Isolated unilateral absence of pulmonary artery (IUAPA) in adults: A case series and review of literature

Abstract

Unilateral Absence of Pulmonary Artery (UAPA) is a rare congenital malformation that is usually associated with cardiac anomalies. When there is no congenital cardiac abnormality it is rarer still and is termed isolated unilateral absence of pulmonary artery (IUAPA) (4). IUAPA may remain undetected until adulthood and frequently found incidentally on imaging for other indications. Symptoms are usually secondary to complications which include pulmonary hypertension, recurrent respiratory tract infections, bronchiectasis and haemoptysis. We report two cases with widely contrasting presentation and trajectories, leading to individualized management strategies.

Embolization treatment of right pulmonary artery agenisis with patent ductus arteriosus causing pulmonary hypertension and hemoptysis: a case report and literature review

“…Selective systemic circulation arterial embolization proves effective in controlling coughing fits along with managing both pulmonary hypertension and infection [ 15 ], offering good hemostatic effects while minimizing trauma; this procedure can be repeated if necessary. It is crucial to ensure complete embolization of responsible vessels in order to avoid missed embolizations that could lead to higher recurrence rates and mortality [ 20 , 21 ].Following selective embolization of the systemic arteries, there was a reduction in blood flow in the right pulmonary circulation and a decrease in pulmonary arterial pressure, with no significant impact on oxygen exchange or lung lobe development (16 years old). For abnormal open systemic circulation arteries (arterial fistula), larger diameter permanent solid embolic agents are preferred due to their irregular surfaces that are more prone to clotting and can prevent occlusion of aberrant small vessels.…”