Pulmonary Hypertension Flashcards by Katharine Pates

What are gold standard diagnostic criteria for pulmonary hypertension?

What are the criteria for breaking down into the subtypes?

Right heart catheter: mean pulmonary artery pressures (mPAP) >20mmHg

Pre-capillary = + PAWP ≤15 + PVR >2
Isolated post-capillary = +PAWP >15 + PVR ≤2
Combined pre + post = +PAWP >15 + PVR >2
Exercised-PH = mPAP/CO slope >3 between rest and exercise

Pulmonary arterial wedge pressure (PAWP)
Pulmonary vascular resistance (PVR) nb. PVR = (mPAP - PAWP)/CO

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What are the criteria for isolated post-capillary pulmonary HTN?

mPAP > 20 + PAWP >15 + PVR ≤2

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What are the criteria for pre-capillary pulmonary HTN?

mPAP >20 + PAWP ≤15 + PVR >2

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What are the criteria for combine pre and post-capillary pulmonary HTN?

mPAP ≥ 20 + PAWP >15 + PVR >2

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What are the criteria for exercise-induced pulmonary HTN?

mPAP/CO slope >3 between rest and exercise

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How is pulmonary HTN classified by cause?

Group 1-5

1 pulmonary arterial hypertension
- idiopathic
- heritable (BMPR-2 = common mutation)
- associated with drugs/toxins (wt loss drugs, metamphetamines, cancer drugs)
- associated with CTD (particularly Ssc), HIV, portal HTN, congenital heart disease, schistosomiasis
- PAH with features of venous/capillary involvement
- persistent PH of newborn

2 associated with L heart disease
- HF, valvular heart disease, congenital/acquired cardiomyopathy

3 associated with lung disease/hypoxia
- COPD, ILD, hypoventilation (inc OSA with hypoventilation)
- hypoxia without lung disease e.g. altitute

4 associated with pulmonary arterial obstruction
- CTEPH
- Malignancy, arteritis w/o CTD

5 unclear/multifactorial
- sarcoid
- haematological disorders e.g. haemolytic anaemia
- renal failure
- Langerhans

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What are causes of type 1 PH and how what are the diagnostic criteria?

mPAP >20
+ PAWP ≤15 and PVR >2 (as pre-capillary PH)

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What are the causes of type 2 PH and what are the diagnostic criteria?

2 associated with L heart disease
- HF, valvular heart disease, congenital/acquired cardiomyopathy

mPAP >20
+ PAWP >15 and PVR ≤2 (isolated post-cap) or PVR >2 (combine pre and post cap)

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What are the causes of type 3 PH and what are diagnostic criteria?

3 associated with lung disease/hypoxia
- COPD, ILD, hypoventilation (inc OSA with hypoventilation)
- hypoxia without lung disease e.g. altitude

mPAP >20
+ PAWP ≤15 and PVR >2 (as pre-capillary PH)

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What are the causes of type 4 PH and what are the diagnostic criteria?

4 associated with pulmonary arterial obstruction
- CTEPH
- Malignancy, arteritis w/o CTD

mPAP >20
+ PAWP ≤15 and PVR >2 (as pre-capillary PH)

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What are the causes of type 5 PH and what are the diagnostic criteria?

5 unclear/multifactorial
- sarcoid
- haematological disorders e.g. haemolytic anaemia
- renal failure
- Langerhans

mPAP >20
+ PAWP ≤15 and PVR >2 (as pre-capillary PH)

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What is the prevalence of PH?

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What is the most common mutation associated with PH?

BMPR-2

Autosomal dominant with variable penetrance
Accounts for 70% case of heritable PAH

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Which CTD has the highest prevalence of PH?

Systemic sclerosis (5-19%) - screen annually with echo and risk score

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What is the leading cause of PH?

Left heart disease

COPD second

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What are the symptoms of PH?

SOBOE, fatigue, SOB bending forward (bendopnoea), palpitations, haemoptysis, exercise induced abdo distension and nausea, wt gain from fluid retention, syncope during or shortly after exercise

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What clinical findings might you see with PH?

TR/PR, fluid overload, underlying disease

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What is Ortner’s syndrome?

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Compression of left recurrent laryngeal nerve - causes hoarse voice

What features might you see on ECG in PH?

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Signs of R heart dysfunction - p-pulmonale, R axis deviation, RV hypertrophy, RBBB, ST depression/Twave inversion in V1-4 II III avF

What features might you see on echo in PH?

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R heart dysfunction
Raised systolic PA pressure (PASP or sPAP) - measured as TR pressure gradient + estimated RA pressure
–> note this is not prognostic or reflective of response to treatment/disease progression
Peak TR velocity can be used to separate in low/int/high probability of PH

peak TRV ≤2.8 + no other PH signs on echo = low

peak TRV 2.8 - 3.4 + no other signs or ≤2.8 with other signs - int

peak TRV >3.4 or 2.8 - 3.4 with other signs = high

What might you see on spirometry in PH?

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Mild restriction + reduced DLCO (and any underlying lung disease if a cause)

What is vasoreactivity testing in PH? What is considered positive?

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done in idiopathic, heritable or drug-induced PAH.
give iloprost or nitric oxide
positive response is decrease in mPAP by ≥10mmHg to get value ≤40mmHg with increased or unchanged cardiac output
means that can use high dose CCB (nifedipine) to treat

Who should be screened for PH?

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Asymptomatic high risk
- systemic sclerosis
- BMPR2 carriers
- 1 degree relatives of pt with heritable PH
- those undergoing liver tx assessment

Symptomatic at-risk
- portal HTN
- HIV
- non-SSc CTD

What is pathophysiology of type 1 PH?

