Abstract
Purpose of Review
The purpose of this review is to define pulmonary hypertension in the setting of left heart disease (PH-LHD), discuss its epidemiology and pathophysiology, and highlight the cause and effect relationship it has with disease progression in the setting of cardiomyopathy.
Recent Findings
Both pulmonary hypertension (PH) and heart failure are becoming increasingly common. As such, PH-LHD is now the most common form of PH. The pathophysiology of the condition relates to backward transmission of elevated left ventricular filling pressures into the pulmonary circulation and, ultimately, right ventricular (RV) strain/dysfunction. It is evident that these pathophysiologic processes are both the effect and cause of left heart disease progression.
Summary
In this review, we describe the complex relationship between disease progression in left ventricular cardiomyopathy and PH-LHD. Clinicians and researchers should take note of the importance of PH-LHD and RV dysfunction to appropriately risk stratify patients and develop therapies for the condition.
Similar content being viewed by others
References
Papers of particular interest, published recently, have been highlighted as: •• Of major importance
Mazurek JA, Jessup M. Understanding heart failure. Heart Fail Clin. 2017;13:1–19. https://doi.org/10.1016/j.hfc.2016.07.001.
Mozaffarian D, Benjamin EJ, Go AS, Arnett DK, Blaha MJ, Cushman M, et al. Heart disease and stroke statistics—2016 update. Circulation. 2015:CIR.0000000000000350; https://doi.org/10.1161/CIR.0000000000000350.
Yancy CW, Jessup M, Bozkurt B, Butler J, Casey DE, Drazner MH, et al. 2013 ACCF/AHA Guideline for the Management of Heart Failure: a report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines. J Am Coll Cardiol. 2013;62:e147–239. https://doi.org/10.1016/j.jacc.2013.05.019.
•• Galiè N, Humbert M, Vachiery J-L, Gibbs S, Lang I, Torbicki A, et al. 2015 ESC/ERS guidelines for the diagnosis and treatment of pulmonary hypertensionThe 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;37:67–119. https://doi.org/10.1093/eurheartj/ehv317. This is an excellent recent guideline document on the topic of pulmonary hypertension.
Vachiéry J-L, Adir Y, Barberà JA, Champion H, Coghlan JG, Cottin V, et al. Pulmonary hypertension due to left heart diseases. J Am Coll Cardiol. 2013;62:D100–8. https://doi.org/10.1016/j.jacc.2013.10.033.
•• Rosenkranz S, Gibbs JSR, Wachter R, De Marco T, Vonk-Noordegraaf A, Vachiéry J-L. Left ventricular heart failure and pulmonary hypertension. Eur Heart J. 2016;37:942–54. https://doi.org/10.1093/eurheartj/ehv512. This is an excellent update article on the specific topic of PH-LHD.
Gerges C, Gerges M, Lang MB, Zhang Y, Jakowitsch J, Probst P, et al. Diastolic pulmonary vascular pressure gradient. Chest. 2013;143:758–66. https://doi.org/10.1378/chest.12-1653.
Tampakakis E, Leary PJ, Selby VN, Marco TD, Cappola TP, Felker GM, et al. The diastolic pulmonary gradient does not predict survival in patients with pulmonary hypertension due to left heart disease. JACC Heart Fail. 2015;3:9–16. https://doi.org/10.1016/j.jchf.2014.07.010.
Tedford RJ, Beaty CA, Mathai SC, Kolb TM, Damico R, Hassoun PM, et al. Prognostic value of the pre-transplant diastolic pulmonary artery pressure to pulmonary capillary wedge pressure gradient (DPG) in cardiac transplant recipients with pulmonary hypertension. J Heart Lung Transplant Off Publ Int Soc Heart Transplant. 2014;33:289–97. https://doi.org/10.1016/j.healun.2013.11.008.
Al-Naamani N, Preston IR, Paulus JK, Hill NS, Roberts KE. Pulmonary arterial capacitance is an important predictor of mortality in heart failure with a preserved ejection fraction. JACC Heart Fail. 2015;3:467–74. https://doi.org/10.1016/j.jchf.2015.01.013.
Gerges M, Gerges C, Pistritto A-M, Lang MB, Trip P, Jakowitsch J, et al. Pulmonary hypertension in heart failure. epidemiology, right ventricular function, and survival. Am J Respir Crit Care Med. 2015;192:1234–46. https://doi.org/10.1164/rccm.201503-0529OC.
Assad TR, Hemnes AR, Larkin EK, Glazer AM, Xu M, Wells QS, et al. Clinical and biological insights into combined post- and pre-capillary pulmonary hypertension. J Am Coll Cardiol. 2016;68:2525–36. https://doi.org/10.1016/j.jacc.2016.09.942.
Mazurek JA, Horne BD, Saeed W, Sardar MR, Zolty R. Galectin-3 levels are elevated and predictive of mortality in pulmonary hypertension. Heart Lung Circ. 2017; https://doi.org/10.1016/j.hlc.2016.12.012.
