Contemporary survival outcomes of congenital systemic-to-pulmonary shunt in children with borderline pulmonary vascular resistant index compared to Eisenmenger syndrome
Abstract Pulmonary arterial hypertension (PAH) associated with congenital heart disease (PAH-CHD) is a complication that occurs after unrepaired significant systemic-to-pulmonary shunt. Treatment options for PAH-CHD-predominantly left-to-right (L-R) shunt in children with borderline-high pulmonary v...
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2024-07-01
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| Online Access: | https://doi.org/10.1038/s41598-024-67899-6 |
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| author | Nalin Chaiwangyen Chodchanok Vijarnsorn Paweena Chungsomprasong Prakul Chanthong Supaluck Kanjanauthai Ploy Thammasate Thita Pacharapakornpong Densiri Bositthipichet Jantaras Sengsim Jarupim Soongswang Teerapong Tocharoenchok Ekarat Nitiyarom Kriangkrai Tantiwongkosri Thaworn Subtaweesin Kritvikrom Durongpisitkul |
| author_facet | Nalin Chaiwangyen Chodchanok Vijarnsorn Paweena Chungsomprasong Prakul Chanthong Supaluck Kanjanauthai Ploy Thammasate Thita Pacharapakornpong Densiri Bositthipichet Jantaras Sengsim Jarupim Soongswang Teerapong Tocharoenchok Ekarat Nitiyarom Kriangkrai Tantiwongkosri Thaworn Subtaweesin Kritvikrom Durongpisitkul |
| author_sort | Nalin Chaiwangyen |
| collection | DOAJ |
| description | Abstract Pulmonary arterial hypertension (PAH) associated with congenital heart disease (PAH-CHD) is a complication that occurs after unrepaired significant systemic-to-pulmonary shunt. Treatment options for PAH-CHD-predominantly left-to-right (L-R) shunt in children with borderline-high pulmonary vascular resistant index (PVRi) have been debated. We aimed to assess the treatment and survival of children with PAH-CHD-predominantly L-R shunt with borderline to high PVRi, using Eisenmenger syndrome (ES) for comparison. In 1995–2021, a total of 142 patients with ES and 192 children with PAH-CHD-predominantly L-R shunt were eligible for our analysis. The PVRi in ES patients was 26.7 ± 16.8 WU m2. Most patients (91%) received PAH-targeted therapy. Of the 192 children with PAH-CHD-predominantly L-R shunt, the baseline PVRi was 9.2 ± 5.8 WU m2. A total of 64 patients (33.3%) had borderline PVRi (4–8 WU m2) and 98 patients (51%) had high PVRi (> 8 WU m2). Most patients (88.5%) responded to acute pulmonary vasodilatory testing and underwent repair, with 158 undergoing defect closure and 12 having fenestrated closure. A treat-and-repair strategy was used in 33 children (17.1%). The 10- and 15-year survival rates for patients with ES were 79.3% and 72.4%, respectively, which was significantly inferior to children with borderline PVRi [97.3% and 87.8% (p = 0.02)]; and high PVRi [91.6% and 89.5% (p = 0.06)], respectively. The survival rate of children receiving treat-and-repair was slightly higher than that of ES (p = 0.16). The independent mortality risk in children with PAH-CHD-predominantly L-R shunt was persistent PAH following the defect correction (adjusted hazard ratio 5.8, 95% CI 1.7–19.9, p = 0.005). Trial registration: TCTR20200420004. |
| format | Article |
| id | doaj-art-142adf22b6294ab68bd0f7c64d01c58b |
| institution | Kabale University |
| issn | 2045-2322 |
| language | English |
| publishDate | 2024-07-01 |
| publisher | Nature Portfolio |
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| series | Scientific Reports |
| spelling | doaj-art-142adf22b6294ab68bd0f7c64d01c58b2025-02-02T12:25:27ZengNature PortfolioScientific Reports2045-23222024-07-0114111110.1038/s41598-024-67899-6Contemporary survival outcomes of congenital systemic-to-pulmonary shunt in children with borderline pulmonary vascular resistant index compared to Eisenmenger syndromeNalin Chaiwangyen0Chodchanok Vijarnsorn1Paweena Chungsomprasong2Prakul Chanthong3Supaluck Kanjanauthai4Ploy Thammasate5Thita Pacharapakornpong6Densiri Bositthipichet7Jantaras Sengsim8Jarupim Soongswang9Teerapong Tocharoenchok10Ekarat Nitiyarom11Kriangkrai Tantiwongkosri12Thaworn Subtaweesin13Kritvikrom Durongpisitkul14Department of Pediatrics, Faculty of Medicine Siriraj Hospital, Mahidol UniversityDepartment of Pediatrics, Faculty of Medicine Siriraj Hospital, Mahidol UniversityDepartment of Pediatrics, Faculty of Medicine Siriraj Hospital, Mahidol UniversityDepartment of Pediatrics, Faculty of Medicine Siriraj Hospital, Mahidol UniversityDepartment of Pediatrics, Faculty of Medicine Siriraj Hospital, Mahidol UniversityDepartment of Pediatrics, Faculty of Medicine Siriraj Hospital, Mahidol UniversityDepartment of Pediatrics, Faculty