Outcomes of cardiac surgery in trisomy 18 patients

Muneuchi, Jun; Yamamoto, Jun; Takahashi, Yoshio; Watanabe, Mamie; Yuge, Tetsuji; Ohno, Takuro; Imoto, Yutaka; Sese, Akira; Joo, Kunitaka

doi:10.1017/s1047951110001848

Cited by 47 publications

(52 citation statements)

References 13 publications

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“…A Japanese study of 34 patients with T18 reported 22% two-year survival following corrective or palliative cardiac surgery, compared to 9% survival with only medical therapy [7]. Similarly, improved survival with surgical management has been reported by other small studies [8, 9].…”

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confidence: 75%

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Long-Term Outcomes of Children With Trisomy 13 and 18 After Congenital Heart Disease Interventions

Peterson

Kochilas

Catton

et al. 2017

The Annals of Thoracic Surgery

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Background The purpose of this study is to report short and long term outcomes following congenital heart defect (CHD) interventions in patients with Trisomy 13 or 18. Methods A retrospective review of the Pediatric Cardiac Care Consortium (PCCC) identified children with Trisomy 13 or 18 with interventions for CHD between 1982 and 2008. Long term survival and cause of death was obtained through linkage with the National Death Index. Results A total of 50 patients with Trisomy 13 and 121 with Trisomy 18 were enrolled in PCCC between 1982 and 2008; among them 29 patients with Trisomy 13 and 69 patients with Trisomy 18 underwent intervention for CHD. In-hospital mortality for patients with Trisomy 13 or Trisomy 18 was 27.6% and 13%, respectively. Causes of in-hospital mortality were primarily cardiac (64.7%) or multiple organ system failure (17.6%). National Death Index linkage confirmed 23 post-discharge deaths. Median survival (conditioned to hospital discharge) was 14.8 years (95% CI 12.3–25.6 years) for patients with Trisomy 13 and 16.2 years (95% CI 12–20.4 years) for patients with Trisomy 18. Causes of late death included cardiac (43.5%), respiratory (26.1%), and pulmonary hypertension (13%). Conclusions In-hospital mortality for all surgical risk categories was higher in patients with Trisomy 13 or 18 than reported for the general population. However, patients with Trisomy 13 or 18, who were selected as acceptable candidates for cardiac intervention and who survived CHD intervention, demonstrated longer survival than previously reported. These findings can be used to counsel families and make program-level decisions on offering intervention to carefully selected patients.

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confidence: 75%

“…Studies published in the past decade suggest that survival in children with T13 and T18 may be longer after intervention for CHD [7–10]. A Japanese study of 34 patients with T18 reported 22% two-year survival following corrective or palliative cardiac surgery, compared to 9% survival with only medical therapy [7].…”

mentioning

confidence: 99%

Long-Term Outcomes of Children With Trisomy 13 and 18 After Congenital Heart Disease Interventions

Peterson

Kochilas

Catton

et al. 2017

The Annals of Thoracic Surgery

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“…The present study was one of the few in the literature to evaluate the association of different subgroups of patients with CHDs with other clinical data, in regards to survival. Muneuchi et al [2011] classified the CHDs in complex and not complex abnormalities based on presence of concomitant lesions, differently from us. However, no difference in survival was observed between these kinds of defects in both studies.…”

Section: To the Editormentioning

confidence: 98%

“…Different factors have been associated with this poor prognosis, and congenital heart defects (CHDs) stand out as a common and important feature; in recent studies they have been pointed as the main cause of death of patients in the setting of intensive treatment [Kosho et al, 2006;Kaneko et al, 2008]. However, there is a scarcity of studies in the literature evaluating if there is a difference, concerning survival, among the different subgroups of patients with trisomy 18 and CHDs [Niedrist et al, 2006;Muneuchi et al, 2011]. Thus, the aim of our study was to verify frequency, types, and prognosis of CHDs in a Brazilian series of patients with trisomy 18.…”

Section: To the Editormentioning

confidence: 99%

Trisomy 18: Frequency, types, and prognosis of congenital heart defects in a Brazilian cohort

Rosa

Lorenzen³

et al. 2012

American J of Med Genetics Pt A

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“…however, new studies have demonstrated that good outcomes can be achieved with surgery in these patients and have shifted the discussion to how surgery can positively affect patient's quality of life [10]. Given the growing body of evidence demonstrating the feasibility of operative interventions in these patients, this study examines our institution's experience with ES and PS in the setting of comorbid CHD and presents the outcomes of surgical versus expectant management.…”

Section: Introductionmentioning

confidence: 99%

A Contemporary, Single-Institutional Experience of Surgical Versus Expectant Management of Congenital Heart Disease in Trisomy 13 and 18 Patients

et al. 2015

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The objective of this study was to examine a large institutional experience of patients with trisomy 13 and trisomy 18 in the setting of comorbid congenital heart disease and present the outcomes of surgical versus expectant management. It is a retrospective single-institution cohort study. Institutional review board approved this study. Thirteen consecutive trisomy 18 patients and three consecutive trisomy 13 patients (sixteen patients in total) with comorbid congenital heart disease who were evaluated by our institution's Division of Cardiovascular Surgery between January 2008 and December 2013 were included in the study. The primary outcome measures evaluated were operative mortality (for patients who received surgical management), overall mortality (for patients who received expectant management), and total length of survival during follow-up. Of the thirteen trisomy 18 patients, seven underwent surgical management and six received expectant management. With surgical management, operative mortality was 29 %, and 80 % of patients were alive after a median follow-up of 116 days. With expectant management, 50 % of patients died before hospital discharge. Of the three patients with trisomy 13, one patient underwent surgical management and two received expectant management. The patient who received surgical management with complete repair was alive at last follow-up over 2 years after surgery; both patients managed expectantly died before hospital discharge. Trisomy 13 and trisomy 18 patients with comorbid congenital heart disease can undergo successful cardiac surgical intervention. In this population, we advocate that nearly all patients with cardiovascular indications for operative congenital heart disease intervention should be offered complete surgical repair over palliative approaches for moderately complex congenital cardiac anomalies.

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Outcomes of cardiac surgery in trisomy 18 patients

Cited by 47 publications

References 13 publications

Long-Term Outcomes of Children With Trisomy 13 and 18 After Congenital Heart Disease Interventions

Long-Term Outcomes of Children With Trisomy 13 and 18 After Congenital Heart Disease Interventions

Trisomy 18: Frequency, types, and prognosis of congenital heart defects in a Brazilian cohort

A Contemporary, Single-Institutional Experience of Surgical Versus Expectant Management of Congenital Heart Disease in Trisomy 13 and 18 Patients

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