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A 42-year-old patient presenting with exertional dyspnoea was diagnosed with functional class II pulmonary hypertension (PH) with severe tricuspid valve insufficiency due to cardiac and pulmonary artery obstruction by calcified amorphous tumour (paCAT). Bilateral (sub)segmental perfusion defects were seen on ventilation/perfusion scan (see online supplementary figure S1), while the CT angiogram showed large amounts of calcified radio-opaque material in absence of radiological characteristics of chronic thromboembolic pulmonary hypertension (CTEPH), such as webs, stenosis or pouches (figure 1A, C). After multi-institutional (Amsterdam, NL and Papworth, Cambridge, UK) assessment of the case, a decision was made to perform a complete, bilateral, pulmonary endarterectomy (PEA) combined with tricuspid valve replacement. During PEA bone-hard material was retrieved that lacked the (sub)segmental pulmonary …
Contributors PIB, selected the case, wrote, edited, reviewed and submitted the paper; HJB, selected the case, edited and reviewed the manuscript; IvdB, reviewed and selected radiology images and reviewed the manuscript; HWMN, reviewed the pathology images and the manuscript; AvdW, reviewed and selected the pathology images and reviewed the manuscript; PS, selected the case, edited and reviewed the manuscript.
Competing interests None declared.
Patient consent Obtained.
Provenance and peer review Not commissioned; externally peer reviewed.
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