Perioperative outcomes of Fontan operation: Impact of heterotaxy syndrome

Background/Purpose: Fontan operation is the standard surgical procedure for achieving long-term survival in single-ventricular complex congenital heart diseases (SV-CHD). We aim to identify the perioperative outcomes and impact of heterotaxy syndrome (HS) after Fontan operation in a tertiary pediatr...
Ausführliche Beschreibung

Gespeichert in:

Autor*in:	Lin, Hsin-Chia [verfasserIn] Wu, Mei-Hwan [verfasserIn] Wang, Jou-Kou [verfasserIn] Lin, Ming-Tai [verfasserIn] Chen, Chun-An [verfasserIn] Lu, Chun-Wei [verfasserIn] Chen, Yih-Sharng [verfasserIn] Huang, Shu-Chien [verfasserIn] Chiu, Shuenn-Nan [verfasserIn]

Format:	E-Artikel
Sprache:	Englisch

Erschienen:	2021

Schlagwörter:	Fontan operation Congenital heart disease Heterotaxy syndrome Pulmonary hypertension

Übergeordnetes Werk:	Enthalten in: Journal of the Formosan Medical Association - Tai wan yi xue hui ; ID: gnd/1042770-3, Hong Kong : Elsevier, 2006, 121, Seite 89-97
Übergeordnetes Werk:	volume:121 ; pages:89-97

DOI / URN:	10.1016/j.jfma.2021.01.014

Katalog-ID:	ELV007137168

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520			\|a Background/Purpose: Fontan operation is the standard surgical procedure for achieving long-term survival in single-ventricular complex congenital heart diseases (SV-CHD). We aim to identify the perioperative outcomes and impact of heterotaxy syndrome (HS) after Fontan operation in a tertiary pediatric cardiology center.Methods: Medical records were reviewed for all patients who received Fontan operation and who were born between 1997 and 2017 in our institution. Preoperative, operative, and postoperative risk factors for perioperative mortality and morbidity were analyzed.Results: Totally, 154 patients were enrolled (103 SV-CHD and 51 HS), and the male to female ratio was 92:62. The mean age of Fontan operation was 5.1 years, and extracardiac conduit comprised the majority (90.9%) of Fontan operation. Overall perioperative event-free survival to discharge was 91.6% (84.3% in HS and 95.1% in other SV-CHD, P = 0.032). For secondary outcomes, length of intensive care stay and duration of pleural effusion drainage were not significantly different between patients with HS and other SV-CHD, but postoperative arrhythmia was more common in HS group (31.4% vs. 12.6%, P = 0.005). In multivariable regression analysis, preoperative risk factors including operation year before 2007 and high PAP and postoperative factors of elevated postoperative CVP were associated with worse outcomes. HS was not a predictor of worse outcome after adjusting for preoperative PAP and operation era.Conclusion: Surgical outcome has improved much in current era. Perioperative outcome is poorer in patients with HS than other SV-CHD, but HS is not a predictor of perioperative mortality after adjusting for hemodynamic factors.
