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Pulmonary Vascular Disease |

Unilateral Pulmonary Vein Atresia Presenting in Adulthood

Paul Brasher, MD; John Fantauzzi, MD; Amit Chopra, MD
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Albany Medical Center, Albany, NY


Chest. 2015;148(4_MeetingAbstracts):996A. doi:10.1378/chest.2232000
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Abstract

SESSION TITLE: Pulmonary Vascular Diseases Student/Resident Cases

SESSION TYPE: Student/Resident Case Report Slide

PRESENTED ON: Tuesday, October 27, 2015 at 04:30 PM - 05:30 PM

INTRODUCTION: Unilateral pulmonary vein atresia is complete obliteration of the luminal continuity of pulmonary venous circulation. Patients often present with hemoptysis and recurrent infections during the first three years of life. A literature search revealed only 8 cases of isolated unilateral pulmonary vein atresia diagnosed in adulthood making diagnosis and decisions regarding treatment challenging.1

CASE PRESENTATION: A 23-year-old Caucasian male presented with acute onset frank hemoptysis. Vital signs were within normal limits with an oxygen saturation of 96% on ambient air. Examination of chest revealed decreased excursion of left hemithorax with poor air entry. Chest radiograph showed a hypoplastic left lung with reticular opacities. A contrast enhanced CT scan of the chest showed a hypoplastic left lung with interlobular septal thickening and a small left pulmonary artery. A 3D reconstruction (Fig 1) of the CT angiogram showed absence of the left sided pulmonary venous circulation. Bronchoscopy (Fig 2) showed dilated sub-mucosal bronchial varices. Given the risk of future life threatening hemoptysis, left pneumonectomy was performed. He was asymptomatic at his one year follow up.

DISCUSSION: Pulmonary vein atresia results from failure of incorporation of the common pulmonary vein into the left atrium. The manifestation of pulmonary vein atresia depends on the stage in which the normal development is affected.2 Rupture of dilated bronchial veins from pulmonary venous obstruction is the main cause of hemoptysis. On radiography, patients have markedly less lung volume on the atretic side. Reticular opacities in the smaller lung correspond with thickened interlobular septae representing chronic changes of pulmonary venous hypertension and impaired lymphatic drainage. CT angiography findings of a small ipsilateral pulmonary artery with the absence of venous opacification on venous phase imaging are virtually diagnostic. Once pulmonary vein obstruction is confirmed, it should be differentiated from acquired pulmonary obstruction such as mediastinal tumors, mediastinitis and constrictive pericarditis. Indication for pneumonectomy is based on; history or risk of life threatening hemoptysis, recurrent infections and development of pulmonary hypertension.

CONCLUSIONS: Unilateral pulmonary vein atresia is a fatal condition in early childhood and rarely diagnosed in adults. The diagnosis and treatment of pulmonary vein atresia is clinically challenging. Pneumonectomy is the ultimate surgical treatment because it will remove the nidus for recurrent infections and hemoptysis as well as relieve significant left-to-right shunt and dead space.

Reference #1: Heyneman LE, Nolan RL, Harrison JK, et al. Congenital unilateral pulmonary vein atresia: radiologic findings in three adult patients. AJR Am J Roentgenol. 2001;177:681-5.

Reference #2: Pourmoghadam KK, Moore JW, Khan M, et al. Congenital unilateral pulmonary venous atresia: definitive diagnosis and treatment. Pediatr Cardiol. 2003;4:73-79.

DISCLOSURE: The following authors have nothing to disclose: Paul Brasher, John Fantauzzi, Amit Chopra

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