Early and long-term outcomes following cardiac surgery for patients with heterotaxy syndromeCentral MessagePerspective
Objective: Heterotaxy syndrome is a complex multisystem abnormality historically associated with high morbidity and mortality. We sought to evaluate the early and long-term outcomes after cardiac surgery in heterotaxy syndrome. Methods: This is a single-center retrospective review of patients with h...
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| Format: | Article |
| Language: | English |
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Elsevier
2024-04-01
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| Series: | JTCVS Open |
| Subjects: | |
| Online Access: | http://www.sciencedirect.com/science/article/pii/S2666273624000469 |
| _version_ | 1827279081325985792 |
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| author | Victor S. Alemany, MD, MS Alexis Crawford, BA Kimberlee Gauvreau, ScD Emily M. Bucholz, MD-PhD, MPH Pedro J. del Nido, MD David N. Schidlow, MD, MMus Meena Nathan, MD, MPH |
| author_facet | Victor S. Alemany, MD, MS Alexis Crawford, BA Kimberlee Gauvreau, ScD Emily M. Bucholz, MD-PhD, MPH Pedro J. del Nido, MD David N. Schidlow, MD, MMus Meena Nathan, MD, MPH |
| author_sort | Victor S. Alemany, MD, MS |
| collection | DOAJ |
| description | Objective: Heterotaxy syndrome is a complex multisystem abnormality historically associated with high morbidity and mortality. We sought to evaluate the early and long-term outcomes after cardiac surgery in heterotaxy syndrome. Methods: This is a single-center retrospective review of patients with heterotaxy syndrome undergoing single-ventricle palliation or primary or staged biventricular repair from 1998 to 2018. Patients were stratified by single ventricle versus biventricular physiology, and the severity of atrioventricular valve regurgitation. Demographics, anatomic characteristics, and early and late outcomes, including the length of stay, mortality, and surgical or catheter reinterventions, were analyzed. Results: Among 250 patients, 150 (60%) underwent biventricular repair. In-hospital mortality was 7.6% (n = 19). Median follow-up was 5.2 (range, 0-16) years. Among survivors to discharge, mortality was 19% (n = 44) and reintervention was 52% (n = 120). Patients with moderate/severe atrioventricular valve regurgitation were older (32 vs 16 months, P = .02), were more likely to experience adverse events during their index surgical admission (72% vs 46%, P < .001), and had longer in-hospital length of stay (20 vs 12 days, P = .009). Among patients with moderate to severe atrioventricular valve regurgitation, single-ventricle palliation is associated with a greater risk of unplanned reintervention compared with patients undergoing biventricular repair (hazard ratio, 2.13; CI, 1.10-4.12; P = .025). Conclusions: There was no significant difference in early or late outcomes in single-ventricle versus biventricular repair strategies in heterotaxy. In the subgroup of patients with moderate/severe atrioventricular valve regurgitation, patients who underwent single-ventricle palliation were 2.5 times more likely to need a late reintervention compared with those undergoing biventricular repair. |
| first_indexed | 2024-04-24T08:12:09Z |
| format | Article |
| id | doaj.art-bf1de985fa05443992cabf141000f1e6 |
| institution | Directory Open Access Journal |
| issn | 2666-2736 |
| language | English |
| last_indexed | 2024-04-24T08:12:09Z |
| publishDate | 2024-04-01 |
| publisher | Elsevier |
| record_format | Article |
| series | JTCVS Open |
| spelling | doaj.art-bf1de985fa05443992cabf141000f1e62024-04-17T04:50:01ZengElsevierJTCVS Open2666-27362024-04-0118167179Early and long-term outcomes following cardiac surgery for patients with heterotaxy syndromeCentral MessagePerspectiveVictor S. Alemany, MD, MS0Alexis Crawford, BA1Kimberlee Gauvreau, ScD2Emily M. Bucholz, MD-PhD, MPH3Pedro J. del Nido, MD4David N. Schidlow, MD, MMus5Meena Nathan, MD, MPH6Department of Cardiac Surgery, Boston Children's Hospital, Boston, MassDepartment of Cardiac Surgery, Boston Children's Hospital, Boston, MassDepartment of Cardiology, Boston Children's Hospital, Boston, Mass; Department of Biostatistics, Harvard School of Public Health, Boston, MassDepartment of Cardiology, Boston Children's Hospital, Boston, MassDepartment