Asymptomatic adults with isolated, unilateral right pulmonary vein atresia: multidetector CT findings

Abstract

ABSTRACT. We report two cases of a very rare congenital anomaly, i.e. isolated unilateral pulmonary vein atresia. The patients were asymptomatic and the diagnosis was made using multidetector CT (MDCT), which also showed cyst formation in the right lung. Asymptomatic adult cases or association with cystic lung lesions have never been reported in this condition before. Unilateral pulmonary vein atresia without associated congenital heart disease is a rare condition [1]. Patients with congenital unilateral pulmonary vein atresia are usually symptomatic and present with recurrent episodes of pneumonia or haemoptysis in infancy or childhood [1]. Although a subclinical course of this anomaly has been recently reported in a 12-year-old boy, so far no asymptomatic adult cases have been described to our knowledge [1]. Also, the presence of pulmonary cysts has never been reported in this condition. We therefore report the CT findings of two asymptomatic adult patients with unilateral pulmonary vein atresia, with associated pulmonary cysts. Case report Case 1A previously healthy 23-year-old male visited the emergency room because of vomiting lasting 1 day. The chest radiograph obtained at the time showed diffuse interstitial infiltration in the right lung field. Multidetector CT (MDCT) showed a heterogeneous lung density, with areas of normal density, ground-glass opacity and small air-cysts (Figure 1a). The cysts were multiple, but predominantly distributed in the subpleural regions. Beaded thickening of the interlobular septum and nodularity along the fissures and costal pleura were noted in the right lower lung (Figure 1b). The volume of the right lung was not diminished. CT also showed a complete lack of the right pulmonary vein and enlargement of the left. The margin of the left atrium where the right pulmonary vein was expected to be was entirely smooth, with no vascular structures connected to it (Figure 1c). The right pulmonary artery was slightly small, with poor contrast enhancement compared with the left pulmonary artery. The mediastinal branches of the right bronchial artery were markedly dilated, suggesting the presence of systemic arterial flow into the hypoplastic pulmonary arteries (Figure 1d). Anomalous pulmonary venous return was excluded on the basis of the absence of pulmonary venous structures connected to systemic venous circulation on CT.Fibre-optic endoscopy revealed findings compatible with reflux oesophagitis and chronic superficial gastritis. The patient recalled being told that his chest radiography showed a ''small right pulmonary artery''. He did not complain of any respiratory, cardiac or other systemic symptoms, and refused further work-up. The patient was in good health 5 years after the initial emergency room visit. Case 2A 39-year-old female with rheumatoid arthritis recently underwent screening chest radiography in preparation for a clinical trial (Figure 2a). This showed a small right hemithorax with significant ipsilateral mediastinal shift, reduced vessel size in the hilar region and...

Unilateral pulmonary veins atresia: evaluation by computed tomography

“…In the present case, the patient had remained asymptomatic for a long period of time. Similarly, Kim et al have reported two cases of adult patients (23 and 37 years) who were asymptomatic at the diagnosis (4) . The time for development of symptoms or pulmonary arterial hypertension seems to be dependent on the balance between the systemic vessels which supply and those vessels which drain the affected lung (2,4) , in association with the lymphatic system drainage capacity.…”

“…Unilateral pulmonary veins atresia is a rare condition, with less than 40 cases reported in the literature until 2010 (4) . Such a condition is characterized by the absence of pulmonary veins on the affected side and presents high morbimortality (2) .…”

“…Recent publications report four cases where the patients were clinically followed-up, with no complication (4,7,8,11) . Two out of such patients underwent follow-up for up to five years (4,8) . Surgical repair of anomalous venous return may be performed in cases where there is no significant left to right shunt.…”

Cystic Lung Changes in a Thin Section CT in an Asymptomatic Young Adult with Unilateral Pulmonary Vein Atresia: A Case Report

“…Based on the gravity of symptoms, coil embolization and pneumonectomy are the usual forms of treatment (5,6). There have been few radiologic reports about the findings of systemic collaterals to the pulmonary vessels such as interlobular septal thickening, ground-glass opacity and subpleural nodules (1,2,4,7). We present the case of a 20-year-old male, who had been an asymptomatic pulmonary vein atresia and systemic-to-pulmonary collaterals patient.…”

“…Unilateral pulmonary vein atresia is a very rare congenital anomaly usually associated with high morbidity and mortality (1)(2)(3)(4). Most of the patients reported were in infancy or early childhood with recurrent episodes of hemoptysis or pneumonia.…”