Isolated unilateral pulmonary vein atresia with hemoptysis in a child

Tang, Changqing; Duan, Hongyu; Zhou, Kaiyu; Hua, Yimin; Liu, Xiaoliang; Li, Yifei; Wang, Chuan

doi:10.1097/md.0000000000011882

Cited by 11 publications

(9 citation statements)

References 16 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…It has been speculated that this abnormal mediastinal soft tissue mass may represent underlying myofibroblast-like soft tissue proliferation in pediatric patients with PVS [13,20,21]. In addition, previously published imaging studies have described similar findings in pediatric patients with complete pulmonary vein atresia [22][23][24]. Therefore, we postulate that perhaps this abnormal mediastinal soft tissue may be one of the underlying causative mechanisms for the narrowing of pulmonary veins in pediatric patients with PVS.…”

Section: Discussionsupporting

confidence: 58%

Extravascular MDCT Findings of Pulmonary Vein Stenosis in Children with Cardiac Septal Defect

et al. 2021

View full text Add to dashboard Cite

To retrospectively investigate the extravascular thoracic MDCT angiography findings of pulmonary vein stenosis (PVS) in children with a cardiac septal defect. Materials and Methods: Pediatric patients (age ≤ 18 years) with cardiac septal defect and PVS, confirmed by echocardiogram and/or conventional angiography, who underwent thoracic MDCT angiography studies from April 2009 to April 2021 were included. Two pediatric radiologists independently evaluated thoracic MDCT angiography studies for the presence of extravascular thoracic abnormalities in: (1) lung and airway (ground-glass opacity (GGO), consolidation, pulmonary nodule, mass, cyst, septal thickening, fibrosis, and bronchiectasis); (2) pleura (pleural thickening, pleural effusion, and pneumothorax); and (3) mediastinum (mass and lymphadenopathy). Interobserver agreement between the two independent pediatric radiology reviewers was evaluated with kappa statistics. Results: The final study group consisted of 20 thoracic MDCT angiography studies from 20 consecutive individual pediatric patients (13 males (65%) and 7 females (35%); mean age: 7.5 months; SD: 12.7; range: 2 days to 7 months) with cardiac septal defect and PVS. The characteristic extravascular thoracic MDCT angiography findings were GGO (18/20; 90%), septal thickening (9/20; 45%), pleural thickening (16/20; 80%), and ill-defined, mildly heterogeneously enhancing, non-calcified soft tissue mass (9/20; 45%) following the contours of PVS in the mediastinum. There was a high interobserver kappa agreement between two independent reviewers for detecting extravascular abnormalities on thoracic MDCT angiography studies (k = 0.99). Conclusion: PVS in children with a cardiac septal defect has a characteristic extravascular thoracic MDCT angiography finding. In the lungs and pleura, GGO, septal thickening, and pleural thickening are frequently seen in children with cardiac septal defect and PVS. In the mediastinum, a mildly heterogeneously enhancing, non-calcified soft tissue mass in the distribution of PVS in the mediastinum is seen in close to half of the pediatric patients with cardiac septal defect and PVS.

show abstract

Section: Discussionsupporting

confidence: 58%

Extravascular MDCT Findings of Pulmonary Vein Stenosis in Children with Cardiac Septal Defect

et al. 2021

View full text Add to dashboard Cite

show abstract

“…These vessels produce numerous vascular channels in the submucosa of the bronchial walls, which may enlarge and rupture. As a result, hemoptysis can be severe and persistent, with a 7% mortality rate [ 13 ]. Our patient had no major complaints until she presented with hemoptysis during late pregnancy.…”

Section: Discussionmentioning

confidence: 99%

Unilateral pulmonary artery agenesis: An unusual cause of hemoptysis

Wong¹,

Abidin²,

Musa³

et al. 2023

Respiratory Medicine Case Reports

View full text Add to dashboard Cite

“…Another case report had described regarding the fiberoptic bronchoscope, which was performed for further scrutiny. 9 The present series also includes one case (patient 6) with a suspected mediastinal tumor, who was referred from a regional hospital to our pediatric oncology center for further evaluation and treatment.…”

Section: Fig 4 Comparison Of Soft Tissue Volume and The Affected/normentioning

confidence: 99%

“…The soft tissue masses resemble those of malignant tumors such as malignant lymphoma, rhabdomyosarcoma, Ewing sarcoma, neuroblastoma, and inflammatory myofibroblastic tumor. Consequently, CUPVA has not always been diagnosed correctly, and the identification of a mediastinal soft tissue mass‐like lesion has led to misdiagnosis, and unnecessary biopsy or bronchoscopy 8,9 . To avoid unnecessary biopsies and other invasive imaging studies, such as bronchoscopy, 123I‐Meta‐iodobenzylguanidine (MIBG) scans, conventional angiography, or magnetic resonance imaging (MRI) with general anesthesia, pediatric radiologists should be alert to the possibility that a mediastinal solid mass associated with CUPVA could be present.…”

Section: Figmentioning

confidence: 99%

Mediastinal and hilar soft tissue mass‐like lesions in congenital unilateral pulmonary vein atresia: A retrospective review of seven pediatric patients

Okamoto

Miyazaki

Aoki

et al. 2020

Pediatrics International

View full text Add to dashboard Cite

Background: Congenital unilateral pulmonary vein atresia (CUPVA) is known to lead to the formation of an abnormal confluent mediastinal and hilar soft tissue mass, thoracic hypoplasia, and interlobular septal thickening on the affected side. The purpose of the present study is to investigate the frequency and severity of mediastinal soft tissue mass-like lesions and examine other abnormal findings associated with CUPVA. Methods: We retrospectively reviewed seven children with CUPVA who underwent contrast-enhanced CT scans and measured the soft tissue mass volume in the bilateral mediastinum (affected and normal side). The location of abnormal soft tissue was divided into three anatomical sections (paratracheal, peribronchial, and the dorsal aspect of the left atrium). The relationships among soft tissue volume and anatomical section were statistically evaluated. Also, the presence of thoracic hypoplasia, small ipsilateral pulmonary arteries, interlobular septal thickening, and ground-glass opacities were investigated. Results: In all cases, CT scans confirmed the presence of confluent soft tissue mass-like lesions in the affected mediastinum. The soft tissue volume on the affected side was 5.5-fold greater than the volume on the normal side (average: 18.0 cm 3 and 4.25 cm 3 respectively, P < 0.01). Thoracic hypoplasia and interlobular septal thickening were found in all patients. Small pulmonary arteries and ground-glass opacities were present in six of the seven patients. Conclusion: Abnormal mediastinal and hilar soft tissue is commonly found in patients with CUPVA. So, if we encounter the mediastinal soft tissue mass in patients with CUPVA, no further test will be indicated.

show abstract

Isolated unilateral pulmonary vein atresia with hemoptysis in a child

Abstract: Supplemental Digital Content is available in the text

Cited by 11 publications

References 16 publications

Extravascular MDCT Findings of Pulmonary Vein Stenosis in Children with Cardiac Septal Defect

Extravascular MDCT Findings of Pulmonary Vein Stenosis in Children with Cardiac Septal Defect

Unilateral pulmonary artery agenesis: An unusual cause of hemoptysis

Mediastinal and hilar soft tissue mass‐like lesions in congenital unilateral pulmonary vein atresia: A retrospective review of seven pediatric patients

Contact Info

Product

Resources

About