A Case of Isolated Peripheral Pulmonary Artery Branch Stenosis Associated with Multiple Pulmonary Artery Aneurysms

Abstract

Selective right pulmonary arteriography and 3-dimensional computed tomography revealed multiple severe stenoses of the peripheral pulmonary artery associated with poststenotic aneurysms in a 65-year-old woman. She was referred to the hospital for evaluation of dry cough, gradually increasing dyspnea and multiple nodular shadows on a chest radiograph. Echocardiography and cardiac catheterization showed severe pulmonary hypertension, though other structural heart diseases or well-characterized congenital syndromes were ruled out. She was diagnosed as isolated peripheral pulmonary artery branch stenosis. Recent advances in CT technology enable a less-invasive assessment of pulmonary artery, and can be useful in the management of pulmonary arterial hypertension.

Peripheral Pulmonary Artery Stenosis: An Unusual Case and Discussion of Genetic Associations

“…Most of the main or proximal segmental pulmonary artery branches are derived from the ventral portions of the sixth branchial arches. The peripheral type of pulmonary branch stenosis may be related to some sort of developmental error in these lesions …”

“…Although pathogenesis is unknown, it is thought to be congenital in most cases because of its association with other embryological malformations. Congenital peripheral stenosis of the pulmonary artery is usually detected in early childhood and is often associated with other structural heart disease . In congenital rubella, peripheral pulmonary artery stenosis commonly is associated with atrial septal defect (ASD) and patent ductus arteriosus (PDA) .…”

“…The peripheral type of pulmonary branch stenosis may be related to some sort of developmental error in these lesions. 4 Currently, the complete etiology of pulmonary artery branch stenosis is undefined. However, it appears that numerous factors and multiple types of pathological changes may manufacture narrowing of the branch pulmonary artery.…”

“…Congenital peripheral stenosis of the pulmonary artery is usually detected in early childhood and is often associated with other structural heart disease. 4 In congenital rubella, peripheral pulmonary artery stenosis commonly is associated with atrial septal defect (ASD) and patent ductus arteriosus (PDA). 5 Peripheral pulmonary artery stenosis is also associated with other congenital heart defects such as tetralogy of Fallot, pulmonary atresia, truncus arteriosus, pulmonary valve stenosis, and PDA as well as syndromes such as Alagille syndrome, Williams syndrome, cutis laxa, Ehlers-Danlos syndrome, Noonan syndrome, and Silver-Russell syndrome.…”

Peripheral pulmonary artery stenosis masquerading as pulmonary hypertension: A diagnostic and therapeutic challenge

“…Branch PA stenosis can occur in adults with systemic inflammatory disease, such as Behçet's disease, in association with PA aneurysmal disease, 3 or as a complication of thromboembolism, but can also occur in isolation. 2 The slow, non-uniform progression to segmental vascular obstruction due to isolated PA stenosis is typically associated with progressive dyspnea and fatigue in the second decade of life. 3 Lung perfusion imaging may reveal segmental defects but diagnosis typically requires direct arterial imaging by contrast-enhanced CT, magnetic resonance angiography or catheter-based arteriography.…”