What are the other Names for this Condition? (Also known as/Synonyms)

• Idiopathic Dilatation of the Pulmonary Artery
• Idiopathic Dilatation of the Pulmonary Trunk 

What is Idiopathic Dilatation of Pulmonary Artery? (Definition/Background Information)

• Idiopathic Dilatation of Pulmonary Artery is a rare, congenital anomaly that develops during the early stages of fetal heart development. In this condition, the pulmonary artery is wider than normal, leading to the development of this condition
• The heart is composed of four chambers; the right atrium, right ventricle, left atrium, and left ventricle
• The deoxygenated blood travels from the right atrium into the right ventricle through a valve, called the tricuspid valve, and from there it enters the right ventricle. Blood, then enters into the lungs through a valve, called the pulmonary valve, into the pulmonary artery. Blood flows through the pulmonary artery and then enters the lung and becomes oxygenated
• In Idiopathic Dilatation of Pulmonary Artery, the pulmonary artery is enlarged in width causing an increase in the amount of blood that enters the lung to get oxygenated, placing a lot of pressure on the lungs. The exact cause of the condition is not known
• The common signs and symptoms of Idiopathic Dilatation of Pulmonary Artery include abnormal heart sounds and breathing difficulties. It may be diagnosed by a complete evaluation of medical history, a thorough physical exam, and imaging studies such as chest X-ray, doppler ultrasound of heart, and MRI scan
• In a majority of individuals, Idiopathic Dilatation of Pulmonary Artery is a benign condition and no treatment may be provided. However, in some individuals this condition can lead to heart failure and lung failure
• The associated prognosis is very good, if the dilatation is not large and causes no symptoms. However, in some individuals, where the dilatation is large and causes significant symptoms, the prognosis can be poor without appropriate timely treatment

Who gets Idiopathic Dilatation of Pulmonary Artery? (Age and Sex Distribution)

• Idiopathic Dilatation of Pulmonary Artery is commonly seen among newly born infants. The condition may or may not progress into childhood or adulthood
• It is distributed among both the male and female population
• There is no distinct racial or ethnic predilection observed

What are the Risk Factors for Idiopathic Dilatation of Pulmonary Artery? (Predisposing Factors)

The risk factors for developing Idiopathic Dilatation of Pulmonary Artery include:

• Improper development of the heart during fetal development
• Other heart-related conditions
• Genetic defects (the presence of certain inherited disorders)

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases ones chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider. 

What are the Causes of Idiopathic Dilatation of Pulmonary Artery? (Etiology)

The exact cause of Idiopathic Dilatation of Pulmonary Artery is not known.

• It is believed that there may be a defect with the heart development, during the embryological stages
• Some reports demonstrate that the condition can result from an underlying condition such as a connective tissue disorder

What are the Signs and Symptoms of Idiopathic Dilatation of Pulmonary Artery?

The signs and symptoms associated with Idiopathic Dilatation of Pulmonary Artery include:

• Abnormal heart sounds
• Difficulty with breathing

In certain cases, the defect may be completely asymptomatic and go unnoticed. It may be only incidentally discovered, while tests are being performed for some other health conditions.

How is Idiopathic Dilatation of Pulmonary Artery Diagnosed?

Idiopathic Dilatation of Pulmonary Artery may be diagnosed using the following measures:

• A complete evaluation of medical history and a thorough physical exam
• During the physical examination, abnormal heart sounds may be detected with the use of a stethoscope
• Imaging studies such as chest X-ray and MRI scan of the chest: A magnetic resonance imaging (MRI) scan is an important test for diagnosing this condition. It is a non-invasive procedure that has many advantages, as it provides accurate and clear images of the structures. This imaging tool may be preferred by the physician over an echocardiography for diagnosing the condition
• Doppler echocardiogram of the heart 

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis. 

What are the possible Complications of Idiopathic Dilatation of Pulmonary Artery?

In a majority of individuals, Idiopathic Dilatation of Pulmonary Artery does not cause any significant complications; however, in individuals where the dilatation is large and significant, the complications may include:

• Pulmonary hypertension
• Acute and chronic lung failure
• Acute and chronic heart failure

How is Idiopathic Dilatation of Pulmonary Artery Treated?

Typically, there is no treatment necessary for Idiopathic Dilatation of Pulmonary Artery, if the dilatation is small and causes no symptoms.

• In those whom the defect is large, it may have to be surgically repaired
• The age at which the corrective surgery is required depends upon one’s specific health condition and individual circumstances. A cardiac surgeon can help advise on the timing of the surgery

How can Idiopathic Dilatation of Pulmonary Artery be Prevented?

Currently, there are no preventive measures available for Idiopathic Dilatation of Pulmonary Artery. 

What is the Prognosis of Idiopathic Dilatation of Pulmonary Artery? (Outcomes/Resolutions)

• The associated prognosis of Idiopathic Dilatation of Pulmonary Artery is excellent, if the dilatation is small and causes no symptoms
• However, in some individuals, where the dilatation is large and causes significant symptoms, the prognosis can be poor without appropriate timely treatment

Additional and Relevant Useful Information for Idiopathic Dilatation of Pulmonary Artery:

The following DoveMed website link is a useful resource for additional information:

http://www.dovemed.com/diseases-conditions/rare-disorders/