Respiratory Flashcards

Question

How might you differentiate PJP from TB in HIV-positive patients with low CD4 counts?

Answer 1

TB often associated with more severe constitutional symptoms eg fever, night sweats, malaise Chest imaging may range from being clear to miliary pattern Note as immunity declines, so too does the frequency of pulmonary cavitation which is the hallmark of pulmonary TB

Answer 2

NTM tends to present as disseminated disease without respiratory symptoms or pulmonary involvement (especially in cases of MAC) in HIV -> quite different from how NTM presents in immunocompetent patients with bronchiectasis

Answer 3

Both may present with fever, cough and diffuse interstitial infiltrates Both may have elevated beta-glucan levels Findings suggestive of histoplasmosis include: - adenopathy - hepatosplenomegaly - oral or mucosal ulcerations (diagnosis made with histoplasmosis antigen testing)

Answer 4

Quite often they are indistinguishable in patients with HIV and low CD4 counts Toxoplasmosis is less common Can identify responsible organism in BAL fluid

Answer 5

No CMV less common - but similar presentation - definitive diagnosis of CMV requires observing CMV inclusion bodies on biopsy Influenza more acute onset with myalgias and headaches cf PJP more subacute COVID-19 often have more peripheral distribution and lower lobe involvement on CT than PJP but nothing pathognomonic

Answer 6

Skin findings commonly present in Kaposi sacroma and absent in PJP - however 20% of patients with KS may have no evidence of cutaneous disease - diagnosis (gold standard) consists of direct visualisation of characteristic lesions on bronchoscopy - failing that, nuclear scans may help differentiate KS from PJP

Answer 7

No, it is very rare and only usually in very advanced HIV or those receiving second-line PJP prophylaxis like dapsone or aerosolised pentamidine Most often involves the eye - typically the choroid layer

Answer 8

B - Prone positioning for 12-16 hours per day Prone positioning >12hr / day shown to reduce mortality Complications: increased rates of pressure sores and ET obstruction The rest haven't been shown to reduce mortality Supportive care with mechanical ventilation remains the cornerstone of ARDS management. * Mechanical ventilation itself can cause and potentiate lung injury * Mixed evidence, but overall research suggests up to a 30% reduction in mortality risk with low tidal volumes and low inspiratory pressures * Recommendation: We recommend that adult patients with ARDS receive mechanical ventilation with strategies that limit tidal volumes (4–8 ml/kg predicted body weight) and inspiratory pressures (plateau pressure < 30 cm H2O) (strong recommendation, moderate confidence in effect estimates)

Answer 9

D - unstable vertebral fracture Absolute contraindications to prone positioning: -Acute bleeding -Multiple # -Spinal instability -Raised ICP or CPP -Tracheal surgery or sternotomy within 2 weeks

Answer 10

A - High res CT to identify Idiopathic Interstitial Pneumonia In this case, organising pneumonia is suspected OP = Rare disease which often mimics community acquired pneumonia * Idiopathic form of organizing pneumonia (aka bronchiolitis obliterans organizing pneumonia [BOOP]) * A diffuse interstitial lung disease arising from injury to the alveolar wall. * Characterised by peripheral lung infiltrates & responsiveness to systemic glucocorticoids * Presenting symptoms generally include * Persistent nonproductive cough (71%) * Dyspnea (62%) * Fever (44%) * Malaise (48%) * Weight loss of greater than 5 kg (57 percent)

Answer 11

- Subacute cough (weeks to months) * Dyspnoea * Flu like illness at disease onset in ~50% * Chest imaging with patchy or diffuse ground-glass infiltrate or consolidation * Lack of response to (often multiple courses of) antibiotics * Investigations for other causes unremarkable

