Pulmonary HTN Flashcards
Pulmonary Hypertension is defined by what?
mPAP > 20 mmHg
Pre-capillary PH is defined as?
mPAP > 20 mmHG
PAWP </= 15 mmHg
PVR > 2WU
Isolated Post-capillary PH is defined as?
mPAP > 20 mmHg
PAWP > 15 mmHg
PVR </= 2 WU
Combined pre and post capillary PH is defined as?
mPAP > 20 mmHg
PAWP > 15 mmHg
PVR > 2 WU
Exercise PH is defined as?
mPAP/CO slope between rest and exercise > 3 mmHg/L/min
Risk Factors for PH
age > 65 years old
Left Heart Disease
COPD
CHD
Some infectious diseases (schistosomiasis, HIV)
high altitude
Clinical Presentation of PH
Early
Dyspnea on Exertion
Fatigue and rapid exhaustion
Dyspnea when bending forward
Palpitations
Hemoptysis
Exercise-induced abdominal distension and nausea
Weight gain d/t fluid retention
Syncope (during or shortly after physical exertion
Clinical Presentation of PH
Late
Exertional chest pain: dynamic compression of the left main coronary artery
Hoarseness (dysphonia): compression o fthe left laryngeal recurrent nerve)
SOB, wheezing, cough, lower respiratory tract infection, atelectasis: compression of the bronchi
The cardinal symptom of PH is
dyspnea on progressively minor exertion
Signs of PH
Central, peripheral or mixed cyanosis
accentuated pulmonary component of the second heart sound
RV third heart sound
Systolic murmur of the tricuspid regurgitation
Diastolic murmur of pulmonary regurgitation
Signs of RV backward failure
Distended and pulsating jugular veins
Abdominal distention
Hepatomegaly
Ascites
Peripheral edema
Signs pointing towards underlying cause of PH
Digital Clubbing
Sclerodactyly
Raynaud’s Phenomenon
digital ulceration
GERD
Signs pointing towards underlying cause of PH
Digital Clubbing
Cyanotic CHD, fibrotic lung disease, bronchiectasis, PVOD, or liver disease
Signs pointing towards underlying cause of PH
Differential clubbing/cyanosis
PDA/Eisenmenger’s syndrome
Signs pointing towards underlying cause of PH
Auscultory findings
crackles or wheezing
murmurs
Lung or heart disease related
Signs pointing towards underlying cause of PH
Sequelae of DVT, venous insufficiency
CTEPH
Signs pointing towards underlying cause of PH
Telangiectasia
Hereditary Hemorrhagic Telangiectasia or systemic sclerosis
Signs pointing towards underlying cause of PH
Sclerodactyly, Raynaud’s Phenomenon, digital ulceration, GORD
systemic sclerosis
Signs of RV forward failure
Peripheral cyanosis (blue lips and tips)
Dizziness
Pallor
Cool Extremities
Prolonged Cap refill
Typical ECG abnormalities in PH
P pulmonale (P > 0.25 mV in lead II)
R or sagittal axis deviation
RV hypertrophy (R/S > 1, w/ R > 0.5 mV in V1; R in V1 + S in lead V5 > 1mV)
RBBB
RV strain pattern (ST depression/ T-wave inversion in the right pre-cordial V1-4 and inferior II, III, aVF)
Prolonged QTc
Radiographic Signs of PH and concomitant abnormalities
R heart enlargement
PA enlargement
Pruning of the peripheral vessels
‘Water-bottle’ shape of cardiac silhouette
Radiographic Signs of LHD/Pulmonary Congestion
Central air space opacification
Interlobular septal thickening ‘Kerley B’ lines
Pleural effusions
LA enlargement
LV dilation
Radiographic signs of lung disease
Flattening of diaphragm (COPD/Emphysema)
Hyperlucency (COPD/Emphysema)
Lung volume loss (fibrotic lung disease)
Reticular opacification (fibrotic lung disease)
PFT findings in patients w/ PAH
usually normal or may show mild restrictive, obstructive or combined abnormalities.