650		4	\|a Fontan operation
650		4	\|a Congenital heart disease
650		4	\|a Heterotaxy syndrome
650		4	\|a Pulmonary hypertension
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700	1		\|a Chen, Chun-An \|e verfasserin \|4 aut
700	1		\|a Lu, Chun-Wei \|e verfasserin \|0 (orcid)0000-0002-6872-4764 \|4 aut
700	1		\|a Chen, Yih-Sharng \|e verfasserin \|4 aut
700	1		\|a Huang, Shu-Chien \|e verfasserin \|4 aut
700	1		\|a Chiu, Shuenn-Nan \|e verfasserin \|4 aut
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allfields	10.1016/j.jfma.2021.01.014 doi (DE-627)ELV007137168 (ELSEVIER)S0929-6646(21)00036-X DE-627 ger DE-627 rda eng 610 DE-600 44.04 bkl Lin, Hsin-Chia verfasserin (orcid)0000-0002-5819-8473 aut Perioperative outcomes of Fontan operation: Impact of heterotaxy syndrome 2021 nicht spezifiziert zzz rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier Background/Purpose: Fontan operation is the standard surgical procedure for achieving long-term survival in single-ventricular complex congenital heart diseases (SV-CHD). We aim to identify the perioperative outcomes and impact of heterotaxy syndrome (HS) after Fontan operation in a tertiary pediatric cardiology center.Methods: Medical records were reviewed for all patients who received Fontan operation and who were born between 1997 and 2017 in our institution. Preoperative, operative, and postoperative risk factors for perioperative mortality and morbidity were analyzed.Results: Totally, 154 patients were enrolled (103 SV-CHD and 51 HS), and the male to female ratio was 92:62. The mean age of Fontan operation was 5.1 years, and extracardiac conduit comprised the majority (90.9%) of Fontan operation. Overall perioperative event-free survival to discharge was 91.6% (84.3% in HS and 95.1% in other SV-CHD, P = 0.032). For secondary outcomes, length of intensive care stay and duration of pleural effusion drainage were not significantly different between patients with HS and other SV-CHD, but postoperative arrhythmia was more common in HS group (31.4% vs. 12.6%, P = 0.005). In multivariable regression analysis, preoperative risk factors including operation year before 2007 and high PAP and postoperative factors of elevated postoperative CVP were associated with worse outcomes. HS was not a predictor of worse outcome after adjusting for preoperative PAP and operation era.Conclusion: Surgical outcome has improved much in current era. Perioperative outcome is poorer in patients with HS than other SV-CHD, but HS is not a predictor of perioperative mortality after adjusting for hemodynamic factors. Fontan operation Congenital heart disease Heterotaxy syndrome Pulmonary hypertension Wu, Mei-Hwan verfasserin aut Wang, Jou-Kou verfasserin aut Lin, Ming-Tai verfasserin (orcid)0000-0002-1718-7137 aut Chen, Chun-An verfasserin aut Lu, Chun-Wei verfasserin (orcid)0000-0002-6872-4764 aut Chen, Yih-Sharng verfasserin aut Huang, Shu-Chien verfasserin aut Chiu, Shuenn-Nan verfasserin aut Enthalten in Tai wan yi xue hui ; ID: gnd/1042770-3 Journal of the Formosan Medical Association Hong Kong : Elsevier, 2006 121, Seite 89-97 Online-Ressource (DE-627)517812649 (DE-600)2250901-X (DE-576)272713511 1876-0821 nnns volume:121 pages:89-97 GBV_USEFLAG_U SYSFLAG_U GBV_ELV SSG-OLC-PHA GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_31 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_73 GBV_ILN_74 GBV_ILN_95 GBV_ILN_100 GBV_ILN_105 GBV_ILN_110 GBV_ILN_151 GBV_ILN_161 GBV_ILN_170 GBV_ILN_206 GBV_ILN_213 GBV_ILN_230 GBV_ILN_285 GBV_ILN_293 GBV_ILN_602 GBV_ILN_2004 GBV_ILN_2014 GBV_ILN_2068 GBV_ILN_4012 GBV_ILN_4037 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4249 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4338 GBV_ILN_4367 GBV_ILN_4700 44.04 Ausbildung Beruf Organisationen Medizin AR 121 89-97
