Idiopathic dilatation of pulmonary artery (IDPA) is a rare congenital structural defect of pulmonary artery. It is characterized by abnormal dilatation of pulmonary artery without any identifiable cause. This anomaly develops during the early developmental stage of fetal heart. Due to widening of the artery, more deoxygenated blood rushes into the lungs. This leads to excess of pressure into the lungs giving rise to breathlessness, abnormal heart sounds etc.
The exact reason of IDPA is not known, but various causes such as connective tissue disease or defect in elasticity of artery have been postulated.In majority of cases the condition is benign and most people do not require any treatment provided there is no lesions in heart valve or other structures of heart. If the widening is too large it may require treatment.
Causes Of Pulmonary Artery Dilatation
The exact reason for idiopathic dilatation of pulmonary artery is not known. The defect develops during the developmental stage of the heart in fetus. There are several theories postulated. According to one theory the defect occurs in the elasticity of the pulmonary arterial tissues during the developmental stage of the heart and lung.
The dilatation can also have an association with disorder of connective tissue as IDPA is often found in people suffering from certain genetic disorder such as Marfan’s syndrome, ED syndrome. Idiopathic dilatation of pulmonary artery is present since birth and the widening may progress or remain limited as it is during childhood or when the person becomes adult.
Symptoms Of Pulmonary Artery Dilation
Idiopathic dilatation of pulmonary artery is a benign condition and in majority of cases it does not present any symptoms. This is because it does not produce any obvious abnormality in the circulation of blood. If at all clinical signs and symptoms are minimal and they consist of difficulty in breathing and abnormal heart sound.
Most often the pulmonary ejection click is palpable which disappears as the patient breathes in air into the lungs. A soft systolic murmur can be heard on auscultation with a stethoscope. A splitting second heart sound can also be heard on auscultation. The electrocardiogram is normal, but X-ray of chest will reveal dilated pulmonary artery.
Complications: There is no complication in most people having idiopathic dilatation of pulmonary artery. However, if the widening of artery is extremely large enough, it may cause pulmonary hypertension which may lead to acute heart and lung failure.
Diagnosis And Treatment For Dilated Pulmonary Artery
Following steps will help in diagnosis of IDPA.
- Complete medical history and clinical examination of the patient.
- While examining the patient, the physician listens to the heart sounds for any murmur with the help of stethoscope. He can hear splitting second heart sound which may be an indication of this condition.
- He will also recommend X-ray and MRI scan of chest which will help in providing accurate diagnosis.
- Another important imaging technique is 2 D echocardiography.
Treatment: Usually idiopathic dilatation of pulmonary artery does not require any treatment as it is a benign condition. If the dilatation is small, patient just needs regular monitoring. However, if dilatation is extremely large and causes discomfort while breathing, or any other physical problem, than patient may require surgical correction of the anomaly.