Incidence and risk factors for the development of pulmonary arteriovenous malformations after stage 2 palliation
Objective: This study evaluated the current incidence of pulmonary arteriovenous malformations (PAVMs) following stage 2 palliation (S2P). Methods: Patients who underwent S2P, either through a bidirectional cavopulmonary shunt (BCPS) or the Kawashima procedure (KP) between 1992 and 2022, were review...
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Elsevier
2025-09-01
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| Series: | International Journal of Cardiology Congenital Heart Disease |
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| Online Access: | http://www.sciencedirect.com/science/article/pii/S2666668525000473 |
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| author | Lea Behrend Thibault Schaeffer Muneaki Matsubara Jonas Palm Teresa Lemmen Nicole Piber Paul Philipp Heinisch Stanimir Georgiev Alfred Hager Peter Ewert Jürgen Hörer Masamichi Ono |
| author_facet | Lea Behrend Thibault Schaeffer Muneaki Matsubara Jonas Palm Teresa Lemmen Nicole Piber Paul Philipp Heinisch Stanimir Georgiev Alfred Hager Peter Ewert Jürgen Hörer Masamichi Ono |
| author_sort | Lea Behrend |
| collection | DOAJ |
| description | Objective: This study evaluated the current incidence of pulmonary arteriovenous malformations (PAVMs) following stage 2 palliation (S2P). Methods: Patients who underwent S2P, either through a bidirectional cavopulmonary shunt (BCPS) or the Kawashima procedure (KP) between 1992 and 2022, were reviewed. The cumulative incidence of PAVMs was compared between BCPS and KP. Risk factors for the development of PAVMs were identified. Results: Among 682 patients who underwent S2P, 661 (96.9 %) underwent BCPS and 21 (3.1 %) KP. Median age at S2P was 5.1 (interquartile ranges (IQR): 3.6–9.6) months. During the median interstage follow-up of 1.6 (IQR: 1.6–2.2) years, PAVMs developed in 11 (1.6 %) patients (1.1 % (n = 7) after BCPS and 19.0 % (n = 4) after KP). Cumulative incidence of PAVMs was higher in patients after KP than those after BCPS (p < 0.001). PAVMs were observed in the right lung in 9 patients and both lungs in two. One patient with biliary atresia died of progressive PAVMs and liver cirrhosis after KP, and the remaining 10 patients underwent Fontan completion with a median interval of 1.9 (IQR: 1.5–2.4) years. PAVMs improved in all patients (9 resolutions and 1 improved). Independent risk factors for the development of PAVMs were KP (hazard ratio (HR): 16.364, p < 0.001) in all patients, anomalous pulmonary venous connection (HR: 6.772, p = 0.023) in BCPS patients, and hypoplastic left heart syndrome (HR: 18.819, p = 0.018) in KP patients. Conclusions: The incidence of PAVMs after S2P is very low after BCPS but still relevant after KP. Resolution or improvement of PAVMs is probable after Fontan completion. |
| format | Article |
| id | doaj-art-4a073991249e4312b59fc76161b5cd4d |
| institution | Kabale University |
| issn | 2666-6685 |
| language | English |
| publishDate | 2025-09-01 |
| publisher | Elsevier |
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| series | International Journal of Cardiology Congenital Heart Disease |
| spelling | doaj-art-4a073991249e4312b59fc76161b5cd4d2025-08-22T04:58:28ZengElsevierInternational Journal of Cardiology Congenital Heart Disease2666-66852025-09-012110061110.1016/j.ijcchd.2025.100611Incidence and risk factors for the development of pulmonary arteriovenous malformations after stage 2 palliationLea Behrend0Thibault Schaeffer1Muneaki Matsubara2Jonas Palm3Teresa Lemmen4Nicole Piber5Paul Philipp Heinisch6Stanimir Georgiev7Alfred Hager8Peter Ewert9Jürgen Hörer10Masamichi Ono11Department of Congenital and Pediatric Heart Surgery, German Heart Center Munich, University Hospital of Technische Universität München, Division of Congenital and Pediatric Heart Surgery, University Hospital of Munich, Ludwig-Maximilians-Universität, Europäisches Kinderherzzentrum München, Munich, GermanyDepartment of Congenital and Pediatric Heart Surgery, German Heart Center Munich, University Hospital of Technische Universität München, Division of Congenital and Pediatric Heart Surgery, University Hospital of Munich, Ludwig-Maximilians-Universität, Europäisches Kinderherzzentrum München, Munich, GermanyDepartment of Congenital and Pediatric Heart Surgery, German Heart Center Munich, University Hospital of Technische Universität München, Division of Congenital and Pediatric Heart Surgery, University Hospital of Munich, Ludwig-Maximilians-Universität, Europäisches Kinderherzzentrum München, Munich, GermanyDepartment of Congenital Heart Disease and Pediatric Cardiology, German Heart Center Munich, University Hospital of Technische Universität München, Munich, GermanyDepartment of Congenital and Pediatric Heart Surgery, German Heart Center Munich, University Hospital of Technische Universität München, Division of Congenital and Pediatric Heart Surgery, University Hospital of Munich, Ludwig-Maximilians-Universität, Europäisches Kinderherzzentrum