The many faces and outcomes of pulmonary vein stenosis in early childhood.

Humpl, Tilman; Fineman, Jeffrey; Qureshi, Athar M (2021). The many faces and outcomes of pulmonary vein stenosis in early childhood. Pediatric pulmonology, 56(3), pp. 649-655. Wiley-Blackwell 10.1002/ppul.24848

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Pulmonary vein stenosis is a rare and poorly understood condition causing obstruction of the large pulmonary veins and of blood flow from the lungs to the left atrium. This results in elevated pulmonary venous pressure and pulmonary edema, pulmonary hypertension, potentially cardiac failure, and death. Clinical signs of the disease include failure to thrive, increasingly severe dyspnea, hemoptysis, respiratory difficulty, recurrent respiratory tract infections/pneumonia, cyanosis, and subcostal retractions. On chest radiograph, the most frequent finding is increased interstitial, ground-glass and/or reticular opacity. Transthoracic echocardiography with pulsed Doppler delineates the stenosis, magnetic resonance imaging and multislice computerized tomography are used for further evaluation. Interventional cardiac catherization, surgical techniques, and medical therapies have been used with varying success as treatment options.

Item Type:

Journal Article (Review Article)

Division/Institute:

04 Faculty of Medicine > Department of Gynaecology, Paediatrics and Endocrinology (DFKE) > Clinic of Paediatric Medicine
04 Faculty of Medicine > Department of Gynaecology, Paediatrics and Endocrinology (DFKE) > Clinic of Paediatric Medicine > Paediatric Intensive Care

UniBE Contributor:

Humpl, Tilman

Subjects:

600 Technology > 610 Medicine & health

ISSN:

8755-6863

Publisher:

Wiley-Blackwell

Language:

English

Submitter:

Tilman Humpl

Date Deposited:

19 Jan 2021 16:00

Last Modified:

11 Feb 2021 01:34

Publisher DOI:

10.1002/ppul.24848

PubMed ID:

32506838

Uncontrolled Keywords:

catheter intervention pulmonary hypertension pulmonary vein stenosis

BORIS DOI:

10.48350/150769

URI:

https://boris.unibe.ch/id/eprint/150769

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