Caravita S, Faini A, Deboeck G, Bondue A, Naeije R, Parati G, et al. Pulmonary hypertension and ventilation during exercise: role of the pre-capillary component. J Heart Lung Transplant. 2017;36:754–62. https://doi.org/10.1016/j.healun.2016.12.011.
Mazurek JA, Forfia PR. Enhancing the accuracy of echocardiography in the diagnosis of pulmonary arterial hypertension: looking at the heart to learn about the lungs. Curr Opin Pulm Med. 2013;19:437–45. https://doi.org/10.1097/MCP.0b013e3283645966.
Mazurek JA, Vaidya A, Grandin EW, Forfia P. RVOT Doppler notching predicts diastolic-to-wedge gradient in left heart disease-associated pulmonary hypertension. J Am Coll Cardiol. 2015;65:A1537. https://doi.org/10.1016/S0735-1097(15)61537-6.
Lam CSP, Borlaug BA, Kane GC, Enders FT, Rodeheffer RJ, Redfield MM. Age-associated increases in pulmonary artery systolic pressure in the general population. Circulation. 2009;119:2663–70. https://doi.org/10.1161/CIRCULATIONAHA.108.838698.
Lam CSP, Roger VL, Rodeheffer RJ, Borlaug BA, Enders FT, Redfield MM. Pulmonary hypertension in heart failure with preserved ejection fraction: a community-based study. J Am Coll Cardiol. 2009;53:1119–26. https://doi.org/10.1016/j.jacc.2008.11.051.
Leung CC, Moondra V, Catherwood E, Andrus BW. Prevalence and risk factors of pulmonary hypertension in patients with elevated pulmonary venous pressure and preserved ejection fraction. Am J Cardiol. 2010;106:284–6. https://doi.org/10.1016/j.amjcard.2010.02.039.
Shah AM, Shah SJ, Anand IS, Sweitzer NK, O’Meara E, Heitner JF, et al. Cardiac structure and function in heart failure with preserved ejection FractionClinical Perspective. Circ Heart Fail. 2014;7:104–15. https://doi.org/10.1161/CIRCHEARTFAILURE.113.000887.
Farr G, Shah K, Markley R, Abbate A, Salloum FN, Grinnan D. Development of pulmonary hypertension in heart failure with preserved ejection fraction. Prog Cardiovasc Dis. 2016;59:52–8. https://doi.org/10.1016/j.pcad.2016.06.002.
Zotter-Tufaro C, Duca F, Kammerlander AA, Koell B, Aschauer S, Dalos D, et al. Diastolic pressure gradient predicts outcome in patients with heart failure and preserved ejection fraction. J Am Coll Cardiol. 2015;66:1308–10. https://doi.org/10.1016/j.jacc.2015.07.011.
Guazzi M, Borlaug BA. Pulmonary hypertension due to left heart disease. Circulation. 2012;126:975–90. https://doi.org/10.1161/CIRCULATIONAHA.111.085761.
Butler J, Chomsky DB, Wilson JR. Pulmonary hypertension and exercise intolerance in patients with heart failure. J Am Coll Cardiol. 1999;34:1802–6. https://doi.org/10.1016/S0735-1097(99)00408-8.
Bursi F, McNallan SM, Redfield MM, Nkomo VT, Lam CSP, Weston SA, et al. Pulmonary pressures and death in heart failure. J Am Coll Cardiol. 2012;59:222–31. https://doi.org/10.1016/j.jacc.2011.06.076.
Salamon JN, Kelesidis I, Msaouel P, Mazurek JA, Mannem S, Adzic A, et al. Outcomes in World Health Organization group II pulmonary hypertension: mortality and readmission trends with systolic and preserved ejection fraction–induced pulmonary hypertension. J Card Fail. 2014;20:467–75. https://doi.org/10.1016/j.cardfail.2014.05.003.
Miller WL, Grill DE, Borlaug BA. Clinical features, hemodynamics, and outcomes of pulmonary hypertension due to chronic heart failure with reduced ejection fraction. JACC Heart Fail. 2013;1:290–9. https://doi.org/10.1016/j.jchf.2013.05.001.
Ghio S, Gavazzi A, Campana C, Inserra C, Klersy C, Sebastiani R, et al. Independent and additive prognostic value of right ventricular systolic function and pulmonary artery pressure in patients with chronic heart failure. J Am Coll Cardiol. 2001;37:183–8. https://doi.org/10.1016/S0735-1097(00)01102-5.
Patel JB, Borgeson DD, Barnes ME, Rihal CS, Daly RC, Redfield MM. Mitral regurgitation in patients with advanced systolic heart failure. J Card Fail. 2004;10:285–91. https://doi.org/10.1016/j.cardfail.2003.12.006.
