Isolated pulmonary vein atresia.

Cullen, Seamus; Deasy, P. F.; Tempany, E.; Duff, Desmond F.

doi:10.1136/hrt.63.6.350

Cited by 39 publications

(28 citation statements)

References 5 publications

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“…Most cases present in infancy or childhood with recurrent respiratory infections or hemoptysis [1,2,4,5]. We present an infant who died from unilateral right-sided pulmonary vein atresia in conjunction with severe contralateral pulmonary vein stenosis.…”

Section: Introductionmentioning

confidence: 94%

Pulmonary Vein Atresia with Severe Contralateral Pulmonary Vein Stenosis in a Child

2012

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Unilateral pulmonary vein atresia is a very rare congenital anomaly associated with high morbidity and mortality. Most cases present in infancy or childhood with recurrent respiratory infections or hemoptysis. Further, the diagnosis can often be difficult to make. We present an infant who died due to unilateral right-sided pulmonary vein atresia in conjunction with severe contralateral pulmonary vein stenosis who was diagnosed with the assistance of cardiac computed tomography scanning.

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Section: Introductionmentioning

confidence: 94%

Pulmonary Vein Atresia with Severe Contralateral Pulmonary Vein Stenosis in a Child

2012

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“…However, MDCT failed to show the severity of pulmonary hypertension or presence of venous drainage through the bronchial vein, which we felt were necessary to make a management decision. Here, although measurements of right and left PA pressures and their differences are available from catheter angiogram [9], quantification of systemic pulmonary shunt flow may be unreliable because of severe turbulence and heterogeneity of PA blood flow (Figure 2c). Likewise, four-dimensional MRI, despite its power in showing temporal change in contrast enhancement of the lung, may be unsuitable for this purpose.…”

Section: Discussionmentioning

confidence: 99%

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

Kim¹,

Yoo²,

Ahn³

et al. 2011

BJR

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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...

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“…Most patients were diagnosed during infancy or early childhood due to episodes of recurrent pneumonia, hemoptysis and pulmonary hypertension due to the systemic collateral connection to pulmonary vessels (5). About 50% of patients had fatal results if they did not receive that did not receive treatment did not survive (6). Due to the serious pulmonary symptoms, such as hemoptysis, recurrent pneumonia, and progressive dyspnea many hypertrophied bronchial and intercostal arteries from the ascending aorta that were connected with the pulmonary vessels at the peripheral subpleural area on coronal maximum intensity projection images (Fig.…”

Section: Case Reportmentioning

confidence: 99%

“…Most of the patients reported were in infancy or early childhood with recurrent episodes of hemoptysis or pneumonia. 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).…”

Section: Introductionmentioning

confidence: 99%

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

Lee

Park

2012

J Korean Soc Radiol

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Isolated pulmonary vein atresia.

Cited by 39 publications

References 5 publications

Pulmonary Vein Atresia with Severe Contralateral Pulmonary Vein Stenosis in a Child

Pulmonary Vein Atresia with Severe Contralateral Pulmonary Vein Stenosis in a Child

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

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

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