Answer 12

* CAP – Antibiotic responsive * Chronic eosinophilic pneumonia – High BAL eosinophil count >25% and more rapid response to steroids (over hours to days) * Hypersensitivity pneumonitis – known exposure agent, micronodules and GGO, higher lymphocyte count on BAL, poorly formed granulomas * Pulmonary lymphoma and lymphomatoid granulomatosis – different histopathology and immunohistochemical appearance * Organizing Pneumonia – can occur in other lung diseases including interstitial idiopathic pneumonias

Answer 13

Often more extensive disease on HRCT than expected from CXR. Radiographic patterns include: * Patchy air-space consolidation * Ground-glass opacities * Small nodular opacities * Bronchial wall thickening with dilation * Peripheral distribution, often in the lower lung zones (as occurs with chronic eosinophilic pneumonia) * Less commonly, nodules, masses with cavitation, irregular reticular opacities, crescent shaped opacities

Answer 14

1) Prednisolone 0.5-1mg/kg/day (up to 60mg) for 4 weeks then wean over 4-6 months Alternatively can trial macrolides OR observation (but only <10% spontaneously resolve)

Answer 15

Allergic Bronchopulmonary Aspergillosis - Complex hypersensitivity reaction to colonization of the airways by Aspergillus fumigatus * Occurs almost exclusively in patients with Asthma or Cystic fibrosis * Episodes of repeated bronchial obstruction, mucoid impaction and inflammation resulting in bronchiectasis and Fibrosis * Elevated TLCO is seen in hyperaemia of the airways and frequently occurs in asthma

Answer 16

No prospectively validated diagnostic criteria 1) Predisposing condition - asthma or CF 2) Positive skin prick test or increased IgE levels to Aspergillus fumigatus AND elevated IgE concentration (>1000 IU) 3) positive aspergillus precipitants or IgA to aspergillus fumigatus +/- radiology consistent with ABPA +/- total eosinophil count >0.5x10^6 in steroid naive patients

Answer 17

Proximal cyclindrical bronchiectasis Mucous plugging Tree in bud opacity Atelectasis Peripheral consolidation GGO Mosaic attenutation with gas trapping However CT is normal in 20%

Answer 18

A heterogenous syndrome caused by defects in proteins that make up the inner and outer dynein arms of cilia

Answer 19

Pulmonary - chronic bronchitis and bronchiectasis Rhinosinusitis Otitis media (more common in children) Situs inversus (although most with situs inversus do not have PCD) Infertility - men infertile, women reduced fertility

Answer 20

D - Hypocapnia OSA is more common than central sleep apnoea (CSA) in the general population * CSA with Cheyne-Stokes Breathing (CSA-CSB) may be more common in patients with heart failure (esp with reduced ejection fraction) * Observational studies suggest CSA-CSB prevalence of 50 – 70% in heart failure patients * Sleep disordered breathing is common in patients with optimally managed heart failure – 61%

Answer 21

The respiratory control centre has a serum CO2 level below which apnoea results (ie the apnoea threshold) CSB had consistently low pCO2 levels (ie chronic hypocapnia * Low CO2 results in increased CSF pH, decreased central chemoreceptor stimulation and decreased RR and apnoea Patients with HF have prolonged circulation time rendering the PCO2-ventilatory relationship unstable During an apnoea in px with advanced HF - PCO2 increases but takes a while for the respiratory control centre to sense this due to increased circulation time (ie lag in ability of brain to respond to elevated CO2); when it eventually does it overcompensates with tachypnoea and hyperpnoea which drives PCO2 down so the cycle repeats (ie loop gain)

Answer 22

C - Pulmonary arterial hypertension On the basis you have disproportionately low DLCO with normal spirometry

Answer 23

Obstructive ventilatory defects most commonly Can get restrictive ventilatory defects in severe disease with lung destruction Variable normal or reduced DLCO

Answer 24

Obstructive ventilatory defects + reduction in DLCO often in proportion to severity of emphysema so

Answer 25

In smokers - airflow obstruction with reduced DLCO is expected In non-smokers - lung function should be normal

