Pericardial disease, pulmonary hypertension and right heart disease
This domain tests pattern recognition in pericardial syndromes and the haemodynamic definition and treatment of pulmonary hypertension.
Pericardial disease
- Acute pericarditis: pleuritic, positional chest pain; diffuse concave ST elevation with PR depression. Treat with NSAID plus colchicine and restrict strenuous exercise.
- Tamponade: Beck triad, pulsus paradoxus above 10 mmHg, electrical alternans, blunted y descent, diastolic collapse of right chambers, plethoric IVC. Treat with pericardiocentesis.
- Constrictive pericarditis: prominent y descent, pericardial knock, septal bounce, annulus paradoxus, ventricular interdependence.
Pulmonary hypertension
- Pre-capillary definition: mean pulmonary artery pressure above 20 mmHg, pulmonary vascular resistance above 2 Wood units, wedge pressure 15 mmHg or less.
- Group 1 pulmonary arterial hypertension: idiopathic, heritable (BMPR2), connective tissue disease, congenital shunts. Group 2 left heart disease. Group 3 lung disease. Group 4 chronic thromboembolic disease, screened with V/Q scanning. Group 5 multifactorial.
- Group 1 therapy: endothelin receptor antagonists, PDE5 inhibitors, riociguat, prostacyclin analogues, selexipag. Calcium channel blockers only in vasoreactive patients.
Right heart failure
- Manage congestion gently, preserve systemic perfusion and avoid abrupt afterload reduction.
Worked example
A 45-year-old with exertional dyspnoea has a loud P2 and RVSP 60 mmHg on echo. Right heart catheterisation shows mean PA pressure 45 mmHg, wedge 10 mmHg and PVR 8 Wood units, confirming group 1 PAH; vasoreactivity testing guides calcium channel blocker use, otherwise dual therapy is started.
Traps
- Aggressive diuresis in right heart failure, causing hypotension.
- Labelling left heart disease as PAH without measuring wedge pressure.
- Missing CTEPH: it is the group with a surgical cure.