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Dilatation of the Pulmonary Artery, Idiopathic

Synonyms of Dilatation of the Pulmonary Artery, Idiopathic

  • IDPA

Disorder Subdivisions

  • No subdivisions found.

General Discussion

Idiopathic dilatation of the pulmonary artery (IDPA) is a rare congenital defect characterized by a wider than normal main pulmonary artery in the absence of any apparent anatomical or physiological cause.

Symptoms

Idiopathic dilatation of the pulmonary artery commonly does not produce symptoms because there is no circulatory abnormality. Clinical signs are minimal, and usually consist of a palpable pulmonary ejection sound that disappears when the patient inhales, a soft pulmonary ejection systolic murmur (abnormal heart sound), and splitting of the second sound on breathing in. IDPA does not cause pulmonary valve disease, nor does bacterial endocarditis occur in patients with this condition. The electrocardiogram is normal, and diagnosis is made when chest X-rays reveal a dilated pulmonary artery without cardiac chamber enlargement.

Causes

The cause of idiopathic dilatation of the pulmonary artery is unknown. A defect in the normal development of pulmonary artery elastic tissue before or after birth has been postulated. The dilatation may also be a consequence of a generalized connective tissue disease as it is occasionally found in Marfan's syndrome or Ehlers-Danlos syndrome. (For more information on these disorders, choose "Marfan" and Ehlers-Danlos" as your search terms in the Rare Disease Database.

Affected Populations

The incidence and prevalence of IDPA are not known. Because the disorder is benign in most instances, neither clinicians nor epidemiologists are able to measure the distribution of the disease with confidence.

Related Disorders

N/A

Standard Therapies

Treatment for idiopathic dilatation of the pulmonary artery is not required. People with this condition have a normal life expectancy, provided they have no cardiac lesions.

Investigational Therapies

Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov. All studies receiving U.S. government funding, and some supported by private industry, are posted on this government web site.

For information about clinical trials being conducted at the NIH Clinical Center in Bethesda, MD, contact the NIH Patient Recruitment Office:

Tollfree: (800) 411-1222
TTY: (866) 411-1010
Email: prpl@cc.nih.gov

For information about clinical trials sponsored by private sources, contact:
www.centerwatch.com

Organizations related to Dilatation of the Pulmonary Artery, Idiopathic

References

JOURNAL ARTICLES
Mesquita SM, Castro CR, Ikari NM, et al. Likelihood of left main coronary artery compression based on pulmonary trunk diameter in patients with pulmonary hypertension. Am J Med. 2004;116:369-74.

Ring NJ, Marshall AJ. Idiopathic dilation of the pulmonary artery. Br J Radiol. 2002;75:532-35.

Hoeffel JC. Idiopathic dilation of the pulmonary artery: report of four cases. Magn Reson Imaging 2001;19:761.

Ugolini P, Mousseaux E, Sadou Y, et al. Idiopathic dilation of the pulmonary artery: report of four cases. Magn Reson Imaging. 1999;17:933-37.

McLaughlin VV, Genthner DE, Panella MM, et al. Reduction in pulmonary vascular resistance with long-term epoprostenol (prostacyclin) therapy in primary pulmonary hypertension. N Engl J Med. 1998;338:273-77.

FROM THE INTERNET
Idiopathic dilation of the main pulmonary artery - nd. 1p.
http://info.med.yale.edu/intmed/cardio/chd/e_idio_pa/

The information in NORD’s Rare Disease Database is for educational purposes only. It should never be used for diagnostic or treatment purposes. If you have questions regarding a medical condition, always seek the advice of your physician or other qualified health professional. NORD’s reports provide a brief overview of rare diseases. For more specific information, we encourage you to contact your personal physician or the agencies listed as “Resources” on this report.

Report last updated: 2008/04/23 00:00:00 GMT+0

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