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.
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.
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.
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.
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.
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
For information about clinical trials sponsored by private sources, contact:
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.
The information in NORD’s Rare Disease Database is for educational purposes only and is not intended to replace the advice of a physician or other qualified medical professional.
The content of the website and databases of the National Organization for Rare Disorders (NORD) is copyrighted and may not be reproduced, copied, downloaded or disseminated, in any way, for any commercial or public purpose, without prior written authorization and approval from NORD. Individuals may print one hard copy of an individual disease for personal use, provided that content is unmodified and includes NORD’s copyright.