Echocardiographic estimate of pulmonary artery pressure in sarcoidosis patients, a multi-national study

Main Article Content

Marloes Huitema
Marco Post
Jan Grutters
Athol Wells
Vasilis Kouranos
Oksana Shlobin
Steven Nathan
Daniel Culver
Joseph Barney
Rohit Gupta
Eva Carmona
Esam Alhamad
Mary Scholand
Marlies Wijsenbeek
Sivagini Ganesh
Elyse Lower
Peter Engel
Robert Baughman

Keywords

Sarcoidosis, pulmonary hypetension, hemodynamics, echocardiography

Abstract

Introduction: Echocardiographic measurement of  the right ventricular systolic pressure (RVSP) is commonly used for estimating systolic pulmonary artery pressure (PASP) measured during right heart catheterization (RHC) in patients suspected for pulmonary hypertension (PH). Generally, there is a strong correlation. However, this has been reported as less robust in sarcoidosis. We analyzed factors influencing the relationship between RVSP and PASP in sarcoidosis.


Methods & Results: Data of patients with and without sarcoidosis associated PH who had both a measurable echocardiographic RVSP and invasive PASP were collected from the RESAPH registry, PULSAR study and Cincinnati Sarcoidosis Clinic database (n=173, 60.1% female, mean age 56.0±9.5years). Among them, 124 had PH confirmed by RHC. There was a strong correlation between PASP and RVSP (r=0.640). This correlation was significant in both male and female, white or non-white, (forced vital capacity) FVC >60%, and presence of fibrosis (p<0.001). However, it was less robust in patients with FVC of 50% or less. RVSP was considered inaccurate if the difference with PASP was more than 10mmHg. Inaccurate echocardiographic estimation of the invasive PASP occurred in 50.8%, with overestimation mostly in patients without PH, and underestimation in patients with severe PH. An RVSP>50mmHg was associated with worse survival.


Conclusions: In this multicenter cohort of sarcoidosis patients, we found a significant correlation between RVSP as determined by echocardiography and PASP measured by RHC. Over- or underestimation of PASP occurred frequently. Therefore, echocardiography alone to screen for pulmonary hypertension in sarcoidosis should be used with caution.