München, Munich, GermanyDepartment of Cardiovascular Surgery, German Heart Center Munich, University Hospital of Technische Universität München, Munich, GermanyDepartment of Congenital and Pediatric Heart Surgery, German Heart Center Munich, University Hospital of Technische Universität München, Division of Congenital and Pediatric Heart Surgery, University Hospital of Munich, Ludwig-Maximilians-Universität, Europäisches Kinderherzzentrum München, Munich, GermanyDepartment of Congenital Heart Disease and Pediatric Cardiology, German Heart Center Munich, University Hospital of Technische Universität München, Munich, GermanyDepartment of Congenital Heart Disease and Pediatric Cardiology, German Heart Center Munich, University Hospital of Technische Universität München, Munich, GermanyDepartment of Congenital Heart Disease and Pediatric Cardiology, German Heart Center Munich, University Hospital of Technische Universität München, Munich, GermanyDepartment of Congenital and Pediatric Heart Surgery, German Heart Center Munich, University Hospital of Technische Universität München, Division of Congenital and Pediatric Heart Surgery, University Hospital of Munich, Ludwig-Maximilians-Universität, Europäisches Kinderherzzentrum München, Munich, GermanyDepartment of Congenital and Pediatric Heart Surgery, German Heart Center Munich, University Hospital of Technische Universität München, Division of Congenital and Pediatric Heart Surgery, University Hospital of Munich, Ludwig-Maximilians-Universität, Europäisches Kinderherzzentrum München, Munich, Germany; Corresponding author. Department of Congenital and Pediatric Heart Surgery, German Heart Center Munich, Lazarettstraße 36, 80636, Munich, Germany.Objective: This study evaluated the current incidence of pulmonary arteriovenous malformations (PAVMs) following stage 2 palliation (S2P). Methods: Patients who underwent S2P, either through a bidirectional cavopulmonary shunt (BCPS) or the Kawashima procedure (KP) between 1992 and 2022, were reviewed. The cumulative incidence of PAVMs was compared between BCPS and KP. Risk factors for the development of PAVMs were identified. Results: Among 682 patients who underwent S2P, 661 (96.9 %) underwent BCPS and 21 (3.1 %) KP. Median age at S2P was 5.1 (interquartile ranges (IQR): 3.6–9.6) months. During the median interstage follow-up of 1.6 (IQR: 1.6–2.2) years, PAVMs developed in 11 (1.6 %) patients (1.1 % (n = 7) after BCPS and 19.0 % (n = 4) after KP). Cumulative incidence of PAVMs was higher in patients after KP than those after BCPS (p < 0.001). PAVMs were observed in the right lung in 9 patients and both lungs in two. One patient with biliary atresia died of progressive PAVMs and liver cirrhosis after KP, and the remaining 10 patients underwent Fontan completion with a median interval of 1.9 (IQR: 1.5–2.4) years. PAVMs improved in all patients (9 resolutions and 1 improved). Independent risk factors for the development of PAVMs were KP (hazard ratio (HR): 16.364, p < 0.001) in all patients, anomalous pulmonary venous connection (HR: 6.772, p = 0.023) in BCPS patients, and hypoplastic left heart syndrome (HR: 18.819, p = 0.018) in KP patients. Conclusions: The incidence of PAVMs after S2P is very low after BCPS but still relevant after KP. Resolution or improvement of PAVMs is probable after Fontan completion.http://www.sciencedirect.com/science/article/pii/S2666668525000473Pulmonary arteriovenous malformationsBidirectional cavopulmonary shuntKawashima procedureTotal cavopulmonary connection |
| spellingShingle | Lea Behrend Thibault Schaeffer Muneaki Matsubara Jonas Palm Teresa Lemmen Nicole Piber Paul Philipp Heinisch Stanimir Georgiev Alfred Hager Peter Ewert Jürgen Hörer Masamichi Ono Incidence and risk factors for the development of pulmonary arteriovenous malformations after stage 2 palliation International Journal of Cardiology Congenital Heart Disease Pulmonary arteriovenous malformations Bidirectional cavopulmonary shunt Kawashima procedure Total cavopulmonary connection |
| title | Incidence and risk factors for the development of pulmonary arteriovenous malformations after stage 2 palliation |
| title_full | Incidence and risk factors for the development of pulmonary arteriovenous malformations after stage 2 palliation |
| title_fullStr | Incidence and risk factors for the development of pulmonary arteriovenous malformations after stage 2 palliation |
| title_full_unstemmed | Incidence and risk factors for the development of pulmonary arteriovenous malformations after stage 2 palliation |
| title_short | Incidence and risk factors for the development of pulmonary arteriovenous malformations after stage 2 palliation |
| title_sort | incidence and risk factors for the development of pulmonary arteriovenous malformations after stage 2 palliation |
| topic | Pulmonary arteriovenous malformations Bidirectional cavopulmonary shunt Kawashima procedure Total cavopulmonary connection |
| url | http://www.sciencedirect.com/science/article/pii/S2666668525000473 |
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