Answer 26

C Compared to smokers with COPD, non-smokers with chronic airflow limitation have fewer symptoms, milder disease and lower burden of systemic inflammation. * Never smokers do NOT appear to have an increased risk of lung cancer or cardiovascular disease, compared to those without chronic airflow limitation. * Compared to the general population never smokers have an increased risk of pneumonia and mortality from respiratory failure

Answer 27

B Several studies have shown that blood eosinophil counts predict the magnitude of the effect of ICS (added on top of regular maintenance bronchodilator treatment) in preventing future exacerbations. * Continuous relationship between blood eosinophil counts and ICS effects * Nil/small effects are observed at lower eosinophil counts * Increasing effects observed at higher eosinophil counts * Blood eosinophil count ≥ 300 cells/μL identifies patients with the greatest likelihood of treatment benefit with ICS. * Data modelling indicates that ICS containing regimens have little or no effect at a blood eosinophil count < 100 cells/μL

Answer 28

Reduces mortality + reduces exacerbations + improves lung function, symptoms and health status in symptomatic COPD patients with a history of frequent and/or severe exacerbations BUT increases risk of pneumonia eTG: Consider adding an inhaled corticosteroid (ICS) to long-acting bronchodilator dual therapy if both of the following apply: - the patient has had a severe exacerbation (requiring hospitalisation) or at least two moderate exacerbations in the previous 12 months AND - the patient has significant symptoms despite dual therapy with a LAMA and LABA

Answer 29

Long-term low-dose oral macrolides may reduce the frequency of exacerbations HOWEVER There are many potential significant adverse effects of long-term, low-dose macrolide therapy, including cardiac toxicity, ototoxicity, diarrhoea, and the development of antibiotic resistance Important to first exclude NTM infection prior to starting

Answer 30

A Raised intracranial pressure following head injury * Symptoms include headache, depressed consciousness and vomiting * Signs include CN VI palsy, papilledema, periorbital bruising and the triad of bradycardia, respiratory depression and hypertension (known as Cushing's Triad)

Answer 31

D RB-ILD = smoking-related, usually seen in px 30-40yo with >30pk yr hx; SOB + cough + inspiratory crackles + GGO (upper zones) + centrilobar nodules + subpleural fibrosis + patchy opacification +/- emphysema

Answer 32

Young smoking adults 20-40yo Equal sex distribution HRCT shows small periobronchiolar nodular opacities, multiple irregularly-shaped CYSTS, mid and upper zone predominance, GGO, mosaic attenuation, interlobular septal thickening

Answer 33

In patients >60yo with progressive exertional dyspnoea and non-productive cough Smokers are at increased risk of IPF and have worse survival than never smokers

Answer 34

Subpleural distribution with reticular opacities - peripheral and lower lobe predominant Traction bronchiectasis Honeycombing UIP pattern

Answer 35

Previously called allergic alveolitis A group of immune-mediated pulmonary disorders with inflammation and/or fibrosis affecting parenchyma and small airways (split into fibrotic and non-fibrotic HP) Usually has a history of exposure to an offending antigen eg bird fanciers or farmers lung Smoking is associated REDUCED risk of HP but more chronic and severe course

Answer 36

Coarse reticulations and lung distortion Traction bronchiectasis Honeycoming Ill-defined centrilobar nodules GGO Mosaic attenuation and/or air trapping Mid-zone predominant OR randomly distributed Related lower zone sparing

Answer 37

If pneumothorax is small (ie <3cm at apex, 2cm at hilum) and patient stable (ie RR <24, HR 60-120, BP normal, SpO2 >90%, can speak full sentences Can treat with observation (with repeat CXR in 4 hours) +/- oxygen and discharged

