Elsevier

Disease-a-Month

Volume 62, Issue 11, November 2016, Pages 382-405
Disease-a-Month

Pulmonary arterial hypertension and associated conditions

https://doi.org/10.1016/j.disamonth.2016.03.006Get rights and content

Section snippets

Definition and epidemiology

Pulmonary hypertension (PH) refers to an increase in pulmonary arterial pressure, defined as mean pulmonary artery pressure (mPAP) ≥25 mmHg at rest, that if left untreated may progress to right ventricle dysfunction and failure.1 Worldwide, pulmonary hypertension associated with schistosomiasis is considered the most common form of the disease.2 In the United States, post-capillary pulmonary hypertension due to left heart disease is the most prevalent form of PH.

Pulmonary arterial hypertension

Clinical manifestations

Patients with PH commonly present with nonspecific symptoms and may be initially evaluated for other causes of shortness of breath before a diagnosis of pulmonary hypertension is even considered. As such, delays in the correct diagnosis can occur resulting in manifestations of right ventricle dysfunction prior to referral to a pulmonologist or cardiologist. It is important to appreciate that the signs and symptoms of PH correlate to the progression of underlying right ventricle impairment.

The

Drug and toxin induced PAH

Much of the initial interest and attention on drug-induced PAH focused around the use of potent appetite suppressants (anorexigens), such as aminorex fumarate in 1965 subsequently leading to its discontinuation.77 Today, drug and toxin-induced PAH comprise a relatively small proportion of PAH patients. The recent 2013 Nice guidelines identified specific medications as risk factors for developing PAH and categorized based on the strength of data available (Table 3).3 Aminorex fumarate,

Conclusion

Pulmonary arterial hypertension is a progressive and debilitating disease that requires early recognition and treatment. PAH can be idiopathic, heritable, or associated with various drugs or conditions, all of which share a similar pathophysiology. Recognizing the clinical manifestations of PAH and being able to distinguish patients with PAH from other groups of pulmonary hypertension can be challenging, but once detected can allow for early administration of PAH-specific therapy and improved

First page preview

First page preview
Click to open first page preview

References (124)

  • P. Moceri et al.

    Imaging in pulmonary hypertension: focus on the role of echocardiography

    Arch Cardiovasc Dis

    (2014)
  • L.G. Rudski et al.

    Guidelines for the echocardiographic assessment of the right heart in adults: a report from the American Society of Echocardiography endorsed by the European Association of Echocardiography, a registered branch of the European Society of Cardiology, and the Canadian Society of Echocardiography

    J Am Soc Echocardiogr

    (2010)
  • R.J. Raymond et al.

    Echocardiographic predictors of adverse outcomes in primary pulmonary hypertension

    J Am Coll Cardiol

    (2002)
  • A.L. Curnock et al.

    High prevalence of hypothyroidism in patients with primary pulmonary hypertension

    Am J Med Sci

    (1999)
  • H.H. Leuchte et al.

    Clinical significance of brain natriuretic peptide in primary pulmonary hypertension

    J Am Coll Cardiol

    (2004)
  • M.M. Hoeper et al.

    Complications of right heart catheterization procedures in patients with pulmonary hypertension in experienced centers

    J Am Coll Cardiol

    (2006)
  • J.L. Vachiery et al.

    Pulmonary hypertension due to left heart diseases

    J Am Coll Cardiol

    (2013)
  • N. Galie et al.

    Updated treatment algorithm of pulmonary arterial hypertension

    J Am Coll Cardiol

    (2013)
  • S. Alam et al.

    Standard therapies for pulmonary arterial hypertension

    Clin Chest Med

    (2007)
  • S. Rich et al.

    The short-term effects of digoxin in patients with right ventricular dysfunction from pulmonary hypertension

    Chest

    (1998)
  • A.R. Tonelli et al.

    Pulmonary vasodilator testing and use of calcium channel blockers in pulmonary arterial hypertension

    Respir Med

    (2010)
  • B.J. Schrader et al.

    Comparison of the effects of adenosine and nifedipine in pulmonary hypertension

    J Am Coll Cardiol

    (1992)
  • R.L. Benza et al.

