Congenital anomalies of the pulmonary arterial system in a paediatric cohort: a single- center 28-year experience

Abstract

Background and objective Congenital anomalies of the pulmonary arterial system are rare. They include left pulmonary artery sling (LPAS), unilateral absence of the pulmonary artery (UAPA) and anomalous origin of unilateral pulmonary artery from the ascending aorta (AOPA) or hemitruncus. Our aim was to review 21 paediatric cases and describe their presentation, , diagnosis, management and long-term follow-up. Material and methods A retrospective observational study of all paediatric patients with pulmonary arterial system abnormalities diagnosis (May 1995-March 2023) was conducted. Patients underwent systematic evaluation (medical and family history; echocardiogram; angiography and/or computed tomography or magnetic resonance angiography). Results 21 paediatric patients with pulmonary arterial system abnormalities (LPAS n=11, UAPA n=6 and AOPA n=4) were included. The median age at diagnosis was 2.0 [0.1-12.0] months, the median follow-up time 2.4 [0.4-11.7] for all patients and 8.0 [0.7-12.5] years for survivors. 2 patients (9.5%) were prenatally diagnosed. Echocardiography diagnosed 18 patients (85.7%). The diagnosis was confirmed by MR (n=1), CT (n=17) or angiography (n=3). There were 5 deaths (23.8%) (median age 1.4 [0.6-5.9] months). Conclusion Prompt diagnosis and treatment of patients with pulmonary arterial system abnormalities is paramount to diminish post-operative residual lesions and morbidity, as demonstrated in our study. Nevertheless, further studies including larger cohorts are required not only to better define consensus regarding surgical management of congenital abnormalities of the pulmonary arterial system but also to improve their aetiology understanding and associated anomalies. These will enhance the clinical delineation of these entities leading to an increase of clinicians’ awareness.