Answer 38

A - Pseudomonas colonisation

Answer 39

A Increased A-a gradient = V/Q mismatch or shunt High altitude + hypoventilation (incl respiratory muscle weakness) = reduced A-a gradient The A-a gradient is a nonspecific indicator of the integrity of the alveolocapillary membrane. * Any pathology of the alveolocapillary unit widens the gradient. * Hypoxemia due to ventilation-perfusion (V/Q) mismatch, diffusion limitation, and shunt are associated with a widened gradient, whereas hypoxemia due to hypoventilation has a normal gradient * Normal A-a gradient ~ (Age/4) + 4

Answer 40

B Patients with IPAH and a mean right atrial pressure ≥20 mmHg have a median survival of ~ one month Other prognostic factors = Age >50 Male sex Severe PAH/RHF Hypocapnia Comorbid CTD-associated PAH SSRI Low VWF levels BMPR2 mutations Episode of acute PH crisis

Answer 41

DLCO = diffusion capacity of the lungs for carbon monoxide (or transfer capacity) Measures the ability of the lungs to transfer gas from inhaled air to RBCs Easy to perform - requires patient to hold their breath for 10 seconds Diseases in which the uptake of οхygeո is reduced cause parallel decreases in the uptake of CO, as measured by the DԼСΟ. Older textbooks suggest that thickening of the alveolar-capillary membrane (in interstitial lung disease) and loss of alveolar membrane surface area (in еmрhysеma) are the primary causes of a low DLCՕ. However, subsequent experimental data suggest these and most other diseases that influence the DԼСΟ do so by reducing the volume of red blood cells in the pulmonary capillaries. The total volume of blood in the lungs in healthy adults at rest is less than 150 mL. Diseases in which the alveolar-capillary surface area is reduced (eg, idiopathic pulmonary fibrosis and еmрhyѕema) lead to a reduction in the blood volume in the lungs.

Answer 42

- Exercise - Supine position - During reverse Valsalva manevoure - Left-to-right cardiac shunt is present - high altitude - obesity - polycythaemia

Answer 43

- Emphysema (correlates with degree of anatomic emphysema; loss of DLCО usually occurs after the decline in FEV1) - Interstitial lung disease - Sarcoidosis - Hypersensitivity pneumonitis - Chest irradiation and cancer chemotherapy - Rheumatic diseases - Pulmonary vascular disease eg recurrent PE or CTEPH - Idiopathic pulmonary arterial hypertension - Pulmonary vascular involvement from rheumatic disease or vasculitides - Heart failure - Anaemia - Smoking/increased carboxyhaemoglobin

Answer 44

1) Pulmonary vascular disease 2) Early interstitial lung disease 3) Anaemia 4) Hepatopulmonary syndrome 5) Increased carboxyhaemoglobin level due to smoking

Answer 45

Dupiliumab - an anti-IL4Ra antibody

Answer 46

Dupilumab - anti-IL4Ra antibody - has the best evidence but could also use mepolizumab or omalizumab Not benralizumab - studies show no improvement in symptoms, time to surgery or chronic steroid use

Answer 47

The abnormal dilation of bronchi due to the destruction of the elastic and muscular components of the bronchial wall

Answer 48

More than 60% of adults with bronchiectasis report symptoms from childhood - these patients have worse disease and a poorer prognosis compared with patients with adult-onset bronchiectasis

Answer 49

1) Post-infectious - e.g. post childhood resp viral infections eg measles, influenza, pertussis; prior mycobacterial infections, COVID-19 infection 2) COPD 3) Asthma 4) Connective tissue disease - especially RA and Sjogren's syndrome 5) ABPA 6) Immunodeficiency - Immunoglobulin deficiency OR HIV infection 7) Aspiration or inhalation injury 8) Genetic conditions - Cystic fibrosis - Ciliary dyskinesia or immotile cilia syndrome with or without Kartagener syndrome - alpha-1-antitrypsin deficiency 9) Inflammatory Bowel Disease 10) Focal bronchial obstruction - e.g. foreign body, stenosis, tumour, extrinsic compression 11) Other rarer causes incl Young syndrome, ANCA-assoc vasculitides, Marfan syndrome etc 12) Idiopathic

