The nomenclature of both intralobar pulmonary sequestration (ILS) and aortic origin of a pulmonary artery (AOPA) remains controversial. According to this review, both ILS and AOPA have an anomalous systemic arterial supply to all or part of the lung with venous drainage into the pulmonary veins, which leads to pulmonary hypertension, congestive heart failure, and fatal pulmonary haemorrhage. The purpose of this review was to consider whether these two rare congenital anomalies have similar anatomical, clinical and pathological characteristics.

This review was conducted by researching relevant literature using PubMed and MEDLINE databases to January 2019. All researched literature was related to the anatomical, associated anomalies, pathophysiology and clinical features of the extralobar pulmonary sequestration (ELS), ILS, and AOPA, and the therapeutic method for ILS and AOPA.

Through research literature, it was found that ILS and AOPA may differ in terms of embryonic origin, but some of the anatomical, histopathological, physiological and clinical features of these two congenital malformations are similar. However, ELS and ILS have significant differences in their anatomical, histopathological, physiological, and clinical features.

This study proposes that ILS and AOPA could be classified as one single condition – systemic arterialisation of the lung – and further divided into three subtypes, namely: types I, II and III. This new classification nomenclature permits the appropriate change of novel surgical techniques, which obviate the need for lobectomy or segmentectomy in specific cases, thereby minimising fatal postoperative complications.