spelling	10.1016/j.jfma.2021.01.014 doi (DE-627)ELV007137168 (ELSEVIER)S0929-6646(21)00036-X DE-627 ger DE-627 rda eng 610 DE-600 44.04 bkl Lin, Hsin-Chia verfasserin (orcid)0000-0002-5819-8473 aut Perioperative outcomes of Fontan operation: Impact of heterotaxy syndrome 2021 nicht spezifiziert zzz rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier Background/Purpose: Fontan operation is the standard surgical procedure for achieving long-term survival in single-ventricular complex congenital heart diseases (SV-CHD). We aim to identify the perioperative outcomes and impact of heterotaxy syndrome (HS) after Fontan operation in a tertiary pediatric cardiology center.Methods: Medical records were reviewed for all patients who received Fontan operation and who were born between 1997 and 2017 in our institution. Preoperative, operative, and postoperative risk factors for perioperative mortality and morbidity were analyzed.Results: Totally, 154 patients were enrolled (103 SV-CHD and 51 HS), and the male to female ratio was 92:62. The mean age of Fontan operation was 5.1 years, and extracardiac conduit comprised the majority (90.9%) of Fontan operation. Overall perioperative event-free survival to discharge was 91.6% (84.3% in HS and 95.1% in other SV-CHD, P = 0.032). For secondary outcomes, length of intensive care stay and duration of pleural effusion drainage were not significantly different between patients with HS and other SV-CHD, but postoperative arrhythmia was more common in HS group (31.4% vs. 12.6%, P = 0.005). In multivariable regression analysis, preoperative risk factors including operation year before 2007 and high PAP and postoperative factors of elevated postoperative CVP were associated with worse outcomes. HS was not a predictor of worse outcome after adjusting for preoperative PAP and operation era.Conclusion: Surgical outcome has improved much in current era. Perioperative outcome is poorer in patients with HS than other SV-CHD, but HS is not a predictor of perioperative mortality after adjusting for hemodynamic factors. Fontan operation Congenital heart disease Heterotaxy syndrome Pulmonary hypertension Wu, Mei-Hwan verfasserin aut Wang, Jou-Kou verfasserin aut Lin, Ming-Tai verfasserin (orcid)0000-0002-1718-7137 aut Chen, Chun-An verfasserin aut Lu, Chun-Wei verfasserin (orcid)0000-0002-6872-4764 aut Chen, Yih-Sharng verfasserin aut Huang, Shu-Chien verfasserin aut Chiu, Shuenn-Nan verfasserin aut Enthalten in Tai wan yi xue hui ; ID: gnd/1042770-3 Journal of the Formosan Medical Association Hong Kong : Elsevier, 2006 121, Seite 89-97 Online-Ressource (DE-627)517812649 (DE-600)2250901-X (DE-576)272713511 1876-0821 nnns volume:121 pages:89-97 GBV_USEFLAG_U SYSFLAG_U GBV_ELV SSG-OLC-PHA GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_31 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_73 GBV_ILN_74 GBV_ILN_95 GBV_ILN_100 GBV_ILN_105 GBV_ILN_110 GBV_ILN_151 GBV_ILN_161 GBV_ILN_170 GBV_ILN_206 GBV_ILN_213 GBV_ILN_230 GBV_ILN_285 GBV_ILN_293 GBV_ILN_602 GBV_ILN_2004 GBV_ILN_2014 GBV_ILN_2068 GBV_ILN_4012 GBV_ILN_4037 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4249 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4338 GBV_ILN_4367 GBV_ILN_4700 44.04 Ausbildung Beruf Organisationen Medizin AR 121 89-97
allfields_unstemmed	10.1016/j.jfma.2021.01.014 doi (DE-627)ELV007137168 (ELSEVIER)S0929-6646(21)00036-X DE-627 ger DE-627 rda eng 610 DE-600 44.04 bkl Lin, Hsin-Chia verfasserin (orcid)0000-0002-5819-8473 aut Perioperative outcomes of Fontan operation: Impact of heterotaxy syndrome 2021 nicht spezifiziert zzz rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier Background/Purpose: Fontan operation is the standard surgical procedure for achieving long-term survival in single-ventricular complex congenital heart diseases (SV-CHD). We aim to identify the perioperative outcomes and impact of heterotaxy syndrome (HS) after Fontan operation in a tertiary pediatric cardiology center.Methods: Medical records were reviewed for all patients who received Fontan operation and who were born between 1997 and 2017 in our institution. Preoperative, operative, and postoperative risk factors for perioperative mortality and morbidity were analyzed.Results: Totally, 154 patients were enrolled (103 SV-CHD and 51 HS), and the male to female ratio was 92:62. The mean age of Fontan operation was 5.1 years, and extracardiac conduit comprised the majority (90.9%) of Fontan operation. Overall perioperative event-free survival to discharge was 91.6% (84.3% in HS and 95.1% in other SV-CHD, P = 0.032). For secondary outcomes, length of intensive care stay and duration of pleural effusion drainage were not significantly different between patients with HS and other SV-CHD, but postoperative arrhythmia was more common in HS group (31.4% vs. 12.6%, P = 0.005). In multivariable regression analysis, preoperative risk factors including operation year before 2007 and high PAP and postoperative factors of elevated postoperative CVP were associated with worse outcomes. HS was not a predictor of worse outcome after adjusting for preoperative PAP and operation era.Conclusion: Surgical outcome has improved much in current era. Perioperative outcome is poorer in patients with HS than other SV-CHD, but HS is not a predictor of perioperative mortality after adjusting for hemodynamic factors. Fontan operation Congenital heart disease Heterotaxy syndrome Pulmonary hypertension Wu, Mei-Hwan verfasserin aut Wang, Jou-Kou verfasserin aut Lin, Ming-Tai verfasserin (orcid)0000-0002-1718-7137 aut Chen, Chun-An verfasserin aut Lu, Chun-Wei verfasserin (orcid)0000-0002-6872-4764 aut Chen, Yih-Sharng verfasserin aut Huang, Shu-Chien verfasserin aut Chiu, Shuenn-Nan verfasserin aut Enthalten in Tai wan yi xue hui ; ID: gnd/1042770-3 Journal of the Formosan Medical Association Hong Kong : Elsevier, 2006 121, Seite 89-97 Online-Ressource (DE-627)517812649 (DE-600)2250901-X (DE-576)272713511 1876-0821 nnns volume:121 pages:89-97 GBV_USEFLAG_U SYSFLAG_U GBV_ELV SSG-OLC-PHA GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_31 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_73 GBV_ILN_74 GBV_ILN_95 GBV_ILN_100 GBV_ILN_105 GBV_ILN_110 GBV_ILN_151 GBV_ILN_161 GBV_ILN_170 GBV_ILN_206 GBV_ILN_213 GBV_ILN_230 GBV_ILN_285 GBV_ILN_293 GBV_ILN_602 GBV_ILN_2004 GBV_ILN_2014 GBV_ILN_2068 GBV_ILN_4012 GBV_ILN_4037 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4249 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4338 GBV_ILN_4367 GBV_ILN_4700 44.04 Ausbildung Beruf Organisationen Medizin AR 121 89-97
allfieldsGer	10.1016/j.jfma.2021.01.014 doi (DE-627)ELV007137168 (ELSEVIER)S0929-6646(21)00036-X DE-627 ger DE-627 rda eng 610 DE-600 44.04 bkl Lin, Hsin-Chia verfasserin (orcid)0000-0002-5819-8473 aut Perioperative outcomes of Fontan operation: Impact of heterotaxy syndrome 2021 nicht spezifiziert zzz rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier Background/Purpose: Fontan operation is the standard surgical procedure for achieving long-term survival in single-ventricular complex congenital heart diseases (SV-CHD). We aim to identify the perioperative outcomes and impact of heterotaxy syndrome (HS) after Fontan operation in a tertiary pediatric cardiology center.Methods: Medical records were reviewed for all patients who received Fontan operation and who were born between 1997 and 2017 in our institution. Preoperative, operative, and postoperative risk factors for perioperative mortality and morbidity were analyzed.Results: Totally, 154 patients were enrolled (103 SV-CHD and 51 HS), and the male to female ratio was 92:62. The mean age of Fontan operation was 5.1 years, and extracardiac conduit comprised the majority (90.9%) of Fontan operation. Overall perioperative event-free survival to discharge was 91.6% (84.3% in HS and 95.1% in other SV-CHD, P = 0.032). For