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Chapter 244. Pulmonary Hypertension

Allan J. Walkey, MD, MSc; Harrison W. Farber, MD

  1. How does the classification system for pulmonary hypertension (PH) aid in understanding the pathophysiology, diagnostic evaluation, and treatments for this condition?

  2. What signs and symptoms might increase the suspicion for elevated pulmonary arterial pressures?

  3. What is a rational diagnostic approach to suspected PH?

  4. What is the meaning of an elevated pulmonary pressure determined by echocardiography?

  5. When should right heart catheterization be considered in the evaluation of suspected PH?

  6. What are the key steps in the evaluation and management of acute right heart failure secondary to elevated pulmonary arterial pressure?

  7. What are the common side effects of the medications used to treat PH?

Pulmonary hypertension (PH) is simply defined as a mean pulmonary arterial pressure > 25 mm Hg at rest. However, this deceptively simple definition encompasses a broad spectrum of clinical entities. In this chapter, we will present an evidence-based approach to the care of the patient with elevated pulmonary arterial pressures. Table 244-1 lists landmark studies supporting the approach presented in this chapter.

Table 244-1 Evidence-Based Key References: Pulmonary Hypertension
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Table 244-1 Evidence-Based Key References: Pulmonary Hypertension
TopicSupporting Literature
EpidemiologyHumbert M, Sitbon O, Chaaouat A, et al. Pulmonary hypertension in France: results from a national registry. Am J Resp Crit Care Med. 2006;173:1023–1030.
Lam CSP, Roger VL, Rodeheffer RJ, et al. Pulmonary hypertension in patients with preserved ejection fraction: a community based study. J Am Coll Card. 2009;53:1119–1126.
Badesch DB, Raskob G, Elliott G, et al. Pulmonary arterial hypertension: baseline characteristics from the REVEAL registry. Chest. 2010;137(2):376–387.
Classification and pathophysiologyFarber HW, Loscalzo J. Pulmonary arterial hypertension. N Engl J Med. 2004;351:1655–1665.
Simonneau G, Robbins IM, Beghetti M, et al. Updated clinical classification of pulmonary hypertension. J Am Coll Cardiol. 2009;54:S43–S54.
Overbeek MJ, Vonk MC, Boonstra A, et al. Pulmonary arterial hypertension in limited cutaneous systemic sclerosis: a distinctive vasculopathy. Eur Respir J. 2009;34(2):371–379.
Diagnosis: examination, laboratoryRich S, Dantzker DR, Ayres SM, et al. Primary pulmonary hypertension: a national prospective study. Ann Intern Med. 1987;107:216–223.
Dahlstrom U. Can natriuretic peptides be used for the diagnosis of diastolic heart failure? Eur J Heart Fail. 2004;6:281–287.
Nagaya N, Nishikimi T, Uematsu M, et al. Plasma brain natriuretic peptide as a prognostic indicator in patients with primary pulmonary hypertension. Circulation. 2000;102:865–870.
Diagnosis: echocardiography, imagingAurigemma GP, Zile MR, Gaasch WH. Lack of relationship between Doppler indices of diastolic function and left ventricular pressure transients in patients with definite diastolic heart failure. Am Heart J. 2004;148:E12.
Fisher MR, Forfia PR, Chamera E, et al. Accuracy of doppler echocardiography in the hemodynamic assessment of pulmonary hypertension. Am J Respir Crit Care Med. 2009;179:615–621.
Chetty KG, Brown SE, Light RW. Identification of pulmonary hypertension in chronic obstructive pulmonary disease from routine chest radiographs. Am Rev Respir Dis. 1982;126:338–341.
Tunariu N, Gibbs SJ, Win Z, et al. Ventilation-perfusion scintigraphy is more sensitive than multidetector CTPA in detecting chronic thromboembolic pulmonary disease as a treatable cause of ...

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