of Medicine Siriraj Hospital, Mahidol UniversityDepartment of Pediatrics, Faculty of Medicine Siriraj Hospital, Mahidol UniversityDepartment of Pediatrics, Faculty of Medicine Siriraj Hospital, Mahidol UniversityDepartment of Pediatrics, Faculty of Medicine Siriraj Hospital, Mahidol UniversityDepartment of Surgery, Faculty of Medicine Siriraj Hospital, Mahidol UniversityDepartment of Surgery, Faculty of Medicine Siriraj Hospital, Mahidol UniversityDepartment of Surgery, Faculty of Medicine Siriraj Hospital, Mahidol UniversityDepartment of Surgery, Faculty of Medicine Siriraj Hospital, Mahidol UniversityDepartment of Pediatrics, Faculty of Medicine Siriraj Hospital, Mahidol UniversityAbstract Pulmonary arterial hypertension (PAH) associated with congenital heart disease (PAH-CHD) is a complication that occurs after unrepaired significant systemic-to-pulmonary shunt. Treatment options for PAH-CHD-predominantly left-to-right (L-R) shunt in children with borderline-high pulmonary vascular resistant index (PVRi) have been debated. We aimed to assess the treatment and survival of children with PAH-CHD-predominantly L-R shunt with borderline to high PVRi, using Eisenmenger syndrome (ES) for comparison. In 1995–2021, a total of 142 patients with ES and 192 children with PAH-CHD-predominantly L-R shunt were eligible for our analysis. The PVRi in ES patients was 26.7 ± 16.8 WU m2. Most patients (91%) received PAH-targeted therapy. Of the 192 children with PAH-CHD-predominantly L-R shunt, the baseline PVRi was 9.2 ± 5.8 WU m2. A total of 64 patients (33.3%) had borderline PVRi (4–8 WU m2) and 98 patients (51%) had high PVRi (> 8 WU m2). Most patients (88.5%) responded to acute pulmonary vasodilatory testing and underwent repair, with 158 undergoing defect closure and 12 having fenestrated closure. A treat-and-repair strategy was used in 33 children (17.1%). The 10- and 15-year survival rates for patients with ES were 79.3% and 72.4%, respectively, which was significantly inferior to children with borderline PVRi [97.3% and 87.8% (p = 0.02)]; and high PVRi [91.6% and 89.5% (p = 0.06)], respectively. The survival rate of children receiving treat-and-repair was slightly higher than that of ES (p = 0.16). The independent mortality risk in children with PAH-CHD-predominantly L-R shunt was persistent PAH following the defect correction (adjusted hazard ratio 5.8, 95% CI 1.7–19.9, p = 0.005). Trial registration: TCTR20200420004.https://doi.org/10.1038/s41598-024-67899-6Pulmonary arterial hypertension (PAH)Congenital heart disease (CHD)EisenmengerPredominantly systemic-to-pulmonary shuntTreat-And-repair |
| spellingShingle | Nalin Chaiwangyen Chodchanok Vijarnsorn Paweena Chungsomprasong Prakul Chanthong Supaluck Kanjanauthai Ploy Thammasate Thita Pacharapakornpong Densiri Bositthipichet Jantaras Sengsim Jarupim Soongswang Teerapong Tocharoenchok Ekarat Nitiyarom Kriangkrai Tantiwongkosri Thaworn Subtaweesin Kritvikrom Durongpisitkul Contemporary survival outcomes of congenital systemic-to-pulmonary shunt in children with borderline pulmonary vascular resistant index compared to Eisenmenger syndrome Scientific Reports Pulmonary arterial hypertension (PAH) Congenital heart disease (CHD) Eisenmenger Predominantly systemic-to-pulmonary shunt Treat-And-repair |
| title | Contemporary survival outcomes of congenital systemic-to-pulmonary shunt in children with borderline pulmonary vascular resistant index compared to Eisenmenger syndrome |
| title_full | Contemporary survival outcomes of congenital systemic-to-pulmonary shunt in children with borderline pulmonary vascular resistant index compared to Eisenmenger syndrome |
| title_fullStr | Contemporary survival outcomes of congenital systemic-to-pulmonary shunt in children with borderline pulmonary vascular resistant index compared to Eisenmenger syndrome |
| title_full_unstemmed | Contemporary survival outcomes of congenital systemic-to-pulmonary shunt in children with borderline pulmonary vascular resistant index compared to Eisenmenger syndrome |
| title_short | Contemporary survival outcomes of congenital systemic-to-pulmonary shunt in children with borderline pulmonary vascular resistant index compared to Eisenmenger syndrome |
| title_sort | contemporary survival outcomes of congenital systemic to pulmonary shunt in children with borderline pulmonary vascular resistant index compared to eisenmenger syndrome |
| topic | Pulmonary arterial hypertension (PAH) Congenital heart disease (CHD) Eisenmenger Predominantly systemic-to-pulmonary shunt Treat-And-repair |
| url | https://doi.org/10.1038/s41598-024-67899-6 |
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