Answer 50

A chronic manifestation of bronchiolitis or pneumonitis in childhood characterised by unilateral pulmonary hypoplasia and radiographic hyperlucency

Answer 51

An autosomal recessive condition characterised by the clinical triad of bronchiectasis, situs inversus and chronic sinusitis

Answer 52

A condition (believed to be genetic) characterised by obstructive azoospermia with normal sperm production plus chronic or recurrent sinus and lung infections

Answer 53

An initial insult (e.g. primary infection) leads to airway inflammation and bronchial damage which then serves as a nidus for subsequent colonisation of the airways. A viscous cycle then ensues predisposing to persistent bacterial colonisation and a subsequent chronic inflammatory reaction that leads to progressive airway damage (including the classical dilation and thickening of the bronchi) and recurrent infections The factors that predispose individuals with an initial infection to go on and develop bronchiectasis remain unclear

Answer 54

Cystic - it is the most severe form - often found in CF patients

Answer 55

High-resolution CT chest

Answer 56

Persistent productive cough Daily mucopurulent sputum Dyspnoea Fatigue Rhinosinusitis Haemoptysis/blood tinged sputum - less common Intermittent fevers Weight loss Require a high degree of suspicion if above symptoms/signs accompany an underlying disorder (e.g. CTD, IBD etc)

Answer 57

Digital clubbing Crackles High-pitched inspiratory squeaks or wheeze

Answer 58

1) FBC - elevated WCC may indicate superimposed infection/exac OR eosinophilia may indicate underlying ABPA - elevated platelet count linked to higher mortality 2) Sputum MCS + AFBs - aids in guiding antibiotic selection 3) Sweat sodium chloride concentration and genetic testing for CFTR mutation analysis - to identify cystic fibrosis 4) Serum alpha-1 antitrypsin phenotype and level - to identify alpha-1 antitrypsin disease as underlying cause esp in those with co-existing basal panacinar emphysema (in which case you may consider replacement therapy) 5) Immunoglobulin levels and IgG subclasses and response to pneumovax with strep pneumo 23 serotype titres - to identify individual immunoglobulin deficiencies (which could be treated to reduce frequency of exac) 6) Specific IgE or skin prick test for Aspergillus fumigatus - to evaluate for ABPA 7) HIV - to identify HIV (which predisposes to bronchiectasis due to abnormal B-lymphocyte function) 8) Rheumatoid factor - bronchiectasis more common in RA population 9) Nasal nitric oxide test - if low (<100 parts per billion) indicates a diagnosis of Primary ciliary dyskinesia (if CF excluded) 10) RFTs - obstructive airway disease (ie reduced FEV1/FVC) + may get increased RV/TLC due to air trapping + reduced DLCO in severe disease

Answer 59

Early age at onset Persistent isolation of Staphylococcus aureus on sputum culture Features of malabsorption Male primary infertility Upper lobe bronchiectasis A history of childhood steatorrhoea

Answer 60

≥60 mmol/L

Answer 61

Broncho-arterial dilation ratio of >1 to 1.5

Answer 62

On examination - reduced intensity of bs characterise COPD but are not found in bronchiectasis On CT - may be normal or show emphysema in COPD cf characteristic abnormalities in bronchiectasis eg thickened, dilated airways +/- air fluid levels, varicose constrictions, ballooned cysts, tree-in-bud pattern

Answer 63

On examination - inspiratory squeaks and crackles may be present in bronchiectasis but are not present in asthma On CT - in asthma lack airway dilatation or signet ring sign On RFTs - airway obstruction often reversible in asthma

Answer 64

1) Exercise 2) Improve nutrition 3) Airway clearance therapy 4) Self-management plan 5) Inhaled bronchodilator 6) Mucoactive agent

