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Assessing Disease State in the Pulmonary Vasculature in Clinical Practice and Research

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Pulmonary Hypertension

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Abstract

The pulmonary circulation is normally a high-flow, low-resistance, low-pressure system that carries blood to the pulmonary microcirculation where gas exchange occurs. In pulmonary arterial hypertension (PAH), the vasculature becomes less compliant as a result of vascular smooth muscle cell proliferation and hypertrophy, and endothelial cell proliferation resulting in lumen obliteration. This change results in a highly resistant low compliance system, which places an undue load on the right ventricle, eventually leading to right heart failure. The timeline of events that result in an obliterative vascular remodeling parallels the evolution of right ventricular (RV) remodeling from a normal to a decompensated state. Patients commonly present with non- specific symptoms of exercise limitation and dyspnea. Once a diagnosis is made and patients are started on therapy clinicians and researchers face the challenge of how to serially and objectively follow patients to gauge treatment response. There are a number of approaches available and being developed to assess response to therapy. Consequently there is a need to analyze current techniques and identify the future techniques that might be useful to guide management of PAH.

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Authors and Affiliations

  1. Pulmonary Critical Care Medicine, Center for Pulmonary Heart Disease, Brigham and Women’s Hospital, Heart and Vascular Center, Boston, MA, USA

    Manyoo Agarwal MBBS

  2. Pulmonary and Critical Care Medicine, Cardiovascular Medicine, Brigham and Women’s Hospital Heart and Vascular Center, Harvard Medical School, 75 Francis Street, PBB CA-3, Boston, MA, 02115, USA

    Aaron B. Waxman MD, PhD, FACP, FCCP, FPVRI

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  1. Manyoo Agarwal MBBS
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  2. Aaron B. Waxman MD, PhD, FACP, FCCP, FPVRI
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Corresponding author

Correspondence to Aaron B. Waxman MD, PhD, FACP, FCCP, FPVRI .

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Editors and Affiliations

  1. Cardiovascular Medicine Dept, Harvard Medical School Brigham and Women’s Hospital, Boston, Massachusetts, USA

    Bradley A. Maron

  2. Pulmonary & Critical Care Medicine, Stanford University Medical Center, Stanford, California, USA

    Roham T. Zamanian

  3. Pulmonary Critical Care Medicine Dept Cardiovascular Medicine Dept, Brigham and Women's Hospital Harvard Medical School, Boston, Massachusetts, USA

    Aaron B. Waxman

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Agarwal, M., Waxman, A.B. (2016). Assessing Disease State in the Pulmonary Vasculature in Clinical Practice and Research. In: Maron, B., Zamanian, R., Waxman, A. (eds) Pulmonary Hypertension. Springer, Cham. https://doi.org/10.1007/978-3-319-23594-3_13

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  • DOI: https://doi.org/10.1007/978-3-319-23594-3_13

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-23593-6

  • Online ISBN: 978-3-319-23594-3

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