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Dunbar Ivy, MDThe Children’s Hospital Heart Institute
University of Colorado School of Medicine
Paediatric PAH in the current era
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Dunbar Ivy, MDThe Children’s Hospital Heart Institute
University of Colorado School of Medicine
Paediatric PAH in the current era
& A Gap Analysis
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Disclosures
I have the following financial relationships with the
manufacturer(s) of any commercial product(s) and/or
provider(s) of commercial services discussed in this CME
activity:
• The University of Colorado contracts with Actelion,
Bayer, Lilly, and United Therapeutics for which I am a
consultant
• UC Contract: Steering Committee Actelion / Bayer / Lilly
/ United Therapeutics
• Funding from NIH / FDA
I do intend to discuss an unapproved/investigative use of a
commercial product/device in my presentation.
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Outline
• Definition and Classification
• Epidemiology
• CHD
• BPD
• Survival
• Guidelines for Treatment of PAH
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5
Pulmonary Arterial Hypertension
Definition and Classification
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5th WSPH Consensus Definitions:Right-heart Catheterization Confirmed
Hoeper MM, et al. J Am Coll Cardiol. 2013;62:D42-50.
Pulmonary Hypertension (PH)
Mean pulmonary artery pressure (mPAP) ≥25 mm Hg
Pulmonary Arterial Hypertension (PAH)
Mean pulmonary artery pressure (mPAP) ≥25 mm Hg
and
Mean pulmonary artery wedge pressure (PAWP) ≤15 mm Hg
PH Hemodynamic Definition
Pulmonary vascular resistance (PVR) >3 Wood units
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5th WSPH: Classification of Pulmonary
Arterial Hypertension by Etiology
● Group 1 Pulmonary Arterial Hypertension (PAH)- Idiopathic (IPAH)
- Heritable (HPAH)- BMPR2
- ALK-1, Endoglin, SMAD9, CAV1, KCNK3
- Unknown
- Drugs and toxins induced
- Associated with• Connective Tissue Diseases
• HIV Infection
• Portal Hypertension
• Congenital Heart Diseases
• Schistosomiasis
• Group 1’ Pulmonary Veno Occlusive Disease (PVOD) and/or Pulmonary
Capillary Hemangiomatotis (PCH)
• Group 1” Persistent pulmonary hypertension of the newborn (PPHN)
Simonneau G, et al. J Am Coll Cardiol. 2013;62:D34-41.
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5th WSPH: Classification of Pulmonary
Arterial Hypertension by Etiology
● Group 1 Pulmonary Arterial Hypertension (PAH)- Idiopathic (IPAH)
- Heritable (HPAH)- BMPR2
- ALK-1, endoglin, SMAD9, CAV1, KCNK3, TBX4
- Unknown
- Drugs and toxins induced
- Associated with• Connective Tissue Diseases
• HIV Infection
• Portal Hypertension
• Congenital Heart Diseases
• Schistosomiasis
• Group 1’ Pulmonary Veno Occlusive Disease (PVOD) and/or Pulmonary
Capillary Hemangiomatotis (PCH) EIF2AK4
• Group 1” Persistent pulmonary hypertension of the newborn (PPHN)
Simonneau G, et al. J Am Coll Cardiol. 2013;62:D34-41.
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5th WSPH: Clinical Classification of Other
Forms of Pulmonary Hypertension• Group 2 -- Pulmonary Hypertension Due to Left Heart Disease
– Left Ventricular Systolic Dysfunction
– Left Ventricular Diastolic Dysfunction
– Valvular disease
– Congenital / acquired left heart inflow / outflow tract obstruction
• Group 3 -- Pulmonary Hypertension Due to Lung Diseases and/or
Hypoxia
– Chronic obstructive pulmonary disease
– Interstitial lung disease
– Other pulmonary diseases with mixed restrictive and obstructive
pattern
– Sleep-disordered breathing
– Alveolar hypoventilation disorders
– Chronic exposure to high altitude
– Developmental lung disease
• Group 4 – Chronic Thromboembolic Pulmonary Hypertension (CTEPH)
Simonneau G, et al. J Am Coll Cardiol. 2013;62:D34-41.
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5th WSPH: Clinical Classification of Other
Forms of Pulmonary Hypertension• Group 2 -- Pulmonary Hypertension Due to Left Heart Disease
– Left Ventricular Systolic Dysfunction
– Left Ventricular Diastolic Dysfunction
– Valvular disease
– Congenital / acquired left heart inflow / outflow tract obstruction
• Group 3 -- Pulmonary Hypertension Due to Lung Diseases and/or
Hypoxia
– Chronic obstructive pulmonary disease
– Interstitial lung disease
– Other pulmonary diseases with mixed restrictive and obstructive
pattern
– Sleep-disordered breathing
– Alveolar hypoventilation disorders
– Chronic exposure to high altitude
– Developmental lung disease
• Group 4 – Chronic Thromboembolic Pulmonary Hypertension (CTEPH)
Simonneau G, et al. J Am Coll Cardiol. 2013;62:D34-41.
