Pulmonary artery aneurysmal dilatation in adult patients with congenital heart disease.

BACKGROUND

Aneurysmal dilatation of the main pulmonary artery and its peripheral branches are rare lesions that account for less than 1% of all thoracic aneurysms and have a number of possible pathogenetic causes such as congenital heart disease (CHD), pulmonary artery hypertension, vasculitis, mycotic aneurysm, neoplasm, iatrogenic causes, trauma-related events, or connective tissue abnormalities.

METHODS

We conducted a retrospective review of the demographic data and the results of clinical examinations, laboratory tests, echocardiography, and angiography of patients managed at the Adult Congenital Heart Disease Unit of the Complejo Hospitalario Universitario Insular-Materno Infantil, between January 2004 and May 2010.

RESULTS

A total of 352 adult patients with CHD were studied. Of these, 8 (2.3%) patients had pulmonary artery aneurysmal dilatation (PAAD): 4 with low pressure of the pulmonary artery and 4 with pulmonary hypertension. Only 1 patient showed PAAD-related symptoms. Patients with CHD having PAAD had significantly higher prevalence of pulmonary artery hypertension and higher levels of C-reactive protein (CRP) than those without PAAD (incidence: 4 patients [50%] vs 18 patients [5.2%], P < .000; CRP in mg/dL: 0.52 [0.4; 1.2] vs 0.2 [0.0; 1.6], P = .016). No significant differences were found in cholesterol levels (total cholesterol, low-density lipoprotein [LDL], high-density lipoprotein [HDL], or triglycerides) between CHD patients with or without PAAD.

CONCLUSIONS

In patients with CHD, PAAD is a rare finding. The PAAD size, etiology, symptoms, and association with pulmonary hypertension should guide decisions on whether conservative or surgical treatment should be applied.