Abstract
Pulmonary hypertension commonly accompanies left heart disease (Group 2 Pulmonary Hypertension in the Dana Point classification scheme), and often accompanies hypoxic/parenchymal lung disease (Group 3). The phenotypic and hemodynamic spectrum of pulmonary hypertension in these settings is enormous, and the clinician is often left with uncertainty regarding the appropriate evaluation and management of these patients. A comprehensive understanding of the prognostic significance of pulmonary hypertension, and appropriate strategies for its evaluation and management, is critical for practitioners caring for these challenging patients. Management of pulmonary hypertension in the setting of left heart disease focuses primarily on identification and management of factors such as hypoxemia or pulmonary emboli that may be superimposed on left heart disease, and on optimization of conventional heart failure therapy. An evolving role for pulmonary vasoactive therapy is foreseen. In parenchymal lung disease, pulmonary hypertension often portends a poor prognosis, but whether there is a role for pulmonary vasoactive therapy beyond correction of hypoxemia is a matter of much debate. This chapter provides a comprehensive review of the current understanding of these important issues.
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
Abbreviations
- CO:
-
Cardiac output
- COPD:
-
Chronic obstructive pulmonary disease
- HFpEF:
-
Heart failure with preserved left ventricular ejection fraction
- HFrEF:
-
Heart failure with reduced left ventricular ejection fraction
- LV:
-
Left ventricle
- MPAP:
-
Mean pulmonary artery pressure
- NYHA:
-
New York Heart Association
- PA:
-
Pulmonary artery
- PAH:
-
Pulmonary arterial hypertension
- PCWP:
-
Pulmonary capillary wedge pressure
- PDE5i:
-
Phosphodiesterase type 5 inhibition
- PHHFrEF:
-
Pulmonary hypertension with heart failure with reduced left ventricular ejection fraction
- PVR:
-
Pulmonary vascular resistance
- RV:
-
Right ventricle
- TPG:
-
Transpulmonary gradient
- WHO:
-
World Health Organization
References
Fang JC, DeMarco T, Givertz MM, Borlaug BA, Lewis GD, Rame JE, et al. World Health Organization Pulmonary Hypertension Group 2: Pulmonary hypertension due to left heart disease in the adult—a summary statement from the Pulmonary Hypertension Council of the International Society for Heart and Lung Transplantation. J Heart Lung Transplant. 2012;31(9):913–33.
Miller WL, Grill DE, Borlaug BA. Clinical features, hemodynamics, and outcomes of pulmonary hypertension due to chronic heart failure with reduced ejection fraction pulmonary hypertension and heart failure. JACC Heart Fail. 2013;1(4):290–9.
Adamson PB, Gold MR, Bennett T, Bourge RC, Stevenson LW, Trupp R, et al. continuous hemodynamic monitoring in patients with mild to moderate heart failure: Results of the Reducing Decompensation Events Utilizing Intracardiac Pressures in Patients With Chronic Heart Failure (REDUCEhf) Trial. Congest Heart Fail. 2011;17(5):248–54.
Goland S, Czer LSC, Kass RM, De Robertis MA, Mirocha J, Coleman B, et al. Pre-existing pulmonary hypertension in patients with End-stage heart failure: impact on clinical outcome and hemodynamic follow-up after orthotopic heart transplantation. J Heart Lung Transplant. 2007;26(4):312–8.
Braunwald E, Braunwald NS, Ross J, Morrow AG. Effects of mitral-valve replacement on the pulmonary vascular dynamics of patients with pulmonary hypertension. N Engl J Med. 1965;273(10):509–14.
Vincens JJ, Temizer D, Post JR, Edmunds LH, Herrmann HC. Long-term outcome of cardiac surgery in patients with mitral stenosis and severe pulmonary hypertension. Circulation. 1995;92(9):137–42.
Nair PK, Kormos RL, Teuteberg JJ, Mathier MA, Bermudez CA, Toyoda Y, et al. Pulsatile left ventricular assist device support as a bridge to decision in patients with end-stage heart failure complicated by pulmonary hypertension. J Heart Lung Transplant. 2010;29(2):201–8.
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(2):289–97.
Cheng J, Brockman R, Pina I, Gao Y, Wei S, Aguel F, et al. CardioMEMS Champion HF Monitoring system. FDA review of P100045. http://www.fdagov/downloads/AdvisoryCommittees/CommitteesMeetingMaterials/MedicalDevices/MedicalDevicesAdvisoryCommittee/CirculatorySystemDevicesPanel/UCM284020pdf. Accessed 8 Dec 2011.