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5th WSPH: Developmental Lung Diseases
Associated With Pulmonary Hypertension
• Congenital diaphragmatic hernia
• Bronchopulmonary dysplasia
• Alveolar capillary dysplasia (ACD)
• ACD with misalignment of veins
• Lung hypoplasia (“primary” or “secondary”)
• Surfactant protein abnormalities
– SPB deficiency
– SPC deficiency
– ATP-binding cassette A3 mutation
– Thyroid transcription factor 1/Nkx2.1 homeobox mutation
• Pulmonary interstitial glycogenosis (PIG)
• Pulmonary alveolar proteinosis (PAP)
• Pulmonary lymphangiectasia
Ivy DD, et al. J Am Coll Cardiol. 2013;62:D117-D126.
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5th WSPH: Clinical Classification of Other
Forms of Pulmonary Hypertension
Group 5 – Pulmonary Hypertension with Unclear Multifactorial Mechanisms
• Hematologic disorders: chronic hemolytic anemias, myeloproliferative
disorders, splenectomy
• Systemic disorders: Sarcoidosis, pulmonary Langerhans cell histiocytosis,
Lymphangioleiomyomatosis, neurofibromatosis, vasculitis,
Down Syndrome
• Metabolic disorders: Glycogen storage disease, Gaucher disease, thyroid
disorders
• Others: Segmental PAH, tumoral obstruction, fibrosing mediastinitis,
chronic renal failure
Simonneau G, et al. J Am Coll Cardiol. 2013;62:D34-41.
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Multifactorial Etiology in Pediatric
Pulmonary Hypertensive Vascular Disease
Del Cerro, Abman, Diaz, Freudenthal, Harikrishnan, Ivy, Stenmark, Adatia.
Pulm Circ. 2011;1:286-298.
31%
Multifactorial
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Maeno, Y. V. et al. Circulation 1999;99:1209-1214
In utero pulmonary vascular disease:
Transposition of the Great Arteries
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The broad schema of 10 basic categories of
Pediatric Pulmonary Hypertensive Vascular Disease
1. Prenatal or developmental pulmonary hypertensive vascular disease
2. Perinatal pulmonary vascular maladaptation
3. Pediatric cardiovascular disease
4. Bronchopulmonary dysplasia
5. Isolated pediatric pulmonary hypertensive vascular disease (isolated
pediatric PAH)
6. Multifactorial pulmonary hypertensive vascular disease in congenital
malformation syndromes
7. Pediatric lung disease
8. Pediatric thromboembolic disease
9. Pediatric hypobaric hypoxic exposure
10. Pediatric pulmonary vascular disease associated with other system
disorders
Del Cerro, Abman, Diaz, Freudenthal, Harikrishnan, Ivy, Stenmark, Adatia.
Pulm Circ. 2011;1:286-298.
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Pulmonary Hypertension
Epidemiology
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Van Loon RL, et al. Circulation. 2011;124:1755-1764.
Annual Incidence Rates for Pediatric
PAH90
80
70
60
50
40
5
4
3
2
1
0
An
nu
al
incid
en
t cases p
er
millio
n
1991 1992 1993 1994 1995 1996 1997 1998 1999 2000 2001 2002 2003 2004 2005
Year of diagnosis
total PH
transient PAH
progressive PAH
PAH-CHD
iPAH
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Van Loon RL, et al. Circulation. 2011;124:1755-1764.
Annual Incidence Rates for Pediatric
PAH90
80
70
60
50
40
5
4
3
2
1
0
An
nu
al
incid
en
t cases p
er
millio
n
1991 1992 1993 1994 1995 1996 1997 1998 1999 2000 2001 2002 2003 2004 2005
Year of diagnosis
total PH
transient PAH
progressive PAH
PAH-CHD
iPAH
BPD?
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Pulmonary Arterial Hypertension
Survival
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Natural History of IPAH: NIH Registry1,2
NIH = National Institutes of Health.
Predicted survival according to the NIH equation. Predicted survival rates were 69%, 56%, 46%, and 38% at 1, 2, 3,
and 4 years, respectively. The numbers of patients at risk were 231, 149, 82, and 10 at 1, 2, 3, and 4 years,
respectively. *Patients with primary pulmonary hypertension, now referred to as idiopathic pulmonary hypertension.
1. Rich et al. Ann Intern Med. 1987;107:216-223. 2. D’Alonzo et al. Ann Intern Med. 1991;115:343-349.
69%
56%
46%
38%
Predicted survivalPerc
ent
surv
ival
Years
Median survival: 2.8 years (n=194)
Pediatric median survival: 0.8 years (n=16)
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21
Survival in the Current Era Using Off
Label Therapy (Denver/Dutch/NY)
Zijlstra, et al. J Am Coll Cardiol. 2014:63(20), 2159–69
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22
Del Cerro, et al. Am J Respir Crit Care Med 2014;190:1421–1429
Survival by WSPH Group
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Pulmonary Arterial Hypertension
CHD-PAH
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5th WSPH: Classification of Pulmonary
Arterial Hypertension by Etiology
Simonneau G, et al. J Am Coll Cardiol. 2013;62:D34-41.
