Redefining the Role of Cardiovascular Imaging in Patients with Pulmonary Arterial Hypertension Authors
Echocardiography (RM Lang, Section Editor)
First Online: 12 February 2012 DOI:
10.1007/s11886-012-0253-2 Cite this article as: Freed, B.H., Patel, A.R. & Lang, R.M. Curr Cardiol Rep (2012) 14: 366. doi:10.1007/s11886-012-0253-2
While pulmonary arterial hypertension is a disease primarily affecting the pulmonary vasculature, the right ventricle plays an integral part in the disease process. Although widely used, two-dimensional echocardiography is limited in visualizing the right ventricle and, therefore, assessment of its structure and function has been largely subjective or invasive. Advanced imaging modalities such as real-time three-dimensional echocardiography and cardiovascular magnetic resonance overcome many challenges of two-dimensional echocardiography and have provided further insight into the pathophysiology of pulmonary arterial hypertension. Indices of right ventricular function obtained from these noninvasive techniques are being assessed for their prognostic capabilities as well as their ability to monitor response to pulmonary arterial hypertension–specific therapies. Future research is needed to compare the accuracy, reproducibility, and prognostic value of each of these parameters to definitively establish the role of cardiovascular imaging in the management of patients with pulmonary arterial hypertension.
Pulmonary arterial hypertension
Real-time three-dimensional echocardiography
Cardiovascular magnetic resonance
Clinical end points
References Papers of particular interest, published recently, have been highlighted as: • Of importance •• Of major importance
Badesch DB, Champion HC, Sanchez MAG, et al. Diagnosis and assessment of pulmonary arterial hypertension. J Am Coll Cardiol. 2009;54:S55–66.
Simonneau G, Robbins IM, Beghetti M, et al. Updated clinical classification of pulmonary hypertension. J Am Coll Cardiol. 2009;54:S43–54.
Chin KM, Kim NH, Rubin LJ. The right ventricle in pulmonary hypertension. Coron Arery Dis. 2005;16:13–8.
Humbert M, Sitbon O, Simonneau G. Treatment of pulmonary arterial hypertension. N Engl J Med. 2004;351:1425–36.
D’Alonzo GE, Barst RJ, Ayres SM, et al. Survival in patients with primary pulmonary hypertension. Results from a national prospective registry. Ann Intern Med. 1991;115:343–9.
McLaughlin VV, Badesch DB, Delcroix M, et al. End points and clinical trial design in pulmonary arterial hypertension. J Am Coll Cardiol. 2009;54:S97–S107.
•• Benza RL, Miller DP, Gomberg-Maitland M, et al. Predicting survival in pulmonary arterial hypertension: Insights from the registry to evaluate early and long-term pulmonary arterial hypertension disease management (REVEAL). Circulation. 2010;122:164–72.
This registry includes 2712 patients with PAH and developed a risk score based on numerous clinical, functional, and hemodynamic independent predictors of 1-year mortality
Hinderliter AL, Willis 4th PW, Long W, et al. Frequency and prognostic significance of pericardial effusion in primary pulmonary hypertension. PPH Study Group. Primary pulmonary hypertension. Am J Cardiol. 1999;84:481–84.
Peacock AJ. Endpoints in trials of new therapies for pulmonary arterial hypertension. Eur Respir Dis. 2007;2:41–4.
Agarwal R, Gomberg-Maitland M. Current therapeutics and practical management strategies for pulmonary arterial hypertension. Am Heart J. 2011;162:201–13.
• Bradlow WM, Hughes ML, Keenan NG, et al. Measuring the heart in pulmonary arterial hypertension (PAH): implications for trial study size. J Magn Reson Imaging. 2010;31:117–24.
This is one of the few papers showing how excellent the reproducibility of CMR is in RV assessment in patients with PAH
Haddad F, Doyle R, Murphy DJ, Hunt SA. Right ventricular function in cardiovascular disease, part II: pathophysiology, clinical importance, and management of right ventricular failure. Circulation. 2008;117:1717–31.
