A systematic classification of the congenital bronchopulmonary vascular malformations: dysmorphogeneses of the primitive foregut system and the primitive aortic arch system

Abstract

Purpose: We reviewed the cases of 33 patients from our clinic and 142 patients from the literature with congenital bronchopulmonary vascular malformations (BPVM), systematically analyzed the bronchopulmonary airways, pulmonary arterial supplies, and pulmonary venous drainages, and classified these patients by pulmonary malinosculation (PM).

Materials and methods: From January 1990 to January 2007, a total of 33 patients (17 men or boys and 16 women or girls), aged 1 day to 24 years (median, 2.5 months), with congenital BPVM were included in this study. Profiles of clinical manifestations, chest radiographs, echocardiographs, esophagographs, computer tomography (CT), magnetic resonance imaging (MRI), magnetic resonance angiography (MRA), cardiac catheterizations with angiography, contrast bronchographs, bronchoscopies, chromosomal studies, surgeries, and autopsies of these patients were analyzed to confirm the diagnosis of congenital BPVM. A total of 142 cases from the literature were also reviewed and classified similarly.

Results: The malformations of our 33 patients can be classified as type A isolated bronchial PM in 13 patients, type B isolated arterial PM in three, type C isolated venous PM in two, type D mixed bronchoarterial PM in five, type F mixed arteriovenous PM in one, and type G mixed bronchoarteriovenous PM in nine.

Conclusion: Dysmorphogeneses of the primitive foregut system and the primitive aortic arch system may lead to haphazard malinosculations of the airways, arteries, and veins of the lung. A systematic classification of patients with congenital BPVM is clinically feasible by assessing the three basic bronchovascular systems of the lung independently.

Fig. 1

Pulmonary malinosculation (PM) involving the pulmonary airways, the pulmonary artery, and the pulmonary vein independently. Each circle represents the occurrence of PM involving the pulmonary airways, the pulmonary artery, and the pulmonary vein within the lung parenchyma, respectively. Each single circle converges to intersect (denoted by 3 sets of reverse arrows) another two circles to form the geometric figure of a trefoil (as a mathematical Venn diagram), within which are seven exclusive areas (areas A-G) denoting seven distinct PM. Area A represents type A isolated bronchial PM (with normal pulmonary artery and veins). Area B represents type B isolated arterial PM (with normal pulmonary airways and veins). Area C represents type C isolated venous PM (with normal pulmonary airways and artery). Area D represents type D mixed bronchoarterial PM (with normal pulmonary veins). Area E represents type E mixed bronchovenous PM (with normal pulmonary artery). Area F represents type F mixed arteriovenous PM (with normal pulmonary airways). Area G represents type G mixed bronchoarteriovenous PM.