August 2022 ERS/ESC Guidelines

Question 1

Top two causes of PH in overall population

Answer 1

Overall, most common cause of elevated PA pressures

(1) L. sided heart disease
(2) Lung disease, mostly COPD

Question 2

Top three (in order) most common causes of group I PAH

Answer 2

Group I PAH etiologies

1st- IPAH (idiopathic)
2nd- CTD-related
3rd- Congenital heart disease
4th- Portopulmonary (PoPH)

Question 3

Prevalence of PH in severe L-sided (mitral, aortic) valvular disease

Answer 3

Very high
-severe, symptomatic mitral valve disease: 60-70% have PH
-about 50% in severe symptomatic aortic stenosis

Question 4

Key echocardiogrpahic probability of PH

Answer 4

Peak TRV (tricuspid regurg velocity) as the key variable for assigning echocardiographic probability of PH (and not estimated sPAP)

Question 5

Guideline recommended use of CPET in patients depending on TTE findings

Answer 5

Consider CPET to further determine likelihood of PH in symptomatic patients with intermediate echocardiographic probability of PH- IIb recommendation

Question 6

Utility of DETECT algorithm

Answer 6

Algorithm to identify asymptomatic systemic sclerosis patients with PAH.
-systemic sclerosis > 3 years with FVC >= 40% and DLCO < 60%

Question 7

Difference in risk stratification at time of diagnosis vs. during follow-up

Answer 7

Risk stratification

At time of diagnosis: three-strata model (low, intermediate, high) based on hemodynamics

At f/u: four-strata model (low, intermediate-low, intermediate-high, and high risk based on WHO-FC, 6MWD, BNP

Question 8

Guideline rec for when to
(a) refer for transplant eval
(b) list for transplant

Answer 8

Grade I Recommendations:

(a) Refer for transplant evaluation: potentially eligible candidates with inadequate response to oral combination therapy = intermediate-high or high-risk or REVEAL risk > 7

(b) List: present with high risk of death or REVEAL >=10 despite optimized medical therapy (including SQ or IV prostacyclins)

Question 9

Guideline cutoffs for recommendation of ASD closure based on PVR

Answer 9

A little wishy-washy especially once PVR above 3

-grade I rec to close ASD/VSD/PDA if PVR < 3
-consider shunt closure of ASD/VSD/PDA if PVR 3-5 and if PVR <5 with PAH treatment
-grade III rec to not close ASD if PVR > 5

Question 10

Maneuvers to consider during RHC for patients with borderline PAWP and c/f HFpEF

Answer 10

IIb rec: consider exercise or fluid challenge to uncover post-capillary PH

Question 11

Change in guidelines for use of diagnostic RHC in patients for suspected PH in patients with lung disease

Answer 11

2015- RHC not recommended for group III unless thought to have therapeutic consequence (clinical trial, c/f group I)

2022- grade I rec for RHC in patients with lung disease and suspected PH

Question 12

Recommendation for use of inhaled treprostinil

Answer 12

Grade IIb recommendation to consider inhaled treprostinil for pts with PH associated with ILD

Question 13

2022 definition of exercise PH

(a) Caveat

Answer 13

mPAP/CO slope between rest and exercise > 3 mmHg/L/min

(a) Caveat- defines an abnormal hemodynamic response to exercise but does not differentiate pre and post-capillary cause

Question 14

Ratio to differentiate pre and post-capillary causes of exercise PH?

Answer 14

Definition of exercise PH: mPAP/CO slope between rest and exercise > 3 mmHg/L/min
^doesn’t differentiate pre and post capillary

“PAWP/CO with threshold > 2 may best differentiate between pre and post capillary causes of exercise PH”
-PAWP/CO < 2 for pts with early pulmonary vascular disease
-HfpEF or dynamic MR and normal PAWP at rest usually show steep increase in mPAP and PAWP (and PAWP/CO slope) during exercise

Question 15

Limitation of exercise doppler echocardiography in diagnosis of PH

Answer 15

In most cases increased in sPAP during exercise are 2/2 diastolic LV dysfunction

Question 16

Strengths/weaknesses of normal perfusion scan for excluding CTEPH

Answer 16

Normal perfusion scan excludes CTEPH with a negative predictive value of 98% in ABSENCE of parenchymal lung disease

Question 17

Combination of which three parameters on CT chest are highly predictive of PH diagnosis

Answer 17

1. PA diameter >= 30mm
2. RVOT wall thickness >= 6mm
3. Septal deviation >= 140 degrees (or RV/LV ratio >= 1)

Question 18

TRV cutoffs used for risk stratification of PH

Answer 18

TRV <= 2.8 m/s without other echo signs of PH = PH low probability

TRV 2.9-3.4 m/s without other echo signs of PH = PH probability intermediate
TRV 2.9-3.4 m/s with other echo signs of PH = PH probaility high

TRV > 3/4 m/s regardless of presence of other echo PH signs = PH probability high

Question 19

When is vasoreactivity testing indicated?

