Partial anomalous pulmonary venous drainage in patients presenting with suspected pulmonary hypertension: A series of 90 patients from the ASPIRE registry.
anomalous pulmonary venous drainage
atrial septal defect
pulmonary hypertension
sinus venosus
Journal
Respirology (Carlton, Vic.)
ISSN: 1440-1843
Titre abrégé: Respirology
Pays: Australia
ID NLM: 9616368
Informations de publication
Date de publication:
10 2020
10 2020
Historique:
received:
10
11
2019
revised:
16
03
2020
accepted:
17
03
2020
pubmed:
7
4
2020
medline:
25
6
2021
entrez:
7
4
2020
Statut:
ppublish
Résumé
There are limited data regarding patients with PAPVD with suspected and diagnosed PH. Patients with PAPVD presenting to a large PH referral centre during 2007-2017 were identified from the ASPIRE registry. Ninety patients with PAPVD were identified; this was newly diagnosed at our unit in 71 patients (78%), despite 69% of these having previously undergone CT. Sixty-seven percent had a single right superior and 23% a single left superior anomalous vein. Patients with an SV-ASD had a significantly larger RV area, pulmonary artery and L-R shunt and a higher % predicted DL Undiagnosed PAPVD with or without ASD may be present in patients with suspected PH; cross-sectional imaging should therefore be specifically assessed whenever this diagnosis is considered. Radiological and physiological markers of L-R shunt are higher in patients with an associated SV-ASD. Although many patients with PAPVD and PH may have other potential causes of PH, a proportion of patients diagnosed with PAH have isolated PAPVD in the absence of other causative conditions.
Sections du résumé
BACKGROUND AND OBJECTIVE
There are limited data regarding patients with PAPVD with suspected and diagnosed PH.
METHODS
Patients with PAPVD presenting to a large PH referral centre during 2007-2017 were identified from the ASPIRE registry.
RESULTS
Ninety patients with PAPVD were identified; this was newly diagnosed at our unit in 71 patients (78%), despite 69% of these having previously undergone CT. Sixty-seven percent had a single right superior and 23% a single left superior anomalous vein. Patients with an SV-ASD had a significantly larger RV area, pulmonary artery and L-R shunt and a higher % predicted DL
CONCLUSION
Undiagnosed PAPVD with or without ASD may be present in patients with suspected PH; cross-sectional imaging should therefore be specifically assessed whenever this diagnosis is considered. Radiological and physiological markers of L-R shunt are higher in patients with an associated SV-ASD. Although many patients with PAPVD and PH may have other potential causes of PH, a proportion of patients diagnosed with PAH have isolated PAPVD in the absence of other causative conditions.
Identifiants
pubmed: 32249494
doi: 10.1111/resp.13815
pmc: PMC8653892
doi:
Types de publication
Journal Article
Research Support, Non-U.S. Gov't
Langues
eng
Sous-ensembles de citation
IM
Pagination
1066-1072Subventions
Organisme : Wellcome Trust
Pays : United Kingdom
Organisme : British Heart Foundation
ID : FS/18/13/33281
Pays : United Kingdom
Organisme : Wellcome Trust
ID : 205188/Z/16/Z
Pays : United Kingdom
Commentaires et corrections
Type : CommentIn
Informations de copyright
© 2020 The Authors. Respirology published by John Wiley & Sons Australia, Ltd on behalf of Asian Pacific Society of Respirology.
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