Nick H. Kim, MD, Richard N. Channick, MD, and Vallerie V. McLaughlin, MD, prepared useful Practice Aids pertaining to pulmonary hypertension for this CME activity titled "Pulmonary Hypertension at the Crossroads of Current Clinical Challenges and Novel Therapeutic Strategies." For the full presentation, monograph, complete CME information, and to apply for credit, please visit us at http://bit.ly/2O9QbOh. CME credit will be available until July 30, 2019.

1. Access the activity,“Pulmonary Hypertension at the Crossroads of Current Clinical Challenges
and Novel Therapeutic Strategies,”at www.peerview.com/CQK40.
REVEAL Registry Simplified
PAH Risk Score Calculator1
PRACTICE AID
This Practice Aid has been provided as a quick reference to help learners apply the information to their daily practice and care of patients.
APAH: associated pulmonary arterial hypertension; BNP: brain natriuretic peptide; CTD: connective tissue disease; DLCO: diffusing capacity of the lungs for carbon monoxide; FPAH: familial pulmonary
arterial hypertension; HR: heart rate; mRAP: mean right atrial pressure; NYHA: New York Heart Association; PAH: pulmonary arterial hypertension; PoPH: portal hypertension; PVR: pulmonary vascular
resistance; SBP: systolic blood pressure.
1. Benza RL et al. Chest. 2012;141:354-362.
For each variable, points are added to or
subtracted from the total score as follows:
2 points: Indicating >2-fold change in risk of
mortality
1 point: Indicating smaller but statistically
significant change in risk of mortality
0 points: If unmeasured or not falling above
or below the relevant threshold
Total risk scores, ranging from 0 (lowest
risk) to 22 (highest risk), are correlated with
predicted 1-year survival
Demographics and
Comorbidities
NYHA/WHO
Functional Class
Vital Signs
6-Minute
Walk Test
BNP
Echocardiogram
Pulmonary
Function Test
Right Heart
Catheterization
APAH-CTD
I
APAH-PoPH
III
FPAH
IV
Males age >60 y
HR >92 beats/min-1
DLCO ≤32% predicted
PVR >32 Wood
units
<165 m
>180 pg/mL-1
Pericardial effusion
Renal insufficiency
SBP <110 mmHg
DLCO ≥80% predicted
mRAP >20 mmHg
with 1 year
≥440 m
<50 pg/mL-1
+1
+1
+1
+1
-1
-2
-1
+1
+1
+1
+1
+1
+2
+1
-2
+2
+2
+2
+2
6
= Risk Score
Sum of Above
+
PAH Risk Score
Low Risk Average Risk
Moderate/
High Risk
High Risk Very High Risk
Risk score 1-7 8 9 10-11 ≥12
Predicted 1-year
survival
95% - 100% 90% - <95% 85% - <90% 70% - <85% <70%
WHO Group I
Subgroup

2. 2015 ESC/ERS Guidelines for the Diagnosis
and Treatment of Pulmonary Hypertension:
Diagnostic Algorithm1
Access the activity,“Pulmonary Hypertension at the Crossroads of Current Clinical Challenges
and Novel Therapeutic Strategies,”at www.peerview.com/CQK40.
PRACTICE AID
This Practice Aid has been provided as a quick reference to help learners apply the information to their daily practice and care of patients.
a
CT pulmonary angiography alone may miss diagnosis of chronic thromboembolic pulmonary hypertension.
CHD: congenital heart disease; CTD: connective tissue disease; CTEPH: chronic thromboembolic pulmonary hypertension; DLCO: diffusing capacity of the lungs for carbon monoxide; ERS:
European Respiratory Society; ESC: European Society of Cardiology; HRCT: high-resolution computed tomography; mPAP: mean pulmonary arterial pressure; PA: pulmonary angiography;
PAH: pulmonary arterial hypertension; PAWP: pulmonary artery wedge pressure; PFT: pulmonary function test; PH: pulmonary hypertension; PVOD/PCH: pulmonary veno-occlusive disease/
pulmonary capillary hemangiomatosis; PVR: pulmonary vascular resistance; RHC: right heart catheterization; RV: right ventricular; V/Q: ventilation/perfusion.
1. Galiè N et al; ESC Scientific Document Group. Eur Heart J. 2016;37:67-119.
Symptoms, signs, history suggestive of PH
Consider left heart disease and lung
diseases by symptoms, signs, risk factors,
ECG, PFT + DLCO, chest radiograph and
HRCT, arterial blood gases
Consider other causes
and/or follow-up
Diagnosis of left heart diseases
or lung diseases confirmed?
V/Q scana
Mismatched perfusion defects?
Refer to PH
expert center
PAH likely
Specific diagnostic tests
No signs of severe
PH/RV dysfunction
Treat underlying
disease
CTEPH possible:
CT PA, RHC ± PA
RHC
mPAP ≥25 mmHg,
PAWP ≤15 mmHg,
PVR >3 Wood units
Signs of severe
PH/RV dysfunction
Refer to PH
expert center
Consider other
causes
Group 5
CTD
Drugs – Toxin
HIV
CHD
Idiopathic
PVOD/PCH
Idiopathic
PAH
Heritable
PAH
Heritable
PVOD/PCH
Porto-
pulmonary
Schistosomiasis
Echocardiographic probability of PH
High/intermediate
Yes Yes
Yes
Yes
No
No
No
Low

