Pulmonary Vascular Disease and Cor Pulmonale

The answer first

Pulmonary vascular disease spans three clinical entities — acute pulmonary embolism (PE), chronic thromboembolic pulmonary hypertension (CTEPH), and pulmonary arterial hypertension (PAH) and the broader pulmonary hypertension (PH) spectrum — and the secondary syndromes they produce, of which the most important is cor pulmonale (right heart failure from pulmonary vascular or chronic lung disease). The physician's task is to recognise the clinical pattern, choose the imaging that confirms the diagnosis, risk-stratify by haemodynamic severity, and deliver a sequenced plan that integrates anticoagulation, reperfusion (thrombolysis, catheter-directed thrombolysis, surgical embolectomy), pulmonary vasodilator therapy (endothelin receptor antagonists, phosphodiesterase-5 inhibitors, soluble guanylate cyclase stimulators, prostacyclin analogues, and receptor agonists), and surgical intervention (pulmonary endarterectomy for CTEPH, balloon pulmonary angioplasty for inoperable CTEPH, transplantation for end-stage disease). ^{[1] [5]}

The FRACP candidate must be able to defend three positions without hedging: (1) acute PE is diagnosed by pretest probability assessment (Wells, YEARS) plus D-dimer or computed tomography pulmonary angiography, and risk-stratified by clinical, right-ventricular, and biomarker criteria into low, intermediate-low, intermediate-high, and high-risk groups with corresponding treatment intensity; (2) pulmonary hypertension is classified into five WHO groups, each with distinct evaluation and treatment, and a mean pulmonary artery pressure above 20 mmHg is the contemporary diagnostic threshold; and (3) cor pulmonale is the end-stage of untreated pulmonary vascular or chronic lung disease and is preventable with early diagnosis and modern therapy. Lead with the decision, then the evidence, then the trap. ^{[1] [9]}

References10ShowHide

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