Answer 65

1) because exercise is a form of airway clearance - exercise improves exercise capacity + QOL + cough-related QOL + psychological symptoms 2) higher BMI correlates with beneficial outcomes

Answer 66

1) Maintaining oral hydration 2) Percussion techniques 3) Breathing techniques 4) Coughing techniques 5) Positioning/postural drainage 6) Positive expiratory pressure devices 7) Oscillatory devices Recommended 15-30mins 2-3 times per day

Answer 67

If co-existent COPD or asthma - follow guidelines for COPD or asthma If not - may benefit from nebulised salbutamol prior to mucoactive therapy

Answer 68

Nebulised/humidified sterile water or normal saline Nebulised hypertonic saline - promotes mucus clearance by inducing coughing, shown to reduce inflammatory mediators, improve sputum bacteriology and QOL BUT may cause chest tightness and wheeze (can try bronchodilator before + add hyaluronic acid to improve tolerability) Others eg bromhexine may be considered

Answer 69

1) Oral antibiotics - amoxicillin 1g TDS OR doxycycline 100mg BD OR - IF infection with beta-lactamase producing strain of Haemophilus or Moraxella suspected (eg because the patient had a recent exacerbation caused by one of these pathogens) -> Augmentin DF BD

Answer 70

Using a validated severity score eg. Bronchiectasis severity index (BSI) Or assessing for features of severe exacerbation including: - worsening respiratory distress - worsening hypoxaemia from the patient’s baseline state - acute-onset confusion - signs of sepsis or septic shock

Answer 71

ONLY if all three of the following clinical features are present: 1) increased sputum volume or change in sputum viscosity 2) increased sputum purulence 3) increased cough, which may be associated with wheeze, breathlessness or haemoptysis.

Answer 72

IV Ceftriaxone/cefotaxime OR IV Augmentin

Answer 73

Amoxicillin or doxycycline Only if the patient is not improving on initial therapy AND you isolate pseudomonas on sputum change to Ciprofloxacin 750mg BD for 14 days

Answer 74

Use the results of susceptibility tests if available 1) Ceftazidime 2g Q8H OR 2) Piptaz 4.5g Q6H + Gentamicin (preferred), Tobramycin OR ciprofloxacin (this is called combination therapy - note there is limited evidence to inform the optimal empirical regimen and whether combination antipseudomonal therapy is required)

Answer 75

A free flowing pleural effusion adjacent to an area of pneumonia that is sterile; free flowing means it lacks internal loculations/septae

Answer 76

An exudative effusion that has been infected with bacteria or other microorganisms (ie pH <7.2 OR glucose <40 and/or positive gram stain or biochemical evidence of marked inflammation)

Answer 77

A parapneumonic effusion that contains internal septations/loculated effusion

Answer 78

A collection of pus within the pleural space due to direct invasion by pyogenic bacteria from an adjacent pneumonia OR from blunt trauma or from another source It is a type of complicated pleural effusion

Answer 79

If small uncomplicated (ie sterile effusions) - not required unless the effusion is sizeable enough to impair respiratory function For large uncomplicated effusions, complicated effusions OR empyema - drainage should be performed ASAP for source control (especially in cases of empyema)

Answer 80

In stable patients with long-standing effusions (since the pathogens that cause subacute/chronic empyema differ from those assoc with acute pneumonia eg myobacteria and fungi) Otherwise, should commence empirical antibiotics that mirrors treatment for underlying pneumonia EXCEPT that should pay attention to ensuring appropriate anaerobic bacterial coverage (especially with empyema) and antibiotics that have good penetration into the pleural space (nearly all do EXCEPT aminoglycosides)

Answer 81

Aminoglycosides eg gentamicin, tobramycin, amikacin

Answer 82

Uncomplicated - 1 to 2 weeks Complicated - 2-3 weeks Empyema - 4-6 weeks

Answer 83

No - worth taking into account but complete radiographic resolution may take weeks to months and residual pleural thickening can last longer

Answer 84

May indicate antibiotic coverage or drainage is inadequate therefore: 1) adjusting antibiotics 2) additional drainage procedures 3) intrapleural tissue plasminogen activator (tPA) with deoxyribonuclease (DNase) 4) Video-assisted thoracic surgery

Answer 85

Possibility of residual trapped lung

Answer 86

Patients with complicated parapneumonic effusion OR empyema who fail antibiotic therapy and initial drainage Patients who are not candidates for OR do not want surgery

Answer 87

Chest pain Fever Allergic reactions Pleural haemorrhage - 1-7%, most worrisome, more common in those with underlying risk factors for bleeding

Answer 88

Often you can leave the catheter in situ and treat with prolonged course of antibiotics (often infection due to staph species) and it will resolve

Answer 89

- residual pleural thickening - fibrothorax - pleural calcification - bronchopleural fistula - empyema necessitans (rupture of the empyema through the chest wall) - pleural lymphoma (especially in Japanese patients)

Answer 90

V/Q scan It is more sensitive (and therefore higher negative predictive value) than CTPA although often performed as complementary studies

Answer 91

V/Q scan It is more sensitive (and therefore higher negative predictive value) than CTPA although often performed as complementary studies. CTPA is particularly poor at identifying PE at the level of the segmental level - which is amenable to surgery

Answer 92

V/Q scan is not specific Therefore CTPA will a) confirm the obstruction and b) exclude alternative causes of pulmonary artery obstruction specifically: pulmonary artery tumour, pulmonary artery stenosis, fibrosing mediastinitis

Answer 93

Digital Subtraction Pulmonary Angiography Also helpful for evaluating surgical eligibility

Answer 94

Mean Pulmonary artery pressure >20mmHg at rest with vascular resistance of >= 2 Wood units in the absence of an elevated pulmonary capillary wedge pressure (PCWP <15mmHg)

Answer 95

1) lifelong anticoagulation Plus 2) referral to a PH / CTEPH centre of excellence for consideration of Surgical Pulmonary ThromboEndarterectomy (PTE) If operable candidate - lifelong anticoagulation plus surgical PTE (only curative therapy) If inoperable - lifelong anticoagulation plus PH-medical therapy - WHO functional class 2-3 - riociguat - WHO class 4 - parenteral prostanoid (intravenous epoprostenol or treprostinil) Alternatives / second line agents if medical monotherapy not effective incl sildenafil,

Answer 96

It is a Soluble guanylate cyclase (sGC) stimulator that targets the nitric oxide (NO)-sGC-cyclic guanosine monophosphate (cGMP) pathway, resulting in vasodilation and reduced pulmonary artery pressure.

Answer 97

Tobacco smoking decreases Riociguat concentrations by 50% therefore if patient is a smoker on Riociguat then stops smoking you may need to reduce dose by 50%

Answer 98

Nitrates Phosphodiesterase inhibitors eg sildenafil

Answer 99

Hypotension Peripheral (& pulmonary) oedema Bleeding

Answer 100

Surgical Pulmonary Thromboendarterectomy

Answer 101

Themain focus of the evaluation is to determine whether removal of thromboembolic material would sufficiently reduce the pulmonary artery vascular resistance (PVR). The latter assessment is critical since outcomes are dependent upon the effect of PTE on PVR. Outcomes are favorable when PVR decreases. In contrast, failure of the PVR to decrease following PTE is associated with poor short-term outcome

Answer 102

No. Only thromboembolic disease located proximally in the main, lobar, or segmental pulmonary arteries are amenable to surgical removal while distal disease is not typically surgically accessible

Answer 103

Those with significant haemodynamic abnormalities at rest eg elevated PVR (wood units 2-15). High PVR better response but also potentially higher risk

Answer 104

Only curative treatment modality - assoc with improved pulmonary hemodynamics, functional status, and long-term survival