Congenital Unilateral Pulmonary Venous Atresia: Definitive Diagnosis and Treatment

Pourmoghadam, Kamal; Moore, John W.; Khan, Muhammad; Geary, Elaine M; Madan, Nandini; Wolfson, Barbara J.; Chadarévian, Jean Pierre De; Holsclaw, Douglas S.; Jacobs, Marshall L.

doi:10.1007/s00246-002-0220-6

Cited by 67 publications

(112 citation statements)

References 0 publications

Supporting

Mentioning

108

Contrasting

Unclassified

Order By: Relevance

“…According to the literature review by Pourmoghadam et al [2], there were five cases, including their own, who had been followed up without surgical treatment for an average period of 6.8 years (range 1-18 years) [2]. The clinical characteristics of those six patients have been summarised in Table 1, along with those of our patients.…”

Section: Discussionmentioning

confidence: 90%

“…This anomaly seems to have no particular predilection towards either side [1,2,4]. In these reports, a broad spectrum of clinical severity has been described, ranging from none [7] to recurrent pulmonary infection to severe haemoptysis to death, although fatal outcomes have been confined to those diagnosed before the age of 8 years [2]. In most patients, unilateral PV atresia is diagnosed during infancy [1] and, to our knowledge, only six (including ours) adult cases of this condition have been reported.…”

Section: Discussionmentioning

confidence: 92%

“…In most patients, unilateral PV atresia is diagnosed during infancy [1] and, to our knowledge, only six (including ours) adult cases of this condition have been reported. Four of them, previously reported by Pourmoghadam et al [2] and Heyneman et al [4], presented with serious respiratory symptoms, such as significant haemoptysis, recurrent pneumonia or progressive dyspnoea. For these reasons, as well as for the purpose of pulmonary hypertension prevention, pneumonectomy has been recommended for treatment of this condition [2,5].…”

Section: Discussionmentioning

confidence: 99%

“…To our knowledge, fewer than 40 cases of unilateral pulmonary vein atresia without associated structural abnormalities of the heart have been reported in the medical literature [1][2][3][4][5][6]. This anomaly seems to have no particular predilection towards either side [1,2,4].…”

Section: Discussionmentioning

confidence: 99%

See 3 more Smart Citations

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

Kim¹,

Yoo²,

Ahn³

et al. 2011

BJR

View full text Add to dashboard Cite

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...

show abstract

Section: Discussionmentioning

confidence: 90%

Section: Discussionmentioning

confidence: 92%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

See 2 more Smart Citations

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

Kim¹,

Yoo²,

Ahn³

et al. 2011

BJR

View full text Add to dashboard Cite

show abstract

“…Unilateral pulmonary vein atresia without associated congenital abnormalities is a very rare clinic condition (1,2). It is of prognostic importance with significant mortality and morbidity.…”

Section: Introductionmentioning

confidence: 99%