More severe PFT abnormalities are occasionally found in patients w/ ?
PAH associated w/ CHD
A severely reduced Diffusing Capacity for Carbon Monoxide (<45% of predicted value) in the presence of otherwise normal PFTs can ve found in what patients?
PAH w/ Systemic Sclerosis
Patients w/ PAH have what PaO2 levels?
slightly reduced or normal
Severe reduction of PaO2 in PAH might raise suspicion of what?
patent foramen ovale
hepatic disease
other abnormalities w/ R-to-L shunt (e.g. septal defect)
low-DLCO- associated conditions
Patients w/ PAH have what PaCO2 levels?
typically lower than normal d/t hyperventilation
Elevated PaCO2 is very unusual in PAH and reflects what?
What is recommended in this case?
alveolar hypoventilation, which may be a cause of PAH in itself
Overnight oximetry or polysomnography for suspicion of sleep d/o breathing or hypoventilation
Echo Signs of RV dysfunction on four chamber view
Dilated RV
Enlarged RA area (> 18 cubic cm)
Presence of pericardial effusion
Echo Signs of RV dysfunction on parasternal long axis view
Enlarged RV
Echo Signs of RV dysfunction on parasternal short axis view
Flattened interventricular septum leading to “D-shaped” LV
Presence of pericardial effusion
Echo Signs of RV dysfunction on subcostal view
Distended inferior vena cava w/ diminished inspiratory collapsibility
Presence of pericardial effusion
Other Imaging that can help diagnose PH
Non-con CT
CTPA
Dual Energy CT
Digital Subtraction Angiography
Cardiac MRI
What is the gold standard for diagnosing and classifying PH
R heart cath
Normal Hemodynamic Measures obtained during R heart cath
R atrial pressure?
systolic PAP?
diastolic PAP?
mean PAP?
PAWP?
CO
SvO2
SaO2
Systemic BP
2-6 mmHg
15-30 mmHg
4-12 mmHg
8-20 mmHg
</= 15 mmHg
4-8 L/min
65-80%
95-100%
120/80
Calculated Parameters obtained during R heart cath
PVR
PVRI
TPR
CI
SV
SVI
PAC
0.3 - 2.0 WU
3-3.5 WUm2
< 3 WU
2.5-4 L/min m2
60-100 mL
33-47mL/m2
<2.3 mL/mmHg
Non-pharmacologic recommendations for PAH
Physical activity and supervised rehab w/n symptom limits
Vaccines for PAH
Covid-19
influenza
Streptococcus Pneumoniae
When are diuretics recommended in patients w/ PAH?
With signs of RV failure and fluid retention
When is long term O2 therapy recommended in patients w/ PAH?
PaO2 < 60 mmHg
What is suggested in patients w/ iron-deficiency anemia?
correction of iron status
What medications are not recommended in patients with PAH unless required by comorbidities such as HTN, CAD, L Heart Failure, or arrhythmias?
ACEis
ARBs
ARNIs
SGLT-2is
beta blockers
ivabradine
If a patient w/ PH undergoes R heart cath w/ pulmonary vasoreactivity testing and has >/= 10mmHg mPAP drop from baseline to </= 40 mmHg w/ increased or unchanged CO, what is the suggested therapy?
When to reasses?
CCB and titrate to optimized individual dose
3-6 months
How to reassess response to CCB for PAH?
If reassessment shows good response, when to reassess again?