secondary outcomes, length of intensive care stay and duration of pleural effusion drainage were not significantly different between patients with HS and other SV-CHD, but postoperative arrhythmia was more common in HS group (31.4% vs. 12.6%, P = 0.005). In multivariable regression analysis, preoperative risk factors including operation year before 2007 and high PAP and postoperative factors of elevated postoperative CVP were associated with worse outcomes. HS was not a predictor of worse outcome after adjusting for preoperative PAP and operation era.Conclusion: Surgical outcome has improved much in current era. Perioperative outcome is poorer in patients with HS than other SV-CHD, but HS is not a predictor of perioperative mortality after adjusting for hemodynamic factors. Fontan operation Congenital heart disease Heterotaxy syndrome Pulmonary hypertension Wu, Mei-Hwan verfasserin aut Wang, Jou-Kou verfasserin aut Lin, Ming-Tai verfasserin (orcid)0000-0002-1718-7137 aut Chen, Chun-An verfasserin aut Lu, Chun-Wei verfasserin (orcid)0000-0002-6872-4764 aut Chen, Yih-Sharng verfasserin aut Huang, Shu-Chien verfasserin aut Chiu, Shuenn-Nan verfasserin aut Enthalten in Tai wan yi xue hui ; ID: gnd/1042770-3 Journal of the Formosan Medical Association Hong Kong : Elsevier, 2006 121, Seite 89-97 Online-Ressource (DE-627)517812649 (DE-600)2250901-X (DE-576)272713511 1876-0821 nnns volume:121 pages:89-97 GBV_USEFLAG_U SYSFLAG_U GBV_ELV SSG-OLC-PHA GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_31 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_73 GBV_ILN_74 GBV_ILN_95 GBV_ILN_100 GBV_ILN_105 GBV_ILN_110 GBV_ILN_151 GBV_ILN_161 GBV_ILN_170 GBV_ILN_206 GBV_ILN_213 GBV_ILN_230 GBV_ILN_285 GBV_ILN_293 GBV_ILN_602 GBV_ILN_2004 GBV_ILN_2014 GBV_ILN_2068 GBV_ILN_4012 GBV_ILN_4037 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4249 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4338 GBV_ILN_4367 GBV_ILN_4700 44.04 Ausbildung Beruf Organisationen Medizin AR 121 89-97
allfieldsSound	10.1016/j.jfma.2021.01.014 doi (DE-627)ELV007137168 (ELSEVIER)S0929-6646(21)00036-X DE-627 ger DE-627 rda eng 610 DE-600 44.04 bkl Lin, Hsin-Chia verfasserin (orcid)0000-0002-5819-8473 aut Perioperative outcomes of Fontan operation: Impact of heterotaxy syndrome 2021 nicht spezifiziert zzz rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier Background/Purpose: Fontan operation is the standard surgical procedure for achieving long-term survival in single-ventricular complex congenital heart diseases (SV-CHD). We aim to identify the perioperative outcomes and impact of heterotaxy syndrome (HS) after Fontan operation in a tertiary pediatric cardiology center.Methods: Medical records were reviewed for all patients who received Fontan operation and who were born between 1997 and 2017 in our institution. Preoperative, operative, and postoperative risk factors for perioperative mortality and morbidity were analyzed.Results: Totally, 154 patients were enrolled (103 SV-CHD and 51 HS), and the male to female ratio was 92:62. The mean age of Fontan operation was 5.1 years, and extracardiac conduit comprised the majority (90.9%) of Fontan operation. Overall perioperative event-free survival to discharge was 91.6% (84.3% in HS and 95.1% in other SV-CHD, P = 0.032). For secondary outcomes, length of intensive care stay and duration of pleural effusion drainage were not significantly different between patients with HS and other SV-CHD, but postoperative arrhythmia was more common in HS group (31.4% vs. 12.6%, P = 0.005). In multivariable regression analysis, preoperative risk factors including operation year before 2007 and high PAP and postoperative factors of elevated postoperative CVP were associated with worse outcomes. HS was not a predictor of worse outcome after adjusting for preoperative PAP and operation era.Conclusion: Surgical outcome has improved