Nonidiopathic Pulmonary Hypertension

  • Author: Nader Kamangar, MD, FACP, FCCP, FCCM; Chief Editor: Ryland P Byrd, Jr, MD  more...
 
Updated: Jan 27, 2016
 

Background

Pulmonary hypertension (PH), defined as a mean pulmonary arterial pressure greater than 25 mm Hg at rest or greater than 30 mm Hg during exercise, is often characterized by a progressive and sustained increase in pulmonary vascular resistance that eventually may lead to right ventricular (RV) failure. It can be a life-threatening condition if untreated. Treatment success rates vary according to the specific cause.

Cardiac disorders, pulmonary diseases, or both in combination are the most common causes of nonidiopathic pulmonary hypertension (see the images below). Cardiac diseases produce PH via volume or pressure overload, though subsequent intimal proliferation of pulmonary resistance vessels adds an obstructive element. Perivascular parenchymal changes, along with pulmonary vasoconstriction, are the mechanisms of PH in respiratory diseases.

Gross pathology on patient who died of severe pulm Gross pathology on patient who died of severe pulmonary arterial hypertension secondary to persistent patent ductus arteriosus.
Close-up view of gross pathology on patient who di Close-up view of gross pathology on patient who died of severe arterial pulmonary hypertension secondary to persistent patent ductus arteriosus.

Therapy for PH is targeted at the underlying cause and its effects on the cardiovascular system. Novel therapeutic agents, such as prostacyclin and others undergoing clinical trials, have led to the possibility of specific therapies for these once untreatable disorders.

For patient education resources, see the Lung and Airway Center.

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Etiology

Pulmonary hypertension was previously divided into two categories, primary and secondary, depending on whether a specific cause could be identified. In 1998, the World Health Organization (WHO) proposed a clinical classification of pulmonary hypertension into five main groups on the basis of similarities in pathophysiology, clinical presentation, and therapeutic options. This classification was later revised in Venice in 2003 and again in Dana Point in 2008 to further clarify classifications.[1]

Group 1, pulmonary arterial hypertension (PAH), is further divided into the following 4 subgroups:

  • Subgroup 1 - Idiopathic PAH (IPAH)
  • Subgroup 2 - Heritable PAH, including those with BMPR2 and ALK2 gene mutations
  • Subgroup 3 - Drug- and toxin-induced PAH (Aminorex, fenfluramine derivatives, and toxic rapeseed oil have been identified as definite risk factors for PAH. [1] )
  • Subgroup 4 - Conditions with known localization of lesions in the small pulmonary arterioles, including collagen-vascular disease (scleroderma/ CREST syndrome), congenital left-to-right shunts, portopulmonary hypertension, HIV-associated pulmonary hypertension, schistosomiasis, and chronic hemolytic anemia
  • Subgroup 5 – Persistent pulmonary hypertension of the newborn

Group 2, pulmonary hypertension owing to left-sided heart disease, consists of left-sided myocardial and valvular diseases and extrinsic compression of the pulmonary veins (eg, tumors) and pulmonary veno-occlusive disease.

Group 3, pulmonary hypertension owing to lung diseases and/or hypoxia, consists of diseases that cause inadequate arterial oxygenation. Such conditions include lung disease (eg, chronic obstructive pulmonary disease [COPD] and interstitial lung disease), impaired respiration (eg, obstructive sleep apnea [OSA][2] and alveolar hypoventilation disorders), and long-term exposure to high altitude.

Group 4, chronic thromboembolic pulmonary hypertension (CTEPH). CTEPH occurs in a minority of patients after acute embolism. Approximately 0.1% of survivors develop progressive pulmonary hypertension. Fewer than 1% of these patients have deficiencies of antithrombin 3, protein C, or protein S. No consistent defect in fibrinolytic activity has been identified.

Pathologically, these patients have a full range of pulmonary hypertensive lesions, including plexogenic lesions in the small pulmonary arteries. These patients present with progressive dyspnea and exercise intolerance. Physical examination findings demonstrate RV failure and PAH.

