Aneurysm of the pulmonary artery

Aneurysm of the pulmonary artery

Characteristics

  1. Permanent dilatation of the main and/or segmental pulmonary arterial branches which can be congenital or acquired.
  2. Congenital – rare condition seen in adolescent females with dilatation of the main pulmonary artery. Patients are usually asymptomatic with only a soft ejection systolicmurmur detectable.No significant complications. The diagnosis is made after excluding other acquired conditions.
  3. Acquired
  • Infections (e.g. TB, known as Rasmussen aneurysm, where there is mycotic inflammatory necrosis causing dilatation of the arterial wall).
  • Collagen vascular disorders (e.g. Marfan’s syndrome).
  • Inflammatory (e.g. Takayasu’s arteritis).
  • Behc¸et’s disease. Segmental artery dilatation is a feature of Hughes–Stovin syndrome.
  • Post trauma – blunt (RTA), or direct (following arterial instrumentation).
  • Post stenotic dilatation in pulmonary valvular stenosis.
  • Thromboembolic disease.
  • Associated with primary bronchial neoplasm.

Clinical features

  1. Clinical presentation depends on the underlying cause and location of the aneurysm.
  2. Congenital lesions are uncommon and asymptomatic.
  3. Acquired lesion – most commonly present with haemoptysis. Rupture may complicate the clinical picture and is frequently fatal.
  4. In many cases diagnosis is incidental following investigations for the underlying primary disease.

Radiological features

  1. CXR – isolated main pulmonary artery dilatation in the aortopulmonary window with normal lungs is a rare radiological finding. More commonly the aneurysm lies within an ill-defined area of consolidation and is difficult to fully appreciate.
  2. Contrast-enhanced CT scanning allows clearer appreciation of the size, location and extent of the arterial aneurysm(s).
  3. CT also allows for further assessment of the underlying cause. An increasing focal lung mass raises the possibility of aneurysm. Lack of an arterial wall and a history of trauma support the diagnosis of a false aneurysm of the pulmonary artery.

Differential diagnosis

  1. Themain differential lieswithin the varying underlying causes listed above.

Management

  1. Management is dependent on identifying the abnormalities, correlating with the clinical history, and excluding the various different conditions.
  2. Sputum culture, echocardiography, cardiac catheterisation and arterial wall biopsy may all play a role in the diagnostic pathway.
  3. Treatment options are targeted first at the correct management of the cause.
  4. Surgical repair of the aneurysm and the use of endovascular stent grafts are rarely used strategies, but potentially offer definitive treatment.

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