Unilateral pulmonary veins atresia: evaluation by computed tomography

Abstract

Unilateral pulmonary vein atresia is a rare congenital condition. In addition to cardiac malformations or pulmonary hypertension, patients may present with recurrent pulmonary infections or hemoptysis in childhood or adolescence. The authors report a case where the typical findings of such condition were observed at computed tomography in an adult patient. Keywords: Pulmonary veins; Unilateral pulmonary veins atresia; Computed tomography.A atresia unilateral das veias pulmonares é uma condição congênita rara. Os pacientes podem apresentar infecções recorrentes ou hemoptise na infância ou adolescência, além de malformações cardíacas ou hipertensão pulmonar. Relatamos caso em que os achados característicos dessa alteração foram demonstrados pela tomografia computadorizada em um paciente adulto. Unitermos: Veias pulmonares; Atresia unilateral de veias pulmonares; Tomografia computadorizada. (Figure 1). The patient presented history of dys- Abstract

Left hypoplastic lung and hemoptysis—rare familial unilateral pulmonary vein atresia

“…In addition, collaterals between the pulmonary and bronchial circulation that can misinterpreted as pulmonary arteriovenous malformations (PAVM) may develop 15 . Due to inappropriate gas exchange caused by alterations between ventilation and perfusion, there is a progressive reduction in caliber of the affected PA, which eventually develops flow reversal toward the contralateral artery 13 . In addition, PH and recurrent infection contribute to the development of parenchymal changes such as ground glass opacities, interlobular septal thickening, and bronchial wall thickening.…”

Isolated agenesis of the right pulmonary veins with pulmonary sequestration

“…Nonvisualization of confluence of PVs to LA, presence of systemic collateral, and dilated bronchial veins on CT angiogram are in favor of PV atresia. [3510] Cardiac catheterization conclusively demonstrates atresia of PVs, presence of systemic collaterals, degree of left-to-right shunt, and severity of pulmonary hypertension. [137]…”

Congenital unilateral pulmonary vein atresia: A rare disease with successful outcome in a child

“…CT angiography may show a small ipsilateral pulmonary artery with nonopacification of pulmonary veins and reduced lung volume with areas of ground glass attenuation, consolidation or unilateral septal thickening. 2,4 Systemic-to-pulmonary collaterals may be seen due to associated poor development of pulmonary arteries, leading to preferential flow to the contralateral lung that is a postulated theory for hemoptysis. 4 The management of symptomatic unilateral pulmonary venous atresia is surgical.…”

“…2,4 Systemic-to-pulmonary collaterals may be seen due to associated poor development of pulmonary arteries, leading to preferential flow to the contralateral lung that is a postulated theory for hemoptysis. 4 The management of symptomatic unilateral pulmonary venous atresia is surgical. In patients with focal stenosis, resection and end-to-end anastomosis or pulmonary venous anastomosis with the left atrium can be done 5 ; however, in cases of complete pulmonary venous atresia, lobectomy or pneumonectomy usually is required.…”