Bilateral lung transplantation for pediatric pulmonary arterial hypertension : perioperative management and one-year follow-up
© 2023 Jack, Carlens, Diekmann, Hasan, Chouvarine, Schwerk, Müller, Wieland, Tudorache, Warnecke, Avsar, Horke, Ius, Bobylev and Hansmann..
Background: Bilateral lung transplantation (LuTx) remains the only established treatment for children with end-stage pulmonary arterial hypertension (PAH). Although PAH is the second most common indication for LuTx, little is known about optimal perioperative management and midterm clinical outcomes.
Methods: Prospective observational study on consecutive children with PAH who underwent LuTx with scheduled postoperative VA-ECMO support at Hannover Medical School from December 2013 to June 2020.
Results: Twelve patients with PAH underwent LuTx (mean age 11.9 years; age range 1.9-17.8). Underlying diagnoses included idiopathic (n = 4) or heritable PAH (n = 4), PAH associated with congenital heart disease (n = 2), pulmonary veno-occlusive disease (n = 1), and pulmonary capillary hemangiomatosis (n = 1). The mean waiting time was 58.5 days (range 1-220d). Three patients were bridged to LuTx on VA-ECMO. Intraoperative VA-ECMO/cardiopulmonary bypass was applied and VA-ECMO was continued postoperatively in all patients (mean ECMO-duration 185 h; range 73-363 h; early extubation). The median postoperative ventilation time was 28 h (range 17-145 h). Echocardiographic conventional and strain analysis showed that 12 months after LuTx, all patients had normal biventricular systolic function. All PAH patients are alive 2 years after LuTx (median follow-up 53 months, range 26-104 months).
Conclusion: LuTx in children with end-stage PAH resulted in excellent midterm outcomes (100% survival 2 years post-LuTx). Postoperative VA-ECMO facilitates early extubation with rapid gain of allograft function and sustained biventricular reverse-remodeling and systolic function after RV pressure unloading and LV volume loading.
| Medienart: |
E-Artikel |
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| Erscheinungsjahr: |
2023 |
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| Erschienen: |
2023 |
| Enthalten in: |
Zur Gesamtaufnahme - volume:10 |
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| Enthalten in: |
Frontiers in cardiovascular medicine - 10(2023) vom: 05., Seite 1193326 |
| Sprache: |
Englisch |
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| Beteiligte Personen: |
Jack, Thomas [VerfasserIn] |
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| Links: |
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| Themen: |
Awake ECMO |
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| Anmerkungen: |
Date Revised 18.07.2023 published: Electronic-eCollection Citation Status PubMed-not-MEDLINE |
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| doi: |
10.3389/fcvm.2023.1193326 |
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| Förderinstitution / Projekttitel: |
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| PPN (Katalog-ID): |
NLM359424856 |
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| 520 | |a © 2023 Jack, Carlens, Diekmann, Hasan, Chouvarine, Schwerk, Müller, Wieland, Tudorache, Warnecke, Avsar, Horke, Ius, Bobylev and Hansmann. | ||
| 520 | |a Background: Bilateral lung transplantation (LuTx) remains the only established treatment for children with end-stage pulmonary arterial hypertension (PAH). Although PAH is the second most common indication for LuTx, little is known about optimal perioperative management and midterm clinical outcomes | ||
| 520 | |a Methods: Prospective observational study on consecutive children with PAH who underwent LuTx with scheduled postoperative VA-ECMO support at Hannover Medical School from December 2013 to June 2020 | ||
| 520 | |a Results: Twelve patients with PAH underwent LuTx (mean age 11.9 years; age range 1.9-17.8). Underlying diagnoses included idiopathic (n = 4) or heritable PAH (n = 4), PAH associated with congenital heart disease (n = 2), pulmonary veno-occlusive disease (n = 1), and pulmonary capillary hemangiomatosis (n = 1). The mean waiting time was 58.5 days (range 1-220d). Three patients were bridged to LuTx on VA-ECMO. Intraoperative VA-ECMO/cardiopulmonary bypass was applied and VA-ECMO was continued postoperatively in all patients (mean ECMO-duration 185 h; range 73-363 h; early extubation). The median postoperative ventilation time was 28 h (range 17-145 h). Echocardiographic conventional and strain analysis showed that 12 months after LuTx, all patients had normal biventricular systolic function. All PAH patients are alive 2 years after LuTx (median follow-up 53 months, range 26-104 months) | ||
| 520 | |a Conclusion: LuTx in children with end-stage PAH resulted in excellent midterm outcomes (100% survival 2 years post-LuTx). Postoperative VA-ECMO facilitates early extubation with rapid gain of allograft function and sustained biventricular reverse-remodeling and systolic function after RV pressure unloading and LV volume loading | ||
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| 700 | 1 | |a Chouvarine, Philippe |e verfasserin |4 aut | |
| 700 | 1 | |a Schwerk, Nicolaus |e verfasserin |4 aut | |
| 700 | 1 | |a Müller, Carsten |e verfasserin |4 aut | |
| 700 | 1 | |a Wieland, Ivonne |e verfasserin |4 aut | |
| 700 | 1 | |a Tudorache, Igor |e verfasserin |4 aut | |
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| 700 | 1 | |a Ius, Fabio |e verfasserin |4 aut | |
| 700 | 1 | |a Bobylev, Dmitry |e verfasserin |4 aut | |
| 700 | 1 | |a Hansmann, Georg |e verfasserin |4 aut | |
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