ration

M0Y1

EisenmengerIPAH

0

100

200

300

400

500

Distance walked

M0Y1

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Heart ultrasound

Introduction Objective Material and method Results Conclusion

BPD Post OP ES IPAH0

10

20

30

40

50

60

70

M0Y1

-40.00

-35.00

-30.00

-25.00

-20.00

-15.00

-10.00

-5.00

0.00

5.00

10.00

-9.65

-36.50

6.39 8

mPAP (%)

The increment or decrement of mPAP values in first year of therapy

Mean pulmonary artery pressure

improved in the BPD and the Eisenmenger groups

worsened in the post-operative PAH and iPAH group

Mean pulmonary artery pressure - a major diagnostic criteria

iPAH

Post-op

ES BPD

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Tricuspid Annular Plane Systolic Excursion

Introduction Objective Material and method Results Conclusion

BPD Post OP ES IPAH0

2

4

6

8

10

12

14

16

18

20

M0Y1

TAPSE (mm)

Increment or decrement in TAPSE values (%)

TAPSE –a useful and easily measured parameter in the evaluation of the right ventricle

-15.00

-10.00

-5.00

0.00

5.00

10.00

15.00

20.00

25.00

Idiopathic PAH

Post-operative PAH

Eisenmenger syndrome

BPD

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Parameters with high prognostic value

Introduction Objective Material and method Results Conclusion

BPD Post OP ES IPAH0

5

10

15

20

25

M0Y1

0.00

5.00

10.00

15.00

20.00

25.00

30.00

35.00

40.00 39.87

5.36

21.29

37.93

Idiopathic PAH

Post-operative PAH

Eisenmenger syndrome

Bronchopul-monary dys-plasia

Right atrial area increased in all subgroups

Right atrial area (cm2)

Right atrial area enlargement (%)

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Parameters with high prognostic value

Introduction Objective Material and method Results Conclusion

0.0

5.0

10.0

15.0

20.0

25.0

Pericardial effusion (%)

Idiopathic PAHPost-operative PAHEisenmenger syndromeBPD

The relative presence of pericardial effusion in each subgroup

• iPAH: 1 patient,showing a worsening tendency

•Post-op PAH: 3 patients, with stable or improving tendencies

•Eisenmenger: 2 patients, 1 improving, 1 worsening

•BPD: absent

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Conclusions

Introduction Objective Material and method Results Conclusion

Bosentan and Sildenafil are efficient in improving:

• life quality• functional status (improvement in NYHA class)• exercise capacity (significant increment in distance walked)But do not significantly improve

cardiac status:

• right atrial area increased in all groups• pericardial effusion, if present, persisted or worsened• left ventricular eccentricity index did not present significant changes

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Conclusions

Introduction Objective Material and method Results Conclusion

BPD group

• despite their serious condition, PAH patients with bronchopulmonary dysplasia displayed improvement of most ultrasound parameters

Post-operative PAH group

• patients presented lowering tendencies only in pulmonary pressures

Differences in disease progression can be noted.

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Conclusions

Introduction Objective Material and method Results Conclusion

increased saturation and less pronounced drop during the walk test

increasing TAPSE values, opposed to the decreasing tendency noted in iPAH

prognosis is not as poor as in iPAH

Eisenmenger patients react better to treatment than those suffering from idiopathic PAH:

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References

Personal contribution:

Professor Michael A. Gatzoulis -Pulmonary Arterial Hypertension, Oxford University Press, Great Britain, 2012

Robyn J. Barst –Pulmonary Arterial Hypertension: Diagnosis and Evidence-Based Treatment, Great Britain, 2008

ATS statement: guidelines for the six-minute walk test, Am J Respir Crit Care Med, 2002, 166: 111-7.

Simonneau G, Robbins I, Beghetti M, et al. Updated clinical classification of pulmonary hypertension. J Am Coll Cardiol 2009;54:S43-S54.

Rubin LJ. Primary pulmonary hypertension. N Engl J Med 1997; 336: 111-7

Shafazand S, et al. Health-related quality of life in patients with pulmonary arterial hypertension. Chest 2004; 126: 1452-9.

McLaughlin VV, et.al. Prognosis of pulmonary arterial hypertension. Chest. 2004;126:78S-92S

Badesch DB, Abman SH, et.al. Medical therapy for pulmonary arterial hypertension. Chest. 2004;126:35S-62S

Initiative to expand patient spectrum

Data gathering and processing

Assisting patient follow-up

Statistics Presentation

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Thank you for your kind attention!