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BMJ Case Reports 2018; doi:10.1136/bcr-2018-227393
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Pulmonary capillary haemangiomatosis causing pulmonary arterial hypertension: a clinician’s conundrum

  1. Sanjeev Kumar
  1. Department of Cardiovascular Radiology & Endovascular Interventions, All India Institute of Medical Sciences, New Delhi, India
  1. Correspondence to Dr Sanjeev Kumar, sanjeevradio{at}gmail.com
  • Accepted 20 September 2018
  • Published 7 October 2018

Description 

Pulmonary capillary haemangiomatosis (PCH) is a rare cause of pulmonary hypertension which poses a considerable diagnostic challenge. Here, we describe the case of a 14-year-old boy with gradually progressive exertional dyspnoea and cough for past 4 months. Frontal chest radiograph demonstrated prominent pulmonary artery segment with diffuse nodular opacities in bilateral lung fields (figure 1A). CT angiography was performed to diagnose the aetiology of pulmonary artery hypertension. It revealed dilated central pulmonary arteries (figure 1B) with multiple ill-defined centrilobular nodules diffusely distributed in both lungs (figure 1C,D). No interlobular septal thickening, pulmonary thromboembolism, pleural effusion or …

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