Group 5, pulmonary hypertension with unclear or multifactorial etiologies, is further divided into the following 4 subgroups:

  • Subgroup 1 - Hematologic disorders, including myeloproliferative disorders
  • Subgroup 2 - Systemic disorders, including sarcoidosis, pulmonary Langerhans cell histiocytosis, lymphangioleiomyomatosis, neurofibromatosis, and vasculitis
  • Subgroup 3 - Metabolic disorders, including glycogen storage disease, Gaucher disease, and thyroid disorders
  • Subgroup 4 - Miscellaneous conditions, including tumor obstruction, mediastinal fibrosis, and chronic renal failure on dialysis

On the basis of information adapted from the executive summary of the world symposium on Primary Pulmonary Hypertension in Evian, France, in 1998, pulmonary hypertension may be divided into the following functional classes:

  • Class I – These are patients with pulmonary hypertension but without resulting limitation of physical activity. Ordinary physical activity does not cause undue dyspnea or fatigue, chest pain, or near-syncope in patients.
  • Class II – These are patients with pulmonary hypertension resulting in slight limitation of physical activity. The patients are comfortable at rest, but ordinary physical activity causes undue dyspnea or fatigue, chest pain, or near-syncope.
  • Class III – These are patients with pulmonary hypertension resulting in marked limitation of physical activity. Patients are comfortable at rest, but even less-than-ordinary activity causes undue dyspnea or fatigue, chest pain, or near-syncope.
  • Class IV – These are patients with pulmonary hypertension who are unable to perform any physical activity without symptoms. These patients manifest signs of right-sided heart failure, dyspnea or fatigue may even be present at rest, and discomfort is increased by any physical activity.
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Epidemiology

The overall prevalence of pulmonary hypertension in the general population is unknown, owing to the heterogeneity of the disease. In specific subgroups of pulmonary hypertension patients, studies have estimated the prevalence as follows:

  • In an observational study of 277 patients with HIV infection, 0.46% of patients had pulmonary hypertension [3] . In comparison with prior studies, [4] no change in prevalence rate was seen with modern highly active antiretroviral treatment (HAART).
  • A systematic review of several studies of patients with OSA estimated the prevalence of pulmonary hypertension to be 15-20%. [5]
  • A systematic review of several studies among patients with COPD estimated the prevalence of pulmonary hypertension to be 10-30%. [6]
  • In scleroderma patients, the incidence has been estimated to be 6-60% of all patients, with the variance based on the extent of disease. [7]
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Prognosis

Increasing pulmonary arterial pressure is associated with a progressive decline in survival for patients with COPD or interstitial lung diseases. The prognosis of patients with nonidiopathic pulmonary hypertension is variable and depends on the severity of hemodynamic derangement and the underlying primary disorder.

Patients with severe pulmonary hypertension or right-sided heart failure survive approximately one year. Patients with moderate elevations in pulmonary arterial pressure (mean pressure below 55 mm Hg) and preserved right-sided heart function have a median survival of three years from diagnosis.

On the basis of Centers for Disease Control and Prevention (CDC) Pulmonary Hypertension Surveillance from 1980 to 2002, the following mortality data were reported[8] :

  • The age-standardized death rates for the total US population increased from 5.2 to 5.4 deaths per 100,000 population.
  • The main increase in death rates was seen among women, with an increase from 3.3 to 5.5 deaths per 100,000 population, and blacks, with an increase from 4.6 to 7.3 deaths per 100,000 population.
  • The death rate in males decreased over this time, from 8.2 to 5.4 deaths per 100,000 population.
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Contributor Information and Disclosures
Author

Nader Kamangar, MD, FACP, FCCP, FCCM Professor of Clinical Medicine, University of California, Los Angeles, David Geffen School of Medicine; Chief, Division of Pulmonary and Critical Care Medicine, Vice-Chair, Department of Medicine, Olive View-UCLA Medical Center

Nader Kamangar, MD, FACP, FCCP, FCCM is a member of the following medical societies: Academy of Persian Physicians, American Academy of Sleep Medicine, American Association for Bronchology and Interventional Pulmonology, American College of Chest Physicians, American College of Critical Care Medicine, American College of Physicians, American Lung Association, American Medical Association, American Thoracic Society, Association of Pulmonary and Critical Care Medicine Program Directors, Association of Specialty Professors, California Sleep Society, California Thoracic Society, Clerkship Directors in Internal Medicine, Society of Critical Care Medicine, Trudeau Society of Los Angeles, World Association for Bronchology and Interventional Pulmonology

Disclosure: Nothing to disclose.