of Cardiac Surgery, Boston Children's Hospital, Boston, Mass; Department of Surgery, Harvard Medical School, Boston, MassDepartment of Cardiology, Boston Children's Hospital, Boston, Mass; Department of Pediatrics, Harvard Medical School, Boston, Mass; Address for reprints: Meena Nathan, MD, MPH, and David N. Schidlow MD, MMus, Department of Cardiovascular Surgery and Department of Cardiology, Boston Children's Hospital, 300 Longwood Ave, Boston, MA 02115.Department of Cardiac Surgery, Boston Children's Hospital, Boston, Mass; Department of Surgery, Harvard Medical School, Boston, Mass; Address for reprints: Meena Nathan, MD, MPH, and David N. Schidlow MD, MMus, Department of Cardiovascular Surgery and Department of Cardiology, Boston Children's Hospital, 300 Longwood Ave, Boston, MA 02115.Objective: Heterotaxy syndrome is a complex multisystem abnormality historically associated with high morbidity and mortality. We sought to evaluate the early and long-term outcomes after cardiac surgery in heterotaxy syndrome. Methods: This is a single-center retrospective review of patients with heterotaxy syndrome undergoing single-ventricle palliation or primary or staged biventricular repair from 1998 to 2018. Patients were stratified by single ventricle versus biventricular physiology, and the severity of atrioventricular valve regurgitation. Demographics, anatomic characteristics, and early and late outcomes, including the length of stay, mortality, and surgical or catheter reinterventions, were analyzed. Results: Among 250 patients, 150 (60%) underwent biventricular repair. In-hospital mortality was 7.6% (n = 19). Median follow-up was 5.2 (range, 0-16) years. Among survivors to discharge, mortality was 19% (n = 44) and reintervention was 52% (n = 120). Patients with moderate/severe atrioventricular valve regurgitation were older (32 vs 16 months, P = .02), were more likely to experience adverse events during their index surgical admission (72% vs 46%, P < .001), and had longer in-hospital length of stay (20 vs 12 days, P = .009). Among patients with moderate to severe atrioventricular valve regurgitation, single-ventricle palliation is associated with a greater risk of unplanned reintervention compared with patients undergoing biventricular repair (hazard ratio, 2.13; CI, 1.10-4.12; P = .025). Conclusions: There was no significant difference in early or late outcomes in single-ventricle versus biventricular repair strategies in heterotaxy. In the subgroup of patients with moderate/severe atrioventricular valve regurgitation, patients who underwent single-ventricle palliation were 2.5 times more likely to need a late reintervention compared with those undergoing biventricular repair.http://www.sciencedirect.com/science/article/pii/S2666273624000469aspleniacongenital heart diseaseFontan operationheterotaxy syndromesingle ventricle |
| spellingShingle | Victor S. Alemany, MD, MS Alexis Crawford, BA Kimberlee Gauvreau, ScD Emily M. Bucholz, MD-PhD, MPH Pedro J. del Nido, MD David N. Schidlow, MD, MMus Meena Nathan, MD, MPH Early and long-term outcomes following cardiac surgery for patients with heterotaxy syndromeCentral MessagePerspective JTCVS Open asplenia congenital heart disease Fontan operation heterotaxy syndrome single ventricle |
| title | Early and long-term outcomes following cardiac surgery for patients with heterotaxy syndromeCentral MessagePerspective |
| title_full | Early and long-term outcomes following cardiac surgery for patients with heterotaxy syndromeCentral MessagePerspective |
| title_fullStr | Early and long-term outcomes following cardiac surgery for patients with heterotaxy syndromeCentral MessagePerspective |
| title_full_unstemmed | Early and long-term outcomes following cardiac surgery for patients with heterotaxy syndromeCentral MessagePerspective |
| title_short | Early and long-term outcomes following cardiac surgery for patients with heterotaxy syndromeCentral MessagePerspective |
| title_sort | early and long term outcomes following cardiac surgery for patients with heterotaxy syndromecentral messageperspective |
| topic | asplenia congenital heart disease Fontan operation heterotaxy syndrome single ventricle |
| url | http://www.sciencedirect.com/science/article/pii/S2666273624000469 |
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