3. Access the activity,“Pulmonary Hypertension at the Crossroads of Current Clinical Challenges
and Novel Therapeutic Strategies,”at www.peerview.com/CQK40.
2015 ESC/ERS Guidelines for the Diagnosis
and Treatment of Pulmonary Hypertension:
Treatment Algorithm for CTEPH1
PRACTICE AID
This Practice Aid has been provided as a quick reference to help learners apply the information to their daily practice and care of patients.
a
Technically operable patients with non-acceptable risk/benefit ratio can be considered for BPA.
b
In some centers, medical therapy and BPA are initiated concurrently.
BPA: balloon pulmonary angioplasty; CTEPH: chronic thromboembolic pulmonary hypertension; ERS: European Respiratory Society; ESC: European Society of Cardiology; PH: pulmonary hypertension.
1. Galiè N et al; ESC Scientific Document Group. Eur Heart J. 2016;37:67-119.
Diagnosis confirmed by
CTEPH expert center
Lifelong anticoagulation
Operability assessment by a
multidisciplinary CTEPH team
Technically operable Technically non-operable
Non-acceptable
risk/benefit ratioa
Acceptable
risk/benefit ratio
Pulmonary
endarterectomy
Persistent
symptomatic PH
Targeted medical
therapy
Consider BPA in
expert centerb
Consider lung
transplantation
Persistent severe
symptomatic PH

4. Access the activity,“Pulmonary Hypertension at the Crossroads of Current Clinical Challenges
and Novel Therapeutic Strategies,”at www.peerview.com/CQK40.
2015 ESC/ERS Guidelines for the Diagnosis
and Treatment of Pulmonary Hypertension:
Treatment Algorithm for PAH1
PRACTICE AID
This Practice Aid has been provided as a quick reference to help learners apply the information to their daily practice and care of patients.
a
Some WHO-FC III patients may be considered high risk.
b
Initial combination with ambrisentan + tadalafil has proven to be superior to initial monotherapy with ambrisentan or tadalafil in delaying critical failure.
c
IV epoprostenol should be prioritized as it has reduced the 3-month rate for mortality in high-risk patients with PAH also as monotherapy.
d
Also consider balloon atrial septostomy.
CCB: calcium channel blocker; DPAH: drug-induced pulmonary arterial hypertension; ERS: European Respiratory Society; ESC: European Society of Cardiology; HPAH: heritable pulmonary arterial
hypertension; IPAH: idiopathic pulmonary arterial hypertension; PAH: pulmonary arterial hypertension; PCA: prostacyclin analogues; WHO FC: World Health Organization functional class.
1. Galiè N et al; ESC Scientific Document Group. Eur Heart J. 2016;37:67-119.
PAH confirmed by
expert center
Acute vasoreactivity test
(IPAH/HPAH/DPAH only)
CCB therapy
Initial oral
combinationb
Initial
monotherapyb
Initial combination
including IV PCAc
Double or triple sequential combination
Consider listing for lung transplantationd
Inadequate clinical response
Treatment-naïve
patient
Patient already
on treatment
General measures
Supportive therapy
Vasoreactive
Non-vasoreactive
Inadequate clinical response
Consider referral for
lung transplantation
Low/intermediate risk
(WHO FC II-III)a
High risk
(WHO FC IV)a