Insights in Biomedicine Open Access

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 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 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. Idiopathic dilatation of the pulmonary artery (IDPA) which is a rare condition in which the pulmonary artery dilates without an obvious cause.

Pulmonary artery replacement is indicated in severe cases to prevent serious complications. 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. Because the disorder is benign in most instances, neither clinicians nor epidemiologists are able to measure the distribution of the disease with confidence. Idiopathic dilatation of pulmonary arteries (IDPA) is a rare abnormality of pulmonary arteries. With the improvement in diagnostic modalities, antemortem diagnosis of IDPA has been increasingly established by excluding diseases which induce pulmonary arterial enlargement.

Idiopathic dilatation of the pulmonary artery admitted with shortness of breath where IDPA was diagnosed as an incidental finding using computed tomography pulmonary angiography and cardiac catheterization. Idiopathic dilatation of the pulmonary artery (IDPA) is a rare condition characterized by enlargement of the pulmonary artery in the absence of an obvious trigger with an incidence of 1 per 14,000 autopsies. The diagnostic criteria for IDPA are diastolic change of the pulmonary artery mainstem; no lesions of the arteries that could cause histological changes, such as syphilis and arteriosclerosis; normal right ventricular pressure.

Generally, a patient who meets all the above-mentioned criteria is diagnosed with IDPA. Dilatation of the pulmonary artery may be secondary to an underlying disorder, such as congenital heart, vasculitis, Marfan syndrome, chronic heart failure, chronic respiratory disease, and trauma. In idiopathic dilatation the systolic pressure in the right ventricle and the pulmonary artery are equal. The pressure in the right ventricle is not increased and the oxygen saturation in the various parts of the right heart, the pulmonary artery and the superior vena cava does not show significant differences. If the pressure in the right ventricle exceeds that in the pulmonary artery even though it is in the normal range it is still possible that slight pulmonary stenosis is present.