Christina L. Greene MD , Antonia Schulz MD , Mariana Chávez MD , Steven J. Staffa MS , David Zurakowski MS, PhD , Kevin G. Friedman MD , Sitaram M. Emani MD , Christopher W. Baird MD
{"title":"13 和 18 三体综合征合并先天性心脏病患者的手术和非手术治疗效果","authors":"Christina L. Greene MD , Antonia Schulz MD , Mariana Chávez MD , Steven J. Staffa MS , David Zurakowski MS, PhD , Kevin G. Friedman MD , Sitaram M. Emani MD , Christopher W. Baird MD","doi":"10.1016/j.xjon.2024.06.007","DOIUrl":null,"url":null,"abstract":"<div><h3>Objective</h3><p>To evaluate the short- and long-term outcomes of cardiac repair versus nonoperative management in patients with trisomy 13 and trisomy 18 with congenital heart disease.</p></div><div><h3>Methods</h3><p>An institutional review board-approved, retrospective review was undertaken to identify all patients admitted with trisomy 13/18 and congenital heart disease. Patients were divided into 2 cohorts (operated vs nonoperated) and compared.</p></div><div><h3>Results</h3><p>Between 1985 and 2023, 62 patients (34 operated and 28 nonoperated) with trisomy 13 (n = 9) and trisomy 18 (n = 53) were identified. The operated cohort was 74% girls, underwent mainly The Society of Thoracic Surgeons-European Association for Cardio-Thoracic Surgery mortality category 1 procedures (n = 24 [71%]) at a median age of 2.5 months (interquartile range [IQR], 1.3-4.5 months). This compares with the nonoperative cohort where 64% (n = 18) would have undergone The Society of Thoracic Surgeons-European Association for Cardio-Thoracic Surgery mortality category 1 procedures if surgery would have been elected. The most common diagnosis was ventricular septal defect. Postoperative median intensive care unit stay was 6.5 days (IQR, 3.7-15 days) with a total hospital length of stay of 15 days (IQR, 11-49 days). Thirty-day postoperative survival was 94%. There were 5 in-hospital deaths in the operated and 7 in the nonoperated cohort. Median follow-up was 15.4 months (IQR, 4.3-48.7 months) for the operated and 11.2 months (IQR, 1.2-48.3 months) for the nonoperated cohorts. One-year survival was 79% operated versus 51.5% nonoperated (<em>P</em> < .003). Nonoperative treatment had an increased risk of mortality (hazard ratio, 3.28; 95% CI, 1.46-7.4; <em>P</em> = .004).</p></div><div><h3>Conclusions</h3><p>Controversy exists regarding the role of primary cardiac repair in patients with trisomy 13/18 and congenital heart disease. Cardiac repair can be performed safely with low early mortality and operated patients had higher long-term survival compared with nonoperated in our cohort.</p></div>","PeriodicalId":74032,"journal":{"name":"JTCVS open","volume":"20 ","pages":"Pages 123-131"},"PeriodicalIF":0.0000,"publicationDate":"2024-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2666273624001669/pdfft?md5=7f5c6941eee514d471dc97a60d6160e8&pid=1-s2.0-S2666273624001669-main.pdf","citationCount":"0","resultStr":"{\"title\":\"Operative and nonoperative outcomes in patients with trisomy 13 and 18 with congenital heart disease\",\"authors\":\"Christina L. Greene MD , Antonia Schulz MD , Mariana Chávez MD , Steven J. Staffa MS , David Zurakowski MS, PhD , Kevin G. Friedman MD , Sitaram M. Emani MD , Christopher W. Baird MD\",\"doi\":\"10.1016/j.xjon.2024.06.007\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<div><h3>Objective</h3><p>To evaluate the short- and long-term outcomes of cardiac repair versus nonoperative management in patients with trisomy 13 and trisomy 18 with congenital heart disease.</p></div><div><h3>Methods</h3><p>An institutional review board-approved, retrospective review was undertaken to identify all patients admitted with trisomy 13/18 and congenital heart disease. Patients were divided into 2 cohorts (operated vs nonoperated) and compared.