much in current era. Perioperative outcome is poorer in patients with HS than other SV-CHD, but HS is not a predictor of perioperative mortality after adjusting for hemodynamic factors. Fontan operation Congenital heart disease Heterotaxy syndrome Pulmonary hypertension Wu, Mei-Hwan verfasserin aut Wang, Jou-Kou verfasserin aut Lin, Ming-Tai verfasserin (orcid)0000-0002-1718-7137 aut Chen, Chun-An verfasserin aut Lu, Chun-Wei verfasserin (orcid)0000-0002-6872-4764 aut Chen, Yih-Sharng verfasserin aut Huang, Shu-Chien verfasserin aut Chiu, Shuenn-Nan verfasserin aut Enthalten in Tai wan yi xue hui ; ID: gnd/1042770-3 Journal of the Formosan Medical Association Hong Kong : Elsevier, 2006 121, Seite 89-97 Online-Ressource (DE-627)517812649 (DE-600)2250901-X (DE-576)272713511 1876-0821 nnns volume:121 pages:89-97 GBV_USEFLAG_U SYSFLAG_U GBV_ELV SSG-OLC-PHA GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_31 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_73 GBV_ILN_74 GBV_ILN_95 GBV_ILN_100 GBV_ILN_105 GBV_ILN_110 GBV_ILN_151 GBV_ILN_161 GBV_ILN_170 GBV_ILN_206 GBV_ILN_213 GBV_ILN_230 GBV_ILN_285 GBV_ILN_293 GBV_ILN_602 GBV_ILN_2004 GBV_ILN_2014 GBV_ILN_2068 GBV_ILN_4012 GBV_ILN_4037 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4249 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4338 GBV_ILN_4367 GBV_ILN_4700 44.04 Ausbildung Beruf Organisationen Medizin AR 121 89-97
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author	Lin, Hsin-Chia
spellingShingle	Lin, Hsin-Chia ddc 610 bkl 44.04 misc Fontan operation misc Congenital heart disease misc Heterotaxy syndrome misc Pulmonary hypertension Perioperative outcomes of Fontan operation: Impact of heterotaxy syndrome
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topic_title	610 DE-600 44.04 bkl Perioperative outcomes of Fontan operation: Impact of heterotaxy syndrome Fontan operation Congenital heart disease Heterotaxy syndrome Pulmonary hypertension
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title_sort	perioperative outcomes of fontan operation: impact of heterotaxy syndrome
title_auth	Perioperative outcomes of Fontan operation: Impact of heterotaxy syndrome
abstract	Background/Purpose: Fontan operation is the standard surgical procedure for achieving long-term survival in single-ventricular complex congenital heart diseases (SV-CHD). We aim to identify the perioperative outcomes and impact of heterotaxy syndrome (HS) after Fontan operation in a tertiary pediatric cardiology center.Methods: Medical records were reviewed for all patients who received Fontan operation and who were born between 1997 and 2017 in our institution. Preoperative, operative, and postoperative risk factors for perioperative mortality and morbidity were analyzed.Results: Totally, 154 patients were enrolled (103 SV-CHD and 51 HS), and the male to female ratio was 92:62. The mean age of Fontan operation was 5.1 years, and extracardiac conduit comprised the majority (90.9%) of Fontan operation. Overall perioperative event-free survival to discharge was 91.6% (84.3% in HS and 95.1% in other SV-CHD, P = 0.032). For secondary outcomes, length of intensive care stay and duration of pleural effusion drainage were not significantly different between patients with HS and other SV-CHD, but postoperative arrhythmia was more common in HS group (31.4% vs. 12.6%, P = 0.005). In multivariable regression analysis, preoperative risk factors including operation year before 2007 and high PAP and postoperative factors of elevated postoperative CVP were associated with worse outcomes. HS was not a predictor of worse outcome after adjusting for preoperative PAP and operation era.Conclusion: Surgical outcome has improved much in current era. Perioperative outcome is poorer in patients with HS than other SV-CHD, but HS is not a predictor of perioperative mortality after adjusting for hemodynamic factors.
