Abstract

GAMA, María Eugenia. Hypogenetic lung or Halasz syndrome, or «scimitar sign»: one of the forms of congenital veno-lobar lung syndrome. Rev. Col. Cardiol. [online]. 2005, vol.12, n.1, pp.25-36. ISSN 0120-5633.

Hypogenetic lung syndrome or Halasz syndrome is one of the variants of the congenital veno -lobar lung syndrome. It is a complex congenital malformation of the right lung and its vasculature that may be associated to other cardiovascular malformations. The symptoms may appear from the first days of life or till an adult age. Clinical diagnosis is made by cardiorespiratory symptoms related to a dextrocardia secondary to a small right lung, or just by the visualization of the chest X-Ray scimitar image that leads to the study though cardiac catheterization in order to confirm the diagnosis and to value the anatomical and functional characteristics. Its seriousness is directly related to the age of appearance, the left to right shunt, the degree of pulmonary arterial hypertension, the presence of right lung hypoplasia, the arterial sequestration, the course of the anomalous vein and the presence of associated cardiovascular malformations. These facts are determinant for the treatment and the prognosis. In the forms without complex cardiovascular malformations, the interruption of the arterial sequestration cures or improves in a significant way, and the associated cardiopathy is corrected in a second intervention. Some authors correct the anomalous vein at the same time as the interruption of the arterial sequestration. In the forms with associated complex malformations, the various described surgical techniques for the anomalous vein return show difficulties. Pre-operative appraisal must determine the anomalous vein trajectory and the importance of the left to right shunt, one by one, and in this way the treatment may be decided. Follow-up of pulmonary hypertension is fundamental for the possibility of deciding the conduct before the condition may become permanent.

Keywords : right lung vascular malformation; right lung hypoplasia; pulmonary arterial hypertension.

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