Congenital Heart Surgery on In-Hospital Mortality in Trisomy 13 and 18

Abstract

CHS is associated with decreased in-hospital mortality in T18 and T13. These results suggest CHS may be beneficial in select cases.

Survival in double aneuploidy involving trisomy 18 and sex chromosome trisomy: A case report of a 27‐month‐old child and a review of the literature

“…The most common associated major anomalies were ventricular septal defect (67%), followed by patent ductus arteriosus (40%) and atrial septal defect (17%) as congenital heart disease (CHD) and esophageal atresia (10%) as other than CHD. The common types of associated CHD in the present review were consistent with the previous observations in trisomy 18 (Kosiv et al ). Tube feeding and mechanical ventilation were required in 59% and 31% of patients, respectively.…”

Surgical and anesthetic management for hepatectomy in two pediatric patients with trisomy 18, pulmonary hypertension, and hepatoblastoma

“…3 There is a well-recognized association between HB and trisomy 18, 4 and as a result, resection of HB in children with trisomy 18 needs to be considered. Repair of congenital heart defects early in life reduces in-hospital mortality in these infants, 5 but some of them have chronic issues with Abbreviations: AFP, alpha-fetoprotein; CVP, central venous pressure; HB, hepatoblastoma; IVC, inferior vena cava; VSD, ventricular septal defect pulmonary hypertension. The pulmonary hypertension that a patient with trisomy 18 experiences may complicate liver tumor resection as central venous pressures may be elevated.…”

Congenital heart defects associated with aneuploidy syndromes: New insights into familiar associations

“…Data is sparse regarding the overall ascertainment of fetal echocardiography leading to a diagnosis of aneuploidy, and there are few or no studies regarding fetuses with aneuploidy assessed by other means, and their subsequent echocardiographic findings (Pavlicek et al, 2019;Tuuli et al, 2009). In addition to the expanded prenatal diagnosis of aneuploidy syndromes with frequent CHDs, postnatal diagnosis of the most severe CHDs in CCHD newborn screening programs has grown rapidly worldwide (Bakker et al, 2019;Liberman et al, 2014;Oster et al, 2016 Smallhorn, & Freedom, 1990Wylie et al, 1994Lin et al, 2007Polli et al, 2014Kosiv, Gossett, Bai, & Collins, 2017Domingo, Carey, Eckhauser, Wilkes, & Menon, 2019Van Praagh et al, 1989Musewe et al, 1990Balderston, Shaffer, Washington, & Sondheimer, 1990Baty et al, 1994Crider, Olney, & Cragan, 2008Savva, Walker, & Morris, 2010Kosiv et al, 2017Abdelgadir, Nowaczyk, & Li, 2013Kehinde et al, 2014Bergstrom et al, 2016Pfitzer et al, 2018;Freeman et al, 2008Lange, Guenther, Busch, Hess, & Schreiber, 2007de Graaf et al, 2015 Prevalence of syndrome per 10,000 livebirths Abbreviations: AVSD, atrioventricular septal defect; BAV, bicuspid aortic valve; CHD, congenital heart defect; ETA, elongation of the transverse aorta; HLHS, hypoplastic left heart syndrome; LB, livebirth; PLSVC, persistent left superior vena cava; TAPVC, total anomalous pulmonary venous connection. disability, and high mortality.…”

“…Although a conservative approach (palliative measures) has been the traditional guideline for infants with trisomy 18, more clinicians (48%) were willing to discuss surgery in one study (Kaulfus et al, 2019). There is a growing body of literature analyzing outcome, the impact of natural history, CHDs, surgery, and attitudes of parents and providers (Baty et al, 1994;Domingo et al, 2019;Kaneko et al, 2008;Kosiv et al, 2017;Lakovschek, Streubel, & Ulm, 2011;Meyer et al, 2016;Weaver, Starr, Austin, Stevenson, & Hammel, 2018). Recent outcome studies provide strong support for consideration of CHD surgery (Kosiv et al, 2017), but the impact on survival after 1 year is debated.…”

“…There is a growing body of literature analyzing outcome, the impact of natural history, CHDs, surgery, and attitudes of parents and providers (Baty et al, 1994;Domingo et al, 2019;Kaneko et al, 2008;Kosiv et al, 2017;Lakovschek, Streubel, & Ulm, 2011;Meyer et al, 2016;Weaver, Starr, Austin, Stevenson, & Hammel, 2018). Recent outcome studies provide strong support for consideration of CHD surgery (Kosiv et al, 2017), but the impact on survival after 1 year is debated. Follow-up after cardiac surgery for trisomy 13 and 18 has focused on in-hospital mortality and survival up to two years; longer term studies are not available because of rarity of survivors.…”