</p></div><div><h3>Results</h3><p>Between 1985 and 2023, 62 patients (34 operated and 28 nonoperated) with trisomy 13 (n = 9) and trisomy 18 (n = 53) were identified. The operated cohort was 74% girls, underwent mainly The Society of Thoracic Surgeons-European Association for Cardio-Thoracic Surgery mortality category 1 procedures (n = 24 [71%]) at a median age of 2.5 months (interquartile range [IQR], 1.3-4.5 months). This compares with the nonoperative cohort where 64% (n = 18) would have undergone The Society of Thoracic Surgeons-European Association for Cardio-Thoracic Surgery mortality category 1 procedures if surgery would have been elected. The most common diagnosis was ventricular septal defect. Postoperative median intensive care unit stay was 6.5 days (IQR, 3.7-15 days) with a total hospital length of stay of 15 days (IQR, 11-49 days). Thirty-day postoperative survival was 94%. There were 5 in-hospital deaths in the operated and 7 in the nonoperated cohort. Median follow-up was 15.4 months (IQR, 4.3-48.7 months) for the operated and 11.2 months (IQR, 1.2-48.3 months) for the nonoperated cohorts. One-year survival was 79% operated versus 51.5% nonoperated (<em>P</em> < .003). Nonoperative treatment had an increased risk of mortality (hazard ratio, 3.28; 95% CI, 1.46-7.4; <em>P</em> = .004).</p></div><div><h3>Conclusions</h3><p>Controversy exists regarding the role of primary cardiac repair in patients with trisomy 13/18 and congenital heart disease. Cardiac repair can be performed safely with low early mortality and operated patients had higher long-term survival compared with nonoperated in our cohort.</p></div>\",\"PeriodicalId\":74032,\"journal\":{\"name\":\"JTCVS open\",\"volume\":\"20 \",\"pages\":\"Pages 123-131\"},\"PeriodicalIF\":0.0000,\"publicationDate\":\"2024-08-01\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"https://www.sciencedirect.com/science/article/pii/S2666273624001669/pdfft?md5=7f5c6941eee514d471dc97a60d6160e8&pid=1-s2.0-S2666273624001669-main.pdf\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"JTCVS open\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://www.sciencedirect.com/science/article/pii/S2666273624001669\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"\",\"JCRName\":\"\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"JTCVS open","FirstCategoryId":"1085","ListUrlMain":"https://www.sciencedirect.com/science/article/pii/S2666273624001669","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"","JCRName":"","Score":null,"Total":0}
Operative and nonoperative outcomes in patients with trisomy 13 and 18 with congenital heart disease
Objective
To evaluate the short- and long-term outcomes of cardiac repair versus nonoperative management in patients with trisomy 13 and trisomy 18 with congenital heart disease.
Methods
An institutional review board-approved, retrospective review was undertaken to identify all patients admitted with trisomy 13/18 and congenital heart disease. Patients were divided into 2 cohorts (operated vs nonoperated) and compared.
Results
Between 1985 and 2023, 62 patients (34 operated and 28 nonoperated) with trisomy 13 (n = 9) and trisomy 18 (n = 53) were identified. The operated cohort was 74% girls, underwent mainly The Society of Thoracic Surgeons-European Association for Cardio-Thoracic Surgery mortality category 1 procedures (n = 24 [71%]) at a median age of 2.5 months (interquartile range [IQR], 1.3-4.5 months). This compares with the nonoperative cohort where 64% (n = 18) would have undergone The Society of Thoracic Surgeons-European Association for Cardio-Thoracic Surgery mortality category 1 procedures if surgery would have been elected. The most common diagnosis was ventricular septal defect. Postoperative median intensive care unit stay was 6.5 days (IQR, 3.7-15 days) with a total hospital length of stay of 15 days (IQR, 11-49 days). Thirty-day postoperative survival was 94%. There were 5 in-hospital deaths in the operated and 7 in the nonoperated cohort. Median follow-up was 15.4 months (IQR, 4.3-48.7 months) for the operated and 11.2 months (IQR, 1.2-48.3 months) for the nonoperated cohorts. One-year survival was 79% operated versus 51.5% nonoperated (P < .003). Nonoperative treatment had an increased risk of mortality (hazard ratio, 3.28; 95% CI, 1.46-7.4; P = .004).
Conclusions
Controversy exists regarding the role of primary cardiac repair in patients with trisomy 13/18 and congenital heart disease. Cardiac repair can be performed safely with low early mortality and operated patients had higher long-term survival compared with nonoperated in our cohort.
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