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.
Similar content being viewed by others
References
Spagnolo P, Rossi G, Trisolini R, Sverzellati N, Baughman RP, Wells AU (2018) Pulmonary sarcoidosis. Lancet Respir Med 6:389–402
Huitema MP, Grutters JC, Rensing BJ, Reesink HJ, Post MC (2016) Pulmonary hypertension complicating pulmonary sarcoidosis. Neth Heart J 24:390–399
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
Bourbonnais JM, Samavati L (2008) Clinical predictors of pulmonary hypertension in sarcoidosis. Eur Respir J 32:296–302
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
Nunes H, Humbert M, Capron F et al (2006) Pulmonary hypertension associated with sarcoidosis: mechanisms, haemodynamics and prognosis. Thorax 61:68–74
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
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
Nathan SD. Pulmonary hypertension in interstitial lung disease. International journal of clinical practice Supplement 2008:21–8.
Miller MR, Crapo R, Hankinson J et al (2005) General considerations for lung function testing. Eur Respir J 26:153–161
Scadding JG (1961) Prognosis of intrathoracic sarcoidosis in England: a review of 136 cases after five years' observation. Br Med J 2:1165–1172
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
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
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
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
Yock PG, Popp RL (1984) Noninvasive estimation of right ventricular systolic pressure by Doppler ultrasound in patients with tricuspid regurgitation. Circulation 70:657–662
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
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
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
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
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
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.
Author information
Authors and Affiliations
Corresponding author
Ethics declarations
Conflicts of interest
None of the authors have any conflicts of interest regarding the content of this article.
Additional information
Publisher's Note
Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.
Electronic supplementary material
Below is the link to the electronic supplementary material.
Rights and permissions
About this article
Cite this article
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
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s10554-020-01859-9