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References

1. Nunes H, Humbert M, Capron F, Brauner M, Sitbon O, Battesti JP, Simonneau G, Valeyre D. Pulmonary hypertension associated with sarcoidosis: mechanisms, haemodynamics and prognosis. Thorax 2006;61:68-74.
2. Shlobin OA, Baughman RP. Sarcoidosis-Associated Pulmonary Hypertension. Semin Respir Crit Care Med 2017;38:450-462.
3. Galie N, Hoeper MM, Humbert M, Torbicki A, Vachiery JL, Barbera JA, Beghetti M, Corris P, Gaine S, Gibbs JS, Gomez-Sanchez MA, Jondeau G, Klepetko W, Opitz C, Peacock A, Rubin L, Zellweger M, Simonneau G, Guidelines ESCCfP. Guidelines for the diagnosis and treatment of pulmonary hypertension: the 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 the International Society of Heart and Lung Transplantation (ISHLT). Eur Heart J 2009;30:2493-537.
4. Rudski LG, Lai WW, Afilalo J, Hua L, Handschumacher MD, Chandrasekaran K, Solomon SD, Louie EK, Schiller NB. 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 2010;23:685-713; quiz 786-8.
5. Yock PG, Popp RL. Noninvasive estimation of right ventricular systolic pressure by Doppler ultrasound in patients with tricuspid regurgitation. Circulation 1984;70:657-662.
6. Berger M, Haimowitz A, Van Tosh A, Berdoff RL, Goldberg E. Quantitative assessment of pulmonary hypertension in patients with tricuspid regurgitation using continuous wave Doppler ultrasound. J Am Coll Cardiol 1985;6:359-365.
7. Currie PJ, Seward JB, Chan KL, Fyfe DA, Hagler DJ, Mair DD, Reeder GS, Nishimura RA, Tajik AJ. Continuous wave Doppler determination of right ventricular pressure: a simultaneous Doppler-catheterization study in 127 patients. J Am Coll Cardiol 1985;6:750-756.
8. Arcasoy SM, Christie JD, Ferrari VA, Sutton MS, Zisman DA, Blumenthal NP, Pochettino A, Kotloff RM. Echocardiographic assessment of pulmonary hypertension in patients with advanced lung disease. Am J Resp Crit Care Med 2003;167:735-40.
9. Fisher MR, Forfia PR, Chamera E, Housten-Harris T, Champion HC, Girgis RE, Corretti MC, Hassoun PM. Accuracy of Doppler echocardiography in the hemodynamic assessment of pulmonary hypertension. Am J Resp Crit Care Med 2009;179:615-21.
10. Nathan SD. Pulmonary hypertension in interstitial lung disease. Int J Clin Prac 2008;:21-8.
11. Rich JD, Shah SJ, Swamy RS, Kamp A, Rich S. Inaccuracy of Doppler echocardiographic estimates of pulmonary artery pressures in patients with pulmonary hypertension: implications for clinical practice. Chest 2011;139:988-993.
12. Baughman RP, Shlobin OA, Wells AU, Alhamad EH, Culver DA, Barney J, Cordova FC, Carmona EM, Scholand MB, Wijsenbeek M, Ganesh S, Birring SS, Kouranos V, O'Hare L, Baran JM, Cal JG, Lower EE, Engel PJ, Nathan SD. Clinical features of sarcoidosis associated pulmonary hypertension: Results of a multi-national registry. Respir Med 2018;139:72-78.
13. Huitema M, Bakker A, Mager J, Rensing B, Smits F, Grutters J, Post M. Prevalence of pulmonary hypertension in pulmonary sarcoidosis; the first large European prospective study Eur Resp Journal 2019;
14. SCADDING JG. Prognosis of intrathoracic sarcoidosis in England. A review of 136 cases after five years' observation. Br Med J 1961;2:1165-1172.
15. Galie N, Humbert M, Vachiery JL, Gibbs S, Lang I, Torbicki A, Simonneau G, Peacock A, Vonk Noordegraaf A, Beghetti M, Ghofrani A, Gomez Sanchez MA, Hansmann G, Klepetko W, Lancellotti P, Matucci M, McDonagh T, Pierard LA, Trindade PT, Zompatori M, Hoeper M. 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 Respir J 2015;
16. Janda S, Shahidi N, Gin K, Swiston J. Diagnostic accuracy of echocardiography for pulmonary hypertension: a systematic review and meta-analysis. Heart 2011;97:612-622.
17. Keir GJ, Wort SJ, Kokosi M, George PM, Walsh SLF, Jacob J, Price L, Bax S, Renzoni EA, Maher TM, MacDonald P, Hansell DM, Wells AU. Pulmonary hypertension in interstitial lung disease: Limitations of echocardiography compared to cardiac catheterization. Respirology 2018;23:687-694.
18. Swanson KL, Utz JP, Krowka MJ. Doppler echocardiography-right heart catheterization relationships in patients with idiopathic pulmonary fibrosis and suspected pulmonary hypertension. Med Sci Monit 2008;14:CR177-82.
19. Amsallem M, Boulate D, Kooreman Z, Zamanian RT, Fadel G, Schnittger I, Fadel E, McConnell MV, Dhillon G, Mercier O, Haddad F. Investigating the value of right heart echocardiographic metrics for detection of pulmonary hypertension in patients with advanced lung disease. Int J Cardiovasc Imaging 2017;33:825-835.
20. Amsallem M, Sternbach JM, Adigopula S, Kobayashi Y, Vu TA, Zamanian R, Liang D, Dhillon G, Schnittger I, McConnell MV, Haddad F. Addressing the Controversy of Estimating Pulmonary Arterial Pressure by Echocardiography. J Am Soc Echocardiogr 2016;29:93-102.
21. D'Alto M, Bossone E, Opotowsky AR, Ghio S, Rudski LG, Naeije R. Strengths and weaknesses of echocardiography for the diagnosis of pulmonary hypertension. Int J Cardiol 2018;263:177-183.
22. Boucly A, Cottin V, Nunes H, Jais X, Tazi A, Prevot G, Reynaud-Gaubert M, Dromer C, Viacroze C, Horeau-Langlard D, Pison C, Bergot E, Traclet J, Weatherald J, Simonneau G, Valeyre D, Montani D, Humbert M, Sitbon O, Savale L. Management and long-term outcomes of sarcoidosis-associated pulmonary hypertension. Eur Respir J 2017;50:10.1183/13993003.00465-2017. Print 2017 Oct.