•• 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.
This is the most comprehensive 2D echocardiographic assessment of the right ventricle. These guidelines help to standardize assessment of the right ventricle using the most up-to-date measures of RV size and function
Forfia P, Fisher MR, Mathai SC, et al. Tricuspid annular displacement predicts survival in pulmonary hypertension. Am J Respir Crit Care Med. 2006;174:1034–41.
Yeo TC, Dujardin KS, Tei C, et al. Value of a Doppler-derived index combining systolic and diastolic time intervals in predicting outcome in primary pulmonary hypertension. Am J Cardiol. 1998;81:1157–61.
La Gerche A, Ruxandra J, Voigt JU. Right ventricular function by strain echocardiography. Curr Opin Cardiol. 2010;25:430–6.
• Sachdev A, Villarraga HR, Frantz RP. Right ventricular strain for prediction of survival in patients with pulmonary arterial hypertension. Chest. 2011;139:1299–309.
This is an important paper examining a newly developed parameter for global RV function with 2DE in patients with PAH
Galie N, Hinderliter AL, Torbicki A, et al. Effects of the oral endothelin-receptor anatagonist bosentan on echocardiographic and Doppler measures in patients with pulmonary arterial hypertension. J Am Coll Cardiol. 2003;41:1380–6.
van der Zwaan HB, Geleijnse ML, McGhie JS, et al. Right ventricular quantification in clinical practice: two-dimensional vs. three-dimensional echocardiography compared with cardiac magnetic resonance imaging. Eur J Echocardiogr. 2011;12:656–64.
• Sugeng L, Mor-Avi W, Weinert L, et al. Multimodality comparison of quantitative volumetric analysis of the right ventricle. JACC Cardiovasc Imaging. 2010;3:10–8.
This paper examines both in vitro and in vivo assessment of the right ventricle using multimodality imaging. RV analysis is performed with identical post-processing software
Grapsa J, O’Regan DP, Pavlopoulos H, et al. Right ventricular remodeling in pulmonary arterial hypertension with three-dimensional echocardiography: comparison with cardiac magnetic resonance imaging. Eur J Echocardiogr. 2010;11:64–73.
Benza RL, Biederman R, Murali S, Gupta H. Role of cardiac magnetic resonance imaging in the management of patients with pulmonary arterial hypertension. J Am Coll Cardiol. 2008;52:1683–92.
Fratz S, Schuhbaeck A, Buchner C, et al. Comparison of accuracy of axial slices versus short-axis slices for measuring ventricular volumes by cardiac magnetic resonance in patients with corrected tetralogy of fallot. Am J Cardiol. 2009;103:1764–9.
Lorenz CH, Walker ES, Morgan VL, Klein SS, Graham Jr TP. Normal human left and right ventricular mass, systolic function, and gender differences by cine magnetic resonance imaging. J Cardiovasc Magn Reson. 1999;1:7–21.
• van Wolferen SA, van de Veerdonk MC, Mauritz GJ, et al. Clinically significant change in stroke volume in pulmonary hypertension. Chest. 2011;139:1003–9.
This is one of the first papers that looks at what is actually a clinically meaningful change in RV parameters using CMR
van Wolferen SA, Marcus JT, Boonstra A, et al. Prognostic value of right ventricular mass, volume, and function in idiopathic pulmonary arterial hypertension. Eur Heart J. 2007;28:1250–7.
Hagger D, Condliffe R, Woodhouse N, et al. Ventricular mass index correlates with pulmonary artery pressure and predicts survival in suspected systemic sclerosis-associated pulmonary arterial hypertension. Rheumatology. 2009;48:1137–42.
Gan CT, Lankhaar JW, Westerhof N, et al. Noninvasively assessed pulmonary artery stiffness predicts mortality in pulmonary arterial hypertension. Chest. 2007;132:1906–12.