Guazzi M, Labate V. Pulmonary hypertension in heart failure patients: pathophysiology and prognostic implications. Curr Heart Fail Rep. 2016;13:281–94. https://doi.org/10.1007/s11897-016-0306-8.
Kutty RS, Parameshwar J, Lewis C, Catarino PA, Sudarshan CD, Jenkins DP, et al. Use of centrifugal left ventricular assist device as a bridge to candidacy in severe heart failure with secondary pulmonary hypertension. Eur J Cardiothorac Surg. 2013;43:1237–42. https://doi.org/10.1093/ejcts/ezs678.
Lundgren J, Algotsson L, Kornhall B, Rådegran G. Preoperative pulmonary hypertension and its impact on survival after heart transplantation. Scand Cardiovasc J. 2014;48:47–58. https://doi.org/10.3109/14017431.2013.877153.
Chen Y, Guo H, Xu D, Xu X, Wang H, Hu X, et al. Left ventricular failure produces profound lung remodeling and pulmonary hypertension in mice: heart failure causes severe lung disease. Hypertension. 2012;59:1170–8. https://doi.org/10.1161/HYPERTENSIONAHA.111.186072.
Delgado JF, Delgado JF. The pulmonary circulation in heart failure. Rev Esp Cardiol. 2010;63:334–45. https://doi.org/10.1016/S1885-5857(10)70066-9.
Tedford RJ, Hassoun PM, Mathai SC, Girgis RE, Russell SD, Thiemann DR, et al. Pulmonary capillary wedge pressure augments right ventricular pulsatile loading. Circulation. 2012;125:289–97. https://doi.org/10.1161/CIRCULATIONAHA.111.051540.
Champion HC, Michelakis ED, Hassoun PM. Comprehensive invasive and noninvasive approach to the right ventricle–pulmonary circulation unit. Circulation. 2009;120:992–1007. https://doi.org/10.1161/CIRCULATIONAHA.106.674028.
Guazzi M, Bandera F, Pelissero G, Castelvecchio S, Menicanti L, Ghio S, et al. Tricuspid annular plane systolic excursion and pulmonary arterial systolic pressure relationship in heart failure: an index of right ventricular contractile function and prognosis. Am J Physiol - Heart Circ Physiol. 2013;305:H1373–81. https://doi.org/10.1152/ajpheart.00157.2013.
Guazzi M, Naeije R, Arena R, Corrà U, Ghio S, Forfia P, et al. Echocardiography of right ventriculoarterial coupling combined with cardiopulmonary exercise testing to predict outcome in heart failure. Chest. 2015;148:226–34. https://doi.org/10.1378/chest.14-2065.
Methvin AB, Owens AT, Emmi AG, Allen M, Wiegers SE, Dries DL, et al. Ventilatory inefficiency reflects right ventricular dysfunction in systolic heart failure. Chest. 2011;139:617–25. https://doi.org/10.1378/chest.10-0318.
Lewis GD, Shah RV, Pappagianopolas PP, Systrom DM, Semigran MJ. Determinants of ventilatory efficiency in heart failure. Circ Heart Fail. 2008;1:227–33. https://doi.org/10.1161/CIRCHEARTFAILURE.108.785501.
Bosch L, Lam CSP, Gong L, Chan SP, Sim D, Yeo D, et al. Right ventricular dysfunction in left-sided heart failure with preserved versus reduced ejection fraction: right ventricular dysfunction in heart failure. Eur J Heart Fail. 2017; https://doi.org/10.1002/ejhf.873.
Dexter L. Atrial Septal Defect Heart. 1956;18:209–25. https://doi.org/10.1136/hrt.18.2.209.
Ghio S, Guazzi M, Scardovi AB, Klersy C, Clemenza F, Carluccio E, et al. Different correlates but similar prognostic implications for right ventricular dysfunction in heart failure patients with reduced or preserved ejection fraction. Eur J Heart Fail. 2017;19:873–9. https://doi.org/10.1002/ejhf.664.
Dini FL, Carluccio E, Simioniuc A, Biagioli P, Reboldi G, Galeotti GG, et al. Right ventricular recovery during follow-up is associated with improved survival in patients with chronic heart failure with reduced ejection fraction: right ventricular recovery in heart failure. Eur J Heart Fail. 2016;18:1462–71. https://doi.org/10.1002/ejhf.639.
Author information
Authors and Affiliations
Corresponding author
Ethics declarations
Conflict of Interest
The authors declare that they have no conflicts of interest.
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.
Additional information
This article is part of the Topical Collection on Prevention of Heart Failure
Rights and permissions
About this article
Cite this article
Adusumalli, S., Mazurek, J.A. Pulmonary Hypertension Due to Left Ventricular Cardiomyopathy: Is it the Result or Cause of Disease Progression?. Curr Heart Fail Rep 14, 507–513 (2017). https://doi.org/10.1007/s11897-017-0368-2
Published:
Issue Date:
DOI: https://doi.org/10.1007/s11897-017-0368-2