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Predicting pulmonary hypertension in sarcoidosis; value of PH probability on echocardiography

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Abstract

Pulmonary hypertension (PH) is a well-recognised complication of sarcoidosis. Non-invasive diagnosis is challenging due to limited accuracy of echocardiography in interstitial lung disease. This study evaluates the value of echocardiographic PH probability for diagnosing PH in pulmonary sarcoidosis. All consecutive patients between August 2015 and November 2018 were prospectively screened for PH, and classified as low, intermediate or high PH probability. Patients with intermediate or high PH probability were referred for right heart catheterisation. PH was defined as a mean pulmonary artery pressure of ≥ 25 mm Hg. Additional data on pulmonary function and chest-CT was collected. Of all 479 patients, PH was present in 17 and absent in 19 patients. Six patients refused right heart catheterisation. PH was present in 33% and 75% of patients with intermediate and high PH probability respectively (n = 36). TRV max was measurable in 46% of all patients. Measurability did not correlate with FVC% predicted or presence of significant fibrosis. In intermediate and high PH probability, TRV max < 2.9 m/s successfully ruled out PH whereas a TRV max > 3.4 confirmed PH in all patients. If TRV max was absent or in between 2.9 and 3.4, secondary echocardiographic signs were not able to improve the diagnostic accuracy. PH is unlikely in patients with a TRV max < 2.9 m/s on echocardiography, whereas PH is highly suspected in a TRV max > 3.4 m/s. Discrimination is challenging if the TRV max is between 2.9–3.4 m/s or absent. Additional secondary signs do not improve discrimination. Decision making for further investigations should be made by an expert team.

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References

  1. Spagnolo P, Rossi G, Trisolini R, Sverzellati N, Baughman RP, Wells AU (2018) Pulmonary sarcoidosis. Lancet Respir Med 6:389–402

    Article  Google Scholar 

  2. Huitema MP, Grutters JC, Rensing BJ, Reesink HJ, Post MC (2016) Pulmonary hypertension complicating pulmonary sarcoidosis. Neth Heart J 24:390–399

    Article  CAS  Google Scholar 

  3. Handa T, Nagai S, Miki S et al (2006) Incidence of pulmonary hypertension and its clinical relevance in patients with sarcoidosis. Chest 129:1246–1252

    Article  Google Scholar 

  4. Bourbonnais JM, Samavati L (2008) Clinical predictors of pulmonary hypertension in sarcoidosis. Eur Respir J 32:296–302

    Article  CAS  Google Scholar 

  5. Huitema M, Bakker A, Mager J, et al. (2019) Prevalence of pulmonary hypertension in pulmonary sarcoidosis; the first large European prospective study Eur Respir J

  6. Nunes H, Humbert M, Capron F et al (2006) Pulmonary hypertension associated with sarcoidosis: mechanisms, haemodynamics and prognosis. Thorax 61:68–74

    Article  CAS  Google Scholar 

  7. Authors/Task Force Members, Galie N, Humbert M, et al. (2015) ESC/ERS guidelines for the diagnosis and treatment of pulmonary hypertension: the joint task force for the diagnosis and treatment of pulmonary hypertension of the european society of cardiology (ESC) and the European Respiratory Society (ERS) endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC), International Society for Heart and Lung Transplantation (ISHLT). Eur Heart J

  8. Arcasoy SM, Christie JD, Ferrari VA et al (2003) Echocardiographic assessment of pulmonary hypertension in patients with advanced lung disease. Am J Respir Crit Care Med 167:735–740

    Article  Google Scholar 

  9. Nathan SD. Pulmonary hypertension in interstitial lung disease. International journal of clinical practice Supplement 2008:21–8.

  10. Miller MR, Crapo R, Hankinson J et al (2005) General considerations for lung function testing. Eur Respir J 26:153–161

    Article  CAS  Google Scholar 

  11. Scadding JG (1961) Prognosis of intrathoracic sarcoidosis in England: a review of 136 cases after five years' observation. Br Med J 2:1165–1172

    Article  CAS  Google Scholar 

  12. Amsallem M, Sternbach JM, Adigopula S et al (2016) Addressing the controversy of estimating pulmonary arterial pressure by echocardiography. J Am Soc Echocardiogr 29:93–102

    Article  Google Scholar 

  13. Rudski LG, Lai WW, Afilalo J et al (2010) Guidelines for the echocardiographic assessment of the right heart in adults: a report from the American Society of Echocardiography endorsed by the European Association of Echocardiography, a registered branch of the European Society of Cardiology, and the Canadian Society of Echocardiography. J Am Soc Echocardiogr 23:685–713 quiz 786-8

    Article  Google Scholar 

  14. Kovacs G, Berghold A, Scheidl S, Olschewski H (2009) Pulmonary arterial pressure during rest and exercise in healthy subjects: a systematic review. Eur Respir J 34:888–894

    Article  CAS  Google Scholar 

  15. Amsallem M, Boulate D, Kooreman Z et al (2017) Investigating the value of right heart echocardiographic metrics for detection of pulmonary hypertension in patients with advanced lung disease. Int J Cardiovasc Imaging 33:825–835

    Article  Google Scholar 

  16. Yock PG, Popp RL (1984) Noninvasive estimation of right ventricular systolic pressure by Doppler ultrasound in patients with tricuspid regurgitation. Circulation 70:657–662

    Article  CAS  Google Scholar 

  17. Berger M, Haimowitz A, Van Tosh A, Berdoff RL, Goldberg E (1985) Quantitative assessment of pulmonary hypertension in patients with tricuspid regurgitation using continuous wave Doppler ultrasound. J Am Coll Cardiol 6:359–365

    Article  CAS  Google Scholar 

  18. Currie PJ, Seward JB, Chan KL et al (1985) Continuous wave Doppler determination of right ventricular pressure: a simultaneous Doppler-catheterization study in 127 patients. J Am Coll Cardiol 6:750–756

    Article  CAS  Google Scholar 

  19. Fisher MR, Forfia PR, Chamera E et al (2009) Accuracy of Doppler echocardiography in the hemodynamic assessment of pulmonary hypertension. Am J Respir Crit Care Med 179:615–621

    Article  Google Scholar 

  20. Keir GJ, Wort SJ, Kokosi M et al (2018) Pulmonary hypertension in interstitial lung disease: limitations of echocardiography compared to cardiac catheterization. Respirology 23:687–694

    Article  Google Scholar 

  21. Simonneau G, Montani D, Celermajer DS et al (2019) Haemodynamic definitions and updated clinical classification of pulmonary hypertension. Eur Respir J 53:1801913. https://doi.org/10.1183/13993003.01913-2018

    Article  CAS  PubMed  PubMed Central  Google Scholar 

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Acknowledgements

This study was funded by ZonMW topzorg (project number 842001006). Statistical analysis was performed together with Dr. H. Kelder, epidemiologist. F. Smits, radiologist, scored the presence of fibrosis on chest-CT and reviewed the chest X-ray’s.

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Correspondence to M. P. Huitema.

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Huitema, M.P., Bakker, A.L.M., Mager, J.J. et al. Predicting pulmonary hypertension in sarcoidosis; value of PH probability on echocardiography. Int J Cardiovasc Imaging 36, 1497–1505 (2020). https://doi.org/10.1007/s10554-020-01859-9

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