    The REVEAL Registry risk score calculator in patients newly diagnosed with pulmonary arterial hypertension

    Chest

    (2012)
  • B. Ranchoux et al.

    Chemotherapy-induced pulmonary hypertension: role of alkylating agents

    Am J Pathol

    (2015)
  • R.W. Battle et al.

    Prevalence of pulmonary hypertension in limited and diffuse scleroderma

    Chest

    (1996)
  • C. Haas

    Pulmonary hypertension associated with systemic lupus erythematosus

    Bull Acad Natl Med

    (2004)
  • D. van der Linde et al.

    Birth prevalence of congenital heart disease worldwide: a systematic review and meta-analysis

    J Am Coll Cardiol

    (2011)
  • I.O. Colle et al.

    Diagnosis of portopulmonary hypertension in candidates for liver transplantation: a prospective study

    Hepatology

    (2003)
  • M. Lapa et al.

    Cardiopulmonary manifestations of hepatosplenic schistosomiasis

    Circulation

    (2009)
  • E. Luthy

    Proceedings: the epidemic of primary pulmonary hypertension in Europe

    Pathol Microbiol (Basel)

    (1975)
  • G. Kovacs et al.

    Pulmonary arterial pressure during rest and exercise in healthy subjects: a systematic review

    Eur Respir J

    (2009)
  • P. Wood

    Diseases of the Heart and Circulation

    (1962)
  • M. Humbert et al.

    Cellular and molecular pathobiology of pulmonary arterial hypertension

    J Am Coll Cardiol

    (2004)
  • G.G. Pietra et al.

    Pathologic assessment of vasculopathies in pulmonary hypertension

    J Am Coll Cardiol

    (2004)
  • R.E. Harrison et al.

    Molecular and functional analysis identifies ALK-1 as the predominant cause of pulmonary hypertension related to hereditary haemorrhagic telangiectasia

    J Med Genet

    (2003)
  • E.D. Austin et al.

    The genetics of pulmonary arterial hypertension

    Circ Res

    (2014)
  • J.H. Newman et al.

    Mutation in the gene for bone morphogenetic protein receptor II as a cause of primary pulmonary hypertension in a large kindred

    N Engl J Med

    (2001)
  • M. Eyries et al.

    EIF2AK4 mutations cause pulmonary veno-occlusive disease, a recessive form of pulmonary hypertension

    Nat Genet

    (2014)
  • R. Colman et al.

    Utility of the physical examination in detecting pulmonary hypertension. A mixed methods study

    PloS One

    (2014)
  • F. Van de Werf et al.

    The mechanism of disappearance of the physiologic third heart sound with age

    Circulation

    (1986)
  • J.M. Chua Chiaco et al.

    The jugular venous pressure revisited

    Cleve Clin J Med

    (2013)
  • S. Rich et al.

    Primary pulmonary hypertension. A national prospective study

    Ann Intern Med

    (1987)
  • G. Kopec et al.

    Electrocardiogram for the diagnosis of right ventricular hypertrophy and dilation in idiopathic pulmonary arterial hypertension

    Circ J

    (2012)
  • B. Chang et al.

    Scleroderma patients with combined pulmonary hypertension and interstitial lung disease

    J Rheumatol

    (2003)
  • Q.A. Truong et al.

    Reference values for normal pulmonary artery dimensions by noncontrast cardiac computed tomography: the Framingham Heart Study

    Circ Cardiovasc Imaging

    (2012)
  • T.E. Raymond et al.

    Significance of main pulmonary artery dilation on imaging studies

    Annal Am Thorac Soc

    (2014)
  • A. Mahammedi et al.

    Pulmonary artery measurements in pulmonary hypertension: the role of computed tomography

    J Thorac Imaging

    (2013)
  • E.J. Barbosa et al.

    Current role of imaging in the diagnosis and management of pulmonary hypertension

    Am J Roentgenol

    (2012)
  • G. Lewis et al.

    Multi-detector CT assessment in pulmonary hypertension: techniques, systematic approach to interpretation and key findings

    Quant Imaging Med Surg

    (2015)
  • E.J. Stern et al.

    CT mosaic pattern of lung attenuation: distinguishing different causes

    Am J Roentgenol

    (1995)
  • View full text