Pulmonary arterial hypertension and associated conditions
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
References (124)
- et al.
Definitions and diagnosis of pulmonary hypertension
J Am Coll Cardiol
(2013) - et al.
Updated clinical classification of pulmonary hypertension
J Am Coll Cardiol
(2013) - et al.
Pulmonary arterial hypertension: baseline characteristics from the REVEAL Registry
Chest
(2010) - et al.
Genetics and genomics of pulmonary arterial hypertension
J Am Coll Cardiol
(2009) - et al.
Diagnosis and assessment of pulmonary arterial hypertension
J Am Coll Cardiol
(2009) The abdominojugular reflux sign
Am J Med
(2000)- et al.
Predictive values of the electrocardiogram in diagnosing pulmonary hypertension
Int J Cardiol
(2008) - et al.
Pulmonary function in primary pulmonary hypertension
J Am Coll Cardiol
(2003) - et al.
Multidetector computed tomographic pulmonary angiography: beyond acute pulmonary embolism
Radiol Clin North Am
(2010) - et al.
2013 ACCF/AHA guideline for the management of heart failure: a report of the American College of Cardiology Foundation/American Heart Association Task Force on Practice Guidelines
J Am Coll Cardiol
(2013)
Imaging in pulmonary hypertension: focus on the role of echocardiography
Arch Cardiovasc Dis
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
Echocardiographic predictors of adverse outcomes in primary pulmonary hypertension
J Am Coll Cardiol
High prevalence of hypothyroidism in patients with primary pulmonary hypertension
Am J Med Sci
Clinical significance of brain natriuretic peptide in primary pulmonary hypertension
J Am Coll Cardiol
Complications of right heart catheterization procedures in patients with pulmonary hypertension in experienced centers
J Am Coll Cardiol
Pulmonary hypertension due to left heart diseases
J Am Coll Cardiol
Updated treatment algorithm of pulmonary arterial hypertension
J Am Coll Cardiol
Standard therapies for pulmonary arterial hypertension
Clin Chest Med
The short-term effects of digoxin in patients with right ventricular dysfunction from pulmonary hypertension
Chest
Pulmonary vasodilator testing and use of calcium channel blockers in pulmonary arterial hypertension
Respir Med
Comparison of the effects of adenosine and nifedipine in pulmonary hypertension
J Am Coll Cardiol
The REVEAL Registry risk score calculator in patients newly diagnosed with pulmonary arterial hypertension
Chest
Chemotherapy-induced pulmonary hypertension: role of alkylating agents
Am J Pathol
Prevalence of pulmonary hypertension in limited and diffuse scleroderma
Chest
Pulmonary hypertension associated with systemic lupus erythematosus
Bull Acad Natl Med
Birth prevalence of congenital heart disease worldwide: a systematic review and meta-analysis
J Am Coll Cardiol
Diagnosis of portopulmonary hypertension in candidates for liver transplantation: a prospective study
Hepatology
Cardiopulmonary manifestations of hepatosplenic schistosomiasis
Circulation
Proceedings: the epidemic of primary pulmonary hypertension in Europe
Pathol Microbiol (Basel)
Pulmonary arterial pressure during rest and exercise in healthy subjects: a systematic review
Eur Respir J
Diseases of the Heart and Circulation
Cellular and molecular pathobiology of pulmonary arterial hypertension
J Am Coll Cardiol
Pathologic assessment of vasculopathies in pulmonary hypertension
J Am Coll Cardiol
Molecular and functional analysis identifies ALK-1 as the predominant cause of pulmonary hypertension related to hereditary haemorrhagic telangiectasia
J Med Genet
The genetics of pulmonary arterial hypertension
Circ Res
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
EIF2AK4 mutations cause pulmonary veno-occlusive disease, a recessive form of pulmonary hypertension
Nat Genet
Utility of the physical examination in detecting pulmonary hypertension. A mixed methods study
PloS One
The mechanism of disappearance of the physiologic third heart sound with age
Circulation
The jugular venous pressure revisited
Cleve Clin J Med
Primary pulmonary hypertension. A national prospective study
Ann Intern Med
Electrocardiogram for the diagnosis of right ventricular hypertrophy and dilation in idiopathic pulmonary arterial hypertension
Circ J
Scleroderma patients with combined pulmonary hypertension and interstitial lung disease
J Rheumatol
Reference values for normal pulmonary artery dimensions by noncontrast cardiac computed tomography: the Framingham Heart Study
Circ Cardiovasc Imaging
Significance of main pulmonary artery dilation on imaging studies
Annal Am Thorac Soc
Pulmonary artery measurements in pulmonary hypertension: the role of computed tomography
J Thorac Imaging
Current role of imaging in the diagnosis and management of pulmonary hypertension
Am J Roentgenol
Multi-detector CT assessment in pulmonary hypertension: techniques, systematic approach to interpretation and key findings
Quant Imaging Med Surg
CT mosaic pattern of lung attenuation: distinguishing different causes
Am J Roentgenol
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