Ann Thorac Surg 2001;72:316
© 2001 The Society of Thoracic Surgeons
Correspondence
Classification of pulmonary atresia with ventricular septal defect
Miguel Barbero-Marcial, MDa
a Heart InstituteUniversity of São Paulo Medical School, Av. Dr. Enéas de Carvalho Aguiar, 44, CEP 05403-000 São Paulo, Brazil
e-mail: dcimiguel{at}incor.usp.br
To the Editor
In a recent issue of The Annals, Tchervenkov and Roy [1] proposed a new classification for pulmonary circulationpulmonary 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 classificationtypes A, B, and Cwas published by our group in 1990 (Fig 1) [2]. Our proposed classification has been used by our Institution and by other groups such as Mace and colleagues [3] from the University of Paris. In 1996, they termed it Barbero-Marcials 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.

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Fig 1. Illustration of the anatomy present in our classification. (Reprinted with permission from Barbero-Marcial M, Jatene AD. Surgical management of the anomalies of the pulmonary arteries in the tetralogy of Fallot with pulmonary atresia. Semin Thorac Cardiovasc Surg 1990;2:93107.)
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Fig 2. Barbero-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;89:5618.)
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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.
References
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Tchervenkov C.I., Roy N. Congenital Heart Surgery Nomenclature and Database Project: pulmonary atresiaventricular septal defect. Ann Thorac Surg 2000;69:S97-S105.[Abstract/Free Full Text]
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Barbero-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;2:93-107.[Medline]
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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 Vaiss 1996;89:561-568 (in French).[Medline]
Related Article
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Classification of pulmonary atresia with ventricular septal defect: Reply
- Christo I. Tchervenkov
Ann. Thorac. Surg. 2001 72: 316-317.
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