WHO-FC I/II
BNP < 50 ng/L or NT-proBNP < 300 ng/L
normal or near normal resting hemodynamics
6-12 months
WHO Classification
Patients with PH but without resulting limitation of physical activity. Ordinary physical activity does not cause undue dyspnoea or fatigue, chest pain, or near syncope
WHO-FC I
WHO Classification
Patients with PH resulting in slight limitation of physical activity. They are comfortable at rest. Ordinary physical activity causes undue dyspnoea or fatigue, chest pain, or near syncope
WHO-FC II
WHO Classification
Patients with PH resulting in marked limitation of physical activity. They are comfortable at rest. Less than ordinary activity causes undue dyspnoea or fatigue, chest pain, or near syncope
WHO-FC III
WHO Classification
Patients with PH with an inability to carry out any physical activity without symptoms. These patients manifest signs of right HF. Dyspnoea and/or fatigue may even be present at rest. Discomfort is increased by any physical activity
WHO-FC IV
CCB’s used to treat PAH
include starting dose and target dose
Amlodipine start
Diltiazem start
Felodipine start
Nifedipine start
Amlodipine Starting dose for PAH?
Target dose for PAH?
5 mg once daily
15-30 mg once daily
Diltiazem Starting dose for PAH?
Target dose for PAH?
60 mg BID
120-360 mg BID
Felodipine Starting dose for PAH?
Target dose for PAH?
5 mg once daily
15-30mg once daily
Nifedipine Starting dose for PAH?
Target dose for PAH?
10 mg TID
20-60 mg BID or TID
Endothelin receptor antagonists
Ambrisentan
Bosentan
Macitentan
Ambrisentan Starting dose for PAH?
Target dose for PAH?
5 mg once daily
10 mg once daily
Bosentan Starting dose for PAH?
Target dose for PAH?
62.5 mg BID
125 mg BID
Macitentan Starting dose for PAH?
Target dose for PAH?
10 mg once daily
10 mg once daily
Phosphodiesterase 5 inhibitors
Sildenafil
Tadalafil
Sildenafil Starting dose for PAH?
Target dose for PAH?
20 mg TID
20 mg TID
Tadalafil Starting dose for PAH?
Target dose for PAH?
20 or 40 mg once daily
40 mg once daily
Prostacyclin analogues (oral admin)
Beraprost sodium
Beraprost extended release
Treprostinil
Prostacyclin receptor agonists
selexipag
Soluble guanylate cyclase stimulators
Riociguat
Proscacyclin analogues (inhaled admin)
Iloprost
Treprostinil
Prostacyclin analogues (iv or sc admin)
epoprostenol iv
Treprostinil sc
What is the recommended treatment for in patients w/ IPAH, HPAH, or DPAH who are responders to acute vasoreactivity testing?
CCBs
What is the recommended treatment for patients w/ IPAH, HPAH, or DPAH in WHO-FC I or II w/ marked hemodynamic improvement
Continue high doses of CCBs
Initiating PAH therapy is recommended in patients who?
remain in WHO-FC III or IV or those w/o marked hemodynamic improvement after high doses of CCBs
What is recommended initial treatment for patients w/ IPAH/HPAH/DPAH and are non-vasoreactive, who present at low or intermediate risk of death?
Combination therapy w/ a PDE5i and an ERA is recommended
What is the recommended initial oral drug combo for patients w/ IPAH/HPAH/DPAH w/o cardiopulmonary comorbidities?
Combination of ambrisentan and tadalafil
Combination of macitentan and tadalafil
What is recommended to base treatment escalations on?
risk assessment and general treatment strategies
The addition of what to PDE5is or oral/inhaled PCA is recommended to reduce the risk of morbidity/mortality events?
macitentan
The addition of what to EERAs and/or PDE5is is recommended to reduce the risk of morbidity/mortality events?
selexipag
The addition of what to ERA or PDE5is/riociguat monotherapy is recommended to reduce the risk of morbidity/mortality?
treprostinil
The addition of what to epoprostenol is recommended to improve exercise capacity
sildenafil
When managing patients with right HF in the ICU, it is recommended to?
Involve physicians w/ expertise, treat causative factors and use supportive measures, including inotropes and vasopressors, fluid management and PAH drugs as appropriate
A combination fo what is recommended to diagnose PAH w/ signs of venous and/or capillary involvement (PVOD/PCH)a
clinical and radiological findings, ABG, PFTs and genetic testing