abstractGer	Background/Purpose: Fontan operation is the standard surgical procedure for achieving long-term survival in single-ventricular complex congenital heart diseases (SV-CHD). We aim to identify the perioperative outcomes and impact of heterotaxy syndrome (HS) after Fontan operation in a tertiary pediatric cardiology center.Methods: Medical records were reviewed for all patients who received Fontan operation and who were born between 1997 and 2017 in our institution. Preoperative, operative, and postoperative risk factors for perioperative mortality and morbidity were analyzed.Results: Totally, 154 patients were enrolled (103 SV-CHD and 51 HS), and the male to female ratio was 92:62. The mean age of Fontan operation was 5.1 years, and extracardiac conduit comprised the majority (90.9%) of Fontan operation. Overall perioperative event-free survival to discharge was 91.6% (84.3% in HS and 95.1% in other SV-CHD, P = 0.032). For secondary outcomes, length of intensive care stay and duration of pleural effusion drainage were not significantly different between patients with HS and other SV-CHD, but postoperative arrhythmia was more common in HS group (31.4% vs. 12.6%, P = 0.005). In multivariable regression analysis, preoperative risk factors including operation year before 2007 and high PAP and postoperative factors of elevated postoperative CVP were associated with worse outcomes. HS was not a predictor of worse outcome after adjusting for preoperative PAP and operation era.Conclusion: Surgical outcome has improved much in current era. Perioperative outcome is poorer in patients with HS than other SV-CHD, but HS is not a predictor of perioperative mortality after adjusting for hemodynamic factors.
abstract_unstemmed	Background/Purpose: Fontan operation is the standard surgical procedure for achieving long-term survival in single-ventricular complex congenital heart diseases (SV-CHD). We aim to identify the perioperative outcomes and impact of heterotaxy syndrome (HS) after Fontan operation in a tertiary pediatric cardiology center.Methods: Medical records were reviewed for all patients who received Fontan operation and who were born between 1997 and 2017 in our institution. Preoperative, operative, and postoperative risk factors for perioperative mortality and morbidity were analyzed.Results: Totally, 154 patients were enrolled (103 SV-CHD and 51 HS), and the male to female ratio was 92:62. The mean age of Fontan operation was 5.1 years, and extracardiac conduit comprised the majority (90.9%) of Fontan operation. Overall perioperative event-free survival to discharge was 91.6% (84.3% in HS and 95.1% in other SV-CHD, P = 0.032). For secondary outcomes, length of intensive care stay and duration of pleural effusion drainage were not significantly different between patients with HS and other SV-CHD, but postoperative arrhythmia was more common in HS group (31.4% vs. 12.6%, P = 0.005). In multivariable regression analysis, preoperative risk factors including operation year before 2007 and high PAP and postoperative factors of elevated postoperative CVP were associated with worse outcomes. HS was not a predictor of worse outcome after adjusting for preoperative PAP and operation era.Conclusion: Surgical outcome has improved much in current era. Perioperative outcome is poorer in patients with HS than other SV-CHD, but HS is not a predictor of perioperative mortality after adjusting for hemodynamic factors.
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title_short	Perioperative outcomes of Fontan operation: Impact of heterotaxy syndrome
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author2	Wu, Mei-Hwan Wang, Jou-Kou Lin, Ming-Tai Chen, Chun-An Lu, Chun-Wei Chen, Yih-Sharng Huang, Shu-Chien Chiu, Shuenn-Nan
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We aim to identify the perioperative outcomes and impact of heterotaxy syndrome (HS) after Fontan operation in a tertiary pediatric cardiology center.Methods: Medical records were reviewed for all patients who received Fontan operation and who were born between 1997 and 2017 in our institution. Preoperative, operative, and postoperative risk factors for perioperative mortality and morbidity were analyzed.Results: Totally, 154 patients were enrolled (103 SV-CHD and 51 HS), and the male to female ratio was 92:62. The mean age of Fontan operation was 5.1 years, and extracardiac conduit comprised the majority (90.9%) of Fontan operation. Overall perioperative event-free survival to discharge was 91.6% (84.3% in HS and 95.1% in other SV-CHD, P = 0.032). For secondary outcomes, length of intensive care stay and duration of pleural effusion drainage were not significantly different between patients with HS and other SV-CHD, but postoperative arrhythmia was more common in HS group (31.4% vs. 12.6%, P = 0.005). In multivariable regression analysis, preoperative risk factors including operation year before 2007 and high PAP and postoperative factors of elevated postoperative CVP were associated with worse outcomes. HS was not a predictor of worse outcome after adjusting for preoperative PAP and operation era.Conclusion: Surgical outcome has improved much in current era. 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Perioperative outcomes of Fontan operation: Impact of heterotaxy syndrome

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