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Pulmonary Hypertension and Cor Pulmonale

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Comprehensive Cardiovascular Medicine in the Primary Care Setting

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

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Abstract

Pulmonary hypertension is a clinically heterogeneous, progressive disease. Tremendous recent advances in diagnosis and treatment options have improved clinical outcomes and survival (1, 2). Key to appropriate patient management is categorization of disease and treatment of underlying associated pathology. An expanded array of diagnostic tests allow for better characterization of clinical status; however, cardiac catheterization remains essential for assessment of disease severity, determination of prognosis, and choice of appropriate treatment. Multiple therapies for pulmonary hypertension have emerged in the past decade. These strategies provide more patient choice, are better tolerated, and result in improved functional class, decreased symptoms, and enhanced quality of life.

Key Points

• Pulmonary hypertension is defined as mean PA pressure > 25 mmHg at cardiac catheterization with PCWp ≤ 15 mmHg and PVR > 3 Wood units.

• Common pathological findings include endothelial hyperplasia, smooth muscle hypertrophy, thrombus formation, and plexiform lesions.

• Echocardiogram is the most appropriate screening modality.

• Thorough work-up to elucidate underlying cause is essential.

• Cardiac catheterization is used for confirmation of diagnosis as well as to assess response to therapy.

• Prognostic factors include right atrial pressure, cardiac index, right ventricular function, BNP, functional class, and exercise capacity.

• Determination of therapy is based on pulmonary vasodilator testing during cardiac catheterization followed by evidence-based clinical algorithm.

• Therapeutic objectives include symptom improvement, survival, and quality of life.

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Authors and Affiliations

  1. Department of Cardiology, Boston Adult Congenital Heart Service, Children’s Hospital, Boston, MA, USA

    Mary P. Mullen MD, PhD & Michael J. Landzberg MD

  2. Harvard Medical School, Boston, MA, USA

    Mary P. Mullen MD, PhD & Michael J. Landzberg MD

  3. Division of Cardiology, Brigham and Women’s Hospital, Boston, MA, USA

    Michael J. Landzberg MD

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  1. Mary P. Mullen MD, PhD
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Editors and Affiliations

  1. Sterling Rock Falls Clinic, 101 E. Miller Road, Sterling, 61081, USA

    Peter P. Toth

  2. Brigham & Women's Hospital, Cardiovascular Division, Harvard Medical School, Longwood Ave. 350, Boston, 02115, USA

    Christopher P. Cannon

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Mullen, M.P., Landzberg, M.J. (2011). Pulmonary Hypertension and Cor Pulmonale. In: Toth, P., Cannon, C. (eds) Comprehensive Cardiovascular Medicine in the Primary Care Setting. Contemporary Cardiology. Humana Press, Totowa, NJ. https://doi.org/10.1007/978-1-60327-963-5_18

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