Long-Term Outcomes After an Individualized Strategy in Patients with Pulmonary Atresia and Intact Ventricular Septum.
Balloon valvuloplasty
Catheter intervention
Congenital heart disease
Fontan operation
Pulmonary atresia and intact ventricular septum
Reintervention
Survival
Journal
Pediatric cardiology
ISSN: 1432-1971
Titre abrégé: Pediatr Cardiol
Pays: United States
ID NLM: 8003849
Informations de publication
Date de publication:
Feb 2022
Feb 2022
Historique:
received:
10
07
2021
accepted:
16
09
2021
pubmed:
25
9
2021
medline:
19
2
2022
entrez:
24
9
2021
Statut:
ppublish
Résumé
This retrospective cohort study aimed to explore the long-term outcomes of an individualized strategy in patients with pulmonary atresia and intact ventricular septum (PAIVS). We analyzed survival and reintervention rates and identified risk factors for outcomes in patients with PAIVS treated based on individual right heart structures between 1979 and 2019. Ninety-five patients were included in this study. The z-scores of the pulmonary annulus, tricuspid annulus, and right ventricular end-diastolic volume were - 3.30 (- 15.15 to 1.83), - 0.70 (- 4.65 to 2.33), and - 1.51 (- 6.35 to 1.18), respectively. Right ventricular-dependent coronary circulation occurred in 15% of the patients. Among the 63 patients attempting biventricular strategy at first, 55 patients achieved biventricular circulation, 3 patients had one-and-a-half circulation, and 4 patients died perioperatively. Among the 33 patients attempting univentricular strategy at first, 10 patients died before the completion of Fontan operation, 17 patients (48%) accomplished Fontan operation, and 5 patients waited for Fontan operation. In one patient, conversion to biventricular circulation occurred. During the follow-up period of 720 person-years, the 20-year survival rate was significantly higher in patients with biventricular circulation than in those patients with univentricular circulation (93% vs. 67%, P < 0.001). Freedom from reintervention rates at 20 years was significantly lower in patients with biventricular circulation than in those patients with univentricular circulation (29% vs. 72%, P < 0.001). The pulmonary annulus z-score was an independent risk factor for reintervention in patients with biventricular circulation. Patients with biventricular circulation had an acceptable survival rate, but a high reintervention rate. Meanwhile, patients with univentricular circulation had high mortality before the completion of Fontan operation, although the reintervention rate was relatively low.
Identifiants
pubmed: 34559262
doi: 10.1007/s00246-021-02740-9
pii: 10.1007/s00246-021-02740-9
doi:
Types de publication
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
435-442Informations de copyright
© 2021. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.
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