Answer 19

Pts with idiopathic, hereditary, drug-induced PH

Recommended against vasoreactivity testing in pts with suspected etiology NOT idiopathic, hereditary, drug-induced

Question 20

Contraindications to RHC

Answer 20

-acute infection
-mechanical R heart valve or triclip (for tx of TR)
-tumor or thrombus in RA or RV
-recently implanted (less than 1 month) pacemaker

Question 21

Normal values for

(a) sPAP
(b) dPAP
(c) mPAP

Answer 21

Normal values

(a) sPAP 15-30 mmHg
(b) dPAP 4-12 mmHg
(c) mPAP 8-20 mmHg

Question 22

Normal values for

(a) PVR
(b) TPR

Answer 22

(a) PVR = (mPAP - PCWP) / CO, normal 0.3-2.0 WU
(b) TPR = mPAP / CO, normal < 3 WU

WU x 80 ~~ Dynes/s/cm^-5
(a) Normal PVR = 0.3-2.0 WU = 30-160 Dynes/
(b) Normal TPR < 3 WU < 240 Dynes/

Question 23

Mixed venous cutoff to consider compartmental oximetry to exclude intracardiac shunt

Answer 23

If SvO2 > 75% (PA sat over 75%) on room air- do compartmental oximetry to exclude intracardiac shunt

Question 24

Formula for pulmonary artery compliance

Answer 24

C = V/P

PAC = Stroke volume / (sPAP-dPAP)

Question 25

Normal value for pulmonary artery compliance

Answer 25

PA compliance = stroke volume / (sPAP-dPAP) Normal value: RV (and LV) stroke volume: 60-100ml Normal PAC > 2.3 ml/mmHg (>3 some say, <2 is def bad)

Question 26

Definition of positive vasoreactivity

Answer 26

Reduction in mPAP >= 10mmHg to reach absolute value <= 40mmHg with increased or unchanged CO

Question 27

Guideline rec for when to refer for lung transplant

Answer 27

Inadequate response to oral combination therapy indicated by intermediate-high or high-risk or by REVEAL risk score > 7

Question 28

Guideline rec for when to list for lung transplant

Answer 28

High risk of death or REVEAL >= 10 despite optimized medical therapy

Question 29

PVR cutoffs in pts with intracardiac shunt where (a) shunt closure recommended (b) shunt closure considered (c) shunt closure not recommended

Answer 29

PVR cutoffs for shunt closure

Question 30

Guideline first line medication for pts with Eisenmenger syndrome

Answer 30

Bosentan recommended in symptomatic pts with Eisenmenger syndrome to improve exercise capacity

Question 31

Group III medications: guideline recs (a) inhaled treprostinil (b) ambrisentan (c) riociguat

Answer 31

Question 32

Guidelines- when to consider PDE5i in pts w. ILD-PH

Answer 32

New in 2022: consider PDE5i in pts with severe ILD-PH (IIb, level C evidence) but not recommended to use PDE5i in pts with ILD and non-severe PH

Question 33

Definition of exercise PH (a) Pitfall

Answer 33

mPAP / CO slope > 3 -so need to exercise, repeat mPAP and thermodilution CO measurements -concept: mPAP rises out of proportion to increase in CO (a) Doesn't differentiate pre and post capillary cause of elevated mPAP (also positive in HFpEF/post-capillary causes)

Question 34

How idiopathic are subgrouped in 2022 guidelines

Answer 34

Different from 2015- new subgroups in idiopathic differentiated by non-responders vs. acute responders

Question 35

Why TRV used over sPAP as the key variable for assigning echocardiographic probability of PH

Answer 35

sPAP inaccuracy given inaccuracy of RA pressure estimation and amplification of measurement errors by using derived variables

Question 36

Describe EKG findings of RVH

Answer 36

Question 37

Describe RV strain pattern on EKG as seen on this EKG

Answer 37

ST depressions and T-wave inversions in R precordial leads (V1-V4) and inferior leads (II, III, aVF) Other features of RVH present on this EKG: dominant R wave in V1, R axis deviation

Question 38

Explain this TTE finding suggestive of elevated PA pressure

Answer 38

RVOT velocity recording -pulse wave doppler through RVOT (modified PSAX), mid-systolic notching suggests elevated PAP

Question 39

Negative predictive value of normal V/Q scan for CTEPH

Answer 39

98%

Question 40

Explain the following TTE parameters in the assessment of PH: RVOT acceleration time cut off

Answer 40

RVOT acceleration time- pulse wave doppler through high PSAX. Higher the PA pressure, slower acceleration time (b/c more after load) RVOT AT < 105ms and mid-systolic notch indicative of pre-capillary PH

Question 41

Explain the following TTE parameters in the assessment of PH: RV fractional area change

Answer 41

RV fractional area change, change in area of the RV from diastole to systole, normal > 35% change < 35% change suggestive of PH (elevated PA pressures)

Question 42

Differentiate TAPSE and TDI

Answer 42

TAPSE- M-mode through lateral tricuspid annulus to measure wall movement, abnormal < 18mm TDI- decreased movement of tricuspid valve annulus muscle during systole (< 9.5 cm/s is abnormal)

Question 43

How peak TRV is measured

Answer 43

Peak tricupid valve regurgitation (preferred value for assigning TTE probability of PH) measured by continuous doppler through tricuspid valve Under x-axis (away from probe) will see flow- take peak velocity of this. Then used modified bernoullis (dP = 4 x v^2) to estimate pressure gradient from max velocity

Question 44

How sPASP is calculated on TTE

Answer 44

1. RA pressure via IVC size and collapsibility 2. RV pressure by TR pressure gradient (dP = 4 x v^2) v = peak TR jet velocity EX: sPAP = TR pressure gradient + estimated RAP

Question 45

3 CT chest features of PH (not CTEPH but PH more specifically R heart stuff) Hint: PA/RV stuff not parenchymal

Answer 45

1. Enlarged PA:aorta ratio > 0.9, PA diameter > 30mm (3cm) 2. RVOT wall thickness > 6mm 3. RV:LV ratio >1

Question 46

Guideline lab workup to send for autoimmune comorbidities

Answer 46

1. ANA 2. Anti-centromere (CREST)- not the same as anti-SCl 70 (topoisomerase more seen in systemic sclerosis) 3. Anti-Ro (SJogrens)