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Congenital Heart Disease Associated with Pulmonary Arterial Hypertension

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Pulmonary Hypertension

Part of the book series: Contemporary Cardiology™ ((CONCARD))

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Abstract

Pulmonary arterial hypertension (PAH) associated with congenital heart disease (CHD) is common among the subtypes of PAH. It is quite variable in terms of clinical manifestations, severity of associated PAH, response to therapy, and outcomes, depending on the anatomy of the specific lesion, pulmonary circulatory flows and pressures, and other factors. Genetic predisposition likely plays a role, but no specific genetic abnormality has yet been identified for this form of PAH. Patients with CHD-PAH should undergo a careful evaluation that includes imaging of the defect as well as catheterization to characterize the severity and nature of pulmonary hemodynamic abnormalities. Surgical correction is desirable as long as the chances of reversibility are sufficient, but this may be difficult to ascertain preoperatively in marginal cases. Many patients now respond to pulmonary hypertension therapies if they are not surgical candidates or fail to improve after surgery, and lung or heart/lung transplantation remains an option for selected recalcitrant patients.

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Authors
  1. Michael J. Landzberg
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Editors and Affiliations

  1. Division of Pulmonary, Critical Care and Sleep Medicine Tufts Medical Center, Boston, MA

    Nicholas S. Hill MD

  2. Pulmonary Center, Boston University, Boston, MA

    Harrison W. Farber MD

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© 2008 Humana Press, Totowa, NJ

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Landzberg, M.J. (2008). Congenital Heart Disease Associated with Pulmonary Arterial Hypertension. In: Hill, N.S., Farber, H.W. (eds) Pulmonary Hypertension. Contemporary Cardiology™. Humana Press. https://doi.org/10.1007/978-1-60327-075-5_7

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  • DOI: https://doi.org/10.1007/978-1-60327-075-5_7

  • Publisher Name: Humana Press

  • Print ISBN: 978-1-58829-661-0

  • Online ISBN: 978-1-60327-075-5

  • eBook Packages: MedicineMedicine (R0)

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