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  • Review Article
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Pulmonary hypertension with left-sided heart disease

Nature Reviews Cardiology volume 7, pages 648–659 (2010)Cite this article

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Abstract

Pulmonary hypertension (PH) with left-sided heart disease is defined, according to the latest Venice classification, as a Group 2 PH, which includes left-sided ventricular or atrial disease, and left-sided valvular diseases. These conditions are all associated with increased left ventricular filling pressure. Although PH with left-sided heart disease is a common entity, and long-term follow-up trials have provided firm recognition that development of left-sided PH carries a poor outcome, available data on incidence, pathophysiology, and therapy are sparse. Mitral stenosis was reported as the most frequent cause of PH several decades ago, but PH with left-sided heart disease is now usually caused by systemic hypertension and ischemic heart disease. In patients with these conditions, PH develops as a consequence of impaired left ventricular relaxation and distensibility. Chronic sustained elevation of cardiogenic blood pressure in pulmonary capillaries leads to a cascade of untoward retrograde anatomical and functional effects that represent specific targets for therapeutic intervention. The pathophysiological and clinical importance of the hemodynamic consequences of left-sided heart disease, starting with lung capillary injury and leading to right ventricular overload and failure, are discussed in this Review, focusing on PH as an evolving contributor to heart failure that may be amenable to novel interventions.

Key Points

  • Pulmonary hypertension (PH) with left-sided heart disease is a common clinical entity that usually develops as a consequence of impaired relaxation and distensibility of the left ventricle

  • Although left-sided PH has a poor outcome, and patients with this condition have a high risk of developing right ventricular failure, available data on PH incidence, pathophysiology, and therapy are limited

  • Chronic sustained cardiogenic pressure elevation in pulmonary capillaries leads to a cascade of adverse retrograde anatomical and functional events that represent potential targets for intervention

  • Therapy of left-sided PH is challenging and most therapeutic strategies tested for this condition have had negative results

  • Although precise guidelines on the most beneficial and cost-effective strategies for managing left-sided PH are lacking, promising results have been observed with the use of PDE5 inhibitors

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Figure 1: Elevation in left atrial pressure and proposed sequence of events that lead to capillary stress failure and alveolar membrane remodeling.

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Acknowledgements

This work was supported by a grant from the Monzino Foundation.

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Authors and Affiliations

  1. Cardiopulmonary Unit, University of Milan, San Paolo Hospital, Via A. di Rudinì 8, 20142, Milan, Italy

    Marco Guazzi

  2. Department of Physical Therapy, Virginia Commonwealth University, 901 West Franklin Street, Richmond, 23284, VA, USA

    Ross Arena

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  1. Marco Guazzi
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M. Guazzi and R. Arena contributed to discussion of content for the article, researched data to include in the manuscript, reviewed and edited the manuscript before submission, and revised the manuscript in response to the peer-reviewers' comments.

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Correspondence to Marco Guazzi.

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Guazzi, M., Arena, R. Pulmonary hypertension with left-sided heart disease. Nat Rev Cardiol 7, 648–659 (2010). https://doi.org/10.1038/nrcardio.2010.144

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