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

Abstract

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.

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

“…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]. However, these small series with relatively short postoperative follow up are not sufficient to guide healthcare providers and families regarding long term survival after CHD interventions in patients with T13 or T18.…”

Attitudes of clinicians toward cardiac surgery and trisomy 18

“…Two studies (Graham, Bradley, Shirali, Hills, & Atz, ; Yamagishi, ) published survival rates of infants with T‐18 after undergoing cardiac surgery, reporting 45% 2‐year survival and 86% hospital survival. One study (Costello et al, ) compared the outcomes of those infants with T‐18 who underwent cardiac surgery versus those who had palliative care and concluded that cardiac surgery should be considered for all infants with T‐18 who have moderately complex heart defects. While there are published recommendations regarding the importance of conversations between clinicians and families regarding the option of cardiac surgery (Janvier, Farlow, & Barrington, ), there is an overall lack of consensus in the literature on recommendations for corrective cardiac surgery versus palliative approaches for moderately complex heart defects (Costello et al, ; Graham et al, ).…”

“…One study (Costello et al, 2015) compared the outcomes of those infants with T-18 who underwent cardiac surgery versus those who had palliative care and concluded that cardiac surgery should be considered for all infants with T-18 who have moderately complex heart defects. While there are published recommendations regarding the importance of conversations between clinicians and families regarding the option of cardiac surgery (Janvier, Farlow, & Barrington, 2016), there is an overall lack of consensus in the literature on recommendations for corrective cardiac surgery versus palliative approaches for moderately complex heart defects (Costello et al, 2015;Graham et al, 2004). While corrective cardiac surgery may potentially help negate cardiac-related death and may improve the survival length, some argue that more data demonstrating clinical benefit of corrective cardiac surgery is needed before generalizations can be made on effectiveness (Graham, 2016;Kaneko et al, 2009;Kobayashi et al, 2010;Maeda et al, 2011;Muneuchi et al, 2011).…”

Obstetricians' views on the ethics of cardiac surgery for newborns with common aneuploidies

“…Given the overall poor prognosis of babies with trisomies 18 and 13, historically these heart defects had not been surgically corrected. However, over the past 15 years, there have been a number of manuscripts published in the medical literature describing the outcomes of infants with trisomies 18 and 13 who underwent cardiac surgical repair . These reports have been accompanied by the formation and/or strengthening of advocacy and support organizations for patients and families with trisomies .…”

“…number of manuscripts published in the medical literature describing the outcomes of infants with trisomies 18 and 13 who underwent cardiac surgical repair. [4][5][6][7][8] These reports have been accompanied by the formation and/or strengthening of advocacy and support organizations for patients and families with trisomies. 9,10 These events have empowered parents of babies with trisomy 18 or 13 to initiate discussions about congenital heart surgery, sometimes contacting the offices of pediatric cardiovascular surgeons directly.…”