Idiopathic dilatation of the pulmonary artery is a rather rare congenital disorder which is typified by a broader than normal pulmonary artery. Furthermore, there is no obvious anatomical or physiological etiological factor present.
The precise cause of the condition is not yet known. A fault in the normal development of elastic tissue of the pulmonary artery before or after birth has been a probable theory that has been hypothesized. The dilatation could also be due to a generalized disorder of the connective tissue, as found in Ehlers-Danlos syndrome or Marfan’s syndrome.
Symptoms Of Idiopathic Pulmonary Artery Dilatation
By and large, idiopathic dilatation of the pulmonary artery does not have any symptoms, given that there is no circulatory anomaly.
Clinical signs are negligible as well, and comprises of a palpable pulmonary ejection sound which vanishes when he inhales, a splitting of the second sound when he inhales and a soft pulmonary ejection systolic murmur. The ECG is normal; diagnosis is established when the chest X-ray reveals a dilated pulmonary artery.
Diagnosis & Treatment For Pulmonary Artery Dilatation
Diagnosing idiopathic dilatation of pulmonary artery is always tricky, since clinical detection is tough and other etiological factors of dilated pulmonary artery must be ruled out too.
Idiopathic dilatation of the pulmonary artery is a recognized clinical entity; however the cause and patho-physiology of such a case are ambiguous. There is scarcity of data regarding the history of the disorder along with a deficiency of clearness about the treatment regimen. Some cases are known to advance to big dilatation and subsequent severe ramifications.
- There is no explicit course of action for the management of idiopathic dilatation of the pulmonary artery.
- Some doctors recommend surgical repair if an isolated aneurysm of the pulmonary artery is too big, usually more than 6 cm, or the condition is producing symptoms, or the chances of a rupture or dissection are high.
- The criterion for surgical intervention is based upon the restricted information on natural history.
- A host of techniques for the repair such as – aneurysmorrhaphy, arterioplasty, pericardial patch reconstruction, and interposition grafting with allografts may be employed, based upon the individual circumstance.
- In most cases, no treatment is required, given that, the condition is rather benign. People having idiopathic dilatation of the pulmonary artery have a normal life expectancy, provided there are no heart lesions.
- Prognosis for the condition is fairly good, since, the disorder is a benign abnormality. Most of the reported cases are known to have survived into the 6th or 7th decade of life. The increase in dilation of the artery can be progressive. Thus, regular, periodical follow ups with your health care provider as well as carrying out scans are very vital in order to evaluate any regurgitation or the formation of an aneurysm.