August 2022 ERS/ESC Guidelines Flashcards
Top two causes of PH in overall population
Overall, most common cause of elevated PA pressures
(1) L. sided heart disease
(2) Lung disease, mostly COPD
Top three (in order) most common causes of group I PAH
Group I PAH etiologies
1st- IPAH (idiopathic)
2nd- CTD-related
3rd- Congenital heart disease
4th- Portopulmonary (PoPH)
Prevalence of PH in severe L-sided (mitral, aortic) valvular disease
Very high
-severe, symptomatic mitral valve disease: 60-70% have PH
-about 50% in severe symptomatic aortic stenosis
Key echocardiogrpahic probability of PH
Peak TRV (tricuspid regurg velocity) as the key variable for assigning echocardiographic probability of PH (and not estimated sPAP)
Guideline recommended use of CPET in patients depending on TTE findings
Consider CPET to further determine likelihood of PH in symptomatic patients with intermediate echocardiographic probability of PH- IIb recommendation
Utility of DETECT algorithm
Algorithm to identify asymptomatic systemic sclerosis patients with PAH.
-systemic sclerosis > 3 years with FVC >= 40% and DLCO < 60%
Difference in risk stratification at time of diagnosis vs. during follow-up
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
Guideline rec for when to
(a) refer for transplant eval
(b) list for transplant
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)
Guideline cutoffs for recommendation of ASD closure based on PVR
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
Maneuvers to consider during RHC for patients with borderline PAWP and c/f HFpEF
IIb rec: consider exercise or fluid challenge to uncover post-capillary PH
Change in guidelines for use of diagnostic RHC in patients for suspected PH in patients with lung disease
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
Recommendation for use of inhaled treprostinil
Grade IIb recommendation to consider inhaled treprostinil for pts with PH associated with ILD
2022 definition of exercise PH
(a) Caveat
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
Ratio to differentiate pre and post-capillary causes of exercise PH?
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
Limitation of exercise doppler echocardiography in diagnosis of PH
In most cases increased in sPAP during exercise are 2/2 diastolic LV dysfunction
Strengths/weaknesses of normal perfusion scan for excluding CTEPH
Normal perfusion scan excludes CTEPH with a negative predictive value of 98% in ABSENCE of parenchymal lung disease
Combination of which three parameters on CT chest are highly predictive of PH diagnosis
- PA diameter >= 30mm
- RVOT wall thickness >= 6mm
- Septal deviation >= 140 degrees (or RV/LV ratio >= 1)
TRV cutoffs used for risk stratification of PH
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
When is vasoreactivity testing indicated?
Pts with idiopathic, hereditary, drug-induced PH
Recommended against vasoreactivity testing in pts with suspected etiology NOT idiopathic, hereditary, drug-induced
Contraindications to RHC
-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
Normal values for
(a) sPAP
(b) dPAP
(c) mPAP
Normal values
(a) sPAP 15-30 mmHg
(b) dPAP 4-12 mmHg
(c) mPAP 8-20 mmHg
Normal values for
(a) PVR
(b) TPR
(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/
Mixed venous cutoff to consider compartmental oximetry to exclude intracardiac shunt
If SvO2 > 75% (PA sat over 75%) on room air- do compartmental oximetry to exclude intracardiac shunt
Formula for pulmonary artery compliance
C = V/P
PAC = Stroke volume / (sPAP-dPAP)
Normal value for pulmonary artery compliance
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)
Definition of positive vasoreactivity
Reduction in mPAP >= 10mmHg to reach absolute value <= 40mmHg with increased or unchanged CO
Guideline rec for when to refer for lung transplant
Inadequate response to oral combination therapy indicated by intermediate-high or high-risk or by REVEAL risk score > 7
Guideline rec for when to list for lung transplant
High risk of death or REVEAL >= 10 despite optimized medical therapy
PVR cutoffs in pts with intracardiac shunt where
(a) shunt closure recommended
(b) shunt closure considered
(c) shunt closure not recommended
PVR cutoffs for shunt closure
Guideline first line medication for pts with Eisenmenger syndrome
Bosentan recommended in symptomatic pts with Eisenmenger syndrome to improve exercise capacity
Group III medications: guideline recs
(a) inhaled treprostinil
(b) ambrisentan
(c) riociguat
Guidelines- when to consider PDE5i in pts w. ILD-PH
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
Definition of exercise PH
(a) Pitfall
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)
How idiopathic are subgrouped in 2022 guidelines
Different from 2015- new subgroups in idiopathic differentiated by non-responders vs. acute responders
Why TRV used over sPAP as the key variable for assigning echocardiographic probability of PH
sPAP inaccuracy given inaccuracy of RA pressure estimation and amplification of measurement errors by using derived variables
Describe EKG findings of RVH
Describe RV strain pattern on EKG as seen on this EKG
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
Explain this TTE finding suggestive of elevated PA pressure
RVOT velocity recording
-pulse wave doppler through RVOT (modified PSAX), mid-systolic notching suggests elevated PAP
Negative predictive value of normal V/Q scan for CTEPH
98%
Explain the following TTE parameters in the assessment of PH:
RVOT acceleration time cut off
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
Explain the following TTE parameters in the assessment of PH:
RV fractional area change
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)
Differentiate TAPSE and TDI
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)
How peak TRV is measured
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
How sPASP is calculated on TTE
- RA pressure via IVC size and collapsibility
- RV pressure by TR pressure gradient (dP = 4 x v^2)
v = peak TR jet velocity
EX:
sPAP = TR pressure gradient + estimated RAP
3 CT chest features of PH (not CTEPH but PH more specifically R heart stuff)
Hint: PA/RV stuff not parenchymal
- Enlarged PA:aorta ratio > 0.9, PA diameter > 30mm (3cm)
- RVOT wall thickness > 6mm
- RV:LV ratio >1
Guideline lab workup to send for autoimmune comorbidities
- ANA
- Anti-centromere (CREST)- not the same as anti-SCl 70 (topoisomerase more seen in systemic sclerosis)
- Anti-Ro (SJogrens)