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Children classified according to the Nice-
CHD-classification
Denver Dutch New York
• Co-
morbidities(n=60/134)
– Down (30)
– Noonan (3)
– DiGeorge (1)
– Turner (1)
– Robinow (1)
– VACTERL (1)
– Undefined Syndrome
(3)
– Lung (8)
– HHT/GSD/ABCA3Zijlstra W, et al. Pulm Circ 2016;6(3):302-312.
• Non-classifiable
– TAPVR
– Scimitar/ IPADO
– TGA +/- VSD
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Pulmonary Arterial Hypertension
Lung Disease Associated PH
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Pulmonary Arterial Hypertension
BPD
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Survival in BPD-related PAH
Khemani E, et al. Pediatrics. 2007;120;1260-1269.
N=42 premature infants with BPD-related PAH
0
0.1
0.2
0.3
0.4
0.5
0.6
0.7
0.8
0.9
1.0
Cu
mu
lati
ve
Su
rviv
al
(%)
Months after Diagnosis of PAH
0
PAH not severe
Severe PAH
6 363012 18 24
34
2113
6 4
0
0.1
0.2
0.3
0.4
0.5
0.6
0.7
0.8
0.9
1.0
Pro
ba
bil
ity o
f S
urv
iva
l
0 6 363012 18 24
Months after Diagnosis of PAH
6
4
2 1
44915
24
10
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29
Pulmonary Vein Stenosis
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Pulmonary vein stenosis of ex‐premature infants with pulmonary hypertension and
bronchopulmonary dysplasia, epidemiology, and survival from a multicenter cohort
Pediatr Pulmonol. 2017 Aug;52(8):1063-1070
N=39
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Pulmonary Arterial Hypertension
Guidelines for Treatment of PAH
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LOWER RISK DETERMINANTS OF RISK HIGHER RISK
No Clinical evidence of RV failure Yes
No Progression of Symptoms Yes
No Syncope Yes
Growth Failure to thrive
I,II WHO Functional Class III,IV
Minimally elevated SBNP / NTproBNPSignificantly elevated
Rising level
Echocardiography
Severe RV
enlargement/dysfunction
Pericardial Effusion
Systemic CI > 3.0 L/min/m2
mPAP/mSAP < 0.75
Acute Vasoreactivity
Hemodynamics
Systemic CI < 2.5 L/min/m2
mPAP/mSAP > 0.75
RAP > 10mmHg
PVRI > 20 WU*m2
WSPH 2013 - Consensus Pediatric IPAH/HPAH Treatment AlgorithmHigher risk vs. Lower Risk Factors
Ivy, et al. J Am Coll Cardiol. 2013;62:D117-26
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Echocardiographic Predictors
Kassem E, Humpl T, and Friedberg M. Am Heart J
2013;165(6):1024-1031
Increased risk of
transplant or death
1. FAC <15.5%
2. TAPSE z-score <-4.3
3. RVED z-score >4.8
4. RVESi <18.4
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Treatment goals in Paediatric
Pulmonary Arterial Hypertension
34Ploegstra, et. al., European Respiratory Journal 2014 44: 1616-1626
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3D Echocardiographic Evaluation of Right Ventricular Function and
Strain: a Prognostic Study in Paediatric Pulmonary Hypertension
Eur Heart J Cardiovasc Imaging. Published online August 25, 2017. doi:10.1093/ehjci/jex205
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3D Echocardiographic Evaluation of Right Ventricular Function and
Strain: a Prognostic Study in Paediatric Pulmonary Hypertension:
2014-2016
Eur Heart J Cardiovasc Imaging. Published online August 25, 2017. doi:10.1093/ehjci/jex205
All patients (n = 96) Event free (n = 78) With event (n = 18)
Death, Transplant, IV Prostacyclin, PAH hospitalization, Potts, BAS
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3D Echocardiographic Evaluation of Right Ventricular Function and
Strain: a Prognostic Study in Paediatric Pulmonary Hypertension
Eur Heart J Cardiovasc Imaging. Published online August 25, 2017. doi:10.1093/ehjci/jex205
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3D Echocardiographic Evaluation of Right Ventricular Function and
Strain: a Prognostic Study in Paediatric Pulmonary Hypertension
Eur Heart J Cardiovasc Imaging. Published online August 25, 2017. doi:10.1093/ehjci/jex205
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No
General: Consider Diuretics, Oxygen, Anticoagulation,
Digoxin
Improved / Sustained reactivity
Oral CCB
Continue CCB
Yes
Negative
Lower Risk Higher Risk
Acute Vasoreactivity Testing
Reassess considerearly combo-therapy
Positive + > 1 y.o.
Expert Referral
Lung Transplant
Epoprostenol or
Treprostinil (IV/SQ)
Consider Early Combination
ERA or PDE-5i (oral)
Potts Shunt ?
Atrial septostomy
ERA or PDE-5i (oral)
Treprostinil (oral/inhaled)
Iloprost (inhaled)
Selexipag