Isolated Unilateral Absence of Pulmonary Artery (UAPA) in the Adult:Between Simple Cough and Intensive Care

Unilateral absence of pulmonary artery (UAPA) is a rare congenital condition occurring isolated or in conjunction with other congenital heart defects (CHD). While none-isolated UAPA is mostly detected early in life, detection of isolated UAPA is often delayed due to diverse and unspecific symptoms. While there is consensus on the therapeutic strategy in young symptomatic patients, the incidental finding of UAPA in young or adult asymptomatic patients is a matter of on-going debate and limited evidence. Here, we will present two previously unpublished, illustrative cases with different extent of symptom severity and discuss the difficulties in determining an appropriate therapeutic strategy. Along with the cases, we will review the literature, present typical symptoms and possible pitfalls, and provide an overview of therapeutic options with a focus on the adult patient.