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Cardiac MR and CT imaging in children with suspected or confirmed pulmonary hypertension/pulmonary hypertensive vascular disease. Expert consensus statement on the diagnosis and treatment of paediatric pulmonary hypertension. The European Paediatric Pulmonary Vascular Disease Network, endorsed by ISHLT and DGPK
  1. Heiner Latus1,
  2. Titus Kuehne2,
  3. Philipp Beerbaum3,
  4. Christian Apitz4,
  5. Georg Hansmann3,
  6. Vivek Muthurangu5,
  7. Shahin Moledina6
  1. 1Pediatric Heart Centre, Justus-Liebig-University, Giessen, Germany
  2. 2Unit of Cardiovascular Imaging, Department of Congenital Heart Disease and Pediatric Cardiology, German Heart Institute Berlin, Berlin, Germany
  3. 3Department of Pediatric Cardiology and Critical Care, Hannover Medical School, Hannover, Germany
  4. 4Department of Pediatric Cardiology, University Childrens Hospital Ulm, Ulm, Germany
  5. 5Cardiovascular MRI Department, Great Ormond Street Hospital for Children, London, UK
  6. 6National Paediatric Pulmonary Hypertension Service UK, Great Ormond Street Hospital for Children, London, UK
  1. Correspondence to Dr Heiner Latus, Paediatric Heart Center, Justus-Liebig-University Giessen, Feulgenstr. 10-12, Giessen 35392, Germany; heiner.latus{at}paediat.med.uni-giessen.de

Abstract

Childhood pulmonary hypertension (PH) is a heterogenous disease associated with considerable morbidity and mortality. Invasive assessment of haemodynamics is crucial for accurate diagnosis and guidance of medical therapy. However, adequate imaging is increasingly important in children with PH to evaluate the right heart and the pulmonary vasculature. Cardiac MR (CMR) and computed tomography (CT) represent important non-invasive imaging modalities that may enable comprehensive assessment of right ventricular (RV) function and pulmonary haemodynamics. Here, we present graded consensus recommendations for the evaluation of children with PH by CMR and CT. The article provides a structured approach for the use of CMR and CT imaging, emphasises non-invasive variables of RV function, myocardial tissue and afterload parameters that may be useful for initial diagnosis and monitoring. Furthermore, assessment of pulmonary perfusion and characterisation of the lung parenchyma provides structural information about processes that may cause or be due to PH.

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