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Pulmonary vascular remodelling causes increased resistance (high PVR) and RV dysfunction

How is PAH treated?

Test vasoreactivity + use CCB if eligible (re-assess at 3-6 months after starting) If not suitable: + cardiovascular co-morbidites = oral monotherapy with PDE5i or ERA - cardiovascular co-morbidities + low/int risk of risk stratification = PDE5i + ERA + high risk = PDE5i + ERA + IV/s/c PCA F/u at 3 months and risk stratify into 4 groups: - low risk = no change - low-int risk = add ERA or switch PDE5i to cGMP stimulator - int-high risk = add IV or s/c PCA +/- evaluate for lung tx - high risk = as for int-high risk PDE5i (increases NO levels) - Sildenafil/Tadalafil cGMP stimulator (also increases NO) - Riociguat Endothelin antagonist (older = more SEs) - Bosentan/Ambisentan/Macitentan Prostacyclin analogues (PCA) - Prostacyclin/Epoprostenol (IV) - Iloprost (Neb/IV) - Trepostinil (IV/Neb/s/c) - Selesipag (oral) General measures: - exercise - anticoag NOT routine (individual asessment) - diuretics if RH failure - oxygen if pO2<8 - avoid pregnancy - consider iron replacement even without anaemia if levels low

How is type 3 PH treated?

- treat underlying lung disease - exclude VTE, diastolic HF and sleep disorders - PH ass with COPD rarely severe (can get transient rise is PASP during exacerbation), but with ILD can be severe - no evidence for vasodilators - sometimes try PDE5i in very severe ILD + PH - oxygen can prevent vascular remodelling

How treat type 4 PH?

Anticoag + 1st line: pulmonary endarterectomy (surgery) 2nd line/not eligible for surgery: balloon angioplasty 3rd line: PAH therapies surgery 3yr survival 90% vs 70 for balloon

What are risk factors for CTEPH?

Previous PE Young age Large perfusion deficit Idiopathic PE Splenectomy, VA shunt, thyroid disease, malignancy, anti phospholipid syndrome, non-O blood group

Does a normal CTPA rule out CTEPH?

No. But normal VQ does

What are PDE5 inhibitors?

Sildenafil, Tadalafil

What are enothelin receptor antagonists?

Bosentan, Ambisentan, Macitentan

What are prostacylcin analogues?

Protacyclin/Epoprostenol (IV) Iloprost (IV/neb) Trepostinin (IV/neb/s/c) Selesipag (oral)

Side effects of endothelium receptor antagonists

Headache, cutaneous flushing, oedema. Deranged liver function, leading to cirrhosis and failure (avoid if moderate or severe hepatic impairment).

Young man with progressive dyspnoea, no pmx and non-smoker. Bilateral creps. Reduced DLCO. Pre-cap pulmonary HTN on RHC. CT shows enlarged mediastinal nodes and thickened interlobular septa. What mutation is associated with this?

EIF2AK4 - associated with pulmonary veno-occlusive disease. Rare cause of pulmonary venules cause pulmonary HTN

What are high risk stratification criteria for patient with PAH?

Signs of RH failure Rapid progression of symptoms. Repeated syncope. Grade 4 WHO criteria 6MWT < 165m BNP > 800 or NT-BNP > 1100 RA area >26 RA pressure >14 Moderate or large pericardial effusion Venous SATS <60 Cardiac index < 2 Nb 1 year mortality >20%

What are intermediate risk stratification criteria for patient with PAH?

No signs of RH failure Slow progression of symptoms. Occasional syncope. Grade 3 WHO criteria 6MWT 165-440m BNP 50-800 or NT-BNP 300-1100 RA area 18-26 RA pressure 8-14 Minimal pericardial effusion Venous SATS 60-65 Cardiac index 2-2.4 1 year mortality 5-20%

Patient has VQ scan has two large segmental perfusion defects. What is probability of PE?

High risk Nb large = >75% of segment

Patient has VQ scan has no perfusion defects. What is risk of PE?

Low Other findings also classified as low: Non-segmental defect Defect smaller than CXR opacity Perfusion defect small subsegmental (<25% of segment) Two or more matched VQ defects Large pleural effusion

What symptoms would classify someone as high risk (WHO type 4) pulmonary HTN? And how might this affect management of PAH?

SOB at rest or syncope If vasoreactivity negative then would start with PDE5i, ERA and IV or s/c PCA Nb. Positive vaso = decrease mPAP by 10+ to get value <=40 with increased or unchanged CO Nb. PDE5i = sildenafil, tadalafil Riociguat = cGMP = similar to PDE5i ERA (endothelin antagonist) = bosentan, ambisentan, macicentan PCA (prostanoids) = prostacyclin/epoprostenol (IV), iloprost (IV/neb), trepostonil (IV/sc/neb), selesipag (oral)

What CXR changes might you see in PH?

bulky PA on both sides (look like bulky hilar) increased heart size oligaemic lung fields effusions very unlikely

What is the only cause of pulmonary oedema and effusions in pre-capillary pulmonary HTN?

Pulmonary veno-occlusive disease (part of group 1)

What auscultations findings might you hear in pulmonary hypertension?

Loud second heart sound

What are plexiform and colander lesions?

Plexiform - disorganised proliferation of endothelial cells to from capillary-like plexeus of channels seen in PAH Colander - small areas of recanalisation in occluding organised thrombus associated with CTEPH

Pulmonary Hypertension Flashcards

(43 cards)