Coauthor(s)

Sat Sharma, MD, FRCPC Professor and Head, Division of Pulmonary Medicine, Department of Internal Medicine, University of Manitoba; Site Director, Respiratory Medicine, St Boniface General Hospital

Sat Sharma, MD, FRCPC is a member of the following medical societies: American Academy of Sleep Medicine, American College of Chest Physicians, American College of Physicians-American Society of Internal Medicine, American Thoracic Society, Canadian Medical Association, Royal College of Physicians and Surgeons of Canada, Royal Society of Medicine, Society of Critical Care Medicine, World Medical Association

Disclosure: Nothing to disclose.

Kelvin Chan, MD Resident Physician, Department of Medicine, Division of Pulmonary, Critical Care and Sleep Medicine, Olive View-UCLA Medical Center

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Chief Editor

Ryland P Byrd, Jr, MD Professor of Medicine, Division of Pulmonary Disease and Critical Care Medicine, James H Quillen College of Medicine, East Tennessee State University

Ryland P Byrd, Jr, MD is a member of the following medical societies: American College of Chest Physicians, American Thoracic Society

Disclosure: Nothing to disclose.

Acknowledgements

Oleh Wasyl Hnatiuk, MD Program Director, National Capital Consortium, Pulmonary and Critical Care, Walter Reed Army Medical Center; Associate Professor, Department of Medicine, Uniformed Services University of Health Sciences

Oleh Wasyl Hnatiuk, MD is a member of the following medical societies: American College of Chest Physicians, American College of Physicians, and American Thoracic Society

Disclosure: Nothing to disclose.

Shahriar Pirouz, MD Resident Physician, Department of Internal Medicine, Olive View-UCLA Medical Center

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Reference Salary Employment

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Gross pathology on patient who died of severe pulmonary arterial hypertension secondary to persistent patent ductus arteriosus.
Close-up view of gross pathology on patient who died of severe arterial pulmonary hypertension secondary to persistent patent ductus arteriosus.
During pulmonary arterial thromboendarterectomy, bilateral proximal thrombus was carefully dissected and extracted, leading to resolution of nonidiopathic pulmonary hypertension.
Chest radiograph of patient with nonidiopathic pulmonary hypertension shows enlarged pulmonary arteries. This patient had atrial septal defect.
54-year-old woman with history of scleroderma (CREST variety—ie, calcinosis cutis, Raynaud phenomenon, esophageal motility disorder, sclerodactyly, and telangiectasia) developed dyspnea that worsened upon exertion. The patient was found to have severe pulmonary arterial hypertension.
54-year-old woman with history of scleroderma (CREST variety—ie, calcinosis cutis, Raynaud phenomenon, esophageal motility disorder, sclerodactyly, and telangiectasia) developed dyspnea that worsened on exertion. Spiral CT showed enlarged pulmonary arteries but no evidence of thromboembolism.
Ventilation-perfusion scan of bilateral mismatched segmental and subsegmental defects, suggesting chronic thromboembolic hypertension.
Left pulmonary arterial angiogram shows large central pulmonary arteries and attenuation of peripheral vessels, but thrombosis cannot be identified, because it has organized along vessel walls.
Bilateral angiography should be performed in patients suspected of having chronic thromboembolic pulmonary arterial hypertension. This right pulmonary arterial angiogram shows no evidence of filling defect, therefore excluding acute thrombosis. Angioscopy is potentially useful in this setting.
 
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