Blyth KG, Groenning BA, Martin TN, et al. Contrast enhanced-cardiovascular magnetic resonance imaging in patients with pulmonary hypertension. Eur Heart J. 2005;26:1993–9.
McCann GP, Gan CT, Beek AM, et al. Extent of MRI delayed enhancement of myocardial mass is related to right ventricular dysfunction in pulmonary artery hypertension. Am J Roentgenol. 2007;188:349–55.
Sanz J, Dellegrottaglie S, Kariisa M, et al. Prevalence and correlates of septal delayed contrast enhancement in patients with pulmonary hypertension. Am J Cardiol. 2007;100:731–5.
Junqueira FP, Macedo R, Coutinho AC, et al. Myocardial delayed enhancement in patients with pulmonary hypertension and right ventricular failure: evaluation by cardiac MRI. Br J Radiol. 2009;82:821–6.
Shehata ML, Lossnitzer D, Skrok J, et al. Myocardial delayed enhancement in pulmonary hypertension: pulmonary hemodynamics, right ventricular function, and remodeling. AJR Am J Roentgenol. 2011;196:87–94.
Freed BH, Gomberg-Maitland M, Chandra S. Contrast-enhanced cardiovascular magnetic resonance predicts clinical worsening in patients with pulmonary hypertension. Presented at the Society of Cardiovascular Magnetic Resonance/EuroCMR International Conference. Nice, France; February 3–6, 2011.
Zafrir N, Zingerman B, Solodky A, et al. Use of noninvasive tools in primary pulmonary hypertension to assess the correlation of right ventricular function with functional capacity and to predict outcome. Int J Cardiovasc Imaging. 2007;23:209–15.
Kawut S, Horn EM, Berekashvili KK, et al. New predictors of outcome in idiopathic pulmonary arterial hypertension. Am J Cardiol. 2005;95:199–203.
•• van de Veerdonk MC, Kind T, Marcus JT, et al. Progressive right ventricular dysfunction in patients with pulmonary arterial hypertension responding to therapy. J Am Coll Cardiol. 2011;58:2511–9.
This is one of the first published papers showing the powerful, independent prognostic capability of CMR-derived RVEF in patients with PAH
Wilkins MR, Paul GA, Strange JW, et al. Sildenafil versus endothelin receptor antagonist for pulmonary hypertension (SERAPH) study. Am J Respir Crit Care Med. 2005;171:1292–7.
Sciancalepore MA, Maffessanti F, Patel AR, et al. Three-dimensional analysis of interventricular septal curvature from cardiac magnetic resonance images for the evaluation of patients with pulmonary hypertension. Int J Cardiovasc Imaging 2011 [Epub ahead of print].
Champion HC, Michelakis ED, Hassoun PM. Comprehensive invasive and noninvasive approach to the right ventricle pulmonary circulation unit: state of the art and clinical and research implications. Circulation. 2009;120:992–1007.
Sanz J, Kariisa M, Dellegrottaglie S, et al. Evaluation of pulmonary artery stiffness in pulmonary hypertension with cardiac magnetic resonance. JACC: Cardiovasc Imaging. 2009;2:286–95.
Shehata M, Basha TA, Tantawy WH, et al. Real-time single-heartbeat fast strain-encoded imaging of right ventricular regional function: normal versus chronic pulmonary hypertension. Magn Reson Med. 2010;64:98–106.
Vogel-claussen J, Skrok J, Shehata ML, et al. Right and left ventricular myocardial perfusion reserves correlate with right ventricular function and pulmonary hemodynamics in patients with pulmonary arterial hypertension. Radiology. 2011;258:119–27.
Bokhari S, Raina A, Rosenweig EB, et al. PET imaging may provide a novel biomarker and understanding of right ventricular dysfunction in patients with idiopathic pulmonary arterial hypertension. Circ Cardiovasc Imaging. 2011;4:641–7.
Shah SJ, Thenappan T, Rich S, et al. Value of exercise treadmill testing in the risk stratification of patients with pulmonary hypertension. Circ Heart Fail. 2009;2:278–86.
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