Lewis GD, Murphy RM, Shah RV, Pappagianopoulos PP, Malhotra R, Bloch KD, et al. Pulmonary vascular response patterns during exercise in left ventricular systolic dysfunction predict exercise capacity and outcomes. Circ Heart Fail. 2011;4(3):276–85.
Lewis GD, Shah R, Shahzad K, Camuso JM, Pappagianopoulos PP, Hung J, et al. Sildenafil improves exercise capacity and quality of life in patients with systolic heart failure and secondary pulmonary hypertension. Circulation. 2007;116(14):1555–62.
Ghio S, Bonderman D, Felix SB, Ghofrani HA, Michelakis ED, Mitrovic V, et al. Left ventricular systolic dysfunction associated with pulmonary hypertension riociguat trial (LEPHT): rationale and design. Eur J Heart Fail. 2012;14(8):946–53.
Bonderman D, Ghio S, Felix SB, Ghofrani H-A, Michelakis E, Mitrovic V, et al. Riociguat for patients with pulmonary hypertension caused by systolic left ventricular dysfunction: a phase IIb double-blind, randomized, placebo-controlled, dose-ranging hemodynamic study. Circulation. 2013;128(5):502–11.
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(13):1119–26.
Guazzi M, Vicenzi M, Arena R, Guazzi MD. Pulmonary hypertension in heart failure with preserved ejection fraction: a target of phosphodiesterase-5 inhibition in a 1-year study. Circulation. 2011;124(2):164–74.
Redfield MM, Borlaug BA, Lewis GD, Mohammed SF, Semigran MJ, LeWinter MM, et al. PhosphdiesteRasE-5 inhibition to improve CLinical Status and EXercise capacity in diastolic heart failure (RELAX) trial: rationale and design. Circ Heart Fail. 2012;5(5):653–9.
Redfield MM, Chen HH, Borlaug BA, et al. Effect of phosphodiesterase-5 inhibition on exercise capacity and clinical status in heart failure with preserved ejection fraction: A randomized clinical trial. JAMA. 2013;309(12):1268–77.
Andersen KH, Iversen M, Kjaergaard J, Mortensen J, Nielsen-Kudsk JE, Bendstrup E, et al. Prevalence, predictors, and survival in pulmonary hypertension related to end-stage chronic obstructive pulmonary disease. J Heart Lung Transplant. 2012;31(4):373–80.
Carlsen J, Hasseriis Andersen K, Boesgaard S, Iversen M, Steinbrüchel D, Bøgelund AC. Pulmonary arterial lesions in explanted lungs after transplantation correlate with severity of pulmonary hypertension in chronic obstructive pulmonary disease. J Heart Lung Transplant. 2013;32(3):347–54.
Wrobel JP, Thompson BR, Williams TJ. Mechanisms of pulmonary hypertension in chronic obstructive pulmonary disease: A pathophysiologic review. J Heart Lung Transplant. 2012;31(6):557–64.
Barbera JA. Mechanisms of development of chronic obstructive pulmonary disease-associated pulmonary hypertensoin. Pulm Circ. 2013;3(1):160–4.
Weitzenblum E, Sautegeau A, Ehrhart M, Mammosser M, Pelletier A. Long-term oxygen therapy can reverse the progression of pulmonary hypertension in patients with chronic obstructive pulmonary disease. Am Rev Respir Dis. 1985;131(4):493–8.
Continuous or nocturnal oxygen therapy in hypoxemic chronic obstructive lung disease: a clinical trial. Ann Intern Med 1980;93(3):391–8.
Rietema H, Holverda S, Bogaard HJ, Marcus JT, Smit HJ, Westerhof N, et al. Sildenafil treatment in COPD does not affect stroke volume or exercise capacity. Eur Respir J. 2008;31(4):759–64.
Holverda S, Rietema H, Bogaard HJ, Westerhof N, Postmus PE, Boonstra A, et al. Acute effects of sildenafil on exercise pulmonary hemodynamics and capacity in patients with COPD. Pulm Pharmacol Ther. 2008;21(3):558–64.
Lederer DJ, Bartels MN, Schluger NW, Brogan F, Jellen P, Thomashow BM, et al. Sildenafil for chronic obstructive pulmonary disease: a randomized crossover trial. COPD. 2012;9(3):268–75.
Stolz D, Rasch H, Linka A, Di Valentino M, Meyer A, Brutsche M, et al. A randomised, controlled trial of bosentan in severe COPD. Eur Respir J. 2008;32(3):619–28.
Naeije R, Melot C, Mols P, Hallemans R. Reduction in pulmonary hypertension by prostaglandin E1 in decompensated chronic obstructive pulmonary disease. Am Rev Respir Dis. 1982;125(1):1–5.
Jones K, Higenbottam T, Wallwork J. Pulmonary vasodilation with prostacyclin in primary and secondary pulmonary hypertension. Chest. 1989;96(4):784–9.
Behr J, Ryu JH. Pulmonary hypertension in interstitial lung disease. Eur Respir J. 2008;31(6):1357–67.
Ryu JH, Krowka MJ, Swanson KL, Pellikka PA, McGoon MD. Pulmonary hypertension in patients with interstitial lung diseases. Mayo Clin Proc. 2007;82(3):342–50.
Nadrous HF, Pellikka PA, Krowka MJ, Swanson KL, Chaowalit N, Decker PA, et al. The impact of pulmonary hypertension on survival in patients with idiopathic pulmonary fibrosis. Chest. 2005;128(6 Suppl):616S–7.
Nadrous HF, Pellikka PA, Krowka MJ, Swanson KL, Chaowalit N, Decker PA, et al. Pulmonary hypertension in patients with idiopathic pulmonary fibrosis. Chest. 2005;128(4):2393–9.
Chaowalit N, Pellikka PA, Decker PA, Aubry M-C, Krowka MJ, Ryu JH, et al. Echocardiographic and clinical characteristics of pulmonary hypertension complicating pulmonary langerhans cell histiocytosis. Mayo Clin Proc. 2004;79(10):1269–75.
Belle-van Meerkerk G, Cramer MJ, Kwakkel-van Erp JM, Nugroho MA, Tahri S, de Valk HW, et al. Pulmonary hypertension is a mild comorbidity in end-stage cystic fibrosis patients. J Heart Lung Transplant. 2013;32(6):609–14.
Venuta F, Tonelli AR, Anile M, Diso D, De Giacomo T, Ruberto F, et al. Pulmonary hypertension is associated with higher mortality in cystic fibrosis patients awaiting lung transplantation. J Cardiovasc Surg (Torino). 2012;53(6):810–7.
Damy T, Burgel PR, Pepin JL, Boelle PY, Cracowski C, Murris-Espin M, et al. Pulmonary acceleration time to optimize the timing of lung transplant in cystic fibrosis. Pulm Circ. 2012;2(1):75–83.
Golbin JM, Somers VK, Caples SM. Obstructive sleep apnea, cardiovascular disease, and pulmonary hypertension. Proc Am Thorac Soc. 2008;5(2):200–6.
Arias MA, GarcÃa-RÃo F, Alonso-Fernández A, MartÃnez I, Villamor J. Pulmonary hypertension in obstructive sleep apnoea: effects of continuous positive airway pressure: a randomized, controlled cross-over study. Eur Heart J. 2006;27(9):1106–13.
Marik PE. The malignant obesity hypoventilation syndrome (MOHS). Obes Rev. 2012;13(10):902–9.
Marik PE, Desai H. The emergent malignant obesity hypoventilation syndrome: a New critical care syndrome. J Intensive Care Med. 2013;28(3):200.
Macavei VM, Spurling KJ, Loft J, Makker HK. Diagnostic predictors of obesity-hypoventilation syndrome in patients suspected of having sleep disordered breathing. J Clin Sleep Med. 2013;9(9):879–84.
Basoglu OK, Tasbakan MS. Comparison of clinical characteristics in patients with obesity hypoventilation syndrome and obese obstructive sleep apnea syndrome: a case-control study. Clin Respir J. 2014;8(2):167–74.
Author information
Authors and Affiliations
Corresponding author
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2015 Springer Science+Business Media New York
About this chapter
Cite this chapter
Frantz, R.P. (2015). Pulmonary Hypertension in Chronic Heart and Lung Disease. In: Klinger, J., Frantz, R. (eds) Diagnosis and Management of Pulmonary Hypertension. Respiratory Medicine, vol 12. Humana Press, New York, NY. https://doi.org/10.1007/978-1-4939-2636-7_5
Download citation
DOI: https://doi.org/10.1007/978-1-4939-2636-7_5
Publisher Name: Humana Press, New York, NY
Print ISBN: 978-1-4939-2635-0
Online ISBN: 978-1-4939-2636-7
eBook Packages: MedicineMedicine (R0)