Genetic and Extracardiac Anomalies Are Associated With Inferior Single Ventricle Palliation Outcomes. Alsoufi, B., McCracken, C., Oster, M., Shashidharan, S., & Kanter, K. The Annals of Thoracic Surgery, 106(4):1204–1212, 2018. doi abstract bibtex BACKGROUND: We examined the effect of genetic syndromes and extracardiac (GS/EC) anomalies on single-ventricle (SV) palliation with focus on hospital and interstage death and progression toward subsequent palliation stages. METHODS: First-stage palliation was performed in 530 neonates with SV: Norwood in 284 (53%), shunt in 173 (33%), and band in 73 (14%). Outcomes were compared between those with GS/EC anomalies (121 [23%]) and without GS/EC anomalies (409 [77%]). Regression analyses were adjusted for other risk factors (age, sex, prematurity, weight, SV anomaly, and first-stage palliation operation). RESULTS: GS/EC anomalies varied among SV defects (range, 3% for double-inlet left ventricle to 100% for atrial isomerism). Patients with GS/EC anomalies required significantly longer durations of mechanical ventilation and intensive care unit and hospital stay. Although patients had comparable rates of extracorporeal membrane oxygenation (13% vs 11%, p = 0.552) and unplanned reoperation (16% vs 11%, p = 0.189), hospital mortality was higher in patients with GS/EC anomalies (24% vs 12%, p = 0.0008). After discharge, patients with GS/EC anomalies had higher interstage death, with lower progression to Glenn (60% vs 77%, p = 0.002) and lower 10-year survival (56% vs 76%, p \textless 0.001). After adjustment for other risk factors, GS/EC anomalies significantly affected survival in almost all subgroups of patients. CONCLUSIONS: The presence of GS/EC anomalies varies among SV anomalies and is associated with additional risk factors such as prematurity and low weight. After adjusting for other risk factors, GS/EC anomalies are associated with prolonged recovery after first-stage palliation and increased hospital and interstage death, with subsequently fewer patients progressing toward Glenn shunt. The increased death risk in those patients is highest in the first 6 months and persists for 2 to 3 years after first-stage palliation, suggesting the need for more vigilant monitoring and outpatient care in these high-risk patients.
@article{alsoufi_genetic_2018,
title = {Genetic and {Extracardiac} {Anomalies} {Are} {Associated} {With} {Inferior} {Single} {Ventricle} {Palliation} {Outcomes}},
volume = {106},
issn = {1552-6259},
doi = {10.1016/j.athoracsur.2018.04.043},
abstract = {BACKGROUND: We examined the effect of genetic syndromes and extracardiac (GS/EC) anomalies on single-ventricle (SV) palliation with focus on hospital and interstage death and progression toward subsequent palliation stages.
METHODS: First-stage palliation was performed in 530 neonates with SV: Norwood in 284 (53\%), shunt in 173 (33\%), and band in 73 (14\%). Outcomes were compared between those with GS/EC anomalies (121 [23\%]) and without GS/EC anomalies (409 [77\%]). Regression analyses were adjusted for other risk factors (age, sex, prematurity, weight, SV anomaly, and first-stage palliation operation).
RESULTS: GS/EC anomalies varied among SV defects (range, 3\% for double-inlet left ventricle to 100\% for atrial isomerism). Patients with GS/EC anomalies required significantly longer durations of mechanical ventilation and intensive care unit and hospital stay. Although patients had comparable rates of extracorporeal membrane oxygenation (13\% vs 11\%, p = 0.552) and unplanned reoperation (16\% vs 11\%, p = 0.189), hospital mortality was higher in patients with GS/EC anomalies (24\% vs 12\%, p = 0.0008). After discharge, patients with GS/EC anomalies had higher interstage death, with lower progression to Glenn (60\% vs 77\%, p = 0.002) and lower 10-year survival (56\% vs 76\%, p {\textless} 0.001). After adjustment for other risk factors, GS/EC anomalies significantly affected survival in almost all subgroups of patients.
CONCLUSIONS: The presence of GS/EC anomalies varies among SV anomalies and is associated with additional risk factors such as prematurity and low weight. After adjusting for other risk factors, GS/EC anomalies are associated with prolonged recovery after first-stage palliation and increased hospital and interstage death, with subsequently fewer patients progressing toward Glenn shunt. The increased death risk in those patients is highest in the first 6 months and persists for 2 to 3 years after first-stage palliation, suggesting the need for more vigilant monitoring and outpatient care in these high-risk patients.},
language = {eng},
number = {4},
journal = {The Annals of Thoracic Surgery},
author = {Alsoufi, Bahaaldin and McCracken, Courtney and Oster, Matthew and Shashidharan, Subhadra and Kanter, Kirk},
year = {2018},
pmid = {29775603},
keywords = {Abnormalities, Multiple, Academic Medical Centers, Age Factors, Cause of Death, Chromosome Disorders, Databases, Factual, Female, Fontan Procedure, Heart Defects, Congenital, Hospital Mortality, Human Genetics, Humans, Hypoplastic Left Heart Syndrome, Infant, Newborn, Intensive Care Units, Neonatal, Linear Models, Male, Palliative Care, Prognosis, Retrospective Studies, Risk Assessment, Statistics, Nonparametric, Survival Analysis},
pages = {1204--1212}
}
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METHODS: First-stage palliation was performed in 530 neonates with SV: Norwood in 284 (53%), shunt in 173 (33%), and band in 73 (14%). Outcomes were compared between those with GS/EC anomalies (121 [23%]) and without GS/EC anomalies (409 [77%]). Regression analyses were adjusted for other risk factors (age, sex, prematurity, weight, SV anomaly, and first-stage palliation operation). RESULTS: GS/EC anomalies varied among SV defects (range, 3% for double-inlet left ventricle to 100% for atrial isomerism). Patients with GS/EC anomalies required significantly longer durations of mechanical ventilation and intensive care unit and hospital stay. Although patients had comparable rates of extracorporeal membrane oxygenation (13% vs 11%, p = 0.552) and unplanned reoperation (16% vs 11%, p = 0.189), hospital mortality was higher in patients with GS/EC anomalies (24% vs 12%, p = 0.0008). After discharge, patients with GS/EC anomalies had higher interstage death, with lower progression to Glenn (60% vs 77%, p = 0.002) and lower 10-year survival (56% vs 76%, p \\textless 0.001). After adjustment for other risk factors, GS/EC anomalies significantly affected survival in almost all subgroups of patients. CONCLUSIONS: The presence of GS/EC anomalies varies among SV anomalies and is associated with additional risk factors such as prematurity and low weight. After adjusting for other risk factors, GS/EC anomalies are associated with prolonged recovery after first-stage palliation and increased hospital and interstage death, with subsequently fewer patients progressing toward Glenn shunt. The increased death risk in those patients is highest in the first 6 months and persists for 2 to 3 years after first-stage palliation, suggesting the need for more vigilant monitoring and outpatient care in these high-risk patients.","language":"eng","number":"4","journal":"The Annals of Thoracic Surgery","author":[{"propositions":[],"lastnames":["Alsoufi"],"firstnames":["Bahaaldin"],"suffixes":[]},{"propositions":[],"lastnames":["McCracken"],"firstnames":["Courtney"],"suffixes":[]},{"propositions":[],"lastnames":["Oster"],"firstnames":["Matthew"],"suffixes":[]},{"propositions":[],"lastnames":["Shashidharan"],"firstnames":["Subhadra"],"suffixes":[]},{"propositions":[],"lastnames":["Kanter"],"firstnames":["Kirk"],"suffixes":[]}],"year":"2018","pmid":"29775603","keywords":"Abnormalities, Multiple, Academic Medical Centers, Age Factors, Cause of Death, Chromosome Disorders, Databases, Factual, Female, Fontan Procedure, Heart Defects, Congenital, Hospital Mortality, Human Genetics, Humans, Hypoplastic Left Heart Syndrome, Infant, Newborn, Intensive Care Units, Neonatal, Linear Models, Male, Palliative Care, Prognosis, Retrospective Studies, Risk Assessment, Statistics, Nonparametric, Survival Analysis","pages":"1204–1212","bibtex":"@article{alsoufi_genetic_2018,\n\ttitle = {Genetic and {Extracardiac} {Anomalies} {Are} {Associated} {With} {Inferior} {Single} {Ventricle} {Palliation} {Outcomes}},\n\tvolume = {106},\n\tissn = {1552-6259},\n\tdoi = {10.1016/j.athoracsur.2018.04.043},\n\tabstract = {BACKGROUND: We examined the effect of genetic syndromes and extracardiac (GS/EC) anomalies on single-ventricle (SV) palliation with focus on hospital and interstage death and progression toward subsequent palliation stages.\nMETHODS: First-stage palliation was performed in 530 neonates with SV: Norwood in 284 (53\\%), shunt in 173 (33\\%), and band in 73 (14\\%). Outcomes were compared between those with GS/EC anomalies (121 [23\\%]) and without GS/EC anomalies (409 [77\\%]). Regression analyses were adjusted for other risk factors (age, sex, prematurity, weight, SV anomaly, and first-stage palliation operation).\nRESULTS: GS/EC anomalies varied among SV defects (range, 3\\% for double-inlet left ventricle to 100\\% for atrial isomerism). Patients with GS/EC anomalies required significantly longer durations of mechanical ventilation and intensive care unit and hospital stay. Although patients had comparable rates of extracorporeal membrane oxygenation (13\\% vs 11\\%, p = 0.552) and unplanned reoperation (16\\% vs 11\\%, p = 0.189), hospital mortality was higher in patients with GS/EC anomalies (24\\% vs 12\\%, p = 0.0008). After discharge, patients with GS/EC anomalies had higher interstage death, with lower progression to Glenn (60\\% vs 77\\%, p = 0.002) and lower 10-year survival (56\\% vs 76\\%, p {\\textless} 0.001). After adjustment for other risk factors, GS/EC anomalies significantly affected survival in almost all subgroups of patients.\nCONCLUSIONS: The presence of GS/EC anomalies varies among SV anomalies and is associated with additional risk factors such as prematurity and low weight. After adjusting for other risk factors, GS/EC anomalies are associated with prolonged recovery after first-stage palliation and increased hospital and interstage death, with subsequently fewer patients progressing toward Glenn shunt. 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