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Stem
A candidate is asked to manage a classic presentation of Pulmonary Hypertension in an exam setting. Use precise definitions, scores, doses, and decision thresholds.
Core knowledge (model answer backbone)
Pulmonary hypertension (PH) is a haemodynamic and pathophysiological syndrome defined as a mean pulmonary arterial pressure (mPAP) above 20 mmHg at rest measured by right heart catheterisation (RHC), confirmed by a pulmonary arterial wedge pressure (PAWP) of 15 mmHg or less in pre-capillary disease and pulmonary vascular resistance (PVR) above 2 Wood units (WU) in pulmonary arterial hypertension (PAH). The 2022 ESC/ERS Guidelines classify PH into five groups by aetiology: Group 1 — pulmonary arterial hypertension (PAH) (idiopathic, heritable, drug- and toxin-induced, and associated with connective tissue disease, HIV, portal hypertension, congenital heart disease); Group 2 — PH due to left heart disease (heart failure with preserved or reduced ejection fraction, valvular disease); Group 3 — PH due to lung disease or hypoxia (COPD, interstitial lung disease, sleep-disordered breathing); Group 4 — PH due to pulmonary artery obstruction (chronic thromboembolic pulmonary hypertension, CTEPH); Group 5 — PH with unclear or multifactorial mechanisms (haematological, systemic, metabolic disorders, sarcoidosis). Clinical presentation is dominated by progressive exertional dyspnoea, fatigue,
Red flags
- Syncope or pre-syncope on exertion in a previously well young or middle-aged adult - think pulmonary arterial hypertension (PAH Group 1); urgent echo and right heart catheterisation
- Peripheral oedema, ascites, tender hepatomegaly and raised JVP with clear lungs - right heart failure from cor pulmonale or Group 1 PAH; risk-stratify, oxygen, diuresis, definitive work-up at a PH centre
- Mismatched perfusion defects on V/Q scan in a patient with persistent dyspnoea 6 to 12 months after pulmonary embolism - chronic thromboembolic pulmonary hypertension (CTEPH / Group 4); surgical referral for pulmonary endarterectomy
- Heavy alcohol use or recent anorexigen exposure combined with progressive dyspnoea and signs of right-heart failure - drug- and toxin-induced PAH (Group 1.3); confirmatory RHC
High-yield structure examiners expect
Cover: Overview & Definition, Classification, Epidemiology & Risk Factors, Pathophysiology, Clinical Presentation, Differential Diagnosis.
Key doses / thresholds (from topic teaching)
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- tan 5 to 10 mg daily, macitentan 10 mg daily), phospho
- sildenafil 20 mg TDS or tadalafil 40 mg daily), the solu
- le guanylate-cyclase stimulator riociguat 1 mg TDS titrated to 2
- iloprost 5 mcg inhaled 6 to 9 times daily), and the se
- lective IP-receptor agonist selexipag 200 mcg BD titrated up to 1600 mcg BD**, plus s
Questions
a) Define the condition and give the most important classification or severity framework used in exams. (3 marks)
- Clear one-line definition matching standard teaching.
- Named classification / stages / types with discriminating features.
- One sentence on why classification changes management.
b) Outline pathophysiology in a mechanism chain that explains the main clinical features. (3 marks)
- Initiating insult → intermediate pathway → end-organ effect.
- Link at least two symptoms/signs to mechanism.
- Mention one complication pathway (e.g. shock, perforation, herniation, arrhythmia).
c) List discriminating clinical features and bedside assessment. (3 marks)
- Classic presentation plus one atypical group (elderly, pregnancy, child, immunocompromised).
- Named signs/manoeuvres if relevant.
- What must never be missed on exam/bedside (pregnancy test, airway, glucose, etc.).
d) Investigations with thresholds and one named score if applicable. (3 marks)
- First-line tests and what positive findings mean.
- Gold-standard or definitive investigation when needed.
- Score components reproduced exactly if a named score is standard for this topic.
e) Immediate resuscitation and definitive management with doses where standard. (3 marks)
- ABC / time-critical steps first.
- First-line drug(s) with agent + dose + route (or procedure steps).
- Escalation triggers (theatre, ICU, thrombolysis window, antidote, etc.).
- Disposition and safety-netting.
Marking tips
Full marks require specificity (numbers, names, doses) not generic "give antibiotics/fluids." Regional practice (ICMR / NICE / AHA) may be cited as alternative where relevant.