Cardiovascular disorders in patients with congenital portosystemic shunts: 23 years of experience in a tertiary referral centre.
Adolescent
Cardiovascular Diseases
/ diagnostic imaging
Child
Child, Preschool
Coronary Circulation
Female
Hemodynamics
Hepatopulmonary Syndrome
/ etiology
Humans
Hypertension, Portal
/ etiology
Hypoxia
/ etiology
Infant
Infant, Newborn
Liver Circulation
Male
Portal Vein
/ abnormalities
Prognosis
Pulmonary Arterial Hypertension
/ etiology
Pulmonary Circulation
Referral and Consultation
Registries
Retrospective Studies
Tertiary Care Centers
Vascular Malformations
/ complications
Congenital heart defects
Congenital portosystemic shunt
Heart failure
Hepatopulmonary syndrome
Hypertension pulmonaire
Insuffisance cardiaque
Malformations cardiaques congénitales
Pulmonary hypertension
Shunt portosystémique congénital
Syndrome hépatopulmonaire
Journal
Archives of cardiovascular diseases
ISSN: 1875-2128
Titre abrégé: Arch Cardiovasc Dis
Pays: Netherlands
ID NLM: 101465655
Informations de publication
Date de publication:
Mar 2021
Mar 2021
Historique:
received:
11
06
2020
revised:
16
08
2020
accepted:
06
10
2020
pubmed:
8
12
2020
medline:
30
4
2021
entrez:
7
12
2020
Statut:
ppublish
Résumé
Congenital portosystemic shunts are rare vascular malformations that may have an impact on the heart-lung system. Associated congenital and/or acquired heart diseases are poorly reported. To analyse cardiovascular disorders within a large congenital portosystemic shunt population, and develop a diagnostic strategy. Among the 168 consecutive fetuses and children referred for congenital portosystemic shunt (1996-2019), patients presenting with at least one cardiovascular disorder, including congenital heart disease, heart failure, portopulmonary hypertension and/or hepatopulmonary syndrome, were reviewed retrospectively. Cardiovascular disorders were detected using echocardiography and one or more of the following: right-sided heart catheterization; contrast-enhanced transthoracic echocardiography; or lung perfusion radionuclide scan. Overall, 46/168 patients with a congenital portosystemic shunt (27.4%) had one or more clinically significant cardiovascular disorders. Congenital heart disease was present in 28 patients, including six with left heterotaxy. Heart failure was present in six fetuses and 21 neonates (eight without congenital heart disease, and 13 with congenital heart disease). In neonates without congenital heart disease, heart function recovered by the age of 3years. Portopulmonary hypertension was identified in 11 patients (mean age at diagnosis: 9years); it was fatal in one patient, and remained stable in five of six patients after congenital portosystemic shunt closure. In six patients, hepatopulmonary syndrome presented as hypoxia (mean age at diagnosis: 5.3years), which reversed after congenital portosystemic shunt closure. Evaluation and monitoring of the cardiopulmonary status of patients with a congenital portosystemic shunt is mandatory to detect and prevent cardiovascular complications. Furthermore, congenital portosystemic shunts must be sought in patients with unexplained cardiovascular disorders, especially when malformations are present.
Sections du résumé
BACKGROUND
BACKGROUND
Congenital portosystemic shunts are rare vascular malformations that may have an impact on the heart-lung system. Associated congenital and/or acquired heart diseases are poorly reported.
AIMS
OBJECTIVE
To analyse cardiovascular disorders within a large congenital portosystemic shunt population, and develop a diagnostic strategy.
METHODS
METHODS
Among the 168 consecutive fetuses and children referred for congenital portosystemic shunt (1996-2019), patients presenting with at least one cardiovascular disorder, including congenital heart disease, heart failure, portopulmonary hypertension and/or hepatopulmonary syndrome, were reviewed retrospectively. Cardiovascular disorders were detected using echocardiography and one or more of the following: right-sided heart catheterization; contrast-enhanced transthoracic echocardiography; or lung perfusion radionuclide scan.
RESULTS
RESULTS
Overall, 46/168 patients with a congenital portosystemic shunt (27.4%) had one or more clinically significant cardiovascular disorders. Congenital heart disease was present in 28 patients, including six with left heterotaxy. Heart failure was present in six fetuses and 21 neonates (eight without congenital heart disease, and 13 with congenital heart disease). In neonates without congenital heart disease, heart function recovered by the age of 3years. Portopulmonary hypertension was identified in 11 patients (mean age at diagnosis: 9years); it was fatal in one patient, and remained stable in five of six patients after congenital portosystemic shunt closure. In six patients, hepatopulmonary syndrome presented as hypoxia (mean age at diagnosis: 5.3years), which reversed after congenital portosystemic shunt closure.
CONCLUSIONS
CONCLUSIONS
Evaluation and monitoring of the cardiopulmonary status of patients with a congenital portosystemic shunt is mandatory to detect and prevent cardiovascular complications. Furthermore, congenital portosystemic shunts must be sought in patients with unexplained cardiovascular disorders, especially when malformations are present.
Identifiants
pubmed: 33281106
pii: S1875-2136(20)30237-0
doi: 10.1016/j.acvd.2020.10.003
pii:
doi:
Types de publication
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
221-231Informations de copyright
Copyright © 2020 Elsevier Masson SAS. All rights reserved.