Diagnosis and treatment of aortic origin of the right pulmonary artery: analysis of 14 cases

Abstract

To investigate the diagnosis of aortic origin of the right pulmonary artery (AORPA) and evaluate the efficacy of surgical treatment therefore. The clinical data of 14 AORPA patients, 9 male and 5 female, aged 4 (60 days-23 years), hospitalized from May 1992 to March 2007, twelve of which were surgically treated through 5 different procedure, and two of which were denied surgical procedure due to Eisenmenger syndrome. Follow-up was conducted for (5.15+/-3.48) years. The diagnosis of 12 out of the 14 patients was confirmed before operation, and 2 of them were misdiagnosed by echocardiography. Two patients died during the peri-operational period due to low output syndrome or pulmonary hypertension crisis. One patient died from right cardiac failure 4 yrs after the surgical procedure. Of the 9 surviving patients, 5 were in NYHA functional class I, and 4 in class II. In diagnosis of AORPA right ventriculography and aortic angiography or multi-sliced CT angiography or MRI are necessary to avoid misdiagnosis. The early and mid-long term effects of surgical treatment for AORPA are good, but it was imperative to adopt these procedures as early as possible to heighten the cure efficacy.