Classification of pulmonary atresia with ventricular septal defect

Abstract

In a recent issue of The Annals, Tchervenkov and Roy [1Tchervenkov C.I. Roy N. Congenital Heart Surgery Nomenclature and Database Project pulmonary atresia–ventricular septal defect.Ann Thorac Surg. 2000; 69: S97-S105Abstract Full Text Full Text PDF PubMed Google Scholar] proposed a new classification for pulmonary circulation—pulmonary atresia with ventricular septal defect. This classification is based on the presence or absence of native pulmonary arteries (PAs) and the presence or absence of main pulmonary collateral arteries. Coincidentally, a similar classification—types A, B, and C—was published by our group in 1990 (Fig 1)[2Barbero-Marcial M. Jatene A.D. Surgical management of the anomalies of the pulmonary arteries in the tetralogy of Fallot with pulmonary atresia.Semin Thorac Cardiovasc Surg. 1990; 2: 93-107PubMed Google Scholar]. Our proposed classification has been used by our Institution and by other groups such as Mace and colleagues [3Mace L. Dervanian P. Losay J. et al.Pulmonary arborization abnormalities in complex forms of pulmonary atresia with ventricular septal defect unification, unifocalization and complete repair.Arch Mal Coeur Vaiss. 1996; 89 (in French): 561-568PubMed Google Scholar] from the University of Paris. In 1996, they termed it Barbero-Marcial’s classification(Fig 2), and divided patients with pulmonary atresia with ventricular septal defect into three types according to the anatomy of the native PAs and collaterals: Type A: All the bronchopulmonary segments are connected to the central PAs, and the initial surgical objective consists of ensuring the presence of PAs adequate in size, distribution, and peripheral resistance to ultimately permit the possibility of total correction. We distinguish two subgroups: A1, with normal or underdeveloped (hypoplastic) PAs, and A2, with stenotic or nonconfluent PAs.Type B: Some bronchopulmonary segments are supplied by branches of the central PAs whereas other segments are supplied by major aortopulmonary collateral arteries. Because both central PAs and major collaterals are present, the surgical objective consists of unifying the pulmonary circulation by anastomosing the aortopulmonary collateral arteries to the PAs, and encouraging enlargement and normal development of pulmonary blood flow, techniques that will, hopefully, permit later complete repair.Type C: All the bronchopulmonary segments are supplied exclusively by major aortopulmonary collateral arteries, and central PAs are absent. The surgical objective consists of unifying the pulmonary circulation by anastomosing the aortopulmonary collateral arteries and the lobar arteries at the hilum of the lung, and constructing an “intermediate PA segment” designed to be accessible through a midline sternotomy at the time of future total correction and supplied by a surgically constructed systemic-PA shunt.Fig 2Barbero-Marcial classification. Type A: no defect in pulmonary arborization. Type B: part of the pulmonary arterial segments are dependent on the central pulmonary arteries, the other segments are supplied by collaterals. Type C: the pulmonary arterial arborization depends on the collaterals; there are no central pulmonary arteries. (Reprinted with permission from Mace L, Dervanian P, Losay J, et al. Pulmonary arborization abnormalities in complex forms of pulmonary atresia with ventricular septal defect: unification, unifocalization and complete repair. Arch Mal Coeur 1996;89:561–8.)View Large Image Figure ViewerDownload (PPT) In our opinion, independent of the two terms of nomenclature used to name this complex anomaly, our classification according to the anomalies of PAs and collaterals could be used without restrictions. Classification of pulmonary atresia with ventricular septal defect: ReplyThe Annals of Thoracic SurgeryVol. 72Issue 1Preview Full-Text PDF