Congenital Absence of One of the Pulmonary ArteryBranches

Absence of one of the pulmonary artery branches is an uncommon congenital defect. Most of these patients have other associated cardiac deficiencies. Early diagnosis and surgical intervention may prevent irreversible pulmonary hypertension and collateral circulation formation. A cross-sectional descriptive study was done from 2001 to 2006 on the records of 18 patients with absence of one of the pulmonary artery branches that referred to the EmamReza Hospital, Mashhad University of Medical Sciences (a tertiary referral hospital). Patients’ data including history, physical examination, chest X-Ray, cardiac catheterization and angiography reviewed and analyzed statistically. Among 18 patients, 13 (72.2%) were males; the mean age of those on diagnosis was 11.8±10.8 years (Range 1-38 years). Except one, all of them had associated congenital heart defects. Most of these abnormalities were complex form of CHD, and Tetralogy of Fallo was the highest abnormality (7 cases). Seventeen patients (77.8%) had occult left pulmonary artery (LPA). CXR findings in all patients included heart and mediastinum deviation toward the absent pulmonary artery. Early diagnosis and surgical intervention may allow normal growth of the pulmonary artery and prevent collateral formation and irreversible pulmonary hypertension. Therefore, complete evaluation for the absence of pulmonary artery branch in suspicious infants, especially those with CHD, is mandatory.