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Pulmonary Hypertension Flashcards

1
Q

What are gold standard diagnostic criteria for pulmonary hypertension?

What are the criteria for breaking down into the subtypes?

A

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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2
Q

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

A

mPAP > 20 + PAWP >15 + PVR ≤2

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3
Q

What are the criteria for pre-capillary pulmonary HTN?

A

mPAP >20 + PAWP ≤15 + PVR >2

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4
Q

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

A

mPAP ≥ 20 + PAWP >15 + PVR >2

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5
Q

What are the criteria for exercise-induced pulmonary HTN?

A

mPAP/CO slope >3 between rest and exercise

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6
Q

How is pulmonary HTN classified by cause?

A

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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7
Q

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

A

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

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

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8
Q

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

A

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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9
Q

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

A

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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10
Q

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

A

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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11
Q

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

A

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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12
Q

What is the prevalence of PH?

A

1%

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13
Q

What is the most common mutation associated with PH?

A

BMPR-2

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

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14
Q

Which CTD has the highest prevalence of PH?

A

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

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15
Q

What is the leading cause of PH?

A

Left heart disease

COPD second

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16
Q

What are the symptoms of PH?

A

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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17
Q

What clinical findings might you see with PH?

A

TR/PR, fluid overload, underlying disease

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18
Q

What is Ortner’s syndrome?

A

Compression of left recurrent laryngeal nerve - causes hoarse voice

19
Q

What features might you see on ECG in PH?

A

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

20
Q

What features might you see on echo in PH?

A
  • 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

21
Q

What might you see on spirometry in PH?

A

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

22
Q

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

A
  • 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
23
Q

Who should be screened for PH?

A

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

24
Q

What is pathophysiology of type 1 PH?

A

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

25
Q

How is PAH treated?

A

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

26
Q

How is type 3 PH treated?

A
  • 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
27
Q

How treat type 4 PH?

A

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

28
Q

What are risk factors for CTEPH?

A

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

29
Q

Does a normal CTPA rule out CTEPH?

A

No. But normal VQ does

30
Q

What are PDE5 inhibitors?

A

Sildenafil, Tadalafil

31
Q

What are enothelin receptor antagonists?

A

Bosentan, Ambisentan, Macitentan

32
Q

What are prostacylcin analogues?

A

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

33
Q

Side effects of endothelium receptor antagonists

A

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

34
Q

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?

A

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

35
Q

What are high risk stratification criteria for patient with PAH?

A

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%

36
Q

What are intermediate risk stratification criteria for patient with PAH?

A

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%

37
Q

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

A

High risk

Nb large = >75% of segment

38
Q

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

A

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

39
Q

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

A

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)

40
Q

What CXR changes might you see in PH?

A

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

effusions very unlikely

41
Q

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

A

Pulmonary veno-occlusive disease (part of group 1)

42
Q

What auscultations findings might you hear in pulmonary hypertension?

A

Loud second heart sound

43
Q

What are plexiform and colander lesions?

A

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

revision (17 decks)

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