Cardiac Surgery Outcomes in Patients with Trisomy 13 and 18: An Analysis of the STS Congenital Heart Surgery Database

Purpose Congenital heart disease is common in patients with Trisomy 13 (T13) and Trisomy 18 (T18).  Due to their markedly reduced lifespan and overall poor prognosis, offering cardiac surgery is controversial. We sought to describe perioperative factors and surgical outcomes in T13 and T18 patients.

Methods Patients in the Society of Thoracic Surgeons Congenital Heart Surgery Database (STS-CHSD) with T13 and T18 who underwent cardiac surgery (2010-2013) were included. Preoperative, operative, and outcomes data were described.

Results A total of 134 T13 (n=31) and T18 (n=103) cases were distributed over 58 centers (out of 113 centers in STS-CHSD), with the majority of sites (84%) having 3 or fewer cases. The most common operations were VSD repair (n=35), PA Banding (n=19), PDA closure (n=8), TOF repair (n=8), coarctation/aortic arch repair (n=8), coarctation/aortic arch + VSD repair (n=7), and Blalock-Taussig Shunt (n=4).  Table 1 provides preoperative data and complexity stratified outcomes.

Conclusion Cardiac surgery in patients with T13 and T18 is associated with high mortality and morbidity, even in low complexity stratified operations.  However, in the selected cohort of patients in this study, >80% of patients with these high risk chromosomal abnormalities survive to hospital discharge.  This information should be considered when counseling families.

Table 1 – Preoperative Data and Postoperative Morbidity and Mortality

	Trisomy 13 N=31	Trisomy 18 N=103	Overall STS Data
Age at Surgery (months)	2.4 (0.6, 14.4)	3.3 (1.5, 6.9)
Female Gender	16 (51.6%)	81 (78.6%)
Premature Birth	6 (19.4%)	27 (26.2%)
Birth Weight (kg)	2.7 (2.4, 3.1)	2.2 (1.8, 2.5)
Weight at Surgery (kg)	4.3 (2.7, 9.4)	3.2 (2.4, 5.3)
Non-Cardiac Abnormalities	1 (3.2%)	7 (6.8%)
Previous Cardiothoracic Operation(s)	6 (19.4%)	14 (13.6%)
Pre-operative Mechanical Ventilation	7 (22.6%)	25 (24.3%)
Gastrostomy Present	6 (19.4%)	13 (12.6%)
In Hospital Mortality	5/31 (16.1%)	18/103 (17.5%)
STAT 1	0/7 (0.0%)	6/39 (15.4%)	0.7%
STAT 2	3/7 (42.9%)	2/23 (8.7%)	1.7%
STAT 3	0/3 (0.0%)	2/7 (28.6%)	3.0%
STAT 4	2/12 (16.7%)	6/26 (23.1%)	6.8%
STAT 5	0/1 (0.0%)	1/1 (100.0%)	16.1%
Postoperative Length of Stay (days)	17.0 (7.0, 42.0)	13.0 (7.0, 31.0)
STAT 1	11.0 (4.0, 46.0)	13.0 (6.0, 30.0)	7.2
STAT 2	29.0 (15.0, 107.0)	9.0 (6.0, 19.0)	20.0
STAT 3	17.0 (8.0, 19.0)	28.0 (13.0, 82.0)	17.1
STAT 4	18.0 (6.5, 32.0)	21.5 (10.0, 33.0)	25.4
STAT 5	45.0 (45.0, 45.0)	88.0 (88.0, 88.0)	40.3
Postoperative Complication (any)	20/31 (64.5%)	50/103 (48.5%)
Postoperative Cardiac Arrest	3 (10.7%)	4 (4.6%)

Values are displayed as n (%) or Median (IQR).  STAT=STS-EACTS Congenital Heart Surgery Mortality Category