Echocardiography in Pulmonary Arterial Hypertension
Purpose of Review
Pulmonary arterial hypertension (PAH) is a devastating disease which can result in right heart (RH) failure and death. Herein, we discuss the current clinical applications of echocardiography in PAH.
Advanced echocardiographic techniques (strain, strain rate, 2D-speckle tracking strain, and three-dimensional echocardiography) may reveal in the near future additional important insights into RH structure and function.
Although right-heart catheterization is mandatory for a definitive diagnosis, echocardiography (resting and exercise) represents a key noninvasive imaging test on the diagnostic-prognostic-therapeutic PAH algorithm.
KeywordsEchocardiography Pulmonary arterial hypertension Exercise Doppler echocardiography Strain Three-dimensional echocardiography
Compliance with Ethical Standards
Conflict of Interest
Francesco Ferrara, Xiao Zhou, Karina Wierzbowska-Drabik, Olga Vriz, Bahaa M. Fadel, Anna Agnese Stanziola, Jaroslaw Kasprzak, Mani Vannan, and Eduardo Bossone declare that they have no conflict of interest.
Luna Gargani reports speaker honoraria from General Electric and Glaxo-Smith-Kline.
Human and Animal Rights and Informed Consent
This article does not contain any studies with human or animal subjects performed by any of the authors.
Papers of particular interest, published recently, have been highlighted as: • Of importance •• Of major importance
- 1.McLaughlin VV, Archer SL, Badesch DB, Barst RJ, Farber HW, Lindner JR, et al. ACCF/ AHA 2009 expert consensus document on pulmonary hypertension a report of the American College of Cardiology Foundation task force on expert consensus documents and the American Heart Association developed in collaboration with the American College of Chest Physicians; American Thoracic Society, Inc.; and the Pulmonary Hypertension Association. J Am Coll Cardiol. 2009;53(17):1573–619.CrossRefGoogle Scholar
- 2.Galiè N, Humbert M, Vachiery JL, et al. 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 2016. 2015;37(1):67–119.Google Scholar
- 3.Simonneau G, Gatzoulis MA, Adatia I, Celermajer D, Denton C, Ghofrani A, et al. Updated clinical classification of pulmonary hypertension. J Am Coll Cardiol. 2013;62(25 Suppl):D34–41. https://doi.org/10.1016/j.jacc.2013.10.029 Review. Erratum in: J Am Coll Cardiol. 2014 Feb 25;63(7):746.CrossRefPubMedGoogle Scholar
- 6.Rudski LG, Lai WW, Afilalo J, et al. 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.CrossRefGoogle Scholar
- 8.Marra AM, Naeije R, Ferrara F, Vriz O, Stanziola AA, D'Alto M, et al. Reference Ranges and Determinants of tricuspid regurgitation velocity in healthy adults assessed by two-dimensional Doppler-echocardiography. Respiration. 2018:1–9.Google Scholar
- 10.Nagueh SF, Smiseth OA, Appleton CP, Byrd BF 3rd, Dokainish H, Edvardsen T, et al. Recommendations for the evaluation of left ventricular diastolic function by echocardiography: an update from the American Society of Echocardiography and the European Association of Cardiovascular Imaging. J Am Soc Echocardiogr. 2016;29(4):277–314.CrossRefGoogle Scholar
- 12.Lang RM, Badano LP, Mor-Avi V, Afilalo J, Armstrong A, Ernande L, et al. Recommendations for cardiac chamber quantification by echocardiography in adults: an update from the American Society of Echocardiography and the European Association of Cardiovascular Imaging. J Am Soc Echocardiogr. 2015;28:1–39.CrossRefGoogle Scholar
- 14.•• Rudski LG, Gargani L, Armstrong WF, Lancellotti P, Lester SJ, Grünig E, et al. Stressing the cardiopulmonary vascular system: the role of echocardiography. J Am Soc Echocardiogr. 2018;31(5):527–50 In this state-of-the-art document, the topic of stress echocardiography applied to the cardiopulmonary vascular system is thoroughly addressed, from clinical applications to limitations and future directions.CrossRefGoogle Scholar
- 15.Lancellotti P, Pellikka PA, Budts W, Chaudhry FA, Donal E, Dulgheru R, et al. The clinical use of stress echocardiography in non-ischaemic heart disease: recommendations from the European Association of Cardiovascular Imaging and the American Society of Echocardiography. Eur Heart J Cardiovasc Imaging. 2016;17(11):1191–229.CrossRefGoogle Scholar
- 19.• van Riel AC, Opotowsky AR, Santos M, et al. Accuracy of echocardiography to estimate pulmonary artery pressures with exercise: a simultaneous invasive-noninvasive comparison. Circ Cardiovasc Imaging. 2017;10(4). https://doi.org/10.1161/CIRCIMAGING.116.005711 This work provides useful information regarding the accuracy of noninvasive assessment of pulmonary artery pressures during exercise by Doppler echocardiography compared with right heart catheterization in a large patient population.
- 20.Gruenig E, Weissmann S, Ehlken N, Fijalkowska A, Fischer C, Fourme T, et al. Stress Doppler echocardiography in relatives of patients with idiopathic and familial pulmonary arterial hypertension results of a multicenter European analysis of pulmonary artery pressure response to exercise and hypoxia. Circulation. 2009;119:1747–57.CrossRefGoogle Scholar
- 21.Hinderhofer K, Fischer C, Pfarr N, Szamalek-Hoegel J, Lichtblau M, Nagel C, et al. Identification of a new intronic BMPR2-mutation and early diagnosis of heritable pulmonary arterial hypertension in a large family with mean clinical follow-up of 12 years. PLoS One. 2014;9:e91374.CrossRefGoogle Scholar
- 29.Lewis GD, Bossone E, Naeije R, Grünig E, Saggar R, Lancellotti P, et al. Pulmonary vascular hemodynamic response to exercise in cardiopulmonary diseases. Circulation. 2013;128:1470–9.Google Scholar
- 31.•• Naeije R, Saggar R, Badesch D, Rajagopalan S, Gargani L, Rischard F, et al. Exercise-induced pulmonary hypertension. Translating pathophysiological concepts into clinical practice. Chest. 2018;154(1):10–5 This review provides a thorough description of exercise pulmonary hypertension from pathophysiological concepts to clinical practice. Google Scholar
- 44.Grapsa J, Gibbs JSR, Cabrita IZ, Watson GF, Pavlopoulos H, Dawson D, et al. The association of clinical outcome with right atrial and ventricular remodelling in patients with pulmonary arterial hypertension: study with real-time three-dimensional echocardiography. Eur Heart J Cardiovasc Imaging. 2012;13(8):666–72.CrossRefGoogle Scholar
- 45.Bhave NM, Patel AR, Weinert L, Yamat M, Freed BH, Mor-Avi V, et al. Three-dimensional modeling of the right ventricle from two-dimensional transthoracic echocardiographic images: utility of knowledge-based reconstruction in pulmonary arterial hypertension. J Am Soc Echocardiogr. 2013;26(8):860–7.CrossRefGoogle Scholar