Pulmonary vascular diseases

Learning resources

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CPD framework

The pulmonary vascular diseases CPD framework provides an outline of topics to guide your learning and development.

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1. Anatomy and development including malformations

  1. Pulmonary vasculature and lymphatic drainage
  2. Anatomy of the cardiovascular system
  3. Right ventricular anatomy

3. Ventilation

  1. Physiology of tidal breathing
  2. Active inspiration and passive expiration
  3. Relative elastic properties of the lung and chest wall
  4. Understanding lung volumes
  5. Transpulmonary pressures and breathing
  6. Changes in intrathoracic pressure
  7. Disease-specific effects on ventilation
  8. COPD and interstitial lung disease (ILD)

4. Circulation

  1. Normal pulmonary vascular pressures and flows
  2. Effects of exercise
  3. Active hypoxic and neurohumoral regulation of pulmonary circulation
  4. Pulsatile flow pulmonary haemodynamics
  5. Non-invasive evaluation of pulmonary circulation
  6. Invasive evaluation of pulmonary circulation
  7. Interpretation of pulmonary vascular resistance
  8. Components of pulmonary vascular load, right ventricular function (systolic and diastolic)

5. Gas exchange

  1. Transfer factor of the lung for carbon monoxide (TLCO)
  2. Definition
  3. Technique
  4. Calculation of TLCO and measurement of carbon monoxide transfer coefficient (KCO)
  5. Implications of KCO × alveolar volume (VA) = TLCO

6. Diagnostics – exercise protocols

1.Field tests versus ergometer/treadmill tests

  1. Maximal incremental test
  2. Walking tests: 6-minute walking distance (6MWD) and incremental shuttle walk test (ISWT)
  3. Indications for cardiopulmonary exercise testing (CPET)
  4. Exercise variables and indexes
  5. Maximal V’O2, heart rate (HR) and ventilation
  6. Lactate threshold/ventilatory thresholds
  7. Oxygen pulse-HR reserve and maximum predicted HR: V’E-V’CO2 slope and ventilatory equivalent
  8. Carbon dioxide-breathing reserve
  9. Dynamic hyperinflation
  10. Arterial oxygen desaturation
  11. Tolerable limit of exercise and “isotime” measurements
  12. CPET response patterns
  13. Ventilatory response
  14. Pulmonary gas exchange
  15. Cardiovascular response
  16. Exercise testing in prognostic evaluation
  17. Evaluation of the effects of therapeutic interventions
  18. Somnography/polygraphy
  19. Activity monitoring
  20. Evaluation of psychological and social status: SYMPACT and EMPHASIS10 questionnaires

7. Signs and symptoms suggesting pulmonary vascular disease (PVD)

  1. Evidence of right heart failure
  2. Dyspnoea
  3. Reducing exercise tolerance
  4. Presyncope
  5. Syncope
  6. Chest tightness

8. Disease processes associated with PVD

  1. Connective tissue disease (CTD)
  2. Congenital heart disease
  3. Hepatic disease
  4. HIV
  5. ILD including sarcoidosis
  6. COPD including emphysema
  7. Lung hypoventilation
  8. Pulmonary embolic disease
  9. Left heart failure
  10. Genetic causes of PVD (i.e. BMPR2, EIF2AK4 and HHT genes)
  11. Haematological diseases (e.g. polycythaemia rubra vera)
  12. Portal hypertension

9. Identification of signs of PVD by imaging modalities

  1. Acute pulmonary embolism
  2. Chronic thromboembolic disease
  3. Evidence of congenital heart disease
  4. Abnormal pulmonary venous return
  5. Enlarged pulmonary arteries
  6. Dilated right heart
  7. Right ventricular hypertrophy
  8. Mosaicism 9.Pulmonary arteriovenous malformations
  9. Pulmonary vein stenosis

10. Right heart catheterisation

  1. Use of right heart catheterisation
  2. Definition of pulmonary hypertension (PH)
  3. Indications, physiologic considerations and interpretation of exercise haemodynamic testing

11. PH-specific therapies

  1. Therapy
  2. Different types of delivery
  3. Medical management of right heart failure
  4. Non-medical therapies
  5. Surgery/balloon pulmonary angioplasty for chronic thromboembolic pulmonary hypertension (CTEPH)
  6. Pulmonary artery denervation

12. Pulmonary rehabilitation

Indications for pulmonary rehabilitation

13. Palliative care

  1. Indications for institution of palliative care
  2. End-of-life relief of symptoms by:
  3. Pharmacological interventions
  4. High-flow nasal oxygen
  5. Discussing end-of-life decisions with patients and their relatives

15. Mechanically assisted ventilation

  1. Indications for assisted ventilation
  2. Effects of mechanical ventilation on pulmonary haemodynamics and heart function

16. Indication for lung transplantation

  1. Principles of evaluation for lung transplantation
  2. Risk and particularities of lung transplantation in patients with PVD
  3. Severity grading of lung diseases with respect to referring a patient to a transplant centre
  4. Extracorporeal membrane oxygenation/right ventricular assist device

17. Evaluation of respiratory emergencies

  1. Management of right heart failure
  2. Management of cardiac arrhythmias
  3. Management of acute pulmonary embolism (PE), pulmonary embolism response team (PERT)
  4. Cardiopulmonary arrest in a patient with pulmonary arterial hypertension (PAH)

18. Differential diagnosis, immediate management steps and specific conditions

  1. Differential diagnosis of PVD and PE
  2. Haemoptysis
  3. Dyspnoea
  4. Thoracic pain
  5. PE
  6. Haemoptysis
  7. Dyspnoea
  8. Thoracic pain
  9. Massive acute pulmonary embolism
  10. Management of shock related to right heart failure
  11. Early identification of chronic thromboembolism

19. COPD and emphysema

  1. Severe PH sub-phenotypes in COPD
  2. Polycythaemia
  3. Management of cor pulmonale

20. Obstructive sleep apnoea syndrome

  1. Signs and symptoms associated with sleep-disordered breathing
  2. Recognition of sleep-disorder breathing as a cause of moderate PH
  3. Physiology and pathophysiology of sleep apnoea syndromes relevant to acute respiratory failure

21. Sarcoidosis

  1. Recognition of Sarcoidosis and its association with PH
  2. Recognition of PH as a poor prognostic factor in sarcoidosis
  3. Effects of drug therapy on PH

22. ILD

  1. Prognostic factors associated with ILD and PVD
  2. Classification of ILD
  3. Limitations/dangers of PAH therapies in ILD

23. CTD related to ILD

  1. ILD associated with CTD
  2. Prognostic factors associated with ILD
  3. List treatment choices

24. Drug-induced disease

Identification of causes of PVD from drug history

  1. Anorexigens
  2. Amphetamines
  3. Selected tyrosine kinase inhibitors
  4. Interferon
  5. Chemotherapeutic agents (alkylating agents)

25. Thromboembolic disease

  1. Identification of PE
  2. Differential diagnosis of PE
  3. Aetiology of PE
  4. Pulmonary Embolism Severity Index score and risk stratification of PE
  5. Management of PE without acute hemodynamic compromise
  6. Conditions/diseases associated with an increased risk of thromboembolic disease
  7. Current indications for prophylaxis against thromboembolic disease
  8. Adverse effects associated with anticoagulation therapy
  9. Interpretation of blood cell counts and coagulation laboratory tests
  10. Drug indications and their appropriate dosages
  11. Willingness to consider potential candidates for prophylaxis against thromboembolic disease
  12. Prophylaxis against thromboembolic disease
  13. Conditions/diseases associated with an increased risk of thromboembolic disease
  14. Adverse effects associated with anticoagulation therapy
  15. Interpretation of coagulation laboratory tests
  16. Drug indications and their appropriate dosages
  17. Genetic risk factors for thrombosis and their impact on clinical decisions
  18. Indications for targeted thrombosis (interventional radiology) and embolectomies
  19. Diagnosis and management of CTEPH
  20. Indications for thrombophilia screening
  21. ndications for inferior vena cava filter placement
  22. Follow-up after PE presentation, e.g. risk scoring (such as DASH) to discuss the risks/benefits of anticoagulation therapy
  23. Pancreatic enzyme replacement therapy

26. PH

  1. Pathophysiology of PH
  2. Pharmacological treatment of PH according to underlying disease
  3. Diagnosis of PH and cor pulmonale
  4. Risk stratification
  5. Translating national and international management guidelines to an individual patient
  6. Appropriate decisions for referral and transfer to specialised referral centres
  7. Prognosis of patients with PH in acute care settings
  8. Indications for systemic pharmacotherapy
  9. Indications for combination therapy
  10. Indications for intravenous therapy
  11. Chronic monotherapy or combination systemic therapy
  12. Institute general therapeutic and supportive measures

27. Vasculitis and diffuse pulmonary haemorrhage

  1. Pulmonary haemorrhage and haemoptysis
  2. Differential diagnoses, diagnostic steps and therapeutic options for pulmonary haemorrhage and haemoptysis
  3. Triage by severity
  4. Indications for radiological evaluation
  5. Indications for bronchoscopic evaluation

28. Arteriovenous (AV) malformation

  1. Symptoms of AV malformation
  2. Diseases associated with AV malformation (e.g. hereditary haemorrhagic telangiectasia)
  3. Diagnostic strategies when AV malformation is suspected
  4. Therapeutic interventions

29. Cardiac disease

  1. Differentiate between cardiac and pulmonary disease as a cause of acute respiratory failure
  2. Recognition of cardiac disease as an aetiology of PH
  3. Monitoring cardiac dysfunction in PVD patients via clinical, laboratorial, functional, echocardiographic and radiological means
  4. Principles of invasive cardiovascular monitoring (e.g. Swan-Ganz catheterisation)
  5. Cardiovascular effect of positive pressure ventilation
  6. Congenital heart diseases and Eisenmenger syndrome

30. Gastrointestinal, liver and kidney disease


  1. Portopulmonary hypertension
  2. Hepatopulmonary syndrome


31. Haematological disease

  1. PVD in patients with past or current haematological conditions
  2. Thrombophilia as a potential sign of PVD

32. Connective Tissue Diseases (CTD)

  1. CTD as a cause of PH
  2. Symptoms and clinical presentation
  3. Pathophysiology of CTD and PH
  4. Differential diagnosis and optimal testing
  5. Optimal treatment
  6. Prognostication of identified CTD

33. Hereditary PAH

Familial occurrence of PH

34. High-altitude sickness

  1. High-altitude as a cause of pulmonary oedema (HAPE)
  2. Pathophysiology, symptoms and clinical presentation of high-altitude sickness
  3. Performance of tests to establish the diagnosis
  4. Preventive measures

Guidelines on pulmonary vascular diseases

Access the catalogue of ERS guidelines, statements and technical